Bell Palsy U MHSW

1
Bell Palsy
http://www.emedicine.com/emerg/topic56.htm
Last Updated: November 28, 2005
Synonyms and related keywords: Bell's palsy, facial nerve paralysis, facial paralysis,
idiopathic facial paralysis, unilateral facial paralysis, cranial nerve VII paralysis, seventh
cranial nerve paralysis, neurologic disorder, paralysis on one side of face, weakness on
one side of face, drooling, tearing from eyes, upper respiratory infection, URI, viral
infection, herpes simplex virus, HSV, Bell palsy
Section 1 of 10
AUTHOR INFORMATION
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Pictures Bibliography
Author: Michael Lambert, MD, Fellowship Director of Emergency Ultrasound, Clinical

Assistant Professor, Department of Emergency Medicine, Resurrection Medical Center
Michael Lambert, MD, is a member of the following medical societies: American
Academy of Emergency Medicine, American College of Emergency Physicians,
American Institute of Ultrasound in Medicine, and Society for Academic Emergency
Medicine
Editor(s): Edward Bessman, MD, Chairman, Department of Emergency Medicine, John
Hopkins Bayview Medical Center; Assistant Professor, Department of Emergency
Medicine, Johns Hopkins University; Francisco Talavera, PharmD, PhD, Senior
Pharmacy Editor, eMedicine; J Stephen Huff, MD, Associate Professor of Emergency
Medicine and Neurology, Department of Emergency Medicine, University of Virginia
Health System; John Halamka, MD, Chief Information Officer, CareGroup Healthcare
System, Assistant Professor of Medicine, Department of Emergency Medicine, Beth Israel
Deaconess Medical Center; Assistant Professor of Medicine, Harvard Medical School;
and Jonathan Adler, MD, Attending Physician, Department of Emergency Medicine,
Massachusetts General Hospital; Division of Emergency Medicine, Harvard Medical
School
INTRODUCTION
Section 2 of 10
Background: Bell palsy is one of the most common neurologic

disorders affecting the cranial nerves. It is an abrupt, unilateral,
peripheral facial paresis or paralysis without a detectable
cause. This syndrome of idiopathic facial paralysis was first described more
than a century ago by Sir Charles Bell, yet much controversy still
surrounds its etiology and managemen t.
2
Bell palsy is certainly the most common cause of facial paralysis
worldwide.
Keeping in mind that Bell palsy is a diagnosis of exclusion is
imperative. Other disease states or conditions that present with
facial palsies are often misdiagnosed as idiopathic.
Patients with Bell palsy frequently present to the ED before seeing any other health care
professional. The appearance of a distorted face and the abrupt
functional impairment are the driving forces that prompt
emergency evaluation. Patients often fear they have had a stroke or
have a tumor and that their distorted facial appearance will be
permanent.
The emergency physician's role consists of the following:
Exclude other causes of facial paralysis.

Initiate appropriate treatment.
Protect the eye.
Arrange appropriate medical follow-up care.
Pathophysiology: Actual pathophysiology is unknown; this is an area of

interminable debate. A popular theory champions inflammation of the
facial nerve. During this process, the nerve increases in diameter
and becomes compressed as it courses through the temporal
bone.
The facial nerve courses through a portion of the temporal
bone commonly referred to as the facial canal. The first
portion of the facial canal (the labyrinthine segment) is
narrowest. The tiny opening (about 0.66 mm in diameter) in
this segment is known as the meatal foramen.
The facial nerve is subjected to tight confines on its journey
through the facial canal. It seems logical that various
inflammatory, demyelinating, ischemic, or compressive
processes may impair neural conduction at this unique anatomic
site.
Anatomy
The facial nerve (seventh cranial nerve) has 2 components. The larger portion comprises
efferent fibers that stimulate the muscles of facial expression. The smaller portion
contains taste fibers to the anterior two thirds of the tongue, secretomotor fibers to the
lacrimal and salivary glands, and some pain fibers.
Pathway
The path of the facial nerve is very complex; this may be the reason the nerve is
vulnerable to injury. Two portions of the facial nerve leave the brain at the
cerebellopontine angle, traverse the posterior cranial fossa, dive into the internal acoustic
meatus, pass through the facial canal in the temporal bone, then angle sharply backwards,
where they pass behind the middle ear and exit the cranium at the stylomastoid foramen.
From here, the facial nerve bisects the parotid gland, and then terminal branches burst out
from the parotid plexus to supply the muscles of facial expression.
Frequency:
In the US: The incidence of Bell palsy in the United

States is approximately 23 cases per 100,000 persons.
The condition affects approximately 1 person in 65 in a
lifetime.
Internationally: The incidence is the same as in the United States.
Mortality/Morbidity: Bell palsy can cause aesthetic, functional, and

psychological disturbances in patients who have residual nerve
dysfunction during their recovery phase or in patients with
incomplete healing.
Partial paralysis
Motor synkinesis (involuntary movement accompanying

a voluntary movement)
Autonomic synkinesis (involuntary lacrimation after a

voluntary muscle movement)
Race: Incidence of Bell palsy appears to be slightly higher in

persons of Japanese descent.
Sex: No difference exists in sex distribution in patients with Bell palsy.

Age: Age affects the probability of contracting Bell palsy. The
incidence is highest in persons aged 15-45 years. Bell palsy
is less common in those younger than 15 years and in those
older than 60 years.
CLINICAL
Section 3 of 10
History: Most patients presenting to the ED suspect they have

suffered a stroke or have an intracranial tumor. The most
common complaint is of weakness on one side of their face.
Postauricular pains: Almost 50% of patients experience pain in the

mastoid region. The pain frequently occurs simultaneously with the paresis, but
precedes the paresis by 2-3 days in about 25% of patients.
Tear flow: Two thirds of patients complain about tear flow. This is due to the
reduced function of the orbicularis oculi in transporting the tears. Fewer tears
arrive at the lacrimal sac and overflow occurs. The production of tears is not
accelerated.
Altered taste: While only one third of patients complain about taste
disorders, four fifths of patients show a reduced sense of taste. This may be
explained by only half the tongue being involved.
Dry eyes
Hyperacusis: Impaired tolerance to typical levels of noise due to an

increased irritability to the sensory neural mechanism.
Physical: Findings of facial paralysis are easily recognizable on physical

examination. A careful, complete examination excludes other possible causes of
facial paralysis. Strongly consider other etiologies if all branches of the facial nerve
are not affected.
The classic definition of Bell palsy describes mononeuric

involvement of the facial nerve, yet other cranial nerves are
probably affected.
The facial nerve is the only cranial nerve eliciting

obvious findings on physical examination because of its
unique anatomical course from the brain to the lateral
face.
Remember that weakness and/or paralysis from

involvement of the facial nerve manifests as weakness
of the entire face (upper and lower) on the affected
side. Focus attention on the voluntary movement of the upper part of the face
on the affected side.
In supranuclear lesions such as a cortical stroke (upper

motor neuron; above the facial nucleus in the pons), the
upper third of the face is spared while the lower two
thirds are paralyzed. The orbicularis, frontalis, and
corrugator muscles are innervated bilaterally ,
which explains the pattern of facial paralysis.
Test other cranial nerves; their examination results should be normal.
Tympanic membranes should not be inflamed; presence

of infection raises possibility of complicated otitis media.
Causes: "All that glitters is not gold" (William Shakespeare)

The etiology of Bell palsy remains unclear, although
vascular, infectious, genetic, and immunologic causes have
all been proposed. Patients with other diseases or
conditions sometimes develop a peripheral facial nerve
palsy, but these are not classified as Bell palsy (see
Differentials).
Viral infections: Clinical and epidemiologic data lend credence to an

infectious origin, which triggers an immunologic response, resulting in damage to
the facial nerve. Pathogens leading the list include herpes simplex virus
type 1 (HSV-1); herpes simplex virus type 2 (HSV-2);
human herpesvirus (HHV); varicella zoster virus (VZV);
Mycoplasma pneumoniae; Borrelia burgdorferi;
influenza B; adenovirus; coxsackievirus; Ebstein-Barr
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virus; hepatitis A, B, and C; cytomegalovirus (CMV); and
rubella virus.
Pregnancy: Bell palsy is uncommon in pregnancy; however, the

prognosis is significantly worse in pregnant women
with Bell palsy than among nonpregnant women with
palsy.
Genetics: Recurrence rates (4.5-15%) and familial

incidence (4.1%) have been addressed in various studies. Genetics may
have a role in Bell palsy, but which factors are inherited is unclear.
DIFFERENTIALS
Section 4 of 10
Diabetes Mellitus, Type 1 - A Review

Diabetes Mellitus, Type 2 - A Review
Fractures, Mandible
Herpes Zoster
Multiple Sclerosis
Tick-Borne Diseases, Lyme
Other Problems to be Considered:
Herpes zoster
Pregnancy (especially third trimester)
Polyneuritis
Acute otitis
Chronic otitis
Temporal bone fracture
Infectious mononucleosis
Parotid tumors
Sarcoidosis
Cholesteatoma of the middle ear
Aneurysm of vertebral, basilar artery, or carotid arteries
Carcinomatous meningitis
Facial trauma (blunt, penetrating, iatrogenic)
Leukemic meningitis
Leprosy
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Melkersson-Rosenthal syndrome
Middle ear surgery
Osteomyelitis of the skull base
Skull base tumor
WORKUP
Section 5 of 10
Lab Studies:
No specific laboratory tests exist to confirm the diagnosis of Bell

palsy. Clinical setting determines tests that may be of value. Other potential
causes in the differential diagnosis may be confirmed or suspected based on the
following diagnostic laboratory tests:
o
Complete blood count
Erythrocyte sedimentation rate
Thyroid function studies
Lyme titer
Serum glucose level
Rapid plasma reagin (RPR) or Venereal Disease

Research Laboratory (VDRL) test
Human immunodeficiency virus (HIV)
Cerebral spinal fluid analysis
Immunoglobulin M (IgM), immunoglobulin G (IgG), and

immunoglobulin A (IgA) titers for CMV, rubella, HSV,
hepatitis A, hepatitis B, hepatitis C, VZV, M
pneumoniae, and B burgdorferi.
Imaging Studies:
Bell palsy remains a clinical diagnosis. Imaging studies are

not indicated in the ED. Excluding other causes of facial palsy may require
one of the following imaging studies depending on clinical setting.
o
Facial CT scan or plain radiographs: Perform to rule out

fractures or bony metastasis.
CT scan is indicated when stroke, or acquired immunodeficiency

syndrome (AIDS)-CNS involvement is considered in differential diagnosis
MRI: For a suspected neoplasm of the temporal bone, brain, parotid

gland, or other structure, or to evaluate for multiple sclerosis, MRI is the
superior method of imaging. The course of the facial nerve through the
intratemporal and extratemporal regions from the brain to the facial
muscles and glands can be followed on MRI. MRI also may be considered
in lieu of CT scan.
Other Tests:
Electrodiagnosis of the facial nerve: These studies assess the

function of the facial nerve. These tests are rarely performed on an emergent
basis.
o
Electromyography (EMG) and nerve conduction

velocities produce a graphic readout of the electrical currents
displayed by stimulating the facial nerve and recording the excitability of
the facial muscles it supplies. Comparison to the
contralateral side helps determine the extent of
nerve injury and has prognostic implications. This is
not part of the acute workup.
In the nerve excitability test, the threshold of the electrical

stimulus producing visible muscle twitching is determined.
Electroneurography (ENoG) compares evoked potentials on

the paretic side versus the healthy side.
TREATMENT
Section 6 of 10
Emergency Department Care: The primary treatment of

patients with Bell palsy in the ED is pharmacologic
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management. The remainder of care focuses on
reassurance, eye care instructions, and appropriate followup care.
Steroids
o
Treatment of Bell palsy with steroids remains
controversial. Numerous research articles have been written

on the benefit or uselessness of steroids to treat patients with Bell palsy.
o
Researchers seem to lean more toward using steroids as a means to

optimize outcomes. Once the decision to use steroids is made, the
consensus is to start immediately.
Antiviral agents: Although there is insufficient research evaluating the

efficacy of antiviral medicines in Bell palsy, most experts believe in a viral
etiology. Therefore, antiviral agents seem a logical choice for pharmacologic
management and are commonly recommended.
Eye care: The eyes are frequently unprotected in patients with Bell palsy. This
leaves the eyes at risk for corneal drying and foreign body exposure. Manage with
tear substitutes, lubricants, and eye protection.
o
Artificial tears: Use these during waking hours to replace diminished

or absent lacrimation.
Lubricants are used during sleep. They may be used during waking
hours if artificial tears cannot provide adequate protection. One
disadvantage is blurred vision during waking hours.
Eyeglasses or shields protect the eye from injury and

reduce drying by decreasing the air currents that come directly in contact
with the exposed cornea.
Consultations: The patient's primary care physician or consultant should provide close
follow-up care. Documentation should chart the progress of the patient's recovery.
Opinions vary widely on referral to a specialist. Some specific referral indications are
listed below:
Neurologist: When other neurologic signs are identified on physical

examination and for an atypical presentation of Bell palsy, referral is indicated.
10
Ophthalmologist: For any unexplained ocular pain or abnormal findings on

physical examination of the eyes, the patient should be referred for further
workup.
Otolaryngologist: In patients with persistent paralysis, prolonged weakness

of the facial muscles, or recurrent weakness, referral is warranted.
Surgeon: Surgery to decompress the facial nerve is recommended occasionally

for patients with Bell palsy. Patients with a poor prognosis identified by facial
nerve testing or persistent paralysis appear to benefit the most from surgical
intervention.
Since most patients recover without medication, physicians may be able to manage
patients without prescribing medication. This watchful waiting plan is an option;
however, some individuals with Bell palsy never completely recover. Both medications
listed below have clinical trials that support and dispute their efficacy.
Drug Category: Corticosteroids -- Have anti-inflammatory properties

and cause profound and varied metabolic effects. Modify the body's immune response to
diverse stimuli.
Drug Name
Prednisone (Deltasone, Orasone, Sterapred) -Pharmacologic success may be the result of antiinflammatory effect, which presumably decreases
compression of the facial nerve in the facial canal.
Adult Dose
1 mg/kg/d PO for 7 d
Pediatric Dose
Administer as in adults
Contraindications
Documented hypersensitivity; viral, fungal, connective

tissue, and tubercular skin infections; peptic ulcer disease;
hepatic dysfunction; GI disease
Interactions
Coadministration with estrogens may decrease prednisone

clearance; concurrent use with digoxin may cause digitalis
toxicity secondary to hypokalemia; phenobarbital,
phenytoin, and rifampin may increase metabolism of
glucocorticoids (consider increasing maintenance dose);
monitor for hypokalemia with coadministration of diuretics
Pregnancy
B - Usually safe but benefits must outweigh the risks.
Precautions
Abrupt discontinuation of glucocorticoids may cause

adrenal crisis; hyperglycemia, edema, osteonecrosis,
myopathy, peptic ulcer disease, hypokalemia, osteoporosis,
euphoria, psychosis, myasthenia gravis, growth
suppression, and infections may occur with glucocorticoid
use
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Drug Category: Antiviral -- Herpes simplex infections may be a common cause of

Bell palsy. Acyclovir is the most-used treatment, but other antiviral agents may be
appropriate.
Drug Name
Acyclovir (Zovirax) -- Has demonstrated inhibitory

activity directed against both HSV-1 and HSV-2, and
infected cells selectively take it up.
Adult Dose
4000 mg/24 h PO for 7-10 d
Pediatric Dose
Contraindications
Interactions
<2 years: Not recommended

>2 years: 1000 mg PO qid for 10 d
Documented hypersensitivity
Concomitant use of probenecid or zidovudine prolongs
half-life and increases CNS toxicity of acyclovir
Pregnancy
C - Safety for use during pregnancy has not been

established.
Precautions
Caution in renal failure or when using nephrotoxic drugs
FOLLOW-UP
Section 8 of 10
In/Out Patient Meds:
Consider prednisone at an initial dose of 1 mg/kg/day.

o
Prednisone is a potent drug with a high risk of

side effects. The evidence of its usefulness continues to come under
scrutiny in the literature. Until efficacy can be clearly defined, it should
not be perceived as a standard of care.
With no contraindications and if the physician

chooses to administer steroids, the best choice is
prednisone at a high dose, as early as possible in
the disease course. (Consider tapering on day 5 to 5 mg bid for 5 d.)
Administer acyclovir (Zovirax) 800 mg PO 5 times/d for 10

d; 20 mg/kg in patients younger than 2 years. Recent
evidence supports HSV as the presumed cause in more
than 70% of Bell palsy cases.
Complications:
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Most patients with Bell palsy recover without any

cosmetically obvious deformities; however,
approximately 5% are left with an unacceptably high
degree of sequelae.
Incomplete motor regeneration

o
The largest portion of the facial nerve comprises efferent fibers that
stimulate muscles of facial expression. If the motor portion achieves
suboptimal regeneration, paresis of all or some of these facial muscles
results.
This manifests as (1) oral incompetence, (2) epiphora (excessive
tearing), and (3) nasal obstruction.
Incomplete sensory regeneration

o
Dysgeusia (impairment of taste) may result.
Ageusia (loss of taste) may result.
Dysesthesia (impairment of sensation or

disagreeable sensation to normal stimuli) may
result.
Aberrant reinnervation of the facial nerve

o
After the impaired neural conduction of the facial nerve begins the
regeneration and repair process, some nerve fibers take an unusual
course and connect to neighboring fibers. This aberrant reconnection
produces unusual neurologic pathways.
When voluntary movements are initiated, they are accompanied by

involuntary movements (eg, the movement of a closed eye following
that of the uncovered one). These involuntary movements
accompanying voluntary movement are termed synkinesis.
Prognosis:
The natural course of Bell palsy varies from early complete

recovery to substantial nerve injury with permanent
sequelae. Prognostically, patients fall into 3 groups with
roughly equal numbers in each group.
13
o
Group 1 regains complete recovery of facial motor

function without sequelae.
Group 2 experiences incomplete recovery of facial

motor function, but no cosmetic defects are apparent
to the untrained eye.
Group 3 experiences permanent neurologic sequelae

that are cosmetically and clinically apparent.
Most patients develop an incomplete facial paralysis during the acute phase.
This group has an excellent prognosis for full recovery. Patients
demonstrating complete paralysis are at higher risk for severe sequelae.
Of patients with Bell palsy, 85% achieve complete recovery.

Ten percent are bothered by some asymmetry of facial
muscles, while 5% experience severe sequelae.
Patient Education:
Eye care
o
Protect the eye from foreign objects and sunlight.
Keep the eye well lubricated.
Educate the patient to report new ocular findings

such as pain, discharge, or visual changes.
For excellent patient education resources, visit eMedicine's Brain and Nervous
System Center. Also, see eMedicine's patient education article Bell Palsy.
PICTURES
Section 9 of 10
Caption: Picture 1. The facial nerve.

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BIBLIOGRAPHY
Section 10 of 10
Adams RD, Victor M, eds: Diseases of the spinal cord, peripheral nerve, and
muscle. In: Principles of Neurology. 5th ed. NY: McGraw Hill; 1993:1175-1177.
Cousin GC: Facial nerve palsy following intra-oral surgery performed with local
anaesthesia. J R Coll Surg Edinb 2000 Oct; 45(5): 330-3[Medline].
English JB, Stommel EW, Bernat JL: Recurrent Bell's palsy. Neurology 1996
Aug; 47(2): 604-5[Medline].
Helling TD, Neely JG: Validation of objective measures for facial paralysis.
Laryngoscope 1997 Oct; 107(10): 1345-9[Medline].
Morgan M, Moffat M, Ritchie L, et al: Is Bell's palsy a reactivation of varicella
zoster virus? J Infect 1995 Jan; 30(1): 29-36[Medline].
Morrow MJ: Bell's Palsy and Herpes Zoster Oticus. Curr Treat Options Neurol
2000 Sep; 2(5): 407-416[Medline].
Niparko JK, Mattox DE: Bell's palsy and herpes zoster oticus. In: Current
Therapy in Neurologic Disease. 4th ed. Philadelphia: BC Decker; 1993:355-361.
O'Halloran HS, Sen HA, Baker RS: Accidental ocular perforation from selfinflicted facial palsy. Retina 1997; 17(2): 164-6[Medline].
O'Rahilly R, Muller F: Basic Human Anatomy: A regional Study of Human
Structure. Philadelphia: WB Saunders Co; 1983:391-98.
Olson WH, Brumback RA, Christoferson LA: Practical Neurology for the
Primary Care Physician. Springfield, Ill: Thomas Books; 1981:262.
Peitersen E: Bell's palsy: the spontaneous course of 2,500 peripheral facial nerve
palsies of different etiologies. Acta Otolaryngol Suppl 2002; 4-30[Medline].
Pulec JL: New horizons in facial nerve therapy. Ear Nose Throat J 1997 Jun;
76(6): 360[Medline].
Qiu WW, Yin SS, Stucker FJ, et al: Time course of Bell palsy. Arch Otolaryngol
Head Neck Surg 1996 Sep; 122(9): 967-72[Medline].
Sittel C, Sittel A, Guntinas-Lichius O, et al: Bell's palsy: a 10-year experience
with antiphlogistic-rheologic infusion therapy. Am J Otol 2000 May; 21(3): 42532[Medline].
Smith IM, Cull RE: Bell's palsy--which factors determine final recovery? Clin
Otolaryngol 1994 Dec; 19(6): 465-6[Medline].
Smouha EE, Coyle PK, Shukri S: Facial nerve palsy in Lyme disease: evaluation
of clinical diagnostic criteria. Am J Otol 1997 Mar; 18(2): 257-61[Medline].
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Unlu Z, Aslan A, Ozbakkaloglu B, et al: Serologic examinations of hepatitis,

cytomegalovirus, and rubella in patients with Bell's palsy. Am J Phys Med
Rehabil 2003 Jan; 82(1): 28-32[Medline].
Victor M, Martin J: Disorders of the cranial nerves. WJM 2000; 173: 266-268.
Volter C, Helms J, Weissbrich B, et al: Frequent detection of Mycoplasma
pneumoniae in Bell's palsy. Eur Arch Otorhinolaryngol 2004 Aug; 261(7): 4004[Medline].
Wiederholt WC: Bell's palsy. In: Wiederhold WC, ed. Therapy for Neurologic
Disorders. NY: Wiley; 1992:257.
Williamson IG, Whelan TR: The clinical problem of Bell's palsy: is treatment
with steroids effective? Br J Gen Pract 1996 Dec; 46(413): 743-7[Medline].
Bell Palsy
http://www.emedicine.com/neuro/topic413.htm
Last Updated: June 2, 2005
Synonyms and related keywords: Bell's palsy, idiopathic facial paralysis, facial nerve
compression, acute unilateral facial paralysis, bilateral facial palsy, Guillain-Barr
syndrome, GBS, sarcoidosis, Lyme disease, meningitis, neoplastic meningitis, infectious
meningitis, bilateral neurofibromas, neurofibromatosis type 2, ipsilateral facial palsy
Section 1 of 11
AUTHOR INFORMATION
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures
Bibliography
Author: Kim Monnell, DO, Consulting Staff, Department of Neurology, Sarasota

Memorial Hospital Coauthor(s): Sally B Zachariah, MD, Chief, Department of
Neurology, Veteran Affairs Medical Center of Bay Pines; Director, Associate Professor,
Department of Neurology, Division of Strokes, University of South Florida College of
Medicine; Suzan Khoromi, MD, Fellow, Pain and Neurosensory Mechanisms Branch,
National Institute of Dental and Cranial Research, National Institutes of Health
Kim Monnell, DO, is a member of the following medical societies: American Academy of
Neurology, and American Osteopathic Association
Editor(s): Milind J Kothari, DO, Program Director, Associate Professor, Department of
Internal Medicine, Division of Neurology, Pennsylvania State University Hershey
Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor,
eMedicine; Florian P Thomas, MD, MA, PhD, DrMed, Associate Chief of Staff, St
Louis VA Medical Center; Associate Director, Neurology Residency Program; Professor,
Departments of Neurology, Molecular Virology, and Molecular Microbiology and
Immunology, Saint Louis University School of Medicine; Matthew J Baker, MD,
Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital; and
Nicholas Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology
Specialists and Consultants
16
INTRODUCTION
Section 2 of 11
Bibliography
Background: Facial paralysis is a disfiguring disorder that has a great impact on the
patient. Facial nerve paralysis may be congenital, neoplastic, or result from infection,
trauma, toxic exposures, or iatrogenic causes. The most common cause of unilateral facial
paralysis is Bell palsy, also known as idiopathic facial paralysis. Bell palsy is thought to
account for approximately 60-75% of cases of acute unilateral facial paralysis.
In 1550, Fallopius noted the narrow lumen in the temporal bone through which a part of
the seventh cranial nerve passes. In 1828, Charles Bell made the distinction between the
fifth and seventh cranial nerves; he noted that the seventh nerve was involved mainly in
the motor function of the face and the fifth nerve was concerned mainly with the sensory
perception of the face.
Even today, controversy still surrounds the etiology and treatment of Bell palsy. Clinical
features of Bell palsy that may help distinguish it from other causes of facial paralysis
include sudden onset of unilateral facial paralysis (less than 48 hours), absence of signs
and symptoms of CNS disease, and absence of signs and symptoms of ear or posterior
fossa disease.
Pathophysiology: The course of the facial nerve is tortuous, both centrally and
peripherally (see Image 1).
The facial nerve nucleus lies within the reticular formation of the pons, adjacent to the
fourth ventricle. The facial nerve roots include fibers from the motor, solitary, and
salivatory nuclei. The nervus intermedius comprises fibers from salivatory and solitary
nuclei (it contains sensory fibers from the tongue, mucosa, and postauricular skin as well
as parasympathetic fibers to the salivary and lacrimal glands). The fibers of the facial
nerve then course around the sixth cranial nerve nucleus and exit the pons at the
cerebellopontine angle. The fibers go through the internal auditory canal along with the
vestibular portion of the eighth cranial nerve. The narrowest portion of the internal
auditory canal is the labyrinthine segment. This is the location that is thought to be the
most common site of compression of the facial nerve in Bell palsy.
The seventh cranial nerve contains parasympathetic fibers to the nose, palate, and
lacrimal glands. The preganglionic parasympathetic fibers that originate in the salivatory
17
nucleus join the fibers from nucleus solitarius to form the nervus intermedius. These
fibers then synapse with the submandibular ganglion, which has fibers that supply the
sublingual and submandibular glands. The fibers from the nervus intermedius also supply
the pterygopalatine ganglion, which has parasympathetic fibers that supply the nose,
palate, and lacrimal glands.
The facial nerve passes through the stylomastoid foramen in the skull and terminates into
the zygomatic, buccal, mandibular, and cervical branches.
These nerves serve the muscles of facial expression, which include frontalis, orbicularis
oculi, orbicularis oris, buccinator, and platysma. Other muscles innervated by the facial
nerve include stapedius, stylohyoid, posterior belly of the digastric, occipitalis, and
anterior and posterior auricular muscles. All muscles of the facial nerve are derived from
the second brachial arch.
The location of injury of the facial nerve in Bell palsy is peripheral to the seventh nerve
nucleus. The injury is thought to occur near or at the geniculate ganglion. If the lesion is
proximal to the geniculate ganglion, the motor paralysis is accompanied by gustatory and
autonomic abnormalities. Lesions between the geniculate ganglion and the origin of the
chorda tympani produce the same effect except that they spare lacrimation. If the lesion is
at the stylomastoid foramen, it may result in facial paralysis only.
Bell palsy is thought to be caused by edema and ischemia resulting in compression of the
facial nerve in its course through the bony canal. The cause of the edema and ischemia is
still being debated. In the past, cold exposure (eg chilly wind, cold air conditioning, or
driving with the car window down) were considered the only triggers to Bell palsy.
However, most authors believe that the herpes simplex virus (HSV) is the most likely
cause. Actually studying the causal relationship between HSV and Bell palsy is difficult
because of the ubiquitous nature of HSV.
In 1972, McCormick first suggested that HSV is responsible for idiopathic facial
paralysis. This was based on the analogy that HSV was found in cold sores, and he
hypothesized that HSV may remain latent in the geniculate ganglion. Since then, autopsy
studies have shown HSV in the geniculate ganglion of patients with Bell palsy. Murakami
et al performed polymerase chain reaction (PCR) testing for HSV in the endoneural fluid
of the seventh nerve of patients who underwent surgery for Bell palsy. Eleven of the 14
patients were found to have HSV in the endoneural fluid.
Assuming that HSV is the etiologic agent in Bell palsy is reasonable. If this is true, then
the virus is most likely to travel up the axons of the sensory nerves and reside in the
ganglion cells. At times of stress the virus will reactivate, causing local damage to the
myelin. Thus, Bell palsy may be secondary to viral and/or autoimmune reactions causing
the facial nerve to demyelinate, resulting in unilateral facial paralysis.
Frequency:
18
In the US: The annual incidence of Bell palsy is approximately 23 per 100,000.
o The right side is affected 63% of the time.
o Persons with diabetes have a risk as much as 29% higher than persons
without diabetes of being affected by Bell palsy. Thus, blood glucose
levels at time of diagnosis of Bell palsy may detect undiagnosed diabetics.
Internationally: The highest incidence was found in a study in Seckori, Japan, in

1986 and the lowest incidence was found in Sweden in 1971. Most population
studies generally show an annual incidence of 15-30 per 100,000.
Mortality/Morbidity:
The majority of patients who suffer from Bell palsy have neurapraxia or local
nerve conduction block. These patients are likely to have a prompt and complete
recovery of the nerve. Patients with axonotmesis, with disruption of the axons,
have a fairly good recovery but it is usually not complete. The risk factors thought
to be associated with a poor outcome in patients with Bell palsy include (1) age
greater than 60 years, (2) complete paralysis, and (3) decreased taste or salivary
flow on the side of paralysis (usually 10-25% compared to the patient's normal
side). Other factors thought to be associated with poor outcome include pain in
the posterior auricular area and decreased lacrimation.
Patients generally have a good prognosis; approximately 80-90% of patients

recover without noticeable disfigurement within 6 weeks to 3 months. Patients
aged 60 years or older have an approximately 40% chance of complete recovery
and have a higher rate of sequelae. Patients younger than 30 years have only a 1015% chance of less than complete recovery and sequelae. If no recovery occurs by
4 months, then the patient is more likely to have sequelae from the disease, which
include synkinesis, crocodile tears, and rarely hemifacial spasm.
o Synkinesis is an abnormal contracture of the facial muscles while smiling
or closing the eyes. It may be mild and result in slight movement of the
chin when the patient blinks, eye closure with smiling, or contracture
around the mouth while blinking. Crocodile tears are observed; patients
shed tears while they eat.
o Facial spasm is a very rare complication of Bell palsy. It occurs as tonic
contraction of one side of the face. Spasms are more likely to occur during
times of stress or fatigue and may occur during sleep. This condition may
occur secondary to compression of the root of the seventh nerve by an
aberrant blood vessel, tumor, or demyelination of the nerve root. It occurs
most commonly in the fifth and sixth decades of life, and sometimes the
etiology is not found. The presence of progressive facial hemispasm with
other cranial nerve findings indicates a possibility of a brainstem lesion.
o Diabetic patients are 30% more likely than nondiabetic patients to have
only partial recovery; recurrence of Bell palsy is also more common
among diabetic patients.
19
Bell palsy accounts for only 23% of bilateral facial

paralysis. The majority of patients with bilateral facial
palsy have Guillain-Barr syndrome (GBS), sarcoidosis,
Lyme disease, meningitis (neoplastic or infectious), or
bilateral neurofibromas (in patients with
neurofibromatosis type 2).
Bell palsy recurs in 10-15% of patients. It may recur on the
ipsilateral or contralateral side of the initial palsy. Recurrence usually is
associated with a family history of recurrent Bell palsy.
Approximately 30% of patients with recurrent ipsilateral

facial palsy were found to have tumors of the seventh
nerve or parotid gland. Patients with recurrent ipsilateral
facial palsy should undergo MRI or high-resolution CT
scan to rule out neoplastic or inflammatory (eg, multiple
sclerosis, sarcoidosis) cause of recurrence.
Sex:
Bell palsy appears to affect the sexes equally. However,

young women aged 10-19 years are more likely to be
affected than men in the same age group.
Pregnant women have a 3.3 times higher risk of being

affected by Bell palsy than nonpregnant women; Bell
palsy occurs most frequently in the third trimester.
Age: The lowest incidence is found in persons younger than

10 years and the highest incidence in persons aged 60 years
or older.
CLINICAL
Section 3 of 11
Bibliography
History: Bell palsy is a diagnosis of exclusion. The diagnosis must be made on the basis
of a thorough history and physical examination and use of diagnostic testing when
necessary.
Symptoms of Bell palsy
20
Acute onset of unilateral upper and lower facial

paralysis (over a 48-h period)
Posterior auricular pain
Decreased tearing
Hyperacusis
Taste disturbances
The paralysis must include the forehead and lower aspect of the face. The patient
may report inability to close the eye or to smile on the affected side. He or she
also may report increased saliva on the side of the paralysis. If the paralysis
involves only the lower portion of the face, a central cause should be suspected
(ie, supranuclear).
If the patient complains of contralateral weakness or
diplopia in conjunction with the supranuclear facial
palsy, a stroke or intracerebral lesion should be
strongly suspected.
o
Half of the patients affected with Bell palsy may complain of posterior
auricular pain. Ask the patient if he or she has experienced
trauma, which may account for the pain and facial paralysis.
One third of patients may experience hyperacusis

in the ear ipsilateral to the paralysis, which is secondary to
weakness of the stapedius muscle.
One sixth of patients experience decreased

lacrimation.
Many patients report numbness on the side of the

paralysis. Some authors believe that this is
secondary to involvement of the trigeminal nerve,
whereas other authors argue that this symptom is probably due to lack
of mobility of the facial muscles and not lack of
sensation.
21
If a patient has gradual onset of facial paralysis,

weakness of the contralateral side, or history of
trauma or infection, other causes of facial paralysis
must be strongly considered. Patients who have
bilateral facial palsy must be evaluated for GBS, Lyme
disease, and meningitis.
If a patient is from the Northeast, Lyme disease should be considered as a

cause of facial paralysis, and serologic testing should be performed.
Recurrent ipsilateral facial paralysis must raise the

suspicion of a tumor of the seventh nerve or parotid
gland.
If the patient reports sudden onset of hearing loss and
severe pain with the onset of facial paralysis, Ramsay
Hunt syndrome must be considered.
Symptoms associated with seventh nerve neoplasm

include slowly progressive paralysis, facial
hyperkinesis, severe pain, recurrent palsy, and other
cranial nerve involvement. Cerebellopontine tumors
may affect the seventh, eighth, and fifth cranial nerves
simultaneously. Patients with a progressive paralysis of
the facial nerve lasting longer than 3 weeks should be
evaluated for neoplasm.
Physical:
Initial inspection of the patient demonstrates flattening of the forehead and

nasolabial fold on the side affected with the palsy.
When the patient is asked to raise the eyebrows, the side of the forehead with the
palsy will remain flat.
When the patient is asked to smile, the face becomes distorted and lateralizes to
the side opposite the palsy.
The patient is not able to close the eye completely on the affected side. On
attempted eye closure, the eye rolls upward and inward on the affected side. This
is known as Bell phenomenon and is considered a normal response to eye closure.
22
A careful examination of the head, ears, eyes, nose, and throat (HEENT) must be
carried out in all patients with facial paralysis.
o
The external auditory canal must be inspected for vesicles, injection,

infection, or trauma.
The patient may have decreased sensation to pinprick in the posterior

auricular area.
The patient who has paralysis of the stapedius muscle will report
hyperacusis.
Bell phenomenon is observed on attempted eye closure.
With weakness/paralysis of the orbicularis oculi muscle (facial nerve

innervation) and normal function of the levator muscle (oculomotor nerve
innervation) and Mueller muscle (sympathetic innervation), eye closure
may be partial or absent. The tear reflex may also be absent in many cases
of Bell palsy. For these reasons the patient may have decreased tearing and
susceptibility to corneal abrasion and dryness of the eye. The patient may
appear to have loss of corneal reflex on the affected side; however, the
contralateral eye blinks when testing the corneal reflex on the affected
side.
A careful oral examination must be performed.

o
Taste and salivation are affected in many patients with Bell palsy.
Taste may be assessed by holding the tongue with gauze and testing each
side of the tongue independently with salt, sugar, and vinegar. The mouth
must be washed after testing with different substances. The affected side
has decreased taste as compared to the normal side.
Careful neurologic examination is necessary in patients with facial paralysis. A

neurologic abnormality warrants neurologic referral and further testing such as
MRI of the brain, lumbar puncture, and electromyography (EMG) where
appropriate.
Causes: See Pathophysiology.
23
DIFFERENTIALS
Section 4 of 11
Bibliography
Acute Inflammatory Demyelinating Polyradiculoneuropathy

Amyloid Angiopathy
Anterior Circulation Stroke
Arsenic
Basilar Artery Thrombosis
Benign Skull Tumors
Brainstem Gliomas
Cerebral Aneurysms
Guillain-Barre Syndrome in Childhood
Intracranial Hemorrhage
Low-Grade Astrocytoma
Lyme Disease
Meningioma
Meningococcal Meningitis
Multiple Sclerosis
Mbius Syndrome
Neurofibromatosis, Type 2
Neurosarcoidosis
Neurosyphilis
Tuberculous Meningitis
Basal skull fractures
Barotrauma
Botulism
Carcinomatosis
Carotid disease and stroke
Diphtheria
Facial injuries
Forceps delivery
HIV
Iatrogenic (as in otologic, neurotologic, skull base, or parotid surgery)
Idiopathic
Infection
Intratemporal internal carotid artery aneurysm
Malignant otitis externa
Meningitis
Mumps
Parotid tumor
Ramsay Hunt syndrome
24
Sarcoma
Teratoma
Tetanus
Thalidomide exposure
Trauma
Toxic
Vascular
Wegener vasculitis
WORKUP
Section 5 of 11
Bibliography
Lab Studies:
In areas where Lyme disease is endemic, Lyme titers (IgM and IgG) should be
obtained.
Blood glucose or hemoglobin A1c may be obtained to determine if the patient has
undiagnosed diabetes.
Serum titers for HSV may be obtained, but this is usually not helpful owing to the
ubiquitous nature of this virus.
Imaging Studies:
If the history and physical examination lead to a diagnosis of Bell palsy, then
immediate imaging is not necessary.
Imaging is not required because most patients with Bell

palsy improve within 8-10 weeks. If the paralysis does
not improve or worsens, imaging may be useful.
The MRI of patients with Bell palsy may show enhancement

of the seventh nerve at, or near, the geniculate ganglion.
However, if the paralysis progresses over weeks, the
possibility is high of a neoplasm compressing the
seventh cranial nerve. Tumors that compress or involve
the seventh cranial nerve include schwannoma (most
common), hemangioma, meningioma, and sclerosing
hemangioma.
25
MRI is preferred for imaging the cerebellopontine angle.
If the patient has a history of trauma, CT scan of the

temporal bone may be required.
If the patient has a palpable parotid mass, imaging may be

necessary.
Other Tests:
The following tests may be performed in the office setting. However, they require
both the patient's and physician's time. They may be helpful in assessing the
extent of the damage to the seventh nerve.
o
The stethoscope loudness test may be used to assess the

functioning of the stapedius muscle. The patient wears the stethoscope,
and the activated tuning fork is placed at the bell of the stethoscope. The
loud sound will lateralize to the side of the paralyzed stapedius muscle
The Schirmer blotting test may be used to assess tearing

function. The use of benzene will stimulate the nasolacrimal reflex, and
the degree of tearing can be compared between the paralyzed and normal
sides.
Salivary flow also may be tested. The physician places a

small catheter into both the paralyzed and normal submandibular glands.
The patient is then asked to suck on a lemon, and the salivary flow is
compared between the 2 sides. The normal side is the control.
Useful tests for evaluation of the function of the facial nerve include nerve
conduction testing and EMG.
o
These tests may aid in assessing the outcome of a patient who has
persistent and severe Bell palsy. This test is most useful when performed
3-10 days after the onset of paralysis.
Nerve conduction responses are abnormal if a

difference of 50% in amplitude between the
paralyzed and normal side is detected; a
difference of 90% between the 2 sides suggests a
poorer prognosis.
26
o
May et al demonstrated that prognosis may be

favorable if the motor amplitude of the affected
side was greater than 25% of that of the normal
side.
An incomplete recovery was observed in patients
whose results demonstrated less than 25%
amplitude on the paralyzed side.
Blink reflexes can be used to measure conduction
across the involved segment but they are
commonly absent in Bell palsy.
Brainstem auditory-evoked response (BAER) may be

obtained in patients with peripheral seventh nerve
lesions and other neurologic involvement.
o
BAER measures the transmission of response

through the brain stem and is effective in
detecting, notably, retrocochlear lesions.
Hendrix and Melnick evaluated BAER of 17

patients with Bell palsy. They found no evidence of
retrocochlear lesions of the auditory system in
any of their patients with Bell palsy.
In another study by Shannon et al, BAER was recorded in 27

patients with Bell palsy; only 6 patients had
prolonged brainstem transmission but normal
auditory function.
These studies were small and do not support routine use of BAER in
patients with Bell palsy. However, when a patient
presents with multiple cranial neuropathies, ie, of
CN VII and VIII, BAER may be useful.
Histologic Findings: A review of 12 autopsy cases of patients with Bell palsy was
summarized in Peter Dyck's Peripheral Neuropathy. This stated that the
majority of cases showed inflammatory changes around the
27
mastoid cells and walls of the arteries. The most common
site of involvement was the geniculate ganglion.
Surgical findings described constriction of the nerve at the
stylomastoid foramen with swelling of the nerve itself.
Microscopic findings showed an inflammatory reaction with
infiltration of macrophages on the nerve.
TREATMENT
Section 6 of 11
Bibliography
Medical Care: In general, persons with true Bell palsy have an

excellent prognosis. The goals of treatment are to improve function of the facial
nerve and reduce neuronal damage. Many issues must be addressed in treating patients
with Bell palsy. The most important consideration is the onset of symptoms. Treatment
may be considered for patients who have the onset of paralysis within 1-7 days of the
initial office visit. The American Academy of Neurology published
a practice parameter in 2001 stating that steroids are
probably effective and acyclovir (with prednisone) is
possibly effective for treatment of Bell palsy.
There was insufficient evidence for any

recommendation on facial decompression
surgery. Because treatment of Bell palsy is still controversial, a study is currently
being performed in Scotland. It is a placebo-controlled study that will
determine the efficacy of prednisone versus prednisone and
acyclovir versus placebo.
The most widely accepted treatment for Bell palsy is

corticosteroids. However, the use of steroids is still controversial because
most patients recover without treatment.
o
o
The recommended dose of prednisone is 1 mg/kg or 60 mg/d for 6 days,

followed by a taper, for a total of 10 days.
Many trials have been carried out to study the efficacy of prednisone in
Bell palsy. Early studies had small numbers of patients and variable
outcomes.
28
In 1972, Adour et al conducted a large, controlled clinical trial, which found
that 89% of patients treated with prednisone had full recovery as compared to
64% of patients treated with placebo.
o
When using corticosteroids for the treatment of Bell palsy, caution should
be used in patients with tuberculosis, peptic ulcer disease, diabetes
mellitus, renal or hepatic dysfunction, or malignant hypertension.
Since HSV is widely accepted as the likely etiologic

agent of Bell palsy, trials using acyclovir have been
conducted. The dose of acyclovir is 400 mg orally 5 times
per day.
o
A prospective randomized trial with 101 patients comparing prednisone

and acyclovir demonstrated that the prednisone group had a better clinical
recovery. In another prospective randomized trial with 99 patients,
prednisone monotherapy was compared to the combination of prednisone
and acyclovir. This study demonstrated that
combination therapy was more effective in
preventing nerve degeneration as measured by
electrodiagnostic tests.
Whether to use prednisone alone or combination therapy is left to the

discretion of the physician. For patients who have a contraindication to
steroid therapy, acyclovir may be given as solitary treatment.
That eye care is imperative in Bell palsy is accepted universally. The patient's eye
is at risk for drying, corneal abrasion, and corneal ulcers. Eye care includes
artificial tears for use during the day as well as eyeglasses or shields. At night, eye
lubricants may be used. If artificial tears are not effective during the daytime, then
lubricants may be used; however, they may cause blurring of vision.
Surgical Care: Surgery for Bell palsy is controversial. In the

past, surgical decompression of the facial nerve was
considered for patients whose facial muscles demonstrated
less than 90% of normal activity on electrophysiologic
studies. Surgical decompression of the facial nerve involves
a middle fossa craniotomy with an extradural approach.
However, recent trials suggest this is not beneficial in
patients with Bell palsy.
Consultations: If the initial impression based on the history and physical examination is
not Bell palsy, then a neurologic or otolaryngologic consultation is needed.
29
If the paralysis persists for several months, a neurology or otolaryngology

consultation should be sought.
Patients who report persistent dry eye or painful eye should be referred to an
ophthalmologist.
the goals of pharmacotherapy are to reduce morbidity and prevent complications.
Drug Category: Corticosteroids -- Prednisone can be used but has many adverse
effects including fluid retention, hypokalemia, myopathy, peptic ulcer, headache
(pseudotumor), menstrual irregularities, cataracts, glaucoma, and manifestation of latent
diabetes mellitus. Signs of infection may also be masked in patients taking prednisone.
Physicians should use caution when using prednisone in patients with the aforementioned
conditions.
Drug Name
Prednisone (Deltasone, Orasone, Meticorten) -Glucocorticoid absorbed readily from GI tract.

It has anti-inflammatory and immunemodulating effects, and profound and varied
metabolic effects.
Adult Dose
1 mg/kg or 60 mg PO qd for 7 d followed by

taper for total of 10 d
Pediatric Dose
1 mg/kg PO qd for 6 d followed by taper for

total of 10 d
Documented hypersensitivity; severe

uncontrolled diabetes; systemic fungal
Contraindications infections; peptic ulcer disease; tuberculosis;
severe osteoporosis; severe adverse reactions
to corticosteroids
Interactions
Pregnancy
Precautions
Drugs that induce hepatic enzymes may

increase clearancethese include
phenobarbital, phenytoin, and rifampin;
patients on aspirin or Coumadin must be
monitored closely for GI bleeding
B - Usually safe but benefits must outweigh
the risks.
Patients are at risk for hyperglycemia,
electrolyte abnormalities (especially
hypokalemia in patients taking diuretics),
osteoporosis, avascular necrosis, psychosis,
and myopathy or worsening weakness in
patients with myasthenia gravis; abrupt
30
discontinuation of prednisone without taper
puts patient at risk for adrenal crisis
Drug Category: Antiviral medication -- Acyclovir has been used in the treatment of
Bell palsy in combination with prednisone or used alone in patients who cannot take
prednisone.
Drug Name
Acyclovir (Zovirax) -- Antiviral drug that has

inhibitory activity against HSV-1, HSV-2, and
VZV; selectively taken up by infected cells.
Adult Dose
400 mg PO 5 times/d for 10 d
Pediatric Dose
<2 years: Not established

>2 years: 20 mg/kg PO for 10 d
Contraindications Documented hypersensitivity

Interactions
Probenecid or zidovudine prolongs half-life

and may increase CNS toxicity
Pregnancy
C - Safety for use during pregnancy has not

been established.
Precautions
Caution in renal failure or when using

nephrotoxic drugs
FOLLOW-UP
Section 8 of 11
Bibliography
Further Outpatient Care:
If the paralysis is not resolved or is progressing to complete paralysis, a thorough

neurologic and HEENT examination should be performed to rule out neoplastic
causes of seventh nerve palsy.
The patient should be monitored if the initial EMG

shows the involved facial muscles to have less than
25% of the function of the normal side.
If the residual paralysis is severe, the patient should be referred for counseling.
Complications:
Approximately 30% of patients with Bell palsy experience sequelae of the

paralysis, which include incomplete motor recovery, incomplete sensory
regeneration, and parasympathetic impairment.
31
o
Incomplete motor recovery may manifest as oral incompetence or

epiphora.
Incomplete sensory recovery may result in dysgeusia (impairment of taste)

or ageusia (loss of taste).
Parasympathetic impairment causes aberrant function of lacrimal glands,

which manifests as crocodile tears; patients report shedding tears while
eating.
Prognosis:
The natural course of Bell palsy varies from early complete

recovery to substantial nerve injury resulting in persistent
paralysis and synkinesis.
One third of patients regain complete recovery of facial

motor function without sequelae.
One third of patients have incomplete recovery of facial

motor function. These patients do not have any noticeable
abnormalities.
The remainder of patients suffer from permanent

neurological and cosmetic abnormalities, which are
apparent.
Patient Education:
To prevent corneal abrasions, the patient should be educated concerning eye care.
They also should be encouraged to do facial muscle exercises using passive range
of motion as well as actively closing their eyes and smiling.
For excellent patient education resources, visit eMedicine's Brain and Nervous
System Center. Also, see eMedicine's patient education article Bell Palsy.
MISCELLANEOUS
Section 9 of 11
Bibliography
32
Medical/Legal Pitfalls:
In most cases, the diagnosis of Bell palsy is straightforward as long as the patient
underwent a thorough history and physical examination. Failure to recognize
structural, infectious, or vascular lesions leading to seventh nerve damage may
result in further deterioration of the patient's condition. For example, if other
cranial nerve, motor, or sensory symptoms were present at the time, then treatable
or preventable nervous system diseases should be sought. These may include
stroke, GBS, basilar meningitis, or cerebellar pontine angle tumor.
PICTURES
Section 10 of 11
Bibliography
Caption: Picture 1. The facial nerve

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BIBLIOGRAPHY
Section 11 of 11
33
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Yanagihara N: Incidence of Bell's palsy. Ann Otol Rhinol Laryngol Suppl 1988
Nov-Dec; 137: 3-4[Medline].
Zavlan C, Hou J, Selesnick S: Bell's palsy: an update on causes, recognition,
therapy. The Consultant 1999: 39-48.
Hemifacial Spasm (HFS)

http://emedicine.com/NEURO/topic154.htm
Last Updated: October 11, 2006
Synonyms and related keywords: craniofacial movement disorders, facial myoclonus,

facial dystonia, botulinum toxin, BTX therapy
Section 1 of 10
AUTHOR INFORMATION
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Bibliography
Author: Steven Gulevich, MD, Department of Neurology, Swedish Medical Center of

Englewood, Colorado
Steven Gulevich, MD, is a member of the following medical societies: American
Academy of Neurology, American Medical Association, and Colorado Medical Society
Editor(s): Stephen A Berman, MD, PhD, Professor, Department of Internal Medicine,
Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River
Junction Veterans Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy
36
Editor, eMedicine; Glenn Lopate, MD, Associate Professor, Department of Neurology,
Division of Neuromuscular Diseases, Washington University School of Medicine; Chief
of Neurology, St Louis ConnectCare; Selim R Benbadis, MD, Professor, Director of
Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery,
University of South Florida College of Medicine; and Nicholas Lorenzo, MD, Chief
Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
INTRODUCTION
Section 2 of 10
Background: Facial musculature is subject to the same movement disorders as muscles

of the limbs or trunk. Myoclonus, dystonia, and other movement disorders present with
specific syndromes in the facial musculature. An understanding of the underlying
mechanism leads to appropriate diagnostic evaluation and potential treatment.
Although specific treatments are available for many craniofacial movement disorders,
botulinum toxin (BTX) chemodenervation has proven useful in many of these disorders,
supplanting surgery and medical therapy.
Pathophysiology: First described by Gowers in 1884, hemifacial
spasm (HFS) represents a segmental myoclonus of muscles
innervated by the facial nerve. The disorder presents in the
fifth or sixth decade of life, almost always unilaterally,
although bilateral involvement may occur rarely in severe
cases.
HFS generally begins with brief clonic movements of the
orbicularis oculi and spreads over years to other facial
muscles (corrugator, frontalis, orbicularis oris, platysma,
zygomaticus).
Clonic movements progress to sustained tonic contractions of involved musculature.
Chronic irritation of the facial nerve or nucleus, the near-universal cause of HFS,
may arise from numerous underlying conditions.
Irritation of the facial nerve nucleus is believed to lead to
hyperexcitability of the facial nerve nucleus, while irritation
of the proximal nerve segment may cause ephaptic
transmission within the facial nerve. Either mechanism
explains the rhythmic involuntary myoclonic contractions
observed in HFS.
37
Compressive lesions (eg, tumor, arteriovenous malformation,
Paget disease) and noncompressive lesions (eg, stroke,
multiple sclerosis plaque, basilar meningitis) may present as
HFS.
Most instances of hemifacial spasm previously thought to be
idiopathic were probably caused by aberrant blood vessels
(eg, distal branches of the anterior inferior cerebellar artery
or vertebral artery) compressing the facial nerve within the
cerebellopontine angle.
Race: All races are affected equally.
Sex: A slight female preponderance exists in HFS.
Age:
Idiopathic hemifacial spasm typically begins in the fifth

or sixth decade of life.
Onset of hemifacial spasm in patients younger than 40

years is unusual and often heralds an underlying
neurologic illness (eg, multiple sclerosis).
CLINICAL
Section 3 of 10
History: Involuntary facial movement is the only symptom.

Fatigue, anxiety, or reading may precipitate the movements.
Hemimasticatory spasm
o
Hemimasticatory spasm is analogous to HFS and

occurs with irritation to the motor trigeminal
nerve.
38
This rare condition is a segmental myoclonus and

presents with unilateral involuntary contractions
of the trigeminally innervated muscles of
mastication (usually the masseter).
Similar to HFS, hemimasticatory spasm responds

to treatment with medications and BTX.
However, less evidence exists that exploratory

surgery benefits patients with this condition.
Myoclonic movements
Myoclonic movements affecting facial musculature

also may arise from lesions at the brain or
brainstem level.
These are distinguished from HFS by the
distribution of abnormal movements (more
generalized, possibly bilateral) and possibly by
electrodiagnostic evaluation.
Imaging studies may yield an underlying cause.
Central myoclonus responds to anticonvulsant

management.
Oromandibular dystonia
o
Oromandibular dystonia (OMD) refers to dystonia affecting the lower

facial musculature, predominantly the jaw, pharynx, and tongue.
When OMD occurs in conjunction with blepharospasm, the disorder is

termed Meige syndrome.
Jaw-opening forms of OMD indicate primary involvement of the digastric

and lateral pterygoid. Jaw-closing OMD involves the masseter, temporalis,
and medial pterygoid.
Jaw deviation, indicating predominant involvement of the lateral

pterygoid, is rare.
39
o
BTX is the preferred treatment for OMD and is most effective in the jawclosure type.
Medications seldom yield acceptable results. When medications must be

used, employ the same agents as for blepharospasm.
Because of the risk of aspiration, never inject BTX into the tongue.
Craniofacial tremor
o Craniofacial tremor may occur in association with essential tremor,
Parkinson disease, thyroid dysfunction, or electrolyte disturbance.
o It occurs rarely in isolation.
o Focal motor seizures must occasionally be distinguished from other facial
movement disorders, particularly HFS.
o Postictal weakness and greater involvement of the lower face are
distinguishing features of focal motor seizures.
Facial chorea
o Facial chorea occurs in the context of a systemic movement disorder (eg,
Huntington disease, Sydenham chorea).
o Chorea is a random, flowing, nonpatterned set of movements.
o A related disorder, spontaneous orofacial dyskinesia of the elderly, is
observed primarily in the edentulous. It usually responds to proper fitting
of dentures.
Tics
o
o
o
o
Facial tics are brief, repetitive, coordinated, semipurposeful movements of

grouped facial and neck muscles.
Tics may occur physiologically or in association with diffuse
encephalopathy.
Some medications (ie, anticonvulsants, caffeine, methylphenidate,
antiparkinsonian agents) are associated with producing tics.
Single, repetitive, stereotyped movements (eg, repetitive grimacing, throat
clearing, vocalizations) define a simple tic disorder.
Facial myokymia
o Facial myokymia appears as vermicular twitching under the skin, often
with a wavelike spread.
o This is distinguished from other abnormal facial movements by
characteristic electromyogram discharges presenting as brief, repetitive
bursts of motor unit potentials firing at 2-60 Hz interrupted by periods of
silence of up to a few seconds.
o Facial myokymia may occur with any brainstem process. Severe cases
may benefit from BTX.
40
o
Most cases are idiopathic and resolve without treatment over several
weeks.
Physical:
The only physical finding in hemifacial spasm is involuntary

facial movements.
Spontaneous HFS manifests with facial spasms that

represent myoclonic jerks and are analogous to segmental
myoclonus, which may affect other body regions.
Postparalytic HFS (following facial nerve trauma such as

Bell palsy) manifests as facial synkinesis and contracture.
Causes:
Idiopathic
Vascular compression
Facial nerve compression by mass
Brainstem lesion such as stroke or multiple sclerosis

plaque
Secondary to trauma or Bell palsy
DIFFERENTIALS
Section 4 of 10

Benign essential
Blepharospasm
41
OMD
Craniofacial tremor
Facial chorea
Tics
Facial myokymia
WORKUP
Section 5 of 10
Lab Studies:
Early cases of HFS may be difficult to distinguish from facial myokymia, tics, or
myoclonus originating in the cortex or brainstem.
o
Neurophysiologic testing can be invaluable.
Spread and variable synkinesis on blink reflex

testing and high-frequency discharges on EMG
(with appropriate clinical findings) are diagnostic.
Stimulation of one branch of the facial nerve may

spread and elicit a response in a muscle supplied
by a different branch.
Blink reflex studies may reveal synkinesis, which is not present in

essential blepharospasm, dystonia, or seizures.
Needle EMG shows irregular, brief, high-frequency

bursts (150-400 Hz) of motor unit potentials, which
correlate with clinically observed facial
movements.
Imaging Studies:
Magnetic resonance imaging is the imaging study of choice,

especially if an underlying compressive lesion is suspected.
42
Perform angiography and/or magnetic resonance

angiography prior to a vascular decompression surgical
procedure.
Other Tests:
Cerebral angiography offers little diagnostic value in HFS.

Ectatic blood vessels rarely are identified, and it is difficult to correlate vessels
with the facial nerve. As angiography may identify an aneurysm or vascular
anomaly, it often is performed prior to decompressive surgery to clarify the
vascular anatomy.
Procedures:
In most patients, the treatment of choice is
injection of BTX under EMG guidance .
Chemodenervation safely and effectively treats most patients, especially

those with sustained contractions.
Relief of spasms occurs 3-5 days after injection and lasts approximately 6
months.
Side effects of BTX injection (eg, facial asymmetry, ptosis, facial weakness)
usually are transient.
o
Most patients report a highly satisfactory response.
Caution patients that although BTX ablates the muscular spasm, the
sensation of spasm often persists.
TREATMENT
Section 6 of 10
Medical Care:
Use medications in patients with noncompressive lesions and early idiopathic

HFS.
43
Response to medication varies but can be satisfactory in early or mild cases.
The most helpful agents are carbamazepine and benzodiazepines (eg,

clonazepam).
Often, medication effects attenuate over time, necessitating more aggressive

treatment.
Medications may be used in early HFS (when spasms are mild and infrequent) or
in patients who decline BTX injection.
Surgical Care:
Treat compressive lesions surgically.

o
Ectatic blood vessels cause HFS by compressing the facial nerve as it exits
the brainstem.
Surgical decompression of these blood vessels can yield excellent results.
Patients with apparently idiopathic HFS may benefit from posterior fossa
exploration and microvascular decompression.
Myectomy rarely is required.
MEDICATION
Section 7 of 10
The goal of pharmacotherapy is reduction of abnormal muscle

contractions. Botulinum toxin type A is the treatment of choice.
Carbamazepine, benzodiazepines, and baclofen also may be used
in patients who refuse BTX injections or who are not surgical
candidates.
Drug Category: Toxins -- Botulinum toxin type A is the drug of choice. It causes
presynaptic paralysis of the myoneural junction and reduces abnormal contractions.
Therapeutic effects may last 3-6 months.
Botulinum toxin type B is useful in reducing excessive, abnormal contractions associated
with blepharospasm; binds to receptor sites on the motor nerve terminals and after uptake
inhibits release of acetylcholine, blocking transmission of impulses in neuromuscular
tissue; 7-14 d after administering initial dose, assess patients for a satisfactory response;
44
increase doses 2-fold over previously administered dose for patients who experience
incomplete paralysis of the target muscle.
Drug Name
Botulinum toxin type A (BOTOX) -- Useful

in reducing excessive, abnormal contractions
associated with blepharospasm; binds to
receptor sites on the motor nerve terminals and
after uptake inhibits release of acetylcholine,
blocking transmission of impulses in
neuromuscular tissue; 7-14 d after
administering initial dose, assess patients for a
satisfactory response; increase doses 2-fold
over previously administered dose for patients
who experience incomplete paralysis of the
target muscle.
Adult Dose
Initial dosing: Inject 1.25-2.5 U (0.05-0.1 mL)

IM into abnormally contracting muscles via
hollow EMG needle; not to exceed 25 U when
given as a single injection or 200 U when
given as a cumulative dose in a 30 d period
Pediatric Dose

>12 years: Administer as in adults

Interactions
Caution in patients taking aminoglycoside

antibiotics or any other drug that interferes
with neuromuscular transmission as they may
potentiate effect of botulinum toxin
Pregnancy

been established.
Precautions
Do not exceed recommended dosages and

frequencies of administration; presence of
antibodies may reduce effectiveness of therapy
Drug Name
Botulinum toxin type B (Myobloc) -- When

botulinum toxin injection is indicated and type
A toxin is ineffective, injection with type B
(Myobloc) should be considered.
Adult Dose
Not established: This author suggests starting

dose of 500-1000 U IM, divided among
abnormally contracting muscles
Pediatric Dose
Not established
45
Documented hypersensitivity to drug; patients
with hypersensitivity to type A toxin,
Contraindications hypersensitivity to type B is significant
concern, and use of type B in these patients is
not recommended
Interactions
Caution in patients taking aminoglycoside

antibiotics or any other drug that interferes
with neuromuscular transmission because they
may potentiate effect of botulinum toxin
Pregnancy

been established.
Precautions
Likely to cause pain at injection site for a few

seconds immediately following administration
Drug Category: Benzodiazepines -- May potentiate effects of GABA and facilitate

inhibitory GABA neurotransmission. May act in the spinal cord to induce muscle
relaxation. Individualize treatment for each patient.
Drug Name
Clonazepam (Klonopin) -- Useful in

suppressing muscle contractions by facilitating
inhibitory GABA neurotransmission and other
inhibitory transmitters.
Adult Dose
Initial dose: 1.5 mg PO in 3 divided doses

Maintenance dose: Increase initial dose by 0.51 mg PO q3d to a dose range of 0.05-0.2
mg/kg
Alternative maintenance dose: 7-12 mg/d PO;
not to exceed 20 mg/d
Pediatric Dose
<10 years or <30 kg:

Initial dose: 0.010.03 mg/kg/d PO bid/tid
Maintenance dose: Increase initial dose by 0.5
mg PO q3d to a dose range of 0.1-0.2 mg/kg/d
divided tid; not to exceed 0.2 mg/kg/d
Contraindications
Interactions
Documented hypersensitivity; severe liver

disease; acute narrow-angle glaucoma
Phenytoin and barbiturates may increase
clonazepam clearance and reduce its effects;
toxicity in the CNS is significantly increased
46
when used concurrently with CNS depressants
Pregnancy

been established.
Precautions
Caution with chronic respiratory disease or

impaired renal function; withdrawal symptoms
can result from abrupt discontinuation
Drug Category: Muscle relaxants -- May inhibit transmission of monosynaptic and

polysynaptic reflexes at the spinal cord level.
Drug Name
Baclofen (Lioresal) -- May induce

hyperpolarization of afferent terminals and
inhibit both monosynaptic and polysynaptic
reflexes at the spinal level.
Adult Dose
Administer 5 mg PO tid for 3 d; 10 mg PO tid

for 3 d; 15 mg PO tid for 3 d; 20 mg PO tid for
3 d; thereafter, additional increases may be
necessary; not to exceed 80 mg/d PO divided
qid
Pediatric Dose
Not established

Interactions
Opiate analgesics, benzodiazepines,

hypertensive agents, alcohol, tricyclic
antidepressants, guanabenz, MAOIs, and
clindamycin may increase effects
Pregnancy
D - Unsafe in pregnancy
Precautions
Caution when spasticity is used to obtain

increased function and with patients with a
history of autonomic dysreflexia; withdrawal
can cause autonomic dysreflexia
Drug Category: Anticonvulsants -- Used to manage severe muscle spasms and

provide analgesia and mild sedation. Anticonvulsants are probably the best medications
in terms of efficacy and long-term safety when BTX and/or surgery are not an option.
Drug Name
Carbamazepine (Tegretol) -Effective in treatment of HFS and complex

partial seizures; appears to act by reducing
polysynaptic responses and blocking
posttetanic potentiation; once a response is
attained, attempt to reduce dose to the
47
minimum effective level or discontinue at least
once every 3 mo; in patients who cannot
tolerate carbamazepine, consider
oxcarbazepine (dosage not yet established).
Adult Dose
Pediatric Dose
Contraindications
Interactions
200 mg PO bid (100 mg qid susp)

Increase at weekly intervals by no more than
200 mg/d using a tid/qid regimen (bid with
extended release) until the best response is
obtained; not to exceed 1600 mg/d
Documented hypersensitivity; patients with
history of bone marrow depression
Do not use concomitantly with MAOIs;
discontinue MAOIs at least 14 d before
administration; may alter hepatic metabolism,
causing decrease in primidone and
phenobarbital serum concentrations and
increase in carbamazepine concentrations;
plasma levels may increase and toxicity may
result when taken concurrently with
cimetidine; appears to be more significant
when cimetidine is added to carbamazepine
during the first 4 wk of therapy; levels increase
significantly within 30 d of danazol
administration; avoid concomitant
administration if possible
Pregnancy
D - Unsafe in pregnancy
Precautions
Not a simple analgesic; do not use for relief of

minor aches or pains; caution in patients with
increased intraocular pressure; obtain CBC and
serum-iron baseline prior to initiating
treatment, then monthly CBCs and iron during
the first 2 mo; thereafter, obtain CBC,
differential, and platelet count yearly or every
other year; can cause drowsiness, dizziness,
and blurred vision; patients should observe
caution while driving or performing other tasks
requiring alertness
Drug Name
Trileptal (Oxcarbazepine) -Effective in partial complex epilepsy,

Oxcarbazepine shows promise in HFS.
Oxcarbazepine may be considered when first-
48
line agents (e.g., botulinum toxin,
carbamazepine) have failed or are
contraindicated.
Adult Dose
Pediatric Dose
Monotherapy: 150 mg or 300 mg PO bid

initially; dose may be increased by 300 mg/d
q3d; maximum recommended daily dose of
1200-2400 mg in divided dosing; elderly
patients may require slower titrations
<4 years
Not established
4-16 years
Adjunctive therapy: 8-10 mg/kg/d PO divided
bid initially, not to exceed 600 mg/d; gradually
increase to target dose over 2 wk; target
adjunctive dose is based on body weight; 20-29
kg = 900 mg/d, 29.1-39 kg = 1200 mg/d, >39
kg = 1800 mg/d
Conversion to monotherapy: 8-10 mg/kg/d PO
divided bid initially; gradually reduce the dose
of concomitant anticonvulsants over 3-6 wk;
may gradually increase oxcarbazepine dose if
clinically indicated by increments not to
exceed 10 mg/kg/d at qwk to recommended
monotherapy dose; monitor patients closely
during this transition phase for anticonvulsant
adverse effects
Monotherapy: 8-10 mg/kg/d PO divided bid;
may increase by 5 mg/kg/d q3d to
recommended daily dose; maintenance
monotherapy dose is based on body weight;
20-24 kg = 600-900 mg/kg/d, 25-34 kg = 9001200 mg/kg/d, 35-44 kg = 900-1500 mg/kg/d,
45-49 kg = 1200-1500 mg/kg/d, 50-59 kg =
1200-1800 mg/kd/d, 60-69 kg = 1200-2100
mg/kg/d, >70 kg = 1500-2100 mg/kg/d

Interactions
May decrease levels of dihydropyridine

calcium antagonists and oral contraceptives;
can reduce serum concentrations of
carbamazepine, phenobarbital, phenytoin and
valproic acid; when oxcarbazepine is given in
doses above 1200 mg/d may increase
phenytoin and phenobarbital serum
concentrations significantly; oxcarbazepine can
reduce serum concentrations of oral
49
contraceptives and make oral contraceptives
ineffective; can increase clearance of
felodipine
Pregnancy

been established.
Precautions
May decrease levels of dihydropyridine

calcium antagonists and oral contraceptives;
can reduce serum concentrations of
carbamazepine, phenobarbital, phenytoin and
valproic acid; when oxcarbazepine is given in
doses above 1200 mg/d may increase
phenytoin and phenobarbital serum
concentrations significantly; oxcarbazepine can
reduce serum concentrations of oral
contraceptives and make oral contraceptives
ineffective; can increase clearance of
felodipine
FOLLOW-UP
Section 8 of 10
Prognosis:
HFS is a progressive, nonfatal illness. It almost always

responds favorably to treatment.
Patient Education:
For excellent patient education resources, visit eMedicine's Procedures Center.

Also, see eMedicine's patient education article BOTOX Injections.
MISCELLANEOUS
Section 9 of 10
At initial evaluation, consider HFS a symptom, not a diagnosis.
50
An abnormal neurologic examination (except for the facial

movements) should prompt the search for an underlying
cause (eg, compressive lesion, tumor, stroke).
Look for demyelinating disease as a cause when HFS

presents before 50 years.
BIBLIOGRAPHY
Section 10 of 10
Adler CH, Zimmerman RA, Savino PJ, et al: Hemifacial spasm: evaluation by
magnetic resonance imaging and magnetic resonance tomographic angiography.
Ann Neurol 1992 Oct; 32(4): 502-6[Medline].
Colosimo C, Chianese M, Giovannelli M, et al: Botulinum toxin type B in
blepharospasm and hemifacial spasm. J Neurol Neurosurg Psychiatry 2003 May;
74(5): 687[Medline].
Cruccu G, Inghilleri M, Berardelli A, et al: Pathophysiology of hemimasticatory
spasm. J Neurol Neurosurg Psychiatry 1994 Jan; 57(1): 43-50[Medline].
Elston JS: The management of blepharospasm and hemifacial spasm. J Neurol
1992 Jan; 239(1): 5-8[Medline].
Jankovic J, Schwartz K, Donovan DT: Botulinum toxin treatment of cranialcervical dystonia, spasmodic dysphonia, other focal dystonias and hemifacial
spasm. J Neurol Neurosurg Psychiatry 1990 Aug; 53(8): 633-9[Medline].
Jannetta PJ, Abbasy M, Maroon JC, et al: Etiology and definitive microsurgical
treatment of hemifacial spasm. Operative techniques and results in 47 patients. J
Neurosurg 1977 Sep; 47(3): 321-8[Medline].
Kraft SP, Lang AE: Cranial dystonia, blepharospasm and hemifacial spasm:
clinical features and treatment, including the use of botulinum toxin. CMAJ 1988
Nov 1; 139(9): 837-44[Medline].
Mauriello JA, Leone T, Dhillon S, et al: Treatment choices of 119 patients with
hemifacial spasm over 11 years. Clin Neurol Neurosurg 1996 Aug; 98(3): 2136[Medline].
Moller AR: The cranial nerve vascular compression syndrome: I. A review of
treatment. Acta Neurochir (Wien) 1991; 113(1-2): 18-23[Medline].
Moller AR: The cranial nerve vascular compression syndrome: II. A review of
pathophysiology. Acta Neurochir (Wien) 1991; 113(1-2): 24-30[Medline].
Reimer J, Gilg K, Karow A, et al: Health-related quality of life in blepharospasm
or hemifacial spasm. Acta Neurol Scand 2005 Jan; 111(1): 64-70[Medline].
NOTE:
Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment
therapies daily. The authors, editors, and publisher of this journal have used their best efforts to provide information that is up-todate and accurate and is generally accepted within medical standards at the time of publication. However, as medical science is
constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the
publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions
or errors in the article or for the results of using this information. The reader should confirm the information in this article from other
sources prior to use. In particular, all drug doses, indications, and contraindications should be confirmed in the package insert.
FULL DISCLAIMER
51
Hemifacial Spasm excerpt
Eyelid Myokymia
http://www.emedicine.com/oph/topic607.htm
Last Updated: August 4, 2005
Synonyms and related keywords: eyelid twitching, eyelid jumping, muscle contractions,
blepharospasm, Meige syndrome, hemifacial spasm, spastic-paretic facial
contracture, botulinum toxin A, BOTOX, BOTOX injections
Section 1 of 10
AUTHOR INFORMATION
Author: Byron L Lam, MD, Professor, Department of Ophthalmology, Bascom Palmer

Eye Institute, University of Miami School of Medicine
Byron L Lam, MD, is a member of the following medical societies: Alpha Omega Alpha,
American Academy of Ophthalmology, American Medical Association, Association for
Research in Vision and Ophthalmology, and Phi Beta Kappa
Editor(s): Ron W Pelton, MD, PhD, Consulting Staff, Department of Surgery, Memorial
Hospital; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine;
Mark T Duffy, MD, PhD, Consulting Staff, Advanced Cosmetic Solutions; Consulting
Staff, Department of Oculoplastic and Reconstructive Surgery, Green Bay Eye Clinic,
BayCare Clinic; Lance L Brown, OD, MD, Ophthalmologist, Regional Eye Center,
Affiliated With Freeman Hospital and St John's Hospital, Joplin, Missouri; and Hampton
Roy, Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of
Arkansas for Medical Sciences
INTRODUCTION
Section 2 of 10
Background: Myokymia is the spontaneous, fine fascicular contractions of muscle

without muscular atrophy or weakness. Eyelid myokymia typically involves the
orbicularis oculi muscle of one of the lower eyelids; occasionally, the upper eyelids also
can be affected. In most cases, eyelid myokymia is benign, self-limited, and not
associated with any disease. Intervention is seldom necessary. Rarely, eyelid myokymia
may occur as a precursor of blepharospasm, Meige syndrome, hemifacial spasm, and
spastic-paretic facial contracture.
Pathophysiology: The pathophysiology of typical eyelid myokymia is not well
understood. The focus of irritation is most likely the nerve fibers within the muscle.
However, pontine dysfunction in the region of the facial nerve nucleus also has been
implicated. Possible precipitating factors include stress, fatigue, and excessive caffeine or
alcohol intake.
52
Frequency:
In the US: The incidence and prevalence of eyelid myokymia are unknown, but
symptoms of eyelid myokymia are not infrequently encountered in the ophthalmic
clinic.
Mortality/Morbidity: Eyelid myokymia is a benign and self-limited condition in most

patients.
Age: Eyelid myokymia may occur at any age.
CLINICAL
Section 3 of 10
History:
Patients with eyelid myokymia usually note sporadic "jumping" or "twitching" of

one of the lower eyelids. The upper eyelids also can be involved. The irregular
contractions are usually unilateral and may occur intermittently for days to
months.
In rare cases, the contractions may be severe enough to move the eye to produce
oscillopsia.
A history of stress, fatigue, and excessive caffeine or alcohol intake may be

present.
Physical:
Fine contractions of the orbicularis oculi may be visible, if the patient has the
contractions during examination.
o
If present, the contractions are usually intermittent and are more apparent
to the patient than to the observer.
The symptoms often improve when the eyelid is pulled manually.
Rarely, the contractions may be vigorous enough to cause movement of

the globe, producing fine nystagmuslike eye movements.
If the eyelid myokymia is associated with contraction of other parts of the face,
blepharospasm, Meige syndrome, hemifacial spasm, and spastic-paretic facial
contracture should be excluded.
Causes: The cause is unknown but may be associated with stress, fatigue, and excessive
caffeine or alcohol intake.
53
DIFFERENTIALS
Section 4 of 10
Blepharospasm, Benign Essential

Meige syndrome - Blepharospasm and oral facial dystonia
Hemifacial spasm - Unilateral facial contraction due to seventh nerve dysfunction
Spastic-paretic facial contracture - Unilateral tonic facial contracture due to pontine
dysfunction, associated usually with multiple sclerosis, and brainstem tumors or vascular
lesions
WORKUP
Section 5 of 10
Imaging Studies:
Brain magnetic resonance imaging (MRI) is not needed for typical eyelid
myokymia but should be considered if facial myokymia, hemifacial spasm, or
spastic paretic facial contracture is suspected.
TREATMENT
Section 6 of 10
Medical Care:
Reassurance and reduction in precipitating factors, if identifiable, are appropriate

for most patients.
Treatment is usually not needed except when symptoms are severe or when
oscillopsia is present.
o
Local subcutaneous botulinum toxin A (BOTOX) injections of 2.5-5

units each to the affected eyelid region provide relief for 12-16 weeks.
Adverse effects include temporary lid laxity, which may produce

lagophthalmus and exposure keratopathy.
The efficacy of other agents has not been proven.
54
he goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Toxins -- May inhibit transmission of impulses in neuromuscular

tissue.
Drug Name
Botulinum toxin A (BOTOX) -- Blocks

neuromuscular conduction by binding to
receptor sites on motor nerve terminals,
entering the nerve terminals and inhibiting the
release of acetylcholine. One treatment is
usually sufficient, but in persistent cases, the
injection may be repeated in 4-6 mo. Injection
should be used only when symptoms are severe
or when oscillopsia is present. Treatment of
pediatric patients is not recommended.
Adult Dose
Local injections of 2.5-5 U each to affected

eyelid region
Pediatric Dose
Not recommended

Interactions
Aminoglycosides or drugs that interfere with

neuromuscular transmission may potentiate
effects of botulinum toxin
Pregnancy

been established.
Precautions
Injections of the upper lids may produce ptosis,

which may last for weeks; do not exceed
recommended dosages and frequencies of
administration; presence of antibodies to
botulinum toxin type A may reduce effects of
therapy
FOLLOW-UP
Section 8 of 10
Further Outpatient Care:
Rarely, eyelid myokymia may occur as a precursor of blepharospasm, Meige

syndrome, hemifacial spasm, facial myokymia, and spastic-paretic facial
contracture.
Advise patients to return for reexamination, if there is a change in symptoms.
55
Deterrence/Prevention:
If precipitating factors can be identified, avoidance will reduce the frequency of

episodes.
Prognosis:
Prognosis is excellent in most cases.
Patient Education:
For excellent patient education resources, visit eMedicine's Procedures Center.

Also, see eMedicine's patient education article BOTOX Injections.
MISCELLANEOUS
Section 9 of 10
Note that persistent myokymia followed by spastic paretic facial contracture is an

important (although uncommon) sign of disease in the dorsal pons, which may be
seen in multiple sclerosis and brainstem neoplasms or vascular lesions.
BIBLIOGRAPHY
Section 10 of 10
Andermann F, et al: Facial myokymia in multiple sclerosis. Brain 1961; 8: 31-44.

Givner I, Jaffe NS: Myokymia of the eyelids. A suggestion as to therapy:
preliminary report. Am J Ophthalmol 1949; 32: 51-55.
Krohel GB, Rosenberg PN: Oscillopsia associated with eyelid myokymia. Am J
Ophthalmol 1986 Nov 15; 102(5): 662-3[Medline].
Lowe R: Facial Twitching. Trans Ophthalmol Soc Aust 1951; 11: 129-133.
Reinecke RD: Translated myokymia of the lower eyelid causing uniocular vertical
pseudonystagmus. Am J Ophthalmol 1973 Jan; 75(1): 150-1[Medline].
Rubin M, Root JD: Electrophysiologic investigation of benign eyelid twitching.
Electromyogr Clin Neurophysiol 1991 Sep; 31(6): 377-81[Medline].
Scott AB: Botulinum toxin for blepharospasm. In: Spaeth G, Katz LJ, Parker KW,
eds. Current Therapy in Ophthalmic Surgery. Toronto: Decker; 1989: 322-324.
Sogg RL, Hoyt WF, Boldrey E: Spastic paretic facial contracture. A rare sign of
brain stem tumor. Neurology 1963 Jul; 13: 607-12[Medline].
NOTE:
56
Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment
therapies daily. The authors, editors, and publisher of this journal have used their best efforts to provide information that is up-todate and accurate and is generally accepted within medical standards at the time of publication. However, as medical science is
constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the
publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions
or errors in the article or for the results of using this information. The reader should confirm the information in this article from other
sources prior to use. In particular, all drug doses, indications, and contraindications should be confirmed in the package insert.
FULL DISCLAIMER
Eyelid Myokymia excerpt
Hemifacial spasm
http://www.answers.com/topic/hemifacial-spasm
Definition
A hemifacial spasm is an involuntary contraction of the muscles of facial expression,
resulting in eyelid closure and upturning of the corner of the mouth and accompanied by
facial weakness.
Description
Hemifacial spasm results in involuntary contraction of the facial muscles limited to one
side of the face. The eyelids are involved, and upturning of the corner of the mouth is
observed. The patient may have facial twitching during periods of sleep. If left untreated,
the twitching may worsen and extend to other facial muscles.
Demographics
Females are affected more than males, regardless of race. Typically, patients afflicted
with hemifacial spasm are in their 40s or 50s.
Causes and symptoms
The cause of hemifacial spasm has been linked to overactivity of the seventh cranial
nerve nucleus that signals facial muscle movement. In other instances, hemifacial spasm
may be caused by compression by a massormal blood vessel or by a lack of blood supply
(ischemia) of the seventh cranial nerve at its origin or by the nucleus itself. It is thought
that compression by a convoluted cerebral artery is the most common cause. In some
patients, no underlying cause can be detected, which is termed an idiopathic hemifacial
spasm. In younger patients, multiple sclerosis may be the cause.
57
Patients will usually report involuntary twitching of one side of the face (hemifacial),
lasting seconds to minutes. Family members may observe facial twitching while the
patient sleeps. Pain or numbness is usually not reported.
Diagnosis
When a clinical diagnosis has been established, imaging of the brain is required to rule
out ischemia, mass lesions, or abnormal vasculature. Magnetic resonance imaging (MRI)
of the brain, with and without contrast, as well as MRI-angiography, are advised. Blood
tests are not required for patients believed to have hemifacial spasm.
Treatment team
Ophthalmologists, neuro-ophthalmologists, and neurologists are physicians who can

diagnose and treat hemifacial spasm. If surgery is indicated as a form of treatment, it is
usually performed by a neurological surgeon.
Treatment
The mainstay of treatment is injection of botulinum toxin to the face, which results in
temporary paralysis of selected muscles of facial expression. Botulinum toxin, commonly
known as Botox (Allergen Inc.), is a neuro-toxin produced by the bacterium, Clostridium
botulinum. This toxin weakens facial muscles by inhibiting the release of a
neurotransmitter, acetylcholine, which results in temporary and partial muscle paralysis.
Botulinum toxin has become an accepted and widely used treatment for hemi-facial
spasm. Although its use is relatively safe and easily injected, the effect of botulinum toxin
is temporary, lasting approximately six months. This necessitates the need for re-injection
or increased doses of the toxin, depending on the patient's response.
If botulinum toxin fails to be effective or the patient does not tolerate it well,
decompression of the seventh cranial nerve can be attempted. This procedure, performed
by a neurosurgeon, entails placing a sponge between the seventh nerve and the vessel
compressing the nerve.
Other treatment options include severing branches of the seventh nerve, destruction of
eyelid and facial musculature, and oral anti-seizure medications. However, oral
medications have proven to be limited in their efficacy and have significant side effects.
Recovery and rehabilitation
There is usually no recovery period following the injection of botulinum toxin. The
maximal effects are usually seen four to seven days following injection.
Clinical trials
Currently there are no clinical trials scheduled to study this disorder.
58
Prognosis
The vast majority of patients responds favorably to injections with a low rate of
complications. A small percentage of patients improves spontaneously, and benefits from
psychotherapy, surgery, or oral medications.
Special concerns
Support groups and information for patients and families are excellent resources that may
improve treatment outcomes and psychosocial ramifications.
Resources
BOOKS
Beers, Mark H., and Robert Berkow, editors. "Cranial Nerve Disorders." The Merck
Manual of Diagnosis and Therapy. Whitehouse Station, NJ: Merck Research
Laboratories, 1999.
Burde, Ronald M., Peter J. Savino, and Jonathan D. Trobe. Clinical Decisions in NeuroOphthalmology, 3rd ed. St. Louis, MO: Mosby, 2002.
Liu, Grant T., Nicholas J. Volpe, and Steven L. Galetta. Neuro-Ophthalmology Diagnosis
and Management, 1st ed. Philadelphia: W.B. Saunders Company, 2001.
Acute Facial Nerve Paralysis

http://icarus.med.utoronto.ca/carr/manual/afnp2.html
PD Warrick BScPhm, Meds '98 McMaster University
Reviewed by Barry Maber MD FRCSC, Clinical Associate Professor
(Otolaryngology), College of Medicine, University of Saskatchewan
Anatomy
The complex anatomy of the seventh cranial nerve and its subsections must be
understood in order to discriminate among the peripheral causes of facial nerve
palsy, and to differentiate these from central etiologies.
Subsections of the Facial Nerve
Supranuclear - from the precentral (motor) and postcentral (sensory) gyri

through the genu of the internal capsule to the facial nucleus in the pons
59
(ipsilateral and contralateral nucleus for upper face, contralateral only for
lower face)
Nuclear - facial nerve travels dorsomedially toward 4 th ventricle, loops
laterally around abducent nucleus, then exits anterolaterally and inferiorly
at the medullopontine junction
Cerebellopontine angle (CPA) - travels with nervus intermedius (NI),
alongside CN V, VIII to internal auditory canal
Intratemporal
o Internal auditory canal (ICA) - travels anterosuperiorly with NI within
canal for 5-12 mm alongside cochlear, vestibular nerves; lateralmost portion is surrounded by thicker periosteum
o Labyrinthine - beginning of " fallopian canal" travels almost totally
inferiorly for 3-5 mm, then makes first (posterior) turn; gives off
geniculate branch (NI) to form geniculate ganglion from which
emerges the greater petrosal nerve (which controls lacrimation via
the sympathetic plexus of internal carotid artery and
pterygopalatine ganglion) and the lesser petrosal nerve (which

controls salivation of parotid gland via the otic ganglion)
60
o
o
Temporal - travels mostly posteriorly for 10-12 mm before reaching

the second (inferior) turn just superior to middle ear
Mastoid - gives off branch to stapedius, chorda tympani (which
unites with lingual nerve of CN V3 to cause salivation in
submandibular and sublingual glands); travels 13 mm inferiorly and
becomes more superficial before emerging from the stylomastoid
foramen
Extracranial - travels anteroinferiorly immediately entering parotid space,

gives off branches to occipitalis, intrinsic muscles of ear (posterior
auricular), posterior belly of digastric and stylohyoid, then separates into
temporofacial and cervicofacial divisions - highly variable anatomy from
this point, but essentially 5 main divisions:
o Temporal - function differentiates upper motor from lower motor
neuron lesion
o Zygomatic - most important, as function prevents exposure keratitis
of cornea
o Buccal
o Marginal Mandibular
o Cervical
Helpful Signs
Some of the most helpful findings to differentiate upper motor neuron (UMN)
(otherwise known as central or supranuclear/nuclear) lesions of the facial nerve
from lower motor neuron (LMN) (also referred to as peripheral or infranuclear)
lesions are:
1) CN VII Temporal branch testing - intact forehead movement indicates UMN
lesion
2) CN V1, V2, V3 testing - alteration in facial sensation suggests CN V
involvement, and therefore a CPA lesion
3) Corneal Reflex - corneal hypesthesia suggests afferent corneal lesion, CN V
History
I. Assess Timing
1. Onset - when, sudden vs. progressive
61
2. Duration
3. Is it recurrent
II. Assess Degree of Palsy
1. UMN vs. LMN - other focal neurological signs

2. CN V findings
3. Bells phenomenon (When the patient closes his/her eyes, the eyeballs
rotate upward)
4. Loss of taste
5. Loss of lacrimation
III. Determine presence of associated symptoms
Otalgia/Aural fullness
Vesicles around the auditory meatus (See picture)
CN VIII symptoms
- Hearing loss
- Tinnitus
- Vertigo
CN V symptoms: Facial hemianesthesia/hypesthesia or slow/ absent

afferent corneal reflex
Physical Exam
1. Complete CN exam
2. Gross peripheral motor and sensory function - look for asymmetry
3. Otoscopy
4. Webers and Rinns tests
Investigations
The first 2 should be done in all patients, the rest as indicated:
1. Audiometry (pure tone and speech)
62
2. Acoustic stapedial reflex testing
3. Viral ELISA isolation studies to differentiate herpes zoster from herpes simplex
4. Electroneurongraphy (ENoG)
5. Topognostic testing (Schirmers test, Hitselberger's test etc...probably more
academic than useful)
6. CT head (if skull fracture suspected)
7. MRI with gadolinium enhancement to delineate labyrinthine or geniculate
ganglion portions of nerve
8. High-resolution CT (HRCT) to delineate fallopian canal
Click Differential Diagnosis to see the table of common causes of AFP
Other DDx which are less common...

Central Facial Paralysis
-primarily due to cerebrovascular accident of the lacunar type within the genu of
the internal capsule
-may occur as part of a human immunodeficiency virus (HIV)-related neurologic
disorder
Conditions Associated with Facial Paralysis
Otitis Media
-facial palsy is rare (represents about 3% of all cases of peripheral nerve palsy),
but may be found in either acute or chronic otitis media
-usually due to a dehiscence of the bony (fallopian) canal of facial nerve
-Otitis media with facial palsy as a complication requires urgent treatment, and
most otolaryngologists would, at the least, perform a myringotomy to drain the
middle ear effusion.
Metabolic Disruption
63
Pregnancy - 3.3 x increased risk; most common in third trimester
-preeclampsia may pose even greater (6 x) risk
Diabetes mellitus - 4.5 x increased risk of Bell's palsy; facial palsies often
associated with ophthalmoplegia without pupil involvement
Chronic alcoholism
Collagen vascular disorders
-recurrent triad of symptoms beginning in second decade (only 25% have all
three symptoms):
1) nonpitting orofacial edema (defining symptom)
2) facial palsy (50% of patients)
3) lingua plicata (fissured tongue) (50% of patients)
Human Immunodeficiency Virus (HIV)
-may be more prone to facial paralysis secondary to Bell's palsy, HZO, or in late
disease, systemic lymphoma
Kawasaki's disease (infantile acute febrile mucocutaneous lymph node
syndrome)
-commonly have mucous membrane, skin, lymph node involvement, coronary
artery aneurysms
-about 30% have neurologic involvement including aseptic meningitis, irritability
and facial nerve palsy
Hemifacial spasm or Blepharospasm
-idiopathic, progressive, involuntary spasm of one side of the face or orbicularis
oculus and upper face
64
-blepharospasm can lead to functional blindness; may respond to botulinum toxin
therapy
References
Mattox DE. Clinical disorders of the facial nerve. In: Cummings CW, ed.
Ololaryngology-Head and Neck Surgery, 2nd ed. Toronto: Mosby, 1992, pp. 321732.
Otolaryngology Clinics of North America Vol. 24 No. 3, June 1991
Selesnick SH, Patwardhan A. Acute facial paralysis: evaluation and early
management. Am J Otolaryngol 1994;15(6):387-408.
FACIAL NERVE (VII) DISORDERS
Anatomy
Bell's palsy
Differential diagnosis
Facial nerve: Anatomy
2 roots
Motor from facial nucleus
Nervus intermedius
Sensory afferents: Skin & taste (to nucleus tractus solitarius)
Preganglionic parasympathetics (from superior salivatory nucleus)
Major branches
o Large petrosal: Lacrimation & salivation
o Nerve to stapedius
o Chorda tympani: Taste
o Motor branches
Facial nerve: Anatomical Diagram
External link
o Brainstem anatomy
o Nerve anatomy: USUHS; Yale
o
o
65
Bell's Palsy
Epidemiology
o Lifetime prevalence: 6.4 per 1,000
o Incidence: Increased with age
Overall: 0.5 per year per 1,000
Age 20: 0.1 per year per 1,000
o Male = Female
o Recurrence: 7%
o Side: Right in 63%
o ? Increased incidence with diabetes
o
Clinical Features
o Onset
Facial Paresis: Right
Paralysis: Progresses over 3 to 72
Widened palpebral fissure
hours
Pain (50%): Near mastoid process
Excess tearing (33%)
Other: Hyperacusis; Dysgeusia
o Signs
Facial weakness
o Upper & Lower
o Unilateral
o Degree: Partial (30%); Complete (70%)
Stapedius dysfunction (33%): Hyperacusis
Lacrimation: mildly affected in some patients
Taste: No clinically significant changes in most patients
o Prognosis better
Incomplete paralysis
Early improvement
Slow progression
Younger age
Normal salivary flow
Normal taste
Electrodiagnostic tests normal
o Nerve excitability
o Electrogustometry
o Course
Improvement onset: 10 days to 2 months
Plateau: 6 weeks to 9 months
Residual signs
o Synkinesis: ~50%
66
Face weakness: 30%
Contracture: 20%
Crocodile tears: 6%
Treatment of Bell's palsy
o Corticosteroids within one week of onset
o Prednisone 80 mg qd x 5 days; then taper over 1 week
Laboratory
o CSF: Protein high in 30%; Cells in 10%
o Calorics: Often reduced on affected side
o
o
o
VII disorders: Differential Diagnosis

Unilateral VII weakness
o Idiopathic: Bell's palsy
o Sarcoid & other granulomatous disorders
o Infection
Leprosy: Especially with paralysis of upper face
Otitis media
Lyme disease
Herpes Zoster (Ramsay Hunt syndrome)
o Neoplasm & Masses
o Trauma
o Cardiofacial syndrome: Lower lip or complete facial palsy
o Familial
o CNS lesions
Bilateral VII weakness

o Melkersson syndrome
o Mbius syndrome
o Guillain Barr
o Motor neuron disorders
o Myasthenia gravis
o Myopathies
o Leprosy
o An--lipoproteinemia (Tangier)
Central VII lesions

o Pyramidal: Lower face paralysis with voluntary motion
67
o
Emotional2: Face paralysis with emotion

Anatomy: Dorsolateral pons lesion
Disorders
o Superior cerebellar artery infarction
Unilateral
With Deafness, Horner's, or reduced Sweating
o Extrapyramidal disorders
Bilateral
Parkinsonism
Hemifacial spasm
Chromosome 9p11; Dominant

Clinical
o Onset
5 to 70 years
Incomplete penetrance
o Recurrent facial palsy
Unilateral or Bilateral
May include loss of taste
o Edema: Facial, Lip & Eyelid; Non-pitting
External link: Image
o Onset: < 16 years
o Lingua plicata (Scrotal tongue)
Laboratory
o Eyelid pathology: Granulomatous lymphangitis
Mbius syndrome
Facial paresis: Congenital

Often with ophthalmoplegia
Sporadic
Associated with attempted abortion using misoprostol: Odds ratio = 38
MBS1: Chromosome 13q12.2-q13; Dominant , or
Clinical
o Congenital facial diplegia; Asymmetric
o Ophthalmoplegia, esp VI nerve
o Mental retardation
o Peripheral neuropathy
68
Skeletal: Arthrogryposis; Orofacial malformations; Rib defects
Muscle aplasia
Respiratory failure: Central; Associated with tegmental brainstem
calcification
o Hypogonadotropic hypogonadism
Pathology: Aplasia or hypoplasia of cranial nerves & nuclei
o
o
o
MBS2: Chromosome 3q; Dominant

Facial weakness: Asymmetric; Unequal involvement of the 3 branches of the
facial nerve
MBS3: Chromosome 10q; Dominant1

Variable penetrance
Facial weakness: Unilateral or Bilateral
Hearing loss: Congenital deafness, or Progressive hearing loss with age
Carey-Fineman-Ziter syndrome
Facial Nerve Trauma & Tumors
Trauma
o Petrous bone fracture
o Surgery: Middle ear; Mastoidectomy; Parotid gland
Tumors & Masses
o Neuroma/Neurinoma
o Meningioma
o Cholesteatoma
o Parotid gland
o Metastasis
o Carcinomatous meningitis
o Paget: Osteopetrosis
69
Hemifacial Spasm
Onset
o
Adults
Typically 5th or 6th decade

Younger patients: Rule out brainstem disease, MS
o Location: Orbicularis oculi muscle
o More common in women
Contraction pattern
o Synchronous contraction of facial nerve innervated muscles
o Paroxysmal
o Involuntary
o Duration: Up to 1 minute
o May persist during sleep
o Usually unilateral
Weakness: Unusual
Triggers: Aggravating factors
o Emotion
o Fatigue
Causes
o Recovery from Bell's palsy
Associated with synkinesis & contracture
o Dolichoectatic brainstem artery
Frequency: 30%
Mechanism: Pressure on VII root entry zone
Vessels: PICA; AICA; Vertebral
o Brainstem disease
o Idiopathic
Course: Usually permanent without treatment
Electrophysiology
o Brief bursts of action potentials
High frequency (150-400 Hz)
Normal motor units
o Variable rhythm & amplitude
o Lateral spread response3
Stimulation: Facial nerve branch
Response: In muscles not normally innervated by branch
Related to cross transmission of facial nerve fibers
o Location: Probably at site of compression
Treatment
o Botulinum toxin
o Microvascular decompression of VII nerve
Morbidity & Mortality 5%
Barker
T2 image: Brainstem
Anticonvulsants
MRA: AP
70
Return to Cranial nerve disorders

Go to Facial Paralysis (Baylor)
References
1. Am. J. Hum. Genet., 1999;65:752-756
2. Neurology 2000;54:1217
3. Muscle Nerve 2002;25:845849
2/26/2003
How the doctors knew Julia

did not have a stroke...
When Julia awoke with a crooked smile she thought she had a stroke because she could
not move the right side of her face no matter how hard she tried. Her eye would not close
completely and she could not raise the corner of her mouth. Her grandfather had recently
suffered a stroke and Julia worried that she also had a stroke because, in the mirror, her
face looked like her grandfather's. However, the doctors could see a subtle difference in
the weakness caused by a stroke and weakness caused by "Bell's Palsy"- also known as
facial nerve paralysis. A stroke is a condition that arises when cerebral neurons are
injured by the sudden blockage of blood supply to that part of the brain. These neurons
initiate movements of the face by sending signals to neurons in the brainstem. From the
brainstem nerve fibers spread out to cover the face (see Figure 1).
Figure 1. The facial nerve and muscles of the face*
Why the face looks different following a stroke compared to following a

primary facial nerve injury (Bell's Palsy)
In general, cerebral neurons from the left side of the brain send their signals to brainstem
neurons whose nerves innervate muscles on the right side of the face and right sided
71
cerebral neurons go to brainstem neurons that innervate muscles on the left side.
However, the facial nerve is somewhat unusual in that the fibers that spread to the upper
face (muscles around the eye and the forehead) come from cerebral neurons on BOTH
the right and left side of the brain. This results in a difference between how facial
paralysis looks if there is injury to brain (from a stroke) or injury to the facial nerve itself
(Bell's Palsy). Look at the faces in Figure 2. Why is the woman's face that of someone
who had a stroke and the man's face that of someone with Bell's Palsy?
Figure 2.*
Answer: If you can raise both eyebrows, the facial nerve is intact. Therefore, the woman
has had a stroke involving the left side of the brain producing paralysis of the right lower
face and the man has a right Bell's Palsy.
* Topical Diagnosis in Neurology,

by Peter Duus,
Thieme Medical Publishers, Inc.
TITLE: FACIAL NERVE PARALYSIS 1996

SOURCE: Dept. of Otolaryngology, UTMB, Grand Rounds
DATE: March 13, 1996
RESIDENT PHYSICIAN: Kelly D. Sweeney, M.D.
FACULTY: Jeffrey T. Vrabec, M.D.DISCUSSANT: Kedar K. Adour, M.D., Sir
Charles Bell Society, San Francisco, CASERIES EDITOR: Francis B. Quinn, Jr.,
M.D.
http://www.bellspalsy.ws/facialparalysis96.htm
"This material was prepared by resident physicians in partial fulfillment of educational
requirements established for the Postgraduate Training Program of the UTMB
72
Department of Otolaryngology/Head and Neck Surgery and was not intended for clinical
use in its present form. It was prepared for the purpose of stimulating group discussion in
a conference setting. No warranties, either express or implied, are made with respect to
its accuracy, completeness, or timeliness. The material does not necessarily reflect the
current or past opinions of members of the UTMB faculty and should not be used for
purposes of diagnosis or treatment without consulting appropriate literature sources and
informed professional opinion."
Anatomy
Acute facial nerve paralysis is a common clinical entity with which all practicing
otolaryngologist should be familiar. In order to diagnose and treat the many causes of
facial nerve paralysis, it is important that the clinician have a good understanding of the
anatomy and function of the facial nerve. The facial nerve contains approximately 10,000
fibers. Of these, 7000 myelinated fibers innervate the muscles of facial expression, the
stapedius muscle, the postauricular muscles, the posterior belly of the digastric muscle,
and the platysma. The remaining 3000 fibers form the nervus intermedius (Nerve of
Wrisberg) which contains sensory fibers (taste) from the anterior 2/3 of the tongue, and
parasympathetic secretomotor fibers to the parotid, submandibular, sublingual, and
lacrimal gland. The facial nerve also contains a few general somatic afferent fibers which
join the auricular branch of the vagus to supply sensation to the external auditory meatus,
and visceral afferents which innervate the mucous membranes of the nose, palate, and
pharynx via the greater palatine nerve.
The motor nucleus of the facial nerve lies deep within the reticular formation of the pons
where it receives input from the precentral gyrus of the motor cortex, which innervates
the ipsilateral and contralateral forehead. The cerebral cortical tracts also innervate the
contralateral portion of the remaining face. This accounts for the sparing of the forehead
motion in supranuclear lesions of the facial nerve.
The parasympathetic secretory fibers of the nervous intermedius arise from the superior
salivatory nucleus. These preganglionic fibers travel to the submandibular ganglion via
the chorda tympani nerve to innervate the submandibular and sublingual glands, and to
the sphenopalatine ganglion via the greater superficial petrosal nerve to innervate the
lacrimal, nasal, and palatine glands. The secretory fibers of the lesser superficial petrosal
nerve traverse the tympanic plexus, synapse in the otic ganglion, and travel via the
auriculotemporal nerve to innervate the parotid gland. Taste fibers from the anterior 2/3
of the tongue reach the geniculate ganglion via the chorda tympani nerve and from there
travel to the nucleus of the tractus solitarius.
The facial nerve and nervus intermedius exit the brain stem at the pontomedullary
junction and travel laterally 12 - 14 mm with the eight cranial nerve to enter the internal
acoustic meatus. The meatal segment of the nerve then travels 8 - 10 mm within the
internal auditory canal (anterosuperior quadrant) to the meatal foramen where the canal
narrows from 1.2 mm in diameter to 0.68 mm in diameter (the narrowest part of the
73
canal). The labyrinthine segment then runs 2 - 4 mm to the geniculate ganglion. Here the
greater superficial petrosal nerve exits to carry parasympathetic secretomotor fibers to the
lacrimal gland. Just distal to this branch, the lesser superficial petrosal nerve exits to
supply parasympathetic secretomotor fibers to the parotid. The tympanic segment begins
just distal to the geniculate ganglion where the nerve turns 40 to 80 degrees (first genu)
and runs posteroinferiorly 11 mm across the tympanic cavity to the second genu. A
branch leaves the segment near the pyramidal eminence to supply the stapedius muscle
The nerve then turns about 90 degrees at the second genu inferiorly where the mastoid
segment travels for 12 - 14 mm inferiorly in the anterior mastoid to exit the stylomastoid
foramen. The terminal branch of the nervus intermedius, the chorda tympani, leaves the
mastoid segment 5 mm proximal to the foramen and travels lateral to the incus, medial to
the malleus to exit at the petrotympanic fissure. The extratympanic segment is composed
entirely of motor fibers and enters the parotid gland after giving off the posterior
auricular branch and a branch to the posterior belly of the digastric muscle. The pes
anserus forms 20 mm from the stylomastoid foramen and further divides the nerve into
the upper (temporal, and zygomatic) and lower (buccal, mandibular, and cervical)
branches.
Physiology of Nerve Injury
When an axon is injured, biochemical and histological changes occur in the cell body
proximal and distal to the site of the injury. The severity of the changes depend upon the
distance from the injury to the cell body (proximal injuries usually more severe than
distal injuries), the type of injury (crush injuries more severe than clean transections), the
age of the patient (older individuals sustain more severe injury than younger patients),
and the nutritional and metabolic status of the patient.
Sunderland's classification of nerve injury describes five degrees of injury. The first
degree (neuropraxia) involves a localized conduction block in the nerve with the nerve
fibers responding to electrical stimuli proximal and distal to the lesion, but not across the
injured segment. Axonal continuity is preserved, wallerian degeneration does not occur,
and recovery is usually complete.
The second degree of nerve injury is called axonotmesis. This refers to disruption of the
axon into proximal and distal portions with interrupted axoplasmic flow. Wallerian
degeneration occurs within 24 hours in the distal portion of the axon and to a slight
degree in the proximal portion. The connective tissue elements remain intact, however,
and the axon may regenerate at a rate of 1 mm/day to the original end organ with the
potential for complete recovery.
The third degree of nerve injury refers to endoneurotmesis. In this type of nerve injury,
the endoneurium and axon are destroyed, but the perineurium remains intact. Wallerian
degeneration occurs. Axons may regenerate, but can be blocked by scar tissue. This will
result in partial reinnervation. In addition, misdirection of fibers can occur with resultant
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synkinesis (abnormal mass movement of muscles which do not normally contract
together) and incomplete recovery.
Fourth degree nerve injury is called perineurotmesis. In this type, only the epineurium
remains intact, while the axon, endoneurium, and perineurium are disrupted. With this
type of injury, wallerian degeneration occurs, and there is much greater chance for
aberrant regeneration, synkinesis, and incomplete recovery.
Fifth degree injury or neurotmesis refers to complete disruption of neural continuity.
Without careful repair, there is little to no chance of regeneration and recovery. In
addition, axonal sprouts may escape the confines of the nerve sheath and produce painful
neuromas adjacent to the injured nerve. Except in cases of complete transection, nerve
injury is usually a combination of degrees of injury.
Clinical Evaluation
The first step in evaluating any patient who presents with facial nerve paralysis involves
taking a careful and thorough history. It is important to determine the onset of the
paralysis (sudden vs delayed), the duration, and the rate of progression. It is especially
important to determine whether the paralysis is complete verses incomplete. Patients
should be questioned regarding previous episodes, family history, associated symptoms
(hearing loss, otorrhea, otalgia, vertigo, headaches, blurred vision, parasthesias),
associated medical illnesses (diabetes, pregnancy, autoimmune disorders, cancer), history
of trauma (recent or remote), and previous surgery (otologic, rhytidectomy,
parotidectomy).
A complete head and neck examination must be performed, including microscopic
examination of the ears, careful palpation of the parotid glands and neck, ophthalmologic
examination (r/o papilledema), auscultation of the neck ( r/o carotid bruits), and a
thorough neurological examination. It is important to assess the degree of voluntary
movement present in order to document the grade of facial paralysis as described in the
House classification system: Grade Degree Description
I Normal Normal facial movements; No synkinesis
II Slight Mild deformity, mild synkinesis, good forehead function, slight asymmetry
III Moderate Obvious facial weakness, forehead motion present, good eye closure,
asymmetry, Bell's phenomenon present
IV Moderately Obvious weakness, increasing synkinesis; no forehead motion
V Severe Very obvious facial paralysis, some tone present, cannot close eye
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VI Total Complete facial paralysis, absent tone
It is also important to determine if the paralysis is central versus peripheral. Supranuclear
(central) lesions produce contralateral voluntary lower facial paralysis. The frontalis
muscle is spared because of the bilateral innervation as described previously. Emotional
response (facial motion on laughing or crying) may also be preserved with central
lesions. Presence of Bell's phenomenon (upward outward turning of the eyeball as the
patient attempts to close the eyelids) indicates a peripheral lesion.
Any patient presenting with facial paralysis should undergo formal audiological testing,
including pure tone, air and bone conduction, speech discrimination, reflexes, and
tympanometry. If asymmetry is found on the audiogram, an ABR and/or MRI should be
obtained. Electronystagmography (ENG) is usually not indicated unless vertigo or other
balance disturbance is part of the clinical picture.
Radiologic evaluation may be undertaken in patients with a history of recurrent paralysis,
associated neurological symptoms, suspected CPA lesions, concurrent otologic findings
(AOM, COM, suspected cholesteatoma), history of trauma, gradually developing facial
nerve paralysis, atypical presentation, or if patients show no evidence of recovery after
one month from onset. Gadolinium enhanced MRI is superior for soft tissue evaluation
and will usually reveal the inflammation and edema associated with Bell's palsy and with
Herpes Zoster oticus. It is also considered to be the procedure of choice to rule out a
cerebellopontine angle tumor or other brain tumors. High- resolution computed
tomography provides excellent bony assessment and is the study of choice to rule out a
temporal bone fracture, or to evaluate the middle ear and mastoid.
Topognostic Testing
The principle behind topognostic testing is that lesions distal to the site of a particular
branch of the facial nerve will spare the function of that branch. Moving distally from the
brainstem, these tests include: the schirmer test for lacrimation (GSPN), the stapedial
reflex test (stapedial branch), taste testing (chorda tympani nerve), salivary flow rates and
pH (chorda tympani).
The Schirmer test evaluates the function of the greater superficial petrosal nerve by
determining the rate of lacrimation. Filter paper is placed in the lower conjunctival fornix
bilaterally. After 3 - 5 minutes, the length of the strip that is moist is compared to the
normal side. A value of 25% or less on the involved side or total lacrimation less than 25
mm is considered abnormal. An abnormal result can indicate injury to the GSPN or to the
facial nerve proximal to the geniculate ganglion and may predict patients at risk for
exposure keratitis.
Stapedial reflex testing is routinely done during the audiological evaluation. This test
evaluates the stapedius branch of the facial nerve which leaves the main trunk just past
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the second genu in the mastoid. Of all the topographic tests, this one is the most objective
and reproducible. A loud tone is presented to either the ipsilateral or contralateral ear
which should evoke a reflex movement of the stapedius muscle. This changes the tension
on the TM (which must be intact for a valid test) resulting in a change in the impedance
of the ossicular chain. If the tone is presented to the opposite ear (normal hearing) and the
reflex is elicited, the seventh nerve is considered to be intact up to that point. In the case
of Bell's palsy with an intact stapedial reflex, complete recovery can be expected to begin
within six weeks. Absence of the reflex when either ear is stimulated with normal VIII
nerve function suggests an abnormality of the facial afferent.
In Bell's palsy, however, absence of the stapedial reflex during the first two weeks is
common and is usually of no prognostic significance.
Measurement of taste by the anterior 2/3 of the tongue can be done by placing a small
amount of salt, sugar, or lemon juice on the tongue. Loss of taste may indicate
interruption of the ipsilateral chorda tympani nerve. This test is extremely subjective. A
more reliable indicator of interruption of the chorda tympani nerve involves microscopic
detection of the absence of taste papillae on the involved side of the tongue. Papillae
generally disappear within 10 days post injury. Examination of the middle 1/3 of the
tongue is most indicative, because the anterior 1/3 may receive bilateral input.
Salivary flow rates can also be assessed to evaluate functional integrity of the chorda
tympani nerve. This test involves cannulation of Wharton's ducts bilaterally with
measurement of output after five minutes. A 25% reduction in flow of the involved side
as compared to the normal side is considered significant. This test has largely been
abandoned secondary to technical difficulty of cannulating the ducts and patient
discomfort. Salivary ph may be examined as an indirect measure of flow. As the rate of
flow increases, the ph increases. Therefore, a ph of less than 6.1 may predict loss of
function of the chorda tympani.
Although these tests are of historical interest, they have not been found to be of much use
clinically for determining the site of the lesion in facial paralysis or for predicting the
outcome. Marked discrepancies are often seen. For example, patients may exhibit a
marked decrease in lacrimation with a normal stapedial reflex and intact taste, or they
may have absent lacrimation and an absent stapedial reflex with normal salivation. These
discrepancies are easily explained in Bell's palsy, where there can be multiple sites of
inflammation and demyelinization from the brainstem to the peripheral branches of the
nerve. In addition, in temporal bone fractures, the GSPN and chorda tympani nerves are
very vulnerable to injury and may be disrupted with an intact facial nerve. Also, with
tumors, transmission of nerve impulses can occur through the tumor mass itself until late
in the disease with different areas of the nerve being affected at different times. These
tests may be helpful, however, for predicting the likelihood of development of exposure
keratitis.
Electrophysiologic Tests
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These tests are useful for patients with complete paralysis for determining prognosis for
return of facial function and the endpoint of degeneration by serial testing. They are most
useful when considering decompression surgery and are of no value in patients with
incomplete paralysis.
The nerve excitability test (NET), maximal stimulation test (MST), and
electroneuronography (ENoG) are most useful in the degenerative phase.
These tests will give normal results during the first 72 hours after injury due to the
stimulating and recording electrodes both being distal to the site of the injury. After 3 - 4
days, the nerve degeneration reaches the site of stimulation and useful results will be
obtained. These tests can only be used for unilateral paralysis because all three involve
comparison to the contralateral side which must be normal for valid results.
The nerve excitability test (NET) is the most commonly used secondary to the low cost,
readily available equipment, and ease of performance. This test involves placement of a
stimulating electrode over the stylomastoid foramen. The lowest current necessary to
produce a twitch on the paralyzed side of the face (threshold) is compared with the
contralateral side. A difference of greater than 3.5 milliamps indicates a poor prognosis
for return of facial function. The major draw back to the use of this test is its subjectivity,
with reliance entirely on a visual end point. In addition, since such a small amount of
current is used with this test, a few intact axons may give a visible response leading the
clinician to predict a good prognosis, when in reality most of the fibers are degenerating.
The maximum stimulation test (MST) is a modified version of the NET. A maximal
stimulus is used to depolarize all facial nerve branches. The paralyzed side is then
compared to the contralateral side and the difference is graded as equal, slightly
decreased, markedly decreased, or absent. Testing begins on the third day post onset and
is repeated periodically until return of facial function or absent response. An equal or
slightly decreased response on the involved side is considered favorable for complete
recovery. An absent or markedly decreased response denotes advanced degeneration with
a poor prognosis. The response to this test becomes abnormal sooner than the response to
the NET and is therefore considered superior. However, like the NET, this test is also
subjective.
Electroneuronography (ENoG) is considered to be the most accurate prognostic test
because it provides an objective, qualitative measurement of neural degeneration. The
facial nerve is stimulated with an impulse transcutaneously at the stylomastoid foramen
using bipolar electrodes. The muscular response is then recorded using bipolar electrodes
placed near the nasolabial groove. The peak to peak amplitude of the evoked compound
action potential is considered proportional to the number of intact axons. The two sides
are then compared with the response on the paralyzed side of the face expressed as a
percentage of the response on the normal side of the face. A reduction in amplitude on the
involved side to 10% or less of the normal side indicates a poor prognosis for
spontaneous recovery. A maximal reduction of less than 90% within 3 weeks of onset
gives an expected spontaneous rate of recovery of 80 - 100%. Disadvantages of ENoG
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include discomfort, cost, and test-retest variability which is due to positioning of the
electrodes and excitation of the muscles of mastication (V).
Electromyography (EMG) is of limited value early in the evaluation of facial paralysis
because fibrillation potentials indicating axonal degeneration do not appear until 10 to 14
days post onset. However, EMG becomes important for assessing reinnervation potential
of the muscle two weeks after onset.
By using needle electrodes placed transcutaneously into the muscles of facial expression,
muscle action potentials generated by voluntary activity can be recorded. Electrical
silence can indicate normal muscle in a resting state, severe muscle wasting and fibrosis
or acute facial paralysis in the early stages. During normal voluntary contraction
organized diphasic or triphasic potentials are seen. Fibrillation potentials indicate
degeneration of the neural supply to the muscle in question. Polyphasic potentials
indicate reinnervation. These are important because they usually appear 6 - 12 weeks
before clinical return of function.
Bell's Palsy
Bell's palsy is the most common cause of facial paralysis and accounts for more than half
of all cases. Traditionally, this was considered to be a diagnosis of exclusion after ruling
out all other possible causes. However, it has recently been considered a positive
diagnosis if the following are present: unilateral weakness of all facial muscles of sudden
onset, possibly associated with a viral prodrome, no evidence of central nervous system
pathology, no evidence of a CPA lesion, no history of otologic disease. Patients may
exhibit evidence of concomitant sensory cranial polyneuritis with otalgia or postauricular
pain, dysacousis or hyperacusis, dysgeusia, decreased tearing or epiphora, and facial
hypesthesias/dysesthesias of V or IX . Although the exact etiology of Bell's palsy is still
unclear, most clinicians believe that herpes simplex infection is the most likely agent.
This belief is supported by an increased incidence of HSV antibodies in patients with
Bell's palsy when compared to age-matched controls.
In 1995, Sugita et al were successful in producing an acute and transient facial paralysis
in mice by inoculating herpes simplex virus into their auricles (104) or tongues (30).
Facial paralysis developed in the mice between six and nine days after inoculation, lasted
for three to seven days, and then resolved spontaneously. Histopathological studies of the
facial nerve and nuclei from these mice revealed severe nerve swelling, vacuolar
degeneration, and infiltration of inflammatory cells. HSV antigens were detected in the
facial nerve, geniculate ganglion, and the facial nerve nucleus. They concluded that HSV
could produce an acute and transient facial paralysis through a natural infectious route
from the auricle or tongue to the geniculate ganglion.
Murakami et al (1996) also investigated the role of herpes simplex virus in the
pathogenesis of facial paralysis in mice by inoculating mouse auricles with HSV. On the
third day following inoculation, HSV DNA was noted in the ipsilateral facial nerve. On
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the tenth day, HSV DNA was noted in both facial nerves and brain stem in the mice with
facial paralysis, but absent in these tissues in the mice without facial paralysis. Between
days 4 and 20, the neutralization antibody titer was elevated in all of the mice. In
addition, facial paralysis developed only on the inoculated side. They concluded that
HSV infection in the facial nerve and brain stem must be a prerequisite for the
development of facial paralysis and suggested that an immunologic reaction after a viral
infection plays a role in the pathogenesis.
The incidence of Bell's palsy is estimated to be 20 to 30 per 100,000, but appears to
increase with age. There is an equal male to female ration and a 3.3 times greater
incidence in pregnant females. The left and right sides of the face are equally involved,
and less than 1% of cases are bilateral. The recurrence rate is about 10% and can be
ipsilateral or bilateral. Patients with diabetes have 4 - 5 times more risk of developing the
disease. A family history is positive in about 10% of patients with Bell's palsy.
The most likely site of lesion in Bell's palsy is the meatal foramen (junction of the
internal auditory canal portion of the nerve and the labyrinthine segment of the nerve),
which is considered to be the narrowest portion of the fallopian canal. MRI with
gadolinium will usually show enhancement of the labyrinthine portion of the nerve. As
the edema within the nerve increases, axonal flow and circulation are inhibited resulting
in varying degrees of nerve injury (first, second, and third degree). Patients who are most
severely affected develop a high level of third degree injury which can result in the loss
of endoneural tubules and misdirected axonal regeneration. Histological studies from
patients with Bell's palsy who died of nonrelated causes reveal diffuse demyelination of
the facial nerve with lymphocytic infiltrates.
The prognosis for Bell's palsy is generally good with 85 to 90% of patients recovering
completely within one month. The remaining 15% progress to complete degeneration and
will not usually show signs of recovery for three to six months. The longer the time
needed for recovery, the greater the probability of sequelae. The single most important
prognostic factor is the degree of paralysis. Patients with incomplete paralysis will
recover with no sequelae 95% of the time.
The treatment of Bell's palsy is variable, ranging from observation to surgical
decompression. Regardless of treatment given, all patients must be counselled regarding
proper eye care to prevent exposure keratitis. Patients should use natural tears liberally
during the day and should place lacrilube ointment in the eye at night. Taping of the eye
lids during sleep may be helpful as well as the use of a moisture chamber. Patients should
avoid fans and dust, and should consider wearing eye protection when outside in the
wind.
Oral prednisone in a divided dosage of 1 mg/kg/day may be helpful in preventing or
lessening degeneration, decreasing synkinesis, and relieving pain, and may result in
earlier recovery. Patients should be reevaluated within five days after starting steroids. If
some function is present (paresis), taper the steroids over the next five days. If no
improvement is noted, the full dose should be given for an additional ten days, then
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tapered over five days. Oral acyclovir may help improve recovery in Bell's palsy. The
usual dosage is 500 mg po four times a day for ten days. For patients in whom steroids or
acyclovir is contraindicated, observation and eye care may be all that is possible.
Surgical decompression for Bell's palsy is somewhat controversial. Most surgeons agree,
however, that in patients progressing to total paralysis within two weeks, with an ENoG
demonstrating 90% or greater degeneration, decompression of the facial nerve may
prevent further degeneration and may improve outcome.
The rationale behind surgical decompression is based on the assumption that the site of
maximal facial nerve injury in Bell's palsy is within the meatal foramen. With increasing
edema and decreasing axoplasmic flow and microcirculation a pathological compression
injury of the nerve occurs at this point of maximal constriction. This can range from first
degree to third degree. Removal of the compression, if performed before irreversible
injury to the endoneural tubules occurs (two weeks), will allow for axonal regeneration to
occur. This is usually accomplished via a middle fossa approach. Surgical decompression
should not be done in an only hearing ear.
In a retrospective study, Fisch (1981) compared fourteen patients with >90%
degeneration within 1 to 14 days after the onset of facial paralysis who underwent
decompression using the middle fossa approach to thirteen similar patients who refused
surgical decompression. A subtle but statistically significant improvement in long-term
facial recovery was noted in the operative group as compared to the patients who refused
surgery. Fisch concluded that in order to obtain a satisfactory return of facial function in
all cases of Bell's palsy, surgical decompression of the facial nerve should be performed
within 24 hours when results of ENoG indicate >90% degeneration has occurred.
Trauma
Trauma is the second most common cause of facial nerve paralysis. Longitudinal
fractures of the temporal bone make up 80% of all temporal bone fractures. In this type,
the fracture line extends along the longitudinal axis of the temporal bone resulting in an
external auditory canal laceration, TM perforation, and possible ossicular disruption or
hemotympanum. Facial nerve injury occurs in 10 to 20% of these fractures with the
injury most common in the perigeniculate region. Transverse fractures are much less
common (20% of all temporal bone fractures). These fractures extend along the
transverse axis of the temporal bone across the vestibule, resulting in sensorineural
hearing loss, possible loss of vestibular function, and a 50% chance of facial nerve injury
which is also usually in the perigeniculate region.
For complete facial nerve paralysis with clinical evidence of a temporal bone fracture,
obtain a high resolution CT scan/temporal bone protocol. If an obvious fracture is
present, surgical exploration of the facial nerve should be undertaken as soon as possible
via either a transmastoid/translabyrinthine (+ SNHL) or transmastoid/middle fossa
approach (- SNHL). During exploration the nerve must be fully exposed in order to
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identify all injured segments, and remove any compression from fracture fragments. The
nerve sheath should be incised and any hematomas within the sheath must be carefully
evacuated. If complete transection of the nerve is found during exploration, a direct endto-end anastomosis should be performed if possible. It is important to handle all neural
tissue as atraumatically as possible, using microvascular instruments and techniques. The
nerve endings should be prepared by sharply cutting at a ninety degree angle and
reapproximating under no tension with two to three 9.0 nylon sutures through the
epineurium.
When a direct end-to-end anastomosis creates tension, or when segments of the nerve are
missing or severely damaged, interpositional grafts from the greater auricular, medial
antebrachial cutaneous, or sural nerve should be used.
For incomplete facial nerve paralysis or for delayed onset paralysis associated with a
temporal bone fracture, facial nerve testing should be obtained on day 4 after onset. If
advanced degeneration has occurred, the nerve should be surgically explored and
decompressed.
Gun shot wounds of the temporal bone cause facial paralysis in over 50% of cases. The
nerve may be transected, or may be secondarily injured by the kinetic injury from the
bullet or from bony fragmentation of the temporal bone. The most common sites of injury
are the tympanic and mastoid segments of the nerve and the stylomastoid foramen area.
For a complete paralysis after a gun shot wound, surgical exploration and repair should
be undertaken as soon as the patient is medically stable. Generally, outcome of facial
function is much worse with gun shot wounds to the temporal bone than with temporal
bone fractures.
The facial nerve can also be injured during middle ear and mastoid surgery. If iatrogenic
transection occurs during surgery, the nerve must be repaired. If paralysis occurs
postoperatively and the surgeon is confident that the nerve was intact at the conclusion of
the case, a high resolution CT scan should be obtained and facial nerve testing should be
done on POD #4 - 6. If advanced degeneration is evident, surgical exploration and
decompression should be done. If there is any question as to the integrity of the nerve,
exploration should be done as soon as possible by a surgeon familiar with the
intratemporal anatomy of the facial nerve and reconstructive techniques.
Penetrating facial injuries with immediate facial nerve paralysis should be explored and
repaired as soon as possible while electrical stimulation can still be used to help locate
the distal branches. When the injury is medial to the lateral canthus of the eye, aggressive
exploration is not usually mandatory because the nerve endings are small and a rich
anastomotic network exists in this area.
Herpes Zoster Oticus
Herpes zoster oticus (Ramsay-Hunt Syndrome) is the third most common cause of facial
nerve paralysis. This is a manifestation of a dormant varicella zoster virus reactivating in
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extramedullary cranial nerve ganglia during a period of decreased cell mediated
immunity. The prodromal symptoms are very similar to those seen in Bell's palsy, but are
usually more severe. Symptoms include severe otalgia, facial paralysis, facial numbness,
and a vesicular eruption on the concha, external auditory canal, and palate. Patients may
also have varying degrees of SNHL, vestibular symptoms, and associated cranial nerve
symptoms. Laboratory evaluation will often show the rise and fall of antibody titers
specific for the virus. The palsy is usually more severe and the prognosis much poorer
compared to Bell's palsy. Only about 50% of these patients regain normal facial function
as opposed to 90% with Bell's palsy.
The degeneration occurs more slowly, usually over three weeks instead of two. Treatment
includes steroids, valacyclovir 1 gm po tid, and proper eye care. Surgical decompression
has not been proven beneficial but may be considered for ENoG showing greater than
90% degeneration.
Otitis Media In patients with evidence of acute otitis media, dehiscences in the fallopian
canal may serve as portals for direct bacterial invasion and inflammation along the nerve.
Facial paralysis may begin within a few days of onset of an acute otitis media and is
usually incomplete. Treatment includes a wide myringotomy, drainage, and culture with
antibiotic coverage for gram positive cocci and H. flu. The facial palsy associated with
acute otitis media generally resolves with aggressive management of the infection.
However, if a total paralysis is present, serial ENoG should be obtained. If axonal
degeneration reaches > 90%, surgical exploration and decompression should be
performed.
Patients with chronic otitis media may also develop facial paralysis which is usually
secondary to cholesteatoma or from inflammation/osteitis compressing the facial nerve.
In these cases a high resolution CT should be obtained, and surgery should be performed
as soon as possible (tympanomastoidectomy, facial nerve exploration and
decompression).
Tumors
About 5% of cases of facial nerve paralysis are caused by tumors. Factors which should
increase the clinicians suspicion of a possible tumor include: a slowly developing paresis
over more than three weeks, facial twitching, additional cranial nerve deficits, recurrent
ipsilateral involvement, associated adenopathy, or a palpable neck or parotid mass. In
these cases, MRI and CT should be obtained. The most common benign tumor causing
facial nerve paralysis is a facial nerve schwanomma. The most common malignant
tumors causing facial paralysis are mucoepidermoid carcinoma and adenoid cystic
carcinoma of the parotid gland. The management of facial nerve paralysis caused by
tumors depends upon the lesions location, size, and malignant potential and may include
transposition of the nerve, division and reanastomosis, interposition grafting, and cranial
nerve crossover. After nerve grafts, the best possible result that can be expected is a
House grade III paresis.
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Melkerson-Rosenthal Syndrome
Melkerson-Rosenthal syndrome is a rare disease that consists of a triad of symptoms:
recurrent orofacial edema, recurrent facial palsy, and lingua plicata (fissured tongue). The
defining feature of this disease is the persistent or recurrent nonpitting facial edema that
cannot be explained by infection, malignancy, or connective tissue disorder. It usually
involves the lips and buccal area and may involve the gingiva, palate, and tongue. The
lips become chapped, fissured, and red-brown in appearance and may develop permanent
deformity after numerous recurrences. Facial paralysis and lingua plicata occur in half of
the patients.
The complete triad is only present in 25% of these patients. This condition usually starts
in the second decade of life, and the manifestations usually occur sequentially (rarely
simultaneously). The etiology of this syndrome is unknown. Some consider it a variant of
sarcoidosis secondary to biopsies of the lips which may reveal noncaseating granulomas.
Facial paralysis occurs in 50 to 90% of these patients and tends to be abrupt. A history of
bilateral sequential paralysis and relapse after initial recovery is common. The site of the
paralysis usually corresponds to the facial swelling. Facial nerve decompression may be
indicated if episodes of facial paralysis are frequent and progressive.
Congenital Facial Paralysis
Newborn facial paralysis is estimated to have an incidence of about 0.23% of live births.
The most common cause is birth trauma (80%), which is usually evident by other signs of
injury. These include a history of a difficult delivery with or without forceps, facial
swelling, bruising over the mastoid or extratemporal course of the nerve, and
hemotympanum. The mechanism of injury is thought to be from direct pressure during
forceps use or from pressure on the infants face by the sacral prominence during delivery.
Congenital causes of newborn facial paralysis are much less common. Mobius' syndrome
consists of a broad spectrum of clinical findings which can range from an isolated
unilateral facial paralysis to bilateral absence of facial and abducens nerve function. In
this syndrome, the facial nerve forms but consists of only a fibrotic tract. The muscles of
facial expression may form in some cases, but degeneration to fibrosis generally occurs
rapidly. These children will have no response on EMG at birth and have no chance of
spontaneous recovery of facial function. Many other cranial nerves may be involved (III,
IV, IX, X, XII) and skeletal abnormalities may be present. Treatment for these causes of
newborn facial paralysis is generally delayed until late childhood and usually requires
static slings and muscle transfers.
A rare cause of isolated newborn facial paralysis is dysgenesis of the intratemporal facial
nerve. CT and surgical findings show that the most common site of the lesion is the distal
part of the mastoid segment in the fallopian canal. The nerve is usually very thin and
fibrotic at this area. EMG findings in these cases usually show a few functional motor
units but useful facial function is not usually present.
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Newborns who present with a complete facial nerve paralysis should undergo electrical
testing within the first three days of life to differentiate between congenital and traumatic
causes. After birth trauma, the nerve can be stimulated for up to five days post injury and
fibrillation potentials will be seen on EMG at ten to fourteen days. In congenital cases,
the nerve will usually not stimulate and no fibrillation potentials will be seen on EMG.
The prognosis for trauma related facial nerve paralysis at birth is usually excellent.
Surgical decompression should not be considered until the nerve has had a chance to
recover or until >90% degeneration has occurred.
Other Causes
Sarcoidosis consists of a chronic noncaseating granulomatous disease usually
characterized by hilar or peripheral adenopathy, polyarthralgias, anergy, elevated serum
calcium, and hepatic dysfunction. One variant of sarcoidosis, Heerfordt's disease (a.k.a.
uveoparotid fever) consists of uveitis, mild fever, nonsuppurative parotitis, and cranial
nerve paralysis. The facial nerve is the most commonly involved cranial nerve and the
paralysis usually starts abruptly days to months after the parotitis. In these cases, the
paralysis is considered to be from direct invasion of the nerve by the granulomatous
process. An elevated serum angiotensin-converting enzyme (ACE) generally confirms the
diagnosis, and treatment consists of steroids.
Lyme disease is an infection caused by the tick-borne spirochete Borrelia burgdorferi.
Several species of Ixodes ticks carry the spirochete, and the primary reservoir is the
white-tailed deer and white-footed mouse. The disease generally occurs in several stages.
The first stage consists of a flu-like illness with regional lymphadenopathy, general
malaise, and erythema migrans (erythematous enlarging annular skin lesions found
anywhere on the body). The second stage usually starts several weeks to months later
with neurologic abnormalities. These include meningitis and cranial and peripheral nerve
neuropathies. The final stage occurs months to years later in the form of recurrent
meningitis, subtle mental disorders or neurologic deficits, and chronic arthritis. Ten
percent of these patients develop facial paralysis (unilateral or bilateral) and hearing loss.
The facial paralysis typically resolves completely, but may rarely result in mild
permanent facial weakness. Treatment for Lyme disease consists of IV ceftriaxone (2
g/day) for fourteen days.
Conclusion
Although Bell's palsy remains the most common cause of acute facial nerve paralysis, it
is important for clinicians to consider all causes to avoid overlooking potentially lifethreatening diseases. A good history and physical is mandatory in the work-up of these
patients and will usually help to significantly narrow the possible causes. Although
topognostic testing is of historical interest, it has not proven to be of much use clinically.
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ENoG is the most useful electrophysiologic test and should be performed within 4 - 6
days post-onset in patients with a complete paralysis with surgical decompression
considered for > 90% degeneration. Treatment plans should be individualized in each
patient, but must include education on eye protection.
Editor's Comment:
With one exception, the article by Murakami stands as the most recent and perhaps most
significant contribution to the understanding of "idiopathic" facial nerve paralysis. The
exception is the section in Conn's Current Therapy - 1996 on Acute Facial Paralysis, by
Kedar Adour.
Dr. Adour discusses the etiology and natural history of the disorder with a clarity rarely
matched in our literature. He proposes a diagnostic protocol which discusses
manifestations which do NOT permit the diagnosis of Bell's palsy and Herpes zoster
paralysis ("exclusion criteria.") He proposes a compellingly rational plan of treatment, in
which he suggests that "electrotherpy is of no benefit and may be harmful."
The faculty have invited Dr. Adour to present a Discussion of this Grand Rounds topic,
and we are honored that he has agreed to do so.
Discussion by Kedar K. Adour, M.D. President, Sir Charles Bell Society:
Recent published articles have changed the thinking regarding diagnosis and treatment of
acute facial paralysis:
Bell Palsy and Herpes Simplex Virus: Identification of Viral DNA in Endoneurial Fluid
and Muscle
Shingo Murakami, MD; Mutsuhiko Mizobucbi, MD; Yuki Nakashiro, MD; Takashi Doi,
MD; Naohito Hato, MD; and Naoaki Yanagihara, MD
Objective: To determine whether herpes simplex virus type 1 (HSV-1) causes Bell palsy.
Design: Prospective study.
Setting: University inpatient service.
Patients: 14 patients with Bell palsy, 9 patients with the Ramsay-Hunt syndrome, and 12
other controls.
Measurements: Viral genomes of HSV-1, varicella-zoster virus, and Epstein-Barr virus
were analyzed in clinical samples of facial nerve endoneurial fluid and posterior auricular
86
muscle using polymerase chain reaction (PCR) followed by hybridization with Southern
blot analysis.
Results: Herpes simplex virus type 1 genomes were detected in 11 of 14 patients (79%)
with Bell palsy but not in patients with the Ramsay-Hunt syndrome or in other controls.
The nucleotide sequences of the PCR fragments were identical to those of the HSV-1
genome.
Conclusions: Herpes simplex virus type 1 is the major etiologic agent in Bell palsy.
------------------------------ The editorial comment: "One might now question whether we
should continue using the term "Bell's palsy" to mean "idiopathic facial paralysis" or
whether we should now recognize Bell's palsy as "herpetic facial paralysis," confirming
the hypothesis advanced initially in 1972 by McCormick.
It is also documented that Adour in 1970 suggested to the American Academy of
Otolaryngology that herpes simplex was the cause of Bell's palsy."
Just as the pyramids of Egypt have guarded the riddle of the sphinx, the petrous pyramid
of the temporal bone has guarded the secrets of the facial nerve. Even today there is
question about the anatomic compartments of the facial nerve. The authors of the above
text suggest that the facial nerve carries some parasympathetic secretomotor fibers to the
parotid gland and a few general somatic afferent fibers to the external ear. These two
suggestions are questionable and are omitted in many neuro-anatomical textbooks.
Cutting the facial nerve at the brainstem does not lead to any deficit of parotid secretion
nor somatosensory loss in the ear canal. Sunderland's classification of nerve injury is not
applicable to herpetic facial paralysis (herpes simplex or herpes zoster). Further, Sir
Sydney Sunderland has not written a single word about the facial nerve in his classic
textbook. The five degrees of injury are of increasing severity according to the effect of
the injury not the cause. The statement "The first degree (neuropraxia) involves a
localized conduction block in the nerve with the nerve fibers responding to electrical
stimuli proximal and distal to the lesion, but not across the injured segment." is incorrect.
Segmental demyelination also causes a neuropraxic state. The terms neuropraxia,
axonotmesis, and neurotmesis should not be equated with the 5 degrees of nerve injury.
Under clinical evaluation: The House Grading System cannot be used to document the
grade of facial paralysis at the onset of a paralysis. The House system is an "end-stage"
interpretation. Grade II includes "mild synkinesis", Grade III and IV "increasing
synkinesis". Synkinesis occurs when the nerve has been damaged and then abnormally
regenerated. It is not present in the acute stages. The Brackmann modification somewhat
addresses this problem and is a modification of the original Adour-Swanson Recovery
Profile. "Bell's phenomenon (upward outward tuning of the eyeball as the patient
attempts to close the eyelids) indicates a peripheral lesion." is not correct. The eyeball
motion described is a normal function of eye muscle activity which only became apparent
to Sir Charles Bell because the eyelids did not close. A central lesion with incomplete
closure of the eyelids will also display a "Bell's phenomenon."
87
We agree that topognostic testing is of historical interest only and is invalid in herpetic
facial paralysis, or in temporal bone fractures. The presenting symptom of dysgeusia is
diagnostic for herpetic facial paralysis. No further diagnostic tests need be done. One
hundred per cent of patients with herpetic facial paralysis demonstrate papillitis of the
fungiform tongue papillae and has led to the truism that " Bell's palsy is a tongue blade
diagnosis." Taste tests using electrogustometry are research tools only. With regards to
the stapedial reflex, it should be considered as the "otologists' electromyography". (See
below) When should you question the diagnosis of herpetic facial paralysis formerly
called Bell's palsy and Ramsay Hunt syndrome??
Partial facial paralysis that does not resolve in 3 to 6 WEEKS.

Partial facial paralysis with electrical evidence of nerve degeneration.
MST in one or more branches with moderate (score of 2) to severe (scoreof 1) decrease in
muscle motion.
Global MST less than 3.
Electromyography with fibrillation (denervation) potentials.
Partial facial paralysis with ipsilateral hearing loss.
Any facial paralysis with evidence of chronic otitis media, or history of previous ear
surgery.
Progressive facial paralysis over period of weeks or months is not Bell's palsy. Note:
Damage to the nerve is complete by day 14 in Bell's palsy but not until day 21 in Ramsay
Hunt syndrome.
Total facial paralysis with no regeneration in 4 months. Note: Even with total loss of
nerve excitability by ENOG or Global MST 99.9% of all Bell's palsy or Ramsay Hunt
Syndrome will regenerate with midface contracture with synkinesis. The earliest sign of
regeneration is the return of the stapedial reflex. The next earliest sign is evidence of
mouth motion with voluntary forced closure of the eyes (synkinesis).
Total facial paralysis with electrical evidence of nerve degeneration that resolves
WITHOUT midface contracture with synkinesis. Note: Facial nerve degeneration with
regeneration in Bell's Palsy or Ramsay Hunt syndrome is followed by the sequelae of
midface contracture with synkinesis.
88
Recurrent facial paralysis on the same side with electrical evidence of nerve degeneration
and recovery WITHOUT contracture with synkinesis in 4 months.
Stapedial Reflex: The Otologists' Electromyography. Total facial paralysis with intact
stapedial reflex is a Partial paralysis! An absent stapedial reflex one week post onset with
return of the stapedial reflex by 3 weeks post onset indicates a good prognosis. The
Global MST score will allow you to predict rate and degree of recovery.
When reviewing Bell's palsy treatment results be suspicious of any article when the
number of patients showing electrical evidence of nerve degeneration does not equal the
number of patients with sequelae of contracture with synkinesis.
Electrical stimulation continues to be widely used in the treatment of facial paralysis (20,
23) although there is mounting evidence that it may be contraindicated.
It has been suggested that electrical stimulation may interfere with neural regeneration
post peripheral nerve injury (24,25) and studies proving its efficacy with facial muscles
are lacking in the literature. A 1984 report by the National Center for Health Services
Research concluded that "Electrotherapy treatment for Bell's palsy. . . has no
demonstrable beneficial effect in enhancing the functional or cosmetic outcomes in
patients with Bell's palsy." (26)
Additionally, patients who undergo electrical stimulation acutely may demonstrate more
synkinesis and mass action than those who do not (27). It is difficult to produce an
isolated contraction of the facial muscles using electrical stimulation due to their small
size and close proximity to each other. The contraction produced causes mass action
which reinforces abnormal motor patterns and can be painful. (20)
20. Cole J, Zimmerman S, Spector G: Nonsurgical neuromuscular rehabilitation of facial
muscle paresis, in Rubin LR (ed): The Paralyzed Face. St Louis, Mosby-Year Book Inc.,
1991, pp 107-112.
23. Farragher DJ: Electrical stimulation: A method of treatment for facial paralysis, in
Rose FC, Jones R, Vrbova G (eds): Neuromuscular Stimulation: Basic Concepts and
Clinical Implication. New York, Demos, 1989 vol 3, pp 303-306.
24. Cohan CS, Kater SB: Suppression of neurite elongation and growth cone motility by
electrical activity. Science 1986; 22:1638-1640. 25. Brown MC, Holland RL: A central
role for denervated tissues in causing nerve sprouting. Nature 1979; 282:724.
26. Waxman B: Electrotherapy for treatment of Facial Nerve Paralysis (Bell's Palsy).
Health Technology Assessment Reports, National Center for Health Services Research
1984; 3:27.
Added Reference:
89
Jansen JKS, Lomo T, Nicolaysen K, et al: Hyperinnervation of skeletal muscle fibers:
Dependence on muscle activity. Science 181:559-561, 1973.
Lack of muscle spindles....
Brodal A: Neurological Anatomy: In Relation to Clinical Medicine, ed 3. New York,
Oxford University Press, 1981, pp 495-508.
Dubner R, Seddie BJ, Storey AT: The Neural Basis of Oral and Facial Function. New
York, Plenum Press, 1978, pp 222-229.
March 27, 1996
Kedar K. Adour, MD
President, Sir Charles Bell Society
1000 Green Street #1203
San Francisco, CA 94133
(415) 474-4046 Fax: (415) 474-3541 E-mail: kadour@aol.com
BIBLIOGRAPHY
Bailey, Byron J., ed. Head and Neck Surgery - Otolaryngology. Philadelphia, PA.: J.B.
Lippincott Co., 1993.
Ballenger, John J., ed. Diseases of the Nose, Throat, Ear, Head, and Neck. Philadelphia,
PA: Lea & Febiger, 1991.
Boies, Lawrence R., ed. Fundamentals of Otolaryngology. Philadelphia, PA: J.B.
Cummings, Charles, ed. Otolaryngology - Head and Neck Surgery. St. Louis, MO.:
Mosby - Year Book, Inc., 1993.
Fisch U. Maximal Nerve Excitability Testing vs Electroneuronography. Arch Otolaryngol
106: 352 - 357, 1980.
Fisch U. Surgery for Bell's Palsy. Arch Otolaryngol 107: 1 - 11, 1981.
Gates, George A. Current Therapy in Otolaryngology - Head and Neck Surgery. St.
Louis, MO.: Mosby - Year Book, Inc., 1994.
90
Green JD, et al. Surgical Management of Iatrogenic Facial Nerve Injuries. Otolaryngol
Head Neck Surg 111(5): 606 - 610, 1994.
Mattox, Douglas E., ed. The Otolaryngology Clinics of North America: Management of
Facial Nerve Disorders. Philadelphia, PA: W. B. Saunders, Co., 1991.
May, Mark. The Facial Nerve. New York: Thieme - Stratton, 1986.
May M., et al. Natural History of Bell's Palsy: The Salivary Flow Test and Other
Prognostic Indicators. Laryngoscope 86: 704 - 712, 1976.
Murakami S, et al. Role Of Herpes Simplex Virus Infection in the Pathogenesis of Facial
Paralysis in Mice. Ann Otol Rhinol Laryngol 105: 49 - 53, 1996.
Peitersen E. The Natural History of Bell's Palsy. Am J. Otol 4: 107, 1982.
Rubin, Leonard R., The Paralyzed Face. St. Louis, MO.: Mosby - Year Book, Inc., 1991.
Selesnick SH, Patwardhan A. Acute Facial Paralysis: Evaluation and Early Management.
Am J Otolaryngol 15 (6): 387 - 408, 1994.
Sugita T, et al. Facial Nerve Paralysis Induced by Herpes Simplex Virus in Mice: An
Animal Model of Acute and Transient Facial Paralysis. Ann Otol Rhinol Laryngol
104:574 - 581, 1995.
Acute Facial Nerve Paralysis

http://icarus.med.utoronto.ca/carr/manual/afnp2.html
PD Warrick BScPhm, Meds '98 McMaster University
Reviewed by Barry Maber MD FRCSC, Clinical Associate Professor
(Otolaryngology), College of Medicine, University of Saskatchewan
Anatomy
The complex anatomy of the seventh cranial nerve and its subsections must be
understood in order to discriminate among the peripheral causes of facial nerve
palsy, and to differentiate these from central etiologies.
Subsections of the Facial Nerve
91
Supranuclear - from the precentral (motor) and postcentral (sensory) gyri

through the genu of the internal capsule to the facial nucleus in the pons
(ipsilateral and contralateral nucleus for upper face, contralateral only for
lower face)
Nuclear - facial nerve travels dorsomedially toward 4 th ventricle, loops
laterally around abducent nucleus, then exits anterolaterally and inferiorly
at the medullopontine junction
Cerebellopontine angle (CPA) - travels with nervus intermedius (NI),
alongside CN V, VIII to internal auditory canal
Intratemporal
o Internal auditory canal (ICA) - travels anterosuperiorly with NI within
canal for 5-12 mm alongside cochlear, vestibular nerves; lateralmost portion is surrounded by thicker periosteum
92
o
o
o
Labyrinthine - beginning of " fallopian canal" travels almost totally

inferiorly for 3-5 mm, then makes first (posterior) turn; gives off
geniculate branch (NI) to form geniculate ganglion from which
emerges the greater petrosal nerve (which controls lacrimation via
the sympathetic plexus of internal carotid artery and
pterygopalatine ganglion) and the lesser petrosal nerve (which

controls salivation of parotid gland via the otic ganglion)
Temporal - travels mostly posteriorly for 10-12 mm before reaching
the second (inferior) turn just superior to middle ear
Mastoid - gives off branch to stapedius, chorda tympani (which
unites with lingual nerve of CN V3 to cause salivation in
submandibular and sublingual glands); travels 13 mm inferiorly and
becomes more superficial before emerging from the stylomastoid
foramen
93
Extracranial - travels anteroinferiorly immediately entering parotid space,

gives off branches to occipitalis, intrinsic muscles of ear (posterior
auricular), posterior belly of digastric and stylohyoid, then separates into
temporofacial and cervicofacial divisions - highly variable anatomy from
this point, but essentially 5 main divisions:
o Temporal - function differentiates upper motor from lower motor
neuron lesion
o Zygomatic - most important, as function prevents exposure keratitis
of cornea
o Buccal
o Marginal Mandibular
o Cervical
Helpful Signs
Some of the most helpful findings to differentiate upper motor neuron (UMN)
(otherwise known as central or supranuclear/nuclear) lesions of the facial nerve
from lower motor neuron (LMN) (also referred to as peripheral or infranuclear)
lesions are:
1) CN VII Temporal branch testing - intact forehead movement indicates UMN
lesion
2) CN V1, V2, V3 testing - alteration in facial sensation suggests CN V
3) Corneal Reflex - corneal hypesthesia suggests afferent corneal lesion, CN V
History
I. Assess Timing
1. Onset - when, sudden vs. progressive
2. Duration
3. Is it recurrent
II. Assess Degree of Palsy
1. UMN vs. LMN - other focal neurological signs

2. CN V findings
3. Bells phenomenon (When the patient closes his/her eyes, the eyeballs
rotate upward)
94
4. Loss of taste
5. Loss of lacrimation
III. Determine presence of associated symptoms
Otalgia/Aural fullness
Vesicles around the auditory meatus (See picture)
CN VIII symptoms
- Hearing loss
- Tinnitus
- Vertigo
CN V symptoms: Facial hemianesthesia/hypesthesia or slow/ absent

afferent corneal reflex
Physical Exam
1. Complete CN exam
2. Gross peripheral motor and sensory function - look for asymmetry
3. Otoscopy
4. Webers and Rinns tests
Investigations
The first 2 should be done in all patients, the rest as indicated:
1. Audiometry (pure tone and speech)
2. Acoustic stapedial reflex testing
3. Viral ELISA isolation studies to differentiate herpes zoster from herpes simplex
4. Electroneurongraphy (ENoG)
5. Topognostic testing (Schirmers test, Hitselberger's test etc...probably more
academic than useful)
6. CT head (if skull fracture suspected)
95
7. MRI with gadolinium enhancement to delineate labyrinthine or geniculate
ganglion portions of nerve
8. High-resolution CT (HRCT) to delineate fallopian canal
Click Differential Diagnosis to see the table of common causes of AFP
Other DDx which are less common...

Central Facial Paralysis
-primarily due to cerebrovascular accident of the lacunar type within the genu of
the internal capsule
-may occur as part of a human immunodeficiency virus (HIV)-related neurologic
disorder
Conditions Associated with Facial Paralysis
Otitis Media
-facial palsy is rare (represents about 3% of all cases of peripheral nerve palsy),
but may be found in either acute or chronic otitis media
-usually due to a dehiscence of the bony (fallopian) canal of facial nerve
-Otitis media with facial palsy as a complication requires urgent treatment, and
most otolaryngologists would, at the least, perform a myringotomy to drain the
middle ear effusion.
Metabolic Disruption
Pregnancy - 3.3 x increased risk; most common in third trimester
-preeclampsia may pose even greater (6 x) risk
Diabetes mellitus - 4.5 x increased risk of Bell's palsy; facial palsies often
associated with ophthalmoplegia without pupil involvement
Chronic alcoholism
Collagen vascular disorders
96
-recurrent triad of symptoms beginning in second decade (only 25% have all
three symptoms):
1) nonpitting orofacial edema (defining symptom)
2) facial palsy (50% of patients)
3) lingua plicata (fissured tongue) (50% of patients)
Human Immunodeficiency Virus (HIV)
-may be more prone to facial paralysis secondary to Bell's palsy, HZO, or in late
disease, systemic lymphoma
Kawasaki's disease (infantile acute febrile mucocutaneous lymph node
syndrome)
-commonly have mucous membrane, skin, lymph node involvement, coronary
artery aneurysms
-about 30% have neurologic involvement including aseptic meningitis, irritability
and facial nerve palsy
Hemifacial spasm or Blepharospasm
-idiopathic, progressive, involuntary spasm of one side of the face or orbicularis
oculus and upper face
-blepharospasm can lead to functional blindness; may respond to botulinum toxin
therapy
References
Mattox DE. Clinical disorders of the facial nerve. In: Cummings CW, ed.
Ololaryngology-Head and Neck Surgery, 2nd ed. Toronto: Mosby, 1992, pp. 321732.
Otolaryngology Clinics of North America Vol. 24 No. 3, June 1991
97
Selesnick SH, Patwardhan A. Acute facial paralysis: evaluation and early
management. Am J Otolaryngol 1994;15(6):387-408.
FACIAL NERVE (VII) DISORDERS
Anatomy
Bell's palsy
Differential diagnosis
Facial nerve: Anatomy
2 roots
Motor from facial nucleus
Nervus intermedius
Sensory afferents: Skin & taste (to nucleus tractus solitarius)
Preganglionic parasympathetics (from superior salivatory nucleus)
Major branches
o Large petrosal: Lacrimation & salivation
o Nerve to stapedius
o Chorda tympani: Taste
o Motor branches
Facial nerve: Anatomical Diagram
External link
o Brainstem anatomy
o Nerve anatomy: USUHS; Yale
o
o
Bell's Palsy
Epidemiology
o Lifetime prevalence: 6.4 per 1,000
o Incidence: Increased with age
Overall: 0.5 per year per 1,000
98
o
o
o
o
o
Male = Female
Recurrence: 7%
Side: Right in 63%
? Increased incidence with diabetes
Clinical Features
o Onset
Facial Paresis: Right
Paralysis: Progresses over 3 to 72
Widened palpebral fissure
hours
Pain (50%): Near mastoid process
Excess tearing (33%)
Other: Hyperacusis; Dysgeusia
o Signs
Facial weakness
o Upper & Lower
o Unilateral
o Degree: Partial (30%); Complete (70%)
Stapedius dysfunction (33%): Hyperacusis
Lacrimation: mildly affected in some patients
Taste: No clinically significant changes in most patients
o Prognosis better
Incomplete paralysis
Early improvement
Slow progression
Younger age
Normal salivary flow
Normal taste
Electrodiagnostic tests normal
o Nerve excitability
o Electrogustometry
o Course
Improvement onset: 10 days to 2 months
Plateau: 6 weeks to 9 months
Residual signs
o Synkinesis: ~50%
o Face weakness: 30%
o Contracture: 20%
o Crocodile tears: 6%
Treatment of Bell's palsy
o Corticosteroids within one week of onset
o Prednisone 80 mg qd x 5 days; then taper over 1 week
Laboratory
o CSF: Protein high in 30%; Cells in 10%
o Calorics: Often reduced on affected side
99
VII disorders: Differential Diagnosis

Unilateral VII weakness
o Idiopathic: Bell's palsy
o Sarcoid & other granulomatous disorders
o Infection
Leprosy: Especially with paralysis of upper face
Otitis media
Lyme disease
Herpes Zoster (Ramsay Hunt syndrome)
o Neoplasm & Masses
o Trauma
o Cardiofacial syndrome: Lower lip or complete facial palsy
o Familial
o CNS lesions
Bilateral VII weakness

o Melkersson syndrome
o Mbius syndrome
o Guillain Barr
o Motor neuron disorders
o Myasthenia gravis
o Myopathies
o Leprosy
o An--lipoproteinemia (Tangier)
Central VII lesions

o Pyramidal: Lower face paralysis with voluntary motion
o Emotional2: Face paralysis with emotion
Anatomy: Dorsolateral pons lesion
Disorders
o Superior cerebellar artery infarction
Unilateral
With Deafness, Horner's, or reduced Sweating
o Extrapyramidal disorders
Bilateral
Parkinsonism
100
Hemifacial spasm
Chromosome 9p11; Dominant

Clinical
o Onset
5 to 70 years
Incomplete penetrance
o Recurrent facial palsy
Unilateral or Bilateral
May include loss of taste
o Edema: Facial, Lip & Eyelid; Non-pitting
External link: Image
o Onset: < 16 years
o Lingua plicata (Scrotal tongue)
Laboratory
o Eyelid pathology: Granulomatous lymphangitis
Mbius syndrome
Facial paresis: Congenital

Often with ophthalmoplegia
Sporadic
Associated with attempted abortion using misoprostol: Odds ratio = 38
MBS1: Chromosome 13q12.2-q13; Dominant , or

Clinical
o Congenital facial diplegia; Asymmetric
o Ophthalmoplegia, esp VI nerve
o Mental retardation
o Peripheral neuropathy
o Skeletal: Arthrogryposis; Orofacial malformations; Rib defects
o Muscle aplasia
o Respiratory failure: Central; Associated with tegmental brainstem
calcification
o Hypogonadotropic hypogonadism
Pathology: Aplasia or hypoplasia of cranial nerves & nuclei
MBS2: Chromosome 3q; Dominant

Facial weakness: Asymmetric; Unequal involvement of the 3 branches of the
facial nerve
101
MBS3: Chromosome 10q; Dominant1

Variable penetrance
Facial weakness: Unilateral or Bilateral
Hearing loss: Congenital deafness, or Progressive hearing loss with age
Carey-Fineman-Ziter syndrome
Facial Nerve Trauma & Tumors
Trauma
o Petrous bone fracture
o Surgery: Middle ear; Mastoidectomy; Parotid gland
Tumors & Masses
o Neuroma/Neurinoma
o Meningioma
o Cholesteatoma
o Parotid gland
o Metastasis
o Carcinomatous meningitis
Paget: Osteopetrosis
Hemifacial Spasm
Onset
o
Adults
Typically 5th or 6th decade

Younger patients: Rule out brainstem disease, MS
o Location: Orbicularis oculi muscle
o More common in women
Contraction pattern
o Synchronous contraction of facial nerve innervated muscles
o Paroxysmal
o Involuntary
o Duration: Up to 1 minute
o May persist during sleep
o Usually unilateral
Weakness: Unusual
Triggers: Aggravating factors
o Emotion
o Fatigue
Causes
o Recovery from Bell's palsy
102
Associated with synkinesis & contracture
o Dolichoectatic brainstem artery
Frequency: 30%
Mechanism: Pressure on VII root entry zone
Vessels: PICA; AICA; Vertebral
o Brainstem disease
o Idiopathic
Course: Usually permanent without treatment
Electrophysiology
o Brief bursts of action potentials
High frequency (150-400 Hz)
Normal motor units
o Variable rhythm & amplitude
o Lateral spread response3
Stimulation: Facial nerve branch
Response: In muscles not normally innervated by branch
Related to cross transmission of facial nerve fibers
o Location: Probably at site of compression
Treatment
o Botulinum toxin
o Microvascular decompression of VII nerve
Morbidity & Mortality 5%
Anticonvulsants
References
1. Am. J. Hum. Genet., 1999;65:752-756
2. Neurology 2000;54:1217
3. Muscle Nerve 2002;25:845849
2/26/2003
How the doctors knew Julia

did not have a stroke...
When Julia awoke with a crooked smile she thought she had a stroke because she could
not move the right side of her face no matter how hard she tried. Her eye would not close
completely and she could not raise the corner of her mouth. Her grandfather had recently
suffered a stroke and Julia worried that she also had a stroke because, in the mirror, her
face looked like her grandfather's. However, the doctors could see a subtle difference in
the weakness caused by a stroke and weakness caused by "Bell's Palsy"- also known as
facial nerve paralysis. A stroke is a condition that arises when cerebral neurons are
injured by the sudden blockage of blood supply to that part of the brain. These neurons
103
initiate movements of the face by sending signals to neurons in the brainstem. From the
brainstem nerve fibers spread out to cover the face (see Figure 1).
Figure 1. The facial nerve and muscles of the face*
Why the face looks different following a stroke compared to following a

primary facial nerve injury (Bell's Palsy)
In general, cerebral neurons from the left side of the brain send their signals to brainstem
neurons whose nerves innervate muscles on the right side of the face and right sided
cerebral neurons go to brainstem neurons that innervate muscles on the left side.
However, the facial nerve is somewhat unusual in that the fibers that spread to the upper
face (muscles around the eye and the forehead) come from cerebral neurons on BOTH
the right and left side of the brain. This results in a difference between how facial
paralysis looks if there is injury to brain (from a stroke) or injury to the facial nerve itself
(Bell's Palsy). Look at the faces in Figure 2. Why is the woman's face that of someone
who had a stroke and the man's face that of someone with Bell's Palsy?
Figure 2.*
Answer: If you can raise both eyebrows, the facial nerve is intact. Therefore, the woman
has had a stroke involving the left side of the brain producing paralysis of the right lower
face and the man has a right Bell's Palsy.
104
* Topical Diagnosis in Neurology,

by Peter Duus,
Thieme Medical Publishers, Inc.
TITLE: FACIAL NERVE PARALYSIS 1996

SOURCE: Dept. of Otolaryngology, UTMB, Grand Rounds
DATE: March 13, 1996
RESIDENT PHYSICIAN: Kelly D. Sweeney, M.D.
FACULTY: Jeffrey T. Vrabec, M.D.DISCUSSANT: Kedar K. Adour, M.D., Sir
Charles Bell Society, San Francisco, CASERIES EDITOR: Francis B. Quinn, Jr.,
M.D.
http://www.bellspalsy.ws/facialparalysis96.htm
"This material was prepared by resident physicians in partial fulfillment of educational
requirements established for the Postgraduate Training Program of the UTMB
Department of Otolaryngology/Head and Neck Surgery and was not intended for clinical
use in its present form. It was prepared for the purpose of stimulating group discussion in
a conference setting. No warranties, either express or implied, are made with respect to
its accuracy, completeness, or timeliness. The material does not necessarily reflect the
current or past opinions of members of the UTMB faculty and should not be used for
purposes of diagnosis or treatment without consulting appropriate literature sources and
informed professional opinion."
Anatomy
Acute facial nerve paralysis is a common clinical entity with which all practicing
otolaryngologist should be familiar. In order to diagnose and treat the many causes of
facial nerve paralysis, it is important that the clinician have a good understanding of the
anatomy and function of the facial nerve. The facial nerve contains approximately 10,000
fibers. Of these, 7000 myelinated fibers innervate the muscles of facial expression, the
stapedius muscle, the postauricular muscles, the posterior belly of the digastric muscle,
and the platysma. The remaining 3000 fibers form the nervus intermedius (Nerve of
Wrisberg) which contains sensory fibers (taste) from the anterior 2/3 of the tongue, and
parasympathetic secretomotor fibers to the parotid, submandibular, sublingual, and
lacrimal gland. The facial nerve also contains a few general somatic afferent fibers which
join the auricular branch of the vagus to supply sensation to the external auditory meatus,
and visceral afferents which innervate the mucous membranes of the nose, palate, and
pharynx via the greater palatine nerve.
105
The motor nucleus of the facial nerve lies deep within the reticular formation of the pons
where it receives input from the precentral gyrus of the motor cortex, which innervates
the ipsilateral and contralateral forehead. The cerebral cortical tracts also innervate the
contralateral portion of the remaining face. This accounts for the sparing of the forehead
motion in supranuclear lesions of the facial nerve.
The parasympathetic secretory fibers of the nervous intermedius arise from the superior
salivatory nucleus. These preganglionic fibers travel to the submandibular ganglion via
the chorda tympani nerve to innervate the submandibular and sublingual glands, and to
the sphenopalatine ganglion via the greater superficial petrosal nerve to innervate the
lacrimal, nasal, and palatine glands. The secretory fibers of the lesser superficial petrosal
nerve traverse the tympanic plexus, synapse in the otic ganglion, and travel via the
auriculotemporal nerve to innervate the parotid gland. Taste fibers from the anterior 2/3
of the tongue reach the geniculate ganglion via the chorda tympani nerve and from there
travel to the nucleus of the tractus solitarius.
The facial nerve and nervus intermedius exit the brain stem at the pontomedullary
junction and travel laterally 12 - 14 mm with the eight cranial nerve to enter the internal
acoustic meatus. The meatal segment of the nerve then travels 8 - 10 mm within the
internal auditory canal (anterosuperior quadrant) to the meatal foramen where the canal
narrows from 1.2 mm in diameter to 0.68 mm in diameter (the narrowest part of the
canal). The labyrinthine segment then runs 2 - 4 mm to the geniculate ganglion. Here the
greater superficial petrosal nerve exits to carry parasympathetic secretomotor fibers to the
lacrimal gland. Just distal to this branch, the lesser superficial petrosal nerve exits to
supply parasympathetic secretomotor fibers to the parotid. The tympanic segment begins
just distal to the geniculate ganglion where the nerve turns 40 to 80 degrees (first genu)
and runs posteroinferiorly 11 mm across the tympanic cavity to the second genu. A
branch leaves the segment near the pyramidal eminence to supply the stapedius muscle
The nerve then turns about 90 degrees at the second genu inferiorly where the mastoid
segment travels for 12 - 14 mm inferiorly in the anterior mastoid to exit the stylomastoid
foramen. The terminal branch of the nervus intermedius, the chorda tympani, leaves the
mastoid segment 5 mm proximal to the foramen and travels lateral to the incus, medial to
the malleus to exit at the petrotympanic fissure. The extratympanic segment is composed
entirely of motor fibers and enters the parotid gland after giving off the posterior
auricular branch and a branch to the posterior belly of the digastric muscle. The pes
anserus forms 20 mm from the stylomastoid foramen and further divides the nerve into
the upper (temporal, and zygomatic) and lower (buccal, mandibular, and cervical)
branches.
Physiology of Nerve Injury
When an axon is injured, biochemical and histological changes occur in the cell body
proximal and distal to the site of the injury. The severity of the changes depend upon the
distance from the injury to the cell body (proximal injuries usually more severe than
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distal injuries), the type of injury (crush injuries more severe than clean transections), the
age of the patient (older individuals sustain more severe injury than younger patients),
and the nutritional and metabolic status of the patient.
Sunderland's classification of nerve injury describes five degrees of injury. The first
degree (neuropraxia) involves a localized conduction block in the nerve with the nerve
fibers responding to electrical stimuli proximal and distal to the lesion, but not across the
injured segment. Axonal continuity is preserved, wallerian degeneration does not occur,
and recovery is usually complete.
The second degree of nerve injury is called axonotmesis. This refers to disruption of the
axon into proximal and distal portions with interrupted axoplasmic flow. Wallerian
degeneration occurs within 24 hours in the distal portion of the axon and to a slight
degree in the proximal portion. The connective tissue elements remain intact, however,
and the axon may regenerate at a rate of 1 mm/day to the original end organ with the
potential for complete recovery.
The third degree of nerve injury refers to endoneurotmesis. In this type of nerve injury,
the endoneurium and axon are destroyed, but the perineurium remains intact. Wallerian
degeneration occurs. Axons may regenerate, but can be blocked by scar tissue. This will
result in partial reinnervation. In addition, misdirection of fibers can occur with resultant
synkinesis (abnormal mass movement of muscles which do not normally contract
together) and incomplete recovery.
Fourth degree nerve injury is called perineurotmesis. In this type, only the epineurium
remains intact, while the axon, endoneurium, and perineurium are disrupted. With this
type of injury, wallerian degeneration occurs, and there is much greater chance for
aberrant regeneration, synkinesis, and incomplete recovery.
Fifth degree injury or neurotmesis refers to complete disruption of neural continuity.
Without careful repair, there is little to no chance of regeneration and recovery. In
addition, axonal sprouts may escape the confines of the nerve sheath and produce painful
neuromas adjacent to the injured nerve. Except in cases of complete transection, nerve
injury is usually a combination of degrees of injury.
Clinical Evaluation
The first step in evaluating any patient who presents with facial nerve paralysis involves
taking a careful and thorough history. It is important to determine the onset of the
paralysis (sudden vs delayed), the duration, and the rate of progression. It is especially
important to determine whether the paralysis is complete verses incomplete. Patients
should be questioned regarding previous episodes, family history, associated symptoms
(hearing loss, otorrhea, otalgia, vertigo, headaches, blurred vision, parasthesias),
associated medical illnesses (diabetes, pregnancy, autoimmune disorders, cancer), history
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of trauma (recent or remote), and previous surgery (otologic, rhytidectomy,
parotidectomy).
A complete head and neck examination must be performed, including microscopic
examination of the ears, careful palpation of the parotid glands and neck, ophthalmologic
examination (r/o papilledema), auscultation of the neck ( r/o carotid bruits), and a
thorough neurological examination. It is important to assess the degree of voluntary
movement present in order to document the grade of facial paralysis as described in the
House classification system: Grade Degree Description
I Normal Normal facial movements; No synkinesis
II Slight Mild deformity, mild synkinesis, good forehead function, slight asymmetry
III Moderate Obvious facial weakness, forehead motion present, good eye closure,
asymmetry, Bell's phenomenon present
IV Moderately Obvious weakness, increasing synkinesis; no forehead motion
V Severe Very obvious facial paralysis, some tone present, cannot close eye
VI Total Complete facial paralysis, absent tone
It is also important to determine if the paralysis is central versus peripheral. Supranuclear
(central) lesions produce contralateral voluntary lower facial paralysis. The frontalis
muscle is spared because of the bilateral innervation as described previously. Emotional
response (facial motion on laughing or crying) may also be preserved with central
lesions. Presence of Bell's phenomenon (upward outward turning of the eyeball as the
patient attempts to close the eyelids) indicates a peripheral lesion.
Any patient presenting with facial paralysis should undergo formal audiological testing,
including pure tone, air and bone conduction, speech discrimination, reflexes, and
tympanometry. If asymmetry is found on the audiogram, an ABR and/or MRI should be
obtained. Electronystagmography (ENG) is usually not indicated unless vertigo or other
balance disturbance is part of the clinical picture.
Radiologic evaluation may be undertaken in patients with a history of recurrent paralysis,
associated neurological symptoms, suspected CPA lesions, concurrent otologic findings
(AOM, COM, suspected cholesteatoma), history of trauma, gradually developing facial
nerve paralysis, atypical presentation, or if patients show no evidence of recovery after
one month from onset. Gadolinium enhanced MRI is superior for soft tissue evaluation
and will usually reveal the inflammation and edema associated with Bell's palsy and with
Herpes Zoster oticus. It is also considered to be the procedure of choice to rule out a
cerebellopontine angle tumor or other brain tumors. High- resolution computed
tomography provides excellent bony assessment and is the study of choice to rule out a
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temporal bone fracture, or to evaluate the middle ear and mastoid.
Topognostic Testing
The principle behind topognostic testing is that lesions distal to the site of a particular
branch of the facial nerve will spare the function of that branch. Moving distally from the
brainstem, these tests include: the schirmer test for lacrimation (GSPN), the stapedial
reflex test (stapedial branch), taste testing (chorda tympani nerve), salivary flow rates and
pH (chorda tympani).
The Schirmer test evaluates the function of the greater superficial petrosal nerve by
determining the rate of lacrimation. Filter paper is placed in the lower conjunctival fornix
bilaterally. After 3 - 5 minutes, the length of the strip that is moist is compared to the
normal side. A value of 25% or less on the involved side or total lacrimation less than 25
mm is considered abnormal. An abnormal result can indicate injury to the GSPN or to the
facial nerve proximal to the geniculate ganglion and may predict patients at risk for
exposure keratitis.
Stapedial reflex testing is routinely done during the audiological evaluation. This test
evaluates the stapedius branch of the facial nerve which leaves the main trunk just past
the second genu in the mastoid. Of all the topographic tests, this one is the most objective
and reproducible. A loud tone is presented to either the ipsilateral or contralateral ear
which should evoke a reflex movement of the stapedius muscle. This changes the tension
on the TM (which must be intact for a valid test) resulting in a change in the impedance
of the ossicular chain. If the tone is presented to the opposite ear (normal hearing) and the
reflex is elicited, the seventh nerve is considered to be intact up to that point. In the case
of Bell's palsy with an intact stapedial reflex, complete recovery can be expected to begin
within six weeks. Absence of the reflex when either ear is stimulated with normal VIII
nerve function suggests an abnormality of the facial afferent.
In Bell's palsy, however, absence of the stapedial reflex during the first two weeks is
common and is usually of no prognostic significance.
Measurement of taste by the anterior 2/3 of the tongue can be done by placing a small
amount of salt, sugar, or lemon juice on the tongue. Loss of taste may indicate
interruption of the ipsilateral chorda tympani nerve. This test is extremely subjective. A
more reliable indicator of interruption of the chorda tympani nerve involves microscopic
detection of the absence of taste papillae on the involved side of the tongue. Papillae
generally disappear within 10 days post injury. Examination of the middle 1/3 of the
tongue is most indicative, because the anterior 1/3 may receive bilateral input.
Salivary flow rates can also be assessed to evaluate functional integrity of the chorda
tympani nerve. This test involves cannulation of Wharton's ducts bilaterally with
measurement of output after five minutes. A 25% reduction in flow of the involved side
as compared to the normal side is considered significant. This test has largely been
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abandoned secondary to technical difficulty of cannulating the ducts and patient
discomfort. Salivary ph may be examined as an indirect measure of flow. As the rate of
flow increases, the ph increases. Therefore, a ph of less than 6.1 may predict loss of
function of the chorda tympani.
Although these tests are of historical interest, they have not been found to be of much use
clinically for determining the site of the lesion in facial paralysis or for predicting the
outcome. Marked discrepancies are often seen. For example, patients may exhibit a
marked decrease in lacrimation with a normal stapedial reflex and intact taste, or they
may have absent lacrimation and an absent stapedial reflex with normal salivation. These
discrepancies are easily explained in Bell's palsy, where there can be multiple sites of
inflammation and demyelinization from the brainstem to the peripheral branches of the
nerve. In addition, in temporal bone fractures, the GSPN and chorda tympani nerves are
very vulnerable to injury and may be disrupted with an intact facial nerve. Also, with
tumors, transmission of nerve impulses can occur through the tumor mass itself until late
in the disease with different areas of the nerve being affected at different times. These
tests may be helpful, however, for predicting the likelihood of development of exposure
keratitis.
Electrophysiologic Tests
These tests are useful for patients with complete paralysis for determining prognosis for
return of facial function and the endpoint of degeneration by serial testing. They are most
useful when considering decompression surgery and are of no value in patients with
incomplete paralysis.
The nerve excitability test (NET), maximal stimulation test (MST), and
electroneuronography (ENoG) are most useful in the degenerative phase.
These tests will give normal results during the first 72 hours after injury due to the
stimulating and recording electrodes both being distal to the site of the injury. After 3 - 4
days, the nerve degeneration reaches the site of stimulation and useful results will be
obtained. These tests can only be used for unilateral paralysis because all three involve
comparison to the contralateral side which must be normal for valid results.
The nerve excitability test (NET) is the most commonly used secondary to the low cost,
readily available equipment, and ease of performance. This test involves placement of a
stimulating electrode over the stylomastoid foramen. The lowest current necessary to
produce a twitch on the paralyzed side of the face (threshold) is compared with the
contralateral side. A difference of greater than 3.5 milliamps indicates a poor prognosis
for return of facial function. The major draw back to the use of this test is its subjectivity,
with reliance entirely on a visual end point. In addition, since such a small amount of
current is used with this test, a few intact axons may give a visible response leading the
clinician to predict a good prognosis, when in reality most of the fibers are degenerating.
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The maximum stimulation test (MST) is a modified version of the NET. A maximal
stimulus is used to depolarize all facial nerve branches. The paralyzed side is then
compared to the contralateral side and the difference is graded as equal, slightly
decreased, markedly decreased, or absent. Testing begins on the third day post onset and
is repeated periodically until return of facial function or absent response. An equal or
slightly decreased response on the involved side is considered favorable for complete
recovery. An absent or markedly decreased response denotes advanced degeneration with
a poor prognosis. The response to this test becomes abnormal sooner than the response to
the NET and is therefore considered superior. However, like the NET, this test is also
subjective.
Electroneuronography (ENoG) is considered to be the most accurate prognostic test
because it provides an objective, qualitative measurement of neural degeneration. The
facial nerve is stimulated with an impulse transcutaneously at the stylomastoid foramen
using bipolar electrodes. The muscular response is then recorded using bipolar electrodes
placed near the nasolabial groove. The peak to peak amplitude of the evoked compound
action potential is considered proportional to the number of intact axons. The two sides
are then compared with the response on the paralyzed side of the face expressed as a
percentage of the response on the normal side of the face. A reduction in amplitude on the
involved side to 10% or less of the normal side indicates a poor prognosis for
spontaneous recovery. A maximal reduction of less than 90% within 3 weeks of onset
gives an expected spontaneous rate of recovery of 80 - 100%. Disadvantages of ENoG
include discomfort, cost, and test-retest variability which is due to positioning of the
electrodes and excitation of the muscles of mastication (V).
Electromyography (EMG) is of limited value early in the evaluation of facial paralysis
because fibrillation potentials indicating axonal degeneration do not appear until 10 to 14
days post onset. However, EMG becomes important for assessing reinnervation potential
of the muscle two weeks after onset.
By using needle electrodes placed transcutaneously into the muscles of facial expression,
muscle action potentials generated by voluntary activity can be recorded. Electrical
silence can indicate normal muscle in a resting state, severe muscle wasting and fibrosis
or acute facial paralysis in the early stages. During normal voluntary contraction
organized diphasic or triphasic potentials are seen. Fibrillation potentials indicate
degeneration of the neural supply to the muscle in question. Polyphasic potentials
indicate reinnervation. These are important because they usually appear 6 - 12 weeks
before clinical return of function.
Bell's Palsy
Bell's palsy is the most common cause of facial paralysis and accounts for more than half
of all cases. Traditionally, this was considered to be a diagnosis of exclusion after ruling
out all other possible causes. However, it has recently been considered a positive
diagnosis if the following are present: unilateral weakness of all facial muscles of sudden
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onset, possibly associated with a viral prodrome, no evidence of central nervous system
pathology, no evidence of a CPA lesion, no history of otologic disease. Patients may
exhibit evidence of concomitant sensory cranial polyneuritis with otalgia or postauricular
pain, dysacousis or hyperacusis, dysgeusia, decreased tearing or epiphora, and facial
hypesthesias/dysesthesias of V or IX . Although the exact etiology of Bell's palsy is still
unclear, most clinicians believe that herpes simplex infection is the most likely agent.
This belief is supported by an increased incidence of HSV antibodies in patients with
Bell's palsy when compared to age-matched controls.
In 1995, Sugita et al were successful in producing an acute and transient facial paralysis
in mice by inoculating herpes simplex virus into their auricles (104) or tongues (30).
Facial paralysis developed in the mice between six and nine days after inoculation, lasted
for three to seven days, and then resolved spontaneously. Histopathological studies of the
facial nerve and nuclei from these mice revealed severe nerve swelling, vacuolar
degeneration, and infiltration of inflammatory cells. HSV antigens were detected in the
facial nerve, geniculate ganglion, and the facial nerve nucleus. They concluded that HSV
could produce an acute and transient facial paralysis through a natural infectious route
from the auricle or tongue to the geniculate ganglion.
Murakami et al (1996) also investigated the role of herpes simplex virus in the
pathogenesis of facial paralysis in mice by inoculating mouse auricles with HSV. On the
third day following inoculation, HSV DNA was noted in the ipsilateral facial nerve. On
the tenth day, HSV DNA was noted in both facial nerves and brain stem in the mice with
facial paralysis, but absent in these tissues in the mice without facial paralysis. Between
days 4 and 20, the neutralization antibody titer was elevated in all of the mice. In
addition, facial paralysis developed only on the inoculated side. They concluded that
HSV infection in the facial nerve and brain stem must be a prerequisite for the
development of facial paralysis and suggested that an immunologic reaction after a viral
infection plays a role in the pathogenesis.
The incidence of Bell's palsy is estimated to be 20 to 30 per 100,000, but appears to
increase with age. There is an equal male to female ration and a 3.3 times greater
incidence in pregnant females. The left and right sides of the face are equally involved,
and less than 1% of cases are bilateral. The recurrence rate is about 10% and can be
ipsilateral or bilateral. Patients with diabetes have 4 - 5 times more risk of developing the
disease. A family history is positive in about 10% of patients with Bell's palsy.
The most likely site of lesion in Bell's palsy is the meatal foramen (junction of the
internal auditory canal portion of the nerve and the labyrinthine segment of the nerve),
which is considered to be the narrowest portion of the fallopian canal. MRI with
gadolinium will usually show enhancement of the labyrinthine portion of the nerve. As
the edema within the nerve increases, axonal flow and circulation are inhibited resulting
in varying degrees of nerve injury (first, second, and third degree). Patients who are most
severely affected develop a high level of third degree injury which can result in the loss
of endoneural tubules and misdirected axonal regeneration. Histological studies from
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patients with Bell's palsy who died of nonrelated causes reveal diffuse demyelination of
the facial nerve with lymphocytic infiltrates.
The prognosis for Bell's palsy is generally good with 85 to 90% of patients recovering
completely within one month. The remaining 15% progress to complete degeneration and
will not usually show signs of recovery for three to six months. The longer the time
needed for recovery, the greater the probability of sequelae. The single most important
prognostic factor is the degree of paralysis. Patients with incomplete paralysis will
recover with no sequelae 95% of the time.
The treatment of Bell's palsy is variable, ranging from observation to surgical
decompression. Regardless of treatment given, all patients must be counselled regarding
proper eye care to prevent exposure keratitis. Patients should use natural tears liberally
during the day and should place lacrilube ointment in the eye at night. Taping of the eye
lids during sleep may be helpful as well as the use of a moisture chamber. Patients should
avoid fans and dust, and should consider wearing eye protection when outside in the
wind.
Oral prednisone in a divided dosage of 1 mg/kg/day may be helpful in preventing or
lessening degeneration, decreasing synkinesis, and relieving pain, and may result in
earlier recovery. Patients should be reevaluated within five days after starting steroids. If
some function is present (paresis), taper the steroids over the next five days. If no
improvement is noted, the full dose should be given for an additional ten days, then
tapered over five days. Oral acyclovir may help improve recovery in Bell's palsy. The
usual dosage is 500 mg po four times a day for ten days. For patients in whom steroids or
acyclovir is contraindicated, observation and eye care may be all that is possible.
Surgical decompression for Bell's palsy is somewhat controversial. Most surgeons agree,
however, that in patients progressing to total paralysis within two weeks, with an ENoG
demonstrating 90% or greater degeneration, decompression of the facial nerve may
prevent further degeneration and may improve outcome.
The rationale behind surgical decompression is based on the assumption that the site of
maximal facial nerve injury in Bell's palsy is within the meatal foramen. With increasing
edema and decreasing axoplasmic flow and microcirculation a pathological compression
injury of the nerve occurs at this point of maximal constriction. This can range from first
degree to third degree. Removal of the compression, if performed before irreversible
injury to the endoneural tubules occurs (two weeks), will allow for axonal regeneration to
occur. This is usually accomplished via a middle fossa approach. Surgical decompression
should not be done in an only hearing ear.
In a retrospective study, Fisch (1981) compared fourteen patients with >90%
degeneration within 1 to 14 days after the onset of facial paralysis who underwent
decompression using the middle fossa approach to thirteen similar patients who refused
surgical decompression. A subtle but statistically significant improvement in long-term
facial recovery was noted in the operative group as compared to the patients who refused
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surgery. Fisch concluded that in order to obtain a satisfactory return of facial function in
all cases of Bell's palsy, surgical decompression of the facial nerve should be performed
within 24 hours when results of ENoG indicate >90% degeneration has occurred.
Trauma
Trauma is the second most common cause of facial nerve paralysis. Longitudinal
fractures of the temporal bone make up 80% of all temporal bone fractures. In this type,
the fracture line extends along the longitudinal axis of the temporal bone resulting in an
external auditory canal laceration, TM perforation, and possible ossicular disruption or
hemotympanum. Facial nerve injury occurs in 10 to 20% of these fractures with the
injury most common in the perigeniculate region. Transverse fractures are much less
common (20% of all temporal bone fractures). These fractures extend along the
transverse axis of the temporal bone across the vestibule, resulting in sensorineural
hearing loss, possible loss of vestibular function, and a 50% chance of facial nerve injury
which is also usually in the perigeniculate region.
For complete facial nerve paralysis with clinical evidence of a temporal bone fracture,
obtain a high resolution CT scan/temporal bone protocol. If an obvious fracture is
present, surgical exploration of the facial nerve should be undertaken as soon as possible
via either a transmastoid/translabyrinthine (+ SNHL) or transmastoid/middle fossa
approach (- SNHL). During exploration the nerve must be fully exposed in order to
identify all injured segments, and remove any compression from fracture fragments. The
nerve sheath should be incised and any hematomas within the sheath must be carefully
evacuated. If complete transection of the nerve is found during exploration, a direct endto-end anastomosis should be performed if possible. It is important to handle all neural
tissue as atraumatically as possible, using microvascular instruments and techniques. The
nerve endings should be prepared by sharply cutting at a ninety degree angle and
reapproximating under no tension with two to three 9.0 nylon sutures through the
epineurium.
When a direct end-to-end anastomosis creates tension, or when segments of the nerve are
missing or severely damaged, interpositional grafts from the greater auricular, medial
antebrachial cutaneous, or sural nerve should be used.
For incomplete facial nerve paralysis or for delayed onset paralysis associated with a
temporal bone fracture, facial nerve testing should be obtained on day 4 after onset. If
advanced degeneration has occurred, the nerve should be surgically explored and
decompressed.
Gun shot wounds of the temporal bone cause facial paralysis in over 50% of cases. The
nerve may be transected, or may be secondarily injured by the kinetic injury from the
bullet or from bony fragmentation of the temporal bone. The most common sites of injury
are the tympanic and mastoid segments of the nerve and the stylomastoid foramen area.
For a complete paralysis after a gun shot wound, surgical exploration and repair should
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be undertaken as soon as the patient is medically stable. Generally, outcome of facial
function is much worse with gun shot wounds to the temporal bone than with temporal
bone fractures.
The facial nerve can also be injured during middle ear and mastoid surgery. If iatrogenic
transection occurs during surgery, the nerve must be repaired. If paralysis occurs
postoperatively and the surgeon is confident that the nerve was intact at the conclusion of
the case, a high resolution CT scan should be obtained and facial nerve testing should be
done on POD #4 - 6. If advanced degeneration is evident, surgical exploration and
decompression should be done. If there is any question as to the integrity of the nerve,
exploration should be done as soon as possible by a surgeon familiar with the
intratemporal anatomy of the facial nerve and reconstructive techniques.
Penetrating facial injuries with immediate facial nerve paralysis should be explored and
repaired as soon as possible while electrical stimulation can still be used to help locate
the distal branches. When the injury is medial to the lateral canthus of the eye, aggressive
exploration is not usually mandatory because the nerve endings are small and a rich
anastomotic network exists in this area.
Herpes Zoster Oticus
Herpes zoster oticus (Ramsay-Hunt Syndrome) is the third most common cause of facial
nerve paralysis. This is a manifestation of a dormant varicella zoster virus reactivating in
extramedullary cranial nerve ganglia during a period of decreased cell mediated
immunity. The prodromal symptoms are very similar to those seen in Bell's palsy, but are
usually more severe. Symptoms include severe otalgia, facial paralysis, facial numbness,
and a vesicular eruption on the concha, external auditory canal, and palate. Patients may
also have varying degrees of SNHL, vestibular symptoms, and associated cranial nerve
symptoms. Laboratory evaluation will often show the rise and fall of antibody titers
specific for the virus. The palsy is usually more severe and the prognosis much poorer
compared to Bell's palsy. Only about 50% of these patients regain normal facial function
as opposed to 90% with Bell's palsy.
The degeneration occurs more slowly, usually over three weeks instead of two. Treatment
includes steroids, valacyclovir 1 gm po tid, and proper eye care. Surgical decompression
has not been proven beneficial but may be considered for ENoG showing greater than
90% degeneration.
Otitis Media In patients with evidence of acute otitis media, dehiscences in the fallopian
canal may serve as portals for direct bacterial invasion and inflammation along the nerve.
Facial paralysis may begin within a few days of onset of an acute otitis media and is
usually incomplete. Treatment includes a wide myringotomy, drainage, and culture with
antibiotic coverage for gram positive cocci and H. flu. The facial palsy associated with
acute otitis media generally resolves with aggressive management of the infection.
However, if a total paralysis is present, serial ENoG should be obtained. If axonal
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degeneration reaches > 90%, surgical exploration and decompression should be
performed.
Patients with chronic otitis media may also develop facial paralysis which is usually
secondary to cholesteatoma or from inflammation/osteitis compressing the facial nerve.
In these cases a high resolution CT should be obtained, and surgery should be performed
as soon as possible (tympanomastoidectomy, facial nerve exploration and
decompression).
Tumors
About 5% of cases of facial nerve paralysis are caused by tumors. Factors which should
increase the clinicians suspicion of a possible tumor include: a slowly developing paresis
over more than three weeks, facial twitching, additional cranial nerve deficits, recurrent
ipsilateral involvement, associated adenopathy, or a palpable neck or parotid mass. In
these cases, MRI and CT should be obtained. The most common benign tumor causing
facial nerve paralysis is a facial nerve schwanomma. The most common malignant
tumors causing facial paralysis are mucoepidermoid carcinoma and adenoid cystic
carcinoma of the parotid gland. The management of facial nerve paralysis caused by
tumors depends upon the lesions location, size, and malignant potential and may include
transposition of the nerve, division and reanastomosis, interposition grafting, and cranial
nerve crossover. After nerve grafts, the best possible result that can be expected is a
House grade III paresis.
Melkerson-Rosenthal Syndrome
Melkerson-Rosenthal syndrome is a rare disease that consists of a triad of symptoms:
recurrent orofacial edema, recurrent facial palsy, and lingua plicata (fissured tongue). The
defining feature of this disease is the persistent or recurrent nonpitting facial edema that
cannot be explained by infection, malignancy, or connective tissue disorder. It usually
involves the lips and buccal area and may involve the gingiva, palate, and tongue. The
lips become chapped, fissured, and red-brown in appearance and may develop permanent
deformity after numerous recurrences. Facial paralysis and lingua plicata occur in half of
the patients.
The complete triad is only present in 25% of these patients. This condition usually starts
in the second decade of life, and the manifestations usually occur sequentially (rarely
simultaneously). The etiology of this syndrome is unknown. Some consider it a variant of
sarcoidosis secondary to biopsies of the lips which may reveal noncaseating granulomas.
Facial paralysis occurs in 50 to 90% of these patients and tends to be abrupt. A history of
bilateral sequential paralysis and relapse after initial recovery is common. The site of the
paralysis usually corresponds to the facial swelling. Facial nerve decompression may be
indicated if episodes of facial paralysis are frequent and progressive.
Congenital Facial Paralysis
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Newborn facial paralysis is estimated to have an incidence of about 0.23% of live births.
The most common cause is birth trauma (80%), which is usually evident by other signs of
injury. These include a history of a difficult delivery with or without forceps, facial
swelling, bruising over the mastoid or extratemporal course of the nerve, and
hemotympanum. The mechanism of injury is thought to be from direct pressure during
forceps use or from pressure on the infants face by the sacral prominence during delivery.
Congenital causes of newborn facial paralysis are much less common. Mobius' syndrome
consists of a broad spectrum of clinical findings which can range from an isolated
unilateral facial paralysis to bilateral absence of facial and abducens nerve function. In
this syndrome, the facial nerve forms but consists of only a fibrotic tract. The muscles of
facial expression may form in some cases, but degeneration to fibrosis generally occurs
rapidly. These children will have no response on EMG at birth and have no chance of
spontaneous recovery of facial function. Many other cranial nerves may be involved (III,
IV, IX, X, XII) and skeletal abnormalities may be present. Treatment for these causes of
newborn facial paralysis is generally delayed until late childhood and usually requires
static slings and muscle transfers.
A rare cause of isolated newborn facial paralysis is dysgenesis of the intratemporal facial
nerve. CT and surgical findings show that the most common site of the lesion is the distal
part of the mastoid segment in the fallopian canal. The nerve is usually very thin and
fibrotic at this area. EMG findings in these cases usually show a few functional motor
units but useful facial function is not usually present.
Newborns who present with a complete facial nerve paralysis should undergo electrical
testing within the first three days of life to differentiate between congenital and traumatic
causes. After birth trauma, the nerve can be stimulated for up to five days post injury and
fibrillation potentials will be seen on EMG at ten to fourteen days. In congenital cases,
the nerve will usually not stimulate and no fibrillation potentials will be seen on EMG.
The prognosis for trauma related facial nerve paralysis at birth is usually excellent.
Surgical decompression should not be considered until the nerve has had a chance to
recover or until >90% degeneration has occurred.
Other Causes
Sarcoidosis consists of a chronic noncaseating granulomatous disease usually
characterized by hilar or peripheral adenopathy, polyarthralgias, anergy, elevated serum
calcium, and hepatic dysfunction. One variant of sarcoidosis, Heerfordt's disease (a.k.a.
uveoparotid fever) consists of uveitis, mild fever, nonsuppurative parotitis, and cranial
nerve paralysis. The facial nerve is the most commonly involved cranial nerve and the
paralysis usually starts abruptly days to months after the parotitis. In these cases, the
paralysis is considered to be from direct invasion of the nerve by the granulomatous
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process. An elevated serum angiotensin-converting enzyme (ACE) generally confirms the
diagnosis, and treatment consists of steroids.
Lyme disease is an infection caused by the tick-borne spirochete Borrelia burgdorferi.
Several species of Ixodes ticks carry the spirochete, and the primary reservoir is the
white-tailed deer and white-footed mouse. The disease generally occurs in several stages.
The first stage consists of a flu-like illness with regional lymphadenopathy, general
malaise, and erythema migrans (erythematous enlarging annular skin lesions found
anywhere on the body). The second stage usually starts several weeks to months later
with neurologic abnormalities. These include meningitis and cranial and peripheral nerve
neuropathies. The final stage occurs months to years later in the form of recurrent
meningitis, subtle mental disorders or neurologic deficits, and chronic arthritis. Ten
percent of these patients develop facial paralysis (unilateral or bilateral) and hearing loss.
The facial paralysis typically resolves completely, but may rarely result in mild
permanent facial weakness. Treatment for Lyme disease consists of IV ceftriaxone (2
g/day) for fourteen days.
Conclusion
Although Bell's palsy remains the most common cause of acute facial nerve paralysis, it
is important for clinicians to consider all causes to avoid overlooking potentially lifethreatening diseases. A good history and physical is mandatory in the work-up of these
patients and will usually help to significantly narrow the possible causes. Although
topognostic testing is of historical interest, it has not proven to be of much use clinically.
ENoG is the most useful electrophysiologic test and should be performed within 4 - 6
days post-onset in patients with a complete paralysis with surgical decompression
considered for > 90% degeneration. Treatment plans should be individualized in each
patient, but must include education on eye protection.
Editor's Comment:
With one exception, the article by Murakami stands as the most recent and perhaps most
significant contribution to the understanding of "idiopathic" facial nerve paralysis. The
exception is the section in Conn's Current Therapy - 1996 on Acute Facial Paralysis, by
Kedar Adour.
Dr. Adour discusses the etiology and natural history of the disorder with a clarity rarely
matched in our literature. He proposes a diagnostic protocol which discusses
manifestations which do NOT permit the diagnosis of Bell's palsy and Herpes zoster
paralysis ("exclusion criteria.") He proposes a compellingly rational plan of treatment, in
which he suggests that "electrotherpy is of no benefit and may be harmful."
118
The faculty have invited Dr. Adour to present a Discussion of this Grand Rounds topic,
and we are honored that he has agreed to do so.
Discussion by Kedar K. Adour, M.D. President, Sir Charles Bell Society:
Recent published articles have changed the thinking regarding diagnosis and treatment of
acute facial paralysis:
Bell Palsy and Herpes Simplex Virus: Identification of Viral DNA in Endoneurial Fluid
and Muscle
Shingo Murakami, MD; Mutsuhiko Mizobucbi, MD; Yuki Nakashiro, MD; Takashi Doi,
MD; Naohito Hato, MD; and Naoaki Yanagihara, MD
Objective: To determine whether herpes simplex virus type 1 (HSV-1) causes Bell palsy.
Design: Prospective study.
Setting: University inpatient service.
Patients: 14 patients with Bell palsy, 9 patients with the Ramsay-Hunt syndrome, and 12
other controls.
Measurements: Viral genomes of HSV-1, varicella-zoster virus, and Epstein-Barr virus
were analyzed in clinical samples of facial nerve endoneurial fluid and posterior auricular
muscle using polymerase chain reaction (PCR) followed by hybridization with Southern
blot analysis.
Results: Herpes simplex virus type 1 genomes were detected in 11 of 14 patients (79%)
with Bell palsy but not in patients with the Ramsay-Hunt syndrome or in other controls.
The nucleotide sequences of the PCR fragments were identical to those of the HSV-1
genome.
Conclusions: Herpes simplex virus type 1 is the major etiologic agent in Bell palsy.
------------------------------ The editorial comment: "One might now question whether we
should continue using the term "Bell's palsy" to mean "idiopathic facial paralysis" or
whether we should now recognize Bell's palsy as "herpetic facial paralysis," confirming
the hypothesis advanced initially in 1972 by McCormick.
It is also documented that Adour in 1970 suggested to the American Academy of
Otolaryngology that herpes simplex was the cause of Bell's palsy."
Just as the pyramids of Egypt have guarded the riddle of the sphinx, the petrous pyramid
of the temporal bone has guarded the secrets of the facial nerve. Even today there is
119
question about the anatomic compartments of the facial nerve. The authors of the above
text suggest that the facial nerve carries some parasympathetic secretomotor fibers to the
parotid gland and a few general somatic afferent fibers to the external ear. These two
suggestions are questionable and are omitted in many neuro-anatomical textbooks.
Cutting the facial nerve at the brainstem does not lead to any deficit of parotid secretion
nor somatosensory loss in the ear canal. Sunderland's classification of nerve injury is not
applicable to herpetic facial paralysis (herpes simplex or herpes zoster). Further, Sir
Sydney Sunderland has not written a single word about the facial nerve in his classic
textbook. The five degrees of injury are of increasing severity according to the effect of
the injury not the cause. The statement "The first degree (neuropraxia) involves a
localized conduction block in the nerve with the nerve fibers responding to electrical
stimuli proximal and distal to the lesion, but not across the injured segment." is incorrect.
Segmental demyelination also causes a neuropraxic state. The terms neuropraxia,
axonotmesis, and neurotmesis should not be equated with the 5 degrees of nerve injury.
Under clinical evaluation: The House Grading System cannot be used to document the
grade of facial paralysis at the onset of a paralysis. The House system is an "end-stage"
interpretation. Grade II includes "mild synkinesis", Grade III and IV "increasing
synkinesis". Synkinesis occurs when the nerve has been damaged and then abnormally
regenerated. It is not present in the acute stages. The Brackmann modification somewhat
addresses this problem and is a modification of the original Adour-Swanson Recovery
Profile. "Bell's phenomenon (upward outward tuning of the eyeball as the patient
attempts to close the eyelids) indicates a peripheral lesion." is not correct. The eyeball
motion described is a normal function of eye muscle activity which only became apparent
to Sir Charles Bell because the eyelids did not close. A central lesion with incomplete
closure of the eyelids will also display a "Bell's phenomenon."
We agree that topognostic testing is of historical interest only and is invalid in herpetic
facial paralysis, or in temporal bone fractures. The presenting symptom of dysgeusia is
diagnostic for herpetic facial paralysis. No further diagnostic tests need be done. One
hundred per cent of patients with herpetic facial paralysis demonstrate papillitis of the
fungiform tongue papillae and has led to the truism that " Bell's palsy is a tongue blade
diagnosis." Taste tests using electrogustometry are research tools only. With regards to
the stapedial reflex, it should be considered as the "otologists' electromyography". (See
below) When should you question the diagnosis of herpetic facial paralysis formerly
called Bell's palsy and Ramsay Hunt syndrome??
Partial facial paralysis that does not resolve in 3 to 6 WEEKS.

Partial facial paralysis with electrical evidence of nerve degeneration.
120
MST in one or more branches with moderate (score of 2) to severe (scoreof 1) decrease in
muscle motion.
Global MST less than 3.
Electromyography with fibrillation (denervation) potentials.
Partial facial paralysis with ipsilateral hearing loss.
Any facial paralysis with evidence of chronic otitis media, or history of previous ear
surgery.
Progressive facial paralysis over period of weeks or months is not Bell's palsy. Note:
Damage to the nerve is complete by day 14 in Bell's palsy but not until day 21 in Ramsay
Hunt syndrome.
Total facial paralysis with no regeneration in 4 months. Note: Even with total loss of
nerve excitability by ENOG or Global MST 99.9% of all Bell's palsy or Ramsay Hunt
Syndrome will regenerate with midface contracture with synkinesis. The earliest sign of
regeneration is the return of the stapedial reflex. The next earliest sign is evidence of
mouth motion with voluntary forced closure of the eyes (synkinesis).
Total facial paralysis with electrical evidence of nerve degeneration that resolves
WITHOUT midface contracture with synkinesis. Note: Facial nerve degeneration with
regeneration in Bell's Palsy or Ramsay Hunt syndrome is followed by the sequelae of
midface contracture with synkinesis.
Recurrent facial paralysis on the same side with electrical evidence of nerve degeneration
and recovery WITHOUT contracture with synkinesis in 4 months.
Stapedial Reflex: The Otologists' Electromyography. Total facial paralysis with intact
stapedial reflex is a Partial paralysis! An absent stapedial reflex one week post onset with
return of the stapedial reflex by 3 weeks post onset indicates a good prognosis. The
Global MST score will allow you to predict rate and degree of recovery.
When reviewing Bell's palsy treatment results be suspicious of any article when the
number of patients showing electrical evidence of nerve degeneration does not equal the
number of patients with sequelae of contracture with synkinesis.
Electrical stimulation continues to be widely used in the treatment of facial paralysis (20,
23) although there is mounting evidence that it may be contraindicated.
It has been suggested that electrical stimulation may interfere with neural regeneration
post peripheral nerve injury (24,25) and studies proving its efficacy with facial muscles
are lacking in the literature. A 1984 report by the National Center for Health Services
Research concluded that "Electrotherapy treatment for Bell's palsy. . . has no
121
demonstrable beneficial effect in enhancing the functional or cosmetic outcomes in
patients with Bell's palsy." (26)
Additionally, patients who undergo electrical stimulation acutely may demonstrate more
synkinesis and mass action than those who do not (27). It is difficult to produce an
isolated contraction of the facial muscles using electrical stimulation due to their small
size and close proximity to each other. The contraction produced causes mass action
which reinforces abnormal motor patterns and can be painful. (20)
20. Cole J, Zimmerman S, Spector G: Nonsurgical neuromuscular rehabilitation of facial
muscle paresis, in Rubin LR (ed): The Paralyzed Face. St Louis, Mosby-Year Book Inc.,
1991, pp 107-112.
23. Farragher DJ: Electrical stimulation: A method of treatment for facial paralysis, in
Rose FC, Jones R, Vrbova G (eds): Neuromuscular Stimulation: Basic Concepts and
Clinical Implication. New York, Demos, 1989 vol 3, pp 303-306.
24. Cohan CS, Kater SB: Suppression of neurite elongation and growth cone motility by
electrical activity. Science 1986; 22:1638-1640. 25. Brown MC, Holland RL: A central
role for denervated tissues in causing nerve sprouting. Nature 1979; 282:724.
26. Waxman B: Electrotherapy for treatment of Facial Nerve Paralysis (Bell's Palsy).
Health Technology Assessment Reports, National Center for Health Services Research
1984; 3:27.
Added Reference:
Jansen JKS, Lomo T, Nicolaysen K, et al: Hyperinnervation of skeletal muscle fibers:
Dependence on muscle activity. Science 181:559-561, 1973.
Lack of muscle spindles....
Brodal A: Neurological Anatomy: In Relation to Clinical Medicine, ed 3. New York,
Oxford University Press, 1981, pp 495-508.
Dubner R, Seddie BJ, Storey AT: The Neural Basis of Oral and Facial Function. New
York, Plenum Press, 1978, pp 222-229.
March 27, 1996
Kedar K. Adour, MD
President, Sir Charles Bell Society
1000 Green Street #1203
122
San Francisco, CA 94133
(415) 474-4046 Fax: (415) 474-3541 E-mail: kadour@aol.com
BIBLIOGRAPHY
Bailey, Byron J., ed. Head and Neck Surgery - Otolaryngology. Philadelphia, PA.: J.B.
Ballenger, John J., ed. Diseases of the Nose, Throat, Ear, Head, and Neck. Philadelphia,
PA: Lea & Febiger, 1991.
Boies, Lawrence R., ed. Fundamentals of Otolaryngology. Philadelphia, PA: J.B.
Cummings, Charles, ed. Otolaryngology - Head and Neck Surgery. St. Louis, MO.:
Mosby - Year Book, Inc., 1993.
Fisch U. Maximal Nerve Excitability Testing vs Electroneuronography. Arch Otolaryngol
106: 352 - 357, 1980.
Fisch U. Surgery for Bell's Palsy. Arch Otolaryngol 107: 1 - 11, 1981.
Gates, George A. Current Therapy in Otolaryngology - Head and Neck Surgery. St.
Louis, MO.: Mosby - Year Book, Inc., 1994.
Green JD, et al. Surgical Management of Iatrogenic Facial Nerve Injuries. Otolaryngol
Head Neck Surg 111(5): 606 - 610, 1994.
Mattox, Douglas E., ed. The Otolaryngology Clinics of North America: Management of
Facial Nerve Disorders. Philadelphia, PA: W. B. Saunders, Co., 1991.
May, Mark. The Facial Nerve. New York: Thieme - Stratton, 1986.
May M., et al. Natural History of Bell's Palsy: The Salivary Flow Test and Other
Prognostic Indicators. Laryngoscope 86: 704 - 712, 1976.
Murakami S, et al. Role Of Herpes Simplex Virus Infection in the Pathogenesis of Facial
Paralysis in Mice. Ann Otol Rhinol Laryngol 105: 49 - 53, 1996.
Peitersen E. The Natural History of Bell's Palsy. Am J. Otol 4: 107, 1982.
Rubin, Leonard R., The Paralyzed Face. St. Louis, MO.: Mosby - Year Book, Inc., 1991.
Selesnick SH, Patwardhan A. Acute Facial Paralysis: Evaluation and Early Management.
Am J Otolaryngol 15 (6): 387 - 408, 1994.
123
Sugita T, et al. Facial Nerve Paralysis Induced by Herpes Simplex Virus in Mice: An
Animal Model of Acute and Transient Facial Paralysis. Ann Otol Rhinol Laryngol
104:574 - 581, 1995.
124

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1

Author: Michael Lambert, MD, Fellowship Director of Emergency Ultrasound, Clinical

Background: Bell palsy is one of the most common neurologic

Exclude other causes of facial paralysis.

Pathophysiology: Actual pathophysiology is unknown; this is an area of

In the US: The incidence of Bell palsy in the United

Internationally: The incidence is the same as in the United States.

Mortality/Morbidity: Bell palsy can cause aesthetic, functional, and

Motor synkinesis (involuntary movement accompanying

Autonomic synkinesis (involuntary lacrimation after a

Race: Incidence of Bell palsy appears to be slightly higher in

Sex: No difference exists in sex distribution in patients with Bell palsy.

History: Most patients presenting to the ED suspect they have

Postauricular pains: Almost 50% of patients experience pain in the

Hyperacusis: Impaired tolerance to typical levels of noise due to an

Physical: Findings of facial paralysis are easily recognizable on physical

The classic definition of Bell palsy describes mononeuric

The facial nerve is the only cranial nerve eliciting

Remember that weakness and/or paralysis from

In supranuclear lesions such as a cortical stroke (upper

Test other cranial nerves; their examination results should be normal.

Tympanic membranes should not be inflamed; presence

Causes: "All that glitters is not gold" (William Shakespeare)

Viral infections: Clinical and epidemiologic data lend credence to an

Pregnancy: Bell palsy is uncommon in pregnancy; however, the

Genetics: Recurrence rates (4.5-15%) and familial

Diabetes Mellitus, Type 1 - A Review

No specific laboratory tests exist to confirm the diagnosis of Bell

Complete blood count

Erythrocyte sedimentation rate

Thyroid function studies

Serum glucose level

Rapid plasma reagin (RPR) or Venereal Disease

Human immunodeficiency virus (HIV)

Cerebral spinal fluid analysis

Immunoglobulin M (IgM), immunoglobulin G (IgG), and

Bell palsy remains a clinical diagnosis. Imaging studies are

Facial CT scan or plain radiographs: Perform to rule out

CT scan is indicated when stroke, or acquired immunodeficiency

MRI: For a suspected neoplasm of the temporal bone, brain, parotid

Electrodiagnosis of the facial nerve: These studies assess the

Electromyography (EMG) and nerve conduction

In the nerve excitability test, the threshold of the electrical

Electroneurography (ENoG) compares evoked potentials on

Emergency Department Care: The primary treatment of

Treatment of Bell palsy with steroids remains

controversial. Numerous research articles have been written

Researchers seem to lean more toward using steroids as a means to

Antiviral agents: Although there is insufficient research evaluating the

Artificial tears: Use these during waking hours to replace diminished

Eyeglasses or shields protect the eye from injury and

Neurologist: When other neurologic signs are identified on physical

Ophthalmologist: For any unexplained ocular pain or abnormal findings on

Otolaryngologist: In patients with persistent paralysis, prolonged weakness

Surgeon: Surgery to decompress the facial nerve is recommended occasionally

Drug Category: Corticosteroids -- Have anti-inflammatory properties

Documented hypersensitivity; viral, fungal, connective

Coadministration with estrogens may decrease prednisone

B - Usually safe but benefits must outweigh the risks.

Abrupt discontinuation of glucocorticoids may cause

Drug Category: Antiviral -- Herpes simplex infections may be a common cause of

Acyclovir (Zovirax) -- Has demonstrated inhibitory