OTOLARYNGOLOGY FOR THE INTERNIST

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THE FACIAL NERVE

Current Trends in Diagnosis, Treatment, and Rehabilitation
C. Gary Jackson, MD, FACS, and Peter G. von Doersten, MD

Facial paralysis is a potentially devastating disorder. Few impairments have a more negative effect on socioeconomic capacity in an individual's life. The facial nerve is responsible for facial expression, lacrimation, salivation, taste, and sensation. It is also the most commonly paralyzed nerve of the body. Paralysis may result from infection, tumor growth, trauma, and several other causes. The nerve is further susceptible to spasm from compression by nearby intracranial vessels or tumors. It has a tortuous bony course longer than any other nerve through the densest bone in the body, making surgery on it quite difficult. It has a fragile blood supply. Finally, the facial nerve is difficult to treat, rehabilitate, and reanimate functionally. Advances in microbiology, radiographic imaging, electrodiagnostic testing, and microsurgery have provided great insight into the pathophysiology, diagnosis, treatment, and rehabilitation of the facial nerve. The most important tenet for all physicians in treating facial nerve pathology is proper diagnosis and early treatment. The terms Bell's palsy and idiopathic facial paralysis may no longer be considered synonymous. DNA polymerase chain reaction (PCR) testing on the endoneurial fluid of the facial nerve in patients with sudden ipsilateral facial paralysis has supported the herpes simplex virus (HSV) as the causative agent in paralysis previously considered idi~pathic'~, 70 or secondary to immunologic or vascular proce~ses.'~ A differential diagnosis (to be discussed later) must always be considered because easily diagnosed entities, such as otitis media or parotid neoplasms, should not be overlooked. Early treatment of facial nerve pathology is imperative because paresis treatment within 72 hours can have implications on return of function in terms of both time and degree.s This article focuses on the evaluation, differential diagnosis, medical treat-

From The Otology Group, Nashville, Tennessee (CGJ); and Rocky Mountain Eye and Ear Center, PC, Missoula, Montana (PGV)

MEDICAL CLINICS OF NORTH AMERICA

VOLUME 83 * NUMBER 1 *JANUARY 1999

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ment, and rehabilitation of facial nerve pathology, with primary emphasis on facial paralysis. Surgical management is also discussed, including reanimation of the paralyzed face. FACIAL NERVE ANATOMY AND HISTOPATHOLOGY

A brief synopsis of facial nerve anatomy is crucial in understanding its pathophysiology (Fig. 1).The facial nerve is a mixed nerve with special visceral efferent (facial musculature), general visceral efferent (parasympathetic lacrimal, minor, submandibular, sublingual gland innervation), special visceral afferent (taste, anterior two thirds of tongue), and general somatic afferent (cutaneous innervation of external auditory canal and pinna) functions. It originates from the pontomedullary junction of the brain stem and courses laterally to the internal auditory canal (IAC), which it enters with the cochleovestibular nerve. A thin myelin sheath encompasses the nerve in its intracranial portion. It is subject to demyelinization from vessel loop pressure with resultant ectopic nerve stimulation.66, h7 The intracranial portion of the nerve is able to withstand slow stretching from a tumor but is quite susceptible to trauma. As the nerve travels farther laterally in the IAC, it enters the narrow fallopian canal, becoming encased in periosteum and epineurium. This canal measures roughly 30 to 33 mm in length96 and consists of three portions-the labyrinthine, tympanic, and mastoid. Of significance, the narrowest portion is at the labyrinthine section, which contains the geniculate ganglion. This section averages 0.68 mm in diameterZR The facial nerve occupies 83% of the available space compared with 23% in the mastoid It is the labyrinthine portion where it is most likely

Sensory and motor roots of VII

Geniculate

Ptervqopalatine ganglion etrosal n

Stapedius n

Parotid

+ q x

Submandibular .gland and ganglion

. .

Muscles of Expression

Figure 1. Facial nerve anatomy and histopathology.

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that a vicious cycle of edema from infection, vascular compromise, and ischemia, thought to be the pathophysiologic process in Bell's palsy, can occur. Four branches of the facial nerve occur within the fallopian canal-the greater and lesser superficial petrosal nerves, the nerve to the stapedius muscle, and the chorda tympani nerve. Topognostic testing for facial nerve dysfunction includes examining tearing, salivation, stapedial reflexes, and taste, attributable to the functions of each of these branches. Today, topognostic testing is of little clinical utility. The facial nerve exits the stylomastoid foramen of the skull base; gives off branches to the posterior auricular muscles and the posterior belly of the digastric; then proceeds anteriorly into the parotid gland, where it divides at the pes anserinus into the temporal, zygomatic, buccal, marginal mandibular, and cervical branches. These nerves are responsible for facial expression, eye closure, and assistance with mastication and speech. The facial nerve derives both an intrinsic and an extrinsic blood supply, the former derived from the latter.I2The extrinsic vascular supply comes from three primary sources-the stylomastoid artery, the petrosal artery, and the internal auditory (labyrinthine) artery-and derives the intrinsic The narrow labyrinthine portion of the nerve is a transition watershed zone between blood supply from the vertebral and carotid artery This vasculature courses between the epineurium and the bony periosteum and is jeopardized by trauma, swelling from infection, and surgical injury. During these times, survival of the nerve depends on the tenuous supply of the intrinsic system.

NERVE INJURY STAGES The stages of nerve injury were well described by Seddon in 1943Rh and Sunderland in 197893 and correspond with a spontaneous facial recovery grading scale proposed by House and Brackmann in 1985 (Table l).,O The degrees of injury range from 1 to 5 and correspond with neurapraxia, axonotmesis, neurotmesis, partial transection, and complete transection. Neurapraxiu is a reversible block of conduction caused by an increase in intraneural pressure, with resultant focal demyelinization and axoplasmal damming. Recovery of the nerve is often complete to a House-Brackmann grade 1 level. Axonotmesis is a more permanent compression with axoplasmal flow interruption and distal wallerian degeneration with partial loss of axons, often recovering to a partial facial weakness or House-Brackmann grade 2. Neurotmesis relates to the permanent loss of axonal and myelin tubes. Facial recovery may improve to a moderate-to-severe weakness of the face, or House-Brackmann grade 3 4 . Faulty regeneration of the nerve may also lead to facial synkinesis or contracture. Partial or complete transection of the nerve may lead ultimately to either minimal perceptible facial function (House-Brackmann grade 5) or no function (House-Brackmann grade 6). Gantz et aP0 have used intraoperative evoked electromyography testing to determine the site of lesion in Bell's palsy during operative decompression of the nerve. These surgeons were able to stimulate the facial nerve distal to the geniculate ganglion but not proximal, supporting the theory of a neurapraxic nerve at its labyrinthine portion. Magnetic resonance (MR) imaging has also shown significant enhancement with gadolinium in the labyrinthine region in patients with Bell's palsy, supporting this as the affected site.", R5 Jackson et a147 confirmed this histologically.

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Table 1. HOUSE-BRACKMANN CLASSIFICATION OF FACIAL FUNCTION Grade Characteristics

I. Normal
11. Mild dysfunction

111. Moderate dysfunction

IV. Moderately severe dysfunction

V. Severe dysfunction

VI. Total paralysis

Normal facial function in all areas Gross Slight weakness noticeable on close inspection May have slight synkinesis. At rest, normal symmetry and tone Motion Forehead: moderate-to-good function Eye complete closure with minimal effort Mouth slight asymmetry Gross Obvious, but not disfiguring difference between the two sides. Noticeable but not severe synkinesis, contracture, or hemifacial spasm At rest, normal symmetry and tone Motion Forehead: slight-to-moderate movement Eye: complete closure with effort Mouth: slightly weak with maximum effort Gross Obvious weakness and/or disfiguring asymmetry. At rest, normal symmetry and tone Motion Forehead: none Eye: incomplete closure Mouth: asymmetric with maximum effort Gross Only barely perceptible motion. At rest, asymmetry Motion Forehead: none Eye: incomplete closure Mouth slight movement No movement

From House W, Brackmann D Facial nerve grading system. Otolaryngol Head Neck Surg 93146147,1985.

DIAGNOSIS

The examination of the patient with facial paralysis has three main objectives: to identify (1) the site of the lesion, (2) the degree of dysfunction, and, if possible, (3) the cause.
History and Physical Examination

As with all disease, the history and physical examination are of paramount importance in facial paralysis. The history should detail date of onset, rapidity of onset, associated symptoms, and associated systemic diseases to help determine a traumatic, neoplastic, or infectious cause. (Metabolic, neurologic, toxic, iatrogenic, and systemic causes may also be considered.) The physical examination should include a careful head and neck examination, with careful attention to the ear, parotid gland, other cranial nerves, salivation, tearing, and taste. Tuning forks can crudely assess hearing. Grading paralysis according to the House-Brackmann grading system is helpful, with measurements of range of

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motion of the eyebrows and a quantification of ability to close the eye and to lateralize the oral commissure. A complete audiogram with stapedial reflex testing is helpful in determining eighth cranial nerve function (a unilateral sensorineural hearing loss would raise suspicion for an acoustic or facial neuroma), stapedius muscle function (part of topognostic testing to identify site of lesion), and presence of a conductive loss (raising suspicion for middle ear disease).

Radiologic Imaging

If a parotid mass is palpated or suspicion is aroused for a compressive lesion in the cerebellopontine angle or internal auditory canal, imaging may be obtained. A computed tomography (CT) or MR imaging scan (with fine needle aspiration for specimen diagnosis) may be used for the parotid gland. The best intracranial facial nerve examination is with a gadolinium-enhanced MR imaging scan. As stated earlier, the geniculate ganglion within the labyrinthine facial nerve enhances in Bells palsy. Acoustic neuromas, meningiomas, hemangiomas, and other cerebellopontine angle (CPA) lesions may be diagnosed with this technique. A less expensive test for examining the brain stem, CPA, and IAC is the limited T2-weighted MR imaging scan without contrast using a spinecho technique. Some intracranial masses may be missed with this test, however. A high-resolution CT scan is useful for examining the course of the facial nerve within the temporal bone, particularly after trauma.

Electrical Testing

Although the initial physical examination documents the severity of the paralysis, it does not reveal the neurophysiologic status of the facial nerve itself, the progression of disease, the prognosis, or the indication for further intervention, including surgical decompression. Electrodiagnostic testing has been used in selecting patients for treatment and in predicting o ~ t c o m e . 2h, ~* ~ 83 The most commonly used electrical tests include the minimal nerve excitability test, the maximal stimulation test (MST), and electroneuronography (ENOG). These tests rely on percutaneous stimulation of the facial nerve and do not show abnormalities until 72 hours after nerve degeneration. Quantitative rating systems have been applied to both MST and ENOG, and each has been used to evaluate the facial nerve after paralysis. Opinions differ on the accuracy of each modality, but MST testing is fairly easy to perform and inexpensive, requiring only a Hilger nerve stimulator (WR Medical Electronics Co., Stillwater, MI). A point score is awarded for stimulation of various branches of the facial nerve, and the score is used to predict outcome.83ENOG testing records a compound action potential (CAP) and latency after stimulating the facial nerve, and the percent degeneration of the amplitude of the CAP compared to the control side of the face is used to predict outcome. Several authors have advocated surgical decompression of the facial nerve based on a CAP degeneration of 90% or greater in Bells palsy because of a poor prognosis without surgical inter57 ~ention. ~A ~ ,poor prognosis has not been determined for traumatic paralysis with CAP degeneration of 90% or greater.89

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FACIAL NERVE PATHOLOGY Facial Paralysis

Perhaps the most complete differential diagnosis of facial palsy has been compiled by May and Klein (Table 2).62 These authors divide causes into several large categories: birth, trauma, neurologic, infection, metabolic, neoplastic, toxic, iatrogenic, and idiopathic. A complete discussion of each of these entities is beyond the scope of this article. Several areas of interest over the past several years have been the association of facial paralysis with Lyme disease,'*,36 human 72 human T-lymphotropic virus type I (HTLVimmunodeficiency virus (HIV),53, 1),48and HSV.69, 70, 84 A careful history at the time of presentation includes questioning regarding exposure to tick bites and risk factors for HIV. With regards to HSV, perhaps no other topic has elicited more controversy within the otologic community regarding the facial nerve in the past 20 years.
Infectious Causes

Bell's Palsy Bell's palsy is a lower motor neuron, ipsilateral facial paresis, which is acute in onset, is frequently preceded by a viral prodrome, and is generally regarded as polyneuropathic. Affecting roughly 20 people per 100,000 per year? 35, 69 it is equally distributed between the sexes and sides of the face. The risk to pregnant women is 3.3 times greater than that for nonpregnant women.37The risk of recurrence is lo%, and this recurrence may occur on either side. Of those who experience recurrence three times, the risk of a fourth recurrence is 5Oy0.~' Diabetics are at higher risk6 (4.5 times more likely), and the risk of Bell's palsy increases with each decade of life.35 Both AdourZ and May and Kleidz describe a prodromal illness before the onset of Bell's palsy (60%of patients), with frequent associated facial numbness, epiphora, pain, dysgeusia, hyperacusis, and decreased tearing (50%). Familial tendencies are implicated in 14% of cases, and a majority (900/,)of patients have a decreased or absent stapedial reflex. Adour and May and Klein disagree on calling Bell's palsy a diagnosis of exclusion. Adour et a14 advocate making the diagnosis based on the history and clinical symptoms and the presence of dysgeusia and hyperacusis. May and Klei@ recommend excluding other diagnostic entities based on a defined workup and a lengthy differential diagnosis. Other authors have also described Bell's palsy as a diagnosis of and have frequently used the term idiopathic. With the discoveries using PCR DNA testing, the use of this term has been further questioned. Consideration of the cause as an HSV-mediated viral inflammatory immune mechanism has become more widely accepted.I3, 84 The treatment of Bell's palsy has become less controversial and involves both medical and surgical options. In 1972, Adour' published a large controlled study on the treatment of Bell's palsy with corticosteroids, showing an 89% complete functional recovery in 194 patients treated with steroids compared with a 64% complete recovery in control patients. Despite some criticism of the study and other studies9,94 that corroborated Adour's findings, steroid use for Bell's palsy is widespread today.87, 92 Because a viral cause has long been postulated, a study reviewed the use of antiviral agents5 in the treatment of Bell's palsy. In a randomized, double-

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Table 2. CAUSES OF FACIAL PALSY IDENTIFIED IN A REVIEW OF MEDICAL LITERATURE (1 900-1 990)
Birth Molding Forceps delivery Dystrophia myotonica Mobius syndrome (facial diplegia associated with other cranial nerve deficits) Trauma Basal skull fractures Facial injuries Penetrating injury to middle ear Altitude paralysis (barotrauma) Scuba diving (barotrauma) Lightning Neurologic Opercular syndrome (cortical lesion in facial motor area) Millard-Gubler syndrome (abducens palsy with contralateral hemiplegia caused by lesion in base of pons involving corticospinal tract) Infection External otitis Otitis media Mastoiditis Chickenpox Herpes zoster cephalicus (Ramsey Hunt syndrome) Encephalitis Poliomyelitis (type I) Mumps Mononucleosis Leprosy Influenza Coxsackievirus Malaria Syphilis Scleroma Tuberculosis Botulism Acute hemorrhagic conjunctivitis (enterovims 70) Gnathostomiasis Mucormycosis Lvme disease Cat scratch AIDS Metabolic Diabetes mellitus Hyperthyroidism Pregnancy Hypertension Acute porphyria Vitamin A deficiency Neoplastic Benign lesions of parotid Cholesteatoma Seventh nerve tumor Glomus jugulare tumor Leukemia Meningioma Hemangioblastoma Sarcoma Carcinoma (invading or metastatic) Anomalous sigmoid sinus Carotid artery aneurysm Hemangioma of tympanum Hydradenoma (external canal) Facial nerve tumor (cylindroma) Schwannoma Teratoma Hand-Schuller-Christian disease Fibrous dysplasia Neurofibromatosis I1 Toxic Thalidomide (Miehlke syndrome, cranial nerves VI and VII with congenital malformed external ears and deafness) Ethylene glycol Alcoholism Arsenic intoxication Tetanus Diphtheria Carbon monoxide Iatrogenic Mandibular block anesthesia Antitetanus serum Vaccine treatment for rabies Postimmunization Parotid surgery Mastoid surgery Posttonsillectomy and adenoidectomy Iontophoresis (local anesthesia) Embolization Dental Idiopathic Bells, familial Melkersson-Rosenthal syndrome (recurrent alternating facial palsy, furrowed tongue, faciolabial edema) Hereditary hypertrophic neuropathy (CharcotMarie-Tooth disease, Dkjkrine-Sottas disease) Autoimmune syndrome Amyloidosis Temporal arteritis Thrombotic thrombocytopenic purpura Periarteritis nodosa Landry-Guillain-Barre syndrome (ascending paralysis) Multiple sclerosis Myasthenia gravis Sarcoidosis (Heerfordt syndrome-uveoparotid fever) Osteopetrosis

Adnptedfrom May M, Klein S: Differential diagnosis of facial nerve palsy. Otolaryngol Clin North Am 24613444,
1991.

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blinded, controlled study, patients treated with prednisone and acyclovir were found to have a statistically significant faster time to recovery and more complete recovery than patients treated with prednisone alone. The treatment group received 60 mg prednisone per day for 4 days, tapering over a total of 10 days, with 400 mg acyclovir taken five times per day for 10 days. With the advent of new antivirals with better gastric absorption (famciclovir and valacyclovir), better uptake is now possible without as great a potential side effect of gastric upset. Surgical decompression for Bell's palsy is advocated only in patients who have had an ENOG that demonstrates a CAP amplitude decrease of greater than 90% and who are in a time window roughly 2 to 3 weeks after the onset of paralysis.*' The surgical procedure typically involves a middle fossa craniotomy for decompression of the labyrinthine portion of the nerve and is not recommended for patients in poor health or older than age 60. Contraindications are infection within the middle ear or mastoid or the presence of an only hearing ear (because of the risk of iatrogenic hearing loss). Patients with multiple recurrences of paralysis are also considered candidate^.^^ Expected time of recovery of function ranges from weeks to 12 months. In patients who have partial paralysis (neurapraxia), approximately 94% recover full function within months.79The absence of recovery by 4 months indicates a likely poor outcome with either no return of function or severe synkinesis and contracture. Unsatisfactory results occur in between 15% and 40% of untreated patients.',57. 79 Adour et a15report that patients treated with acyclovir and prednisone have one half the incidence of synkinesis and facial contracture compared with patients treated with prednisone alone. Esslen,z4in data generated by natural history ENOG and that correlate well with the clinical landmark study of Peiter~en?~ determined that in 93% of Bell's palsy patients, the progression of degeneration is ended by day 10. Return of function, even if minimal, always occurs within 6 months.79It is most important to identify areas of a patient's clinical presentation that suggest a diagnosis other than Bell's palsy: Slowly progressive facial paralysis beyond 3 weeks or no return of function within 6 months suggests neoplasm. Simultaneous bilateral facial paralysis excludes Bell's palsy as a diagnosis. Intact forehead movement is seen in 5% of Bell's palsy patients and does not necessarily suggest an upper motor neuron lesion. Recurrent unilateral paralysis is consistent with Bell's palsy but is frequently (30%) seen in tumor patients. It is a cause for concern. Pain is not viewed as prognostically or diagnostically significant. Facial hyperkinesis is troublesome because it is seen in vascular compression and neoplasia. Clinical awareness of these major features helps to avoid misdiagnosis or delayed diagnosis.
Herpes Zoster Oticus (Ramsay Hunt Syndrome)

In 1907, Ramsay H u n P generated his classic description of a syndrome characterized by facial paralysis, herpetiform vesicular eruptions, and vestibulocochlear dysfunction. In contrast to Bell's palsy, the viral cause of Ramsay Hunt syndrome is not disputed." Vesicular eruptions may occur over the ear, face, and neck down to the shoulder. There is more pain than in Bell's palsy, a

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greater likelihood of hearing loss, and a lower incidence of a complete recovery from facial paralysis (54%).'j8 A complete recovery of hearing loss occurs in roughly 45% of patients. Younger patients have a better prognosis.68 Treatment for Ramsay Hunt syndrome is similar to that of Bell's palsy. Steroids have been advocated at 1 mg/kg/d in divided doses tapering over 10 days.76Further, Dickens et alZ1have found that the initiation of intravenous acyclovir within 72 hours of onset has greatly mitigated the neurologic sequelae of Ramsay Hunt syndrome. These authors found earlier facial nerve recovery and return of hearing.
Lyme Disease

Lyme disease is a tick-borne spirochete (Borrelia burgdorferi) infection known to cause erythema chronicum migrans, meningopolyneuritis, myocardial conduction abnormalities, and Lyme arthritis. Initially associated with tick bites in the northeastern United States, the disease is now recognized across North America and Europe. Clark et alzohave reported the incidence of facial palsy at greater than 10% in all patients with Lyme disease, with bilaterality in 25% of these. Symptoms after a tick bite (with associated maculopapular rash with central clearing) include headache, malaise, myalgias, chills and fever, nausea and vomiting, and neurologic sequelae. If Lyme disease is suspect, an enzyme immunoassay may be obtained.90The treatment of choice is doxycycline or tetracycline, with penicillin and erythromycin as alternatives. The prognosis for return of facial function is excellent.20
Facial Paralysis from Bacterial Infection

Facial paralysis may occur from an acute bacterial infection of the middle ear or mastoid, chronic otitis media, or necrotizing otitis externa. In 1982, Pollock and Browns2reported the incidence of facial paralysis in 1250 patients with otitis media at 0.16% compared with 2% in the preantibiotic era. The route of infection is thought to be via the bony dehiscences of the fallopian canal, with resultant edema and vascular c~mprornise.~~ Chronic infection is thought to cause compression of the facial nerve from intrafallopian abscesses, granulation tissue, and edema.'j5Cholesteatoma should be suspected if the onset of paralysis is gradual. This is contrasted to the 2- to 3-day onset of paralysis in acute otitis media. In immunocompromised hosts with otitis externa, a necrotizing chronic infection (typically from Pseudomonas aeruginosa) can occur, which spreads via vascular and fascia1 planes to cause cellulitis of the skull base and facial paralysis." In cases of acute paralysis with otitis media, treatment consists of intrave88 If paralysis persists more than 2 nous antibiotics and surgical myringotomy.20, weeks or if fever persists more than 48 hours, a mastoidectomy with facial nerve decompression is advocated.= For chronic otitis media and cholesteatoma, a mastoidectomy is also indicated. In cases of malignant (necrotizing) otitis externa, facial paralysis is an ominous sign. Occurring in 38% of patients, facial paralysis is typically predictive of poor patient survival.5'j Treatment is largely medical, with intravenous antibiotics and occasionally hyperbaric oxygen. The prognosis is not affected by surgery (other than debridement of granulation tissue in the external canal), and if the patient survives, the chance of facial nerve recovery is roughly

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Noninfectious Causes

Trauma Intratemporal facial paralysis may be of a catastrophic origin (head trauma) or may be iatrogenic in nature. In head trauma, the facial nerve is the most commonly injured cranial nerve, with temporal bone fractures as the likely cause. One study reports 70 documented temporal bone fractures of 5000 cases of head trauma, 27 of which developed facial nerve paralysis.I6Facial paralysis secondary to surgery of the middle ear and mastoid ranges in incidence from 0.2% to 1.4%.3y, 54 Temporal bone fractures are typically described in terms of the relationship of the fracture line to the long axis of the petrous pyramid. Longitudinal Injury commonly fractures are most common and constitute 90% of occurs to the facial nerve in the region of the geniculate ganglion. Transverse fractures are less common but result in 38% to 50% of cases of facial These fractures tend to transect the IAC through which the facial nerve courses. Many fractures do not fall into the classic definitions of longitudinal or transverse and are, in fact, mixed fractures or comminuted fractures. Indications for exploration and repair are fairly well defined, and in contrast to paralysis for infectious causes, no controversy exists regarding the cause. According to May and Shamba~gh;~ if there is firm evidence after head trauma that the nerve has been transected, including a sudden onset of complete paralysis, loss of electrical activity by ENOG or MST testing by the fifth day, and evidence of a displaced fracture involving the fallopian canal, surgical exploration and decompression are warranted. Depending on the site of the lesion, decompression typically entails a middle cranial fossa approach and mastoidectomy. For iatrogenic injuries, reexploration is warranted if other causes, such as local anesthesia or compression from packing, have been excluded. If the paralysis is complete and was noted immediately after surgery (i.e., not delayed in onset), surgicai exploration should be considered. Repairs typically consist of decompressing the nerve proximal and distal to the site of injury; opening the nerve sheath to prevent compression from edema; and, if necessary, performing a microneurorraphy (for injury <So% of nerve diameter) or nerve grafting.
Neoplasm

Intuitively, it would seem that a facial nerve palsy secondary to a tumor would be relatively slow in onset. Characteristic is a paralytic course that is progressive beyond 3 weeks. A sudden onset, however, does not rule out the possibility of a neoplasm. Jackson et a146have noted the incidence of sudden facial palsy in 27% of patients who have neoplastic involvement of the nerve. This has been substantiated by Neely and Alford (28% of patients) and Fisch and Ruttner2y(20% of patients). Symptoms of facial hyperkinesis,61 ipsilateral recurrent facial paralysis, pain, persistence of symptoms, and other cranial nerve involvement all increase suspicion for neoplastic involvement. The differential diagnosis for neoplasms is presented in Table 2. These may include tumors of the facial nerve itself (neuromas, hemangiomas) or tumors anywhere along the course of the facial nerve that cause extrinsic compression or malignant involvement of the nerve. Isolated branch palsies direct attention to the distal nerve in the parotid gland. For a suspicious history, a radiologic examination typically consists of an MR imaging scan with gadolinium with

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attention to the IAC. This examination may require supplementation with a CT scan to examine the bony course of the fallopian canal. For primary tumors of the facial nerve with paralysis, recommended treatment is surgical resection with end-to-end anastomosis or interposition nerve grafting.44 Melkersson-Rosenthal Syndrome Characterized by facial paralysis, episodic facial swelling, and a fissured tongue (scrota1 tongue or lingua plicata), Melkersson-Rosenthal syndromeassociated paralysis typically begtns in adolescence and has been described as both sporadic and familial.s5 The cause is unknown. With each episode of paralysis, facial edema worsens, and progressive disfigurement may occur.52 Because of the relatively low incidence of this disorder, optimal therapy is difficult to deduce statistically. Steroid use has been employed as in Bell's palsy, and some groups have advocated surgical decompression to avoid the denervation associated with recurrent episodes of paralysis.32, 33

PARALYSIS IN CHILDREN

The causes for facial paralysis in children are somewhat different than those for adults. May et a160 have found that Bell's palsy represents the most common congenital cause (42% of cases), followed by trauma (21%), infection (Is%), causes (8%), and neoplasm (2%). Identifiable causes are found in between 58% and 72% of cases,7and are treated based on the cause. In newborns, it is most important to determine whether the paralysis is acquired through trauma or is congenital in nature, so that appropriate therapy may be provided. Smith et aP' have found that of 95 patients with neonatal facial paralysis, 78% were the result of intrauterine or birth trauma, and the rest were congenital. With trauma, a crush injury is typically suspect in forceps delivery, although pressure on the ipsilateral face from the sacrum or in utero molding during delivery are also suspect.s1A complete return of facial function without sequelae has been found in 91% of cases.9' The facial nerve has been shown to be vulnerable in the neonate because of the lateral position of the stylomastoid foramen, placing the nerve in a superficial position at its exit from the skull base. Facial paralysis in the neonate in congenital malformations has a poor prognosis for recovery. With congenital paralysis, there is an absence of obvious physical or radiographic findings for trauma, although associated craniofacial abnormalities, particularly of the first and second branchial arch, may occur.6o Of the associated syndromes, perhaps the most commonly addressed is Mobius' syndrome, with a characteristic bilateral facial and abducens palsy and associated extremity abnormalities. Suspect is intrauterine brain stem necrosis42or nuclear agenesis secondary to anomalous brain stem vessel development in the sixth week of gestation.lg A translocation on chromosome 1 has also been i m p l i ~ a t e d Thalidomide .~~ embryopathy has similar facial findings with limb defects. Other syndromes associated with neonatal paralysis include Goldenhar 's syndrome; neurofibromatosis; Paland syndrome; cardiofacial anomaly (isolated mandibular branch palsy with cardiac defects); and several bone disorders, including McCune-Albright syndrome and osteopetr~sis.~~

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FACIAL HYPERKINESIA

The most common forms of facial hyperkinesia are essential blepharospasm and hemifacial spasm. Blepharospasm is a bilateral asynchronous firing of the orbicularis oculi muscle (with frequent brow involvement), which may have coexisting movement of the lower face, mouth, tongue, and neck (Meiges syndrome). The cause is unclear but an extrapyramidal disorder is Treatment consists initially of the injection of botulinum toxin (Botox) into the this treatment is not effective or if complications affected m u ~ c u l a t u r e .If ~~ 0ccur,2~ a partial or complete surgical myectomy is adv0~ated.I~ Hemifacial spasm is a vascular compressidn syndrome and can be differentiated from essential blepharospasm by its synchronous muscle twitches, lack of disappearance in sleep, degree of spasticity (more complete and longer lasting), unilaterality, cause (vascular compression at the nerve root entry zone), and treatment.zzThe condition is rare, with a prevalence of 7.4 men per 100,000, and 14.5 women per 100,000 in the white population of the United States.* Multiple causes have been suggested, but evidence based on electrophysiologic data and surgical observation suggests compression at the nerve root entry zone, most commonly by the posterior-inferior cerebellar artery.22, 49 The kindling theory postulates that vessel compression causes demyelinization, resulting in an ectopic focus of excitation of the nerve.66 Treatment is typically with an initial trial of botulinum toxin, with subsequent injections every 4 to 6 months. More definitive treatment is with microvascular decompression of the facial nerve via a retrosigmoid craniotomy. In a review of the literature, Illingworth et a1 report that in microvascular decompression surgery, 62% to 97% of patients had complete resolution of spasm, 1.3% to 25% had partial relief, and 1.3% to 15% had no relief in the large studies p e r f ~ r m e dIn . ~their ~ own series, these authors patients had a 92% free-of-spasm rate at a mean follow-up of 8 years.
REHABILITATION

With facial paralysis, either temporary or permanent, great attention is paid to protection of the eye. Major factors affecting the eye are lagophthalmos, paralytic ectropion of the lower lid, concomitant trigeminal nerve loss, and decreased tear p r o d u ~ t i o n After . ~ ~ paralysis, it is imperative to provide the patient with artificial tears and ophthalmic ointments. Glasses or goggles are important in protection from light, wind, and dust. Protection of the eye at night is frequently required and requires proper technique. Long-term treatment for paralysis may include tarsorrhaphy, lateral canthoplasty, and gold weight implant to the upper lid. Ophthalmologic consultation is indicated. Priorities of rehabilitation are corneal protection, comfort, preservation of vision, improved function, and esthetic r e s t ~ r a t i o n . ~ ~ Restoration of function to the face is possible with several techniques. After a traumatic injury to the nerve, a microsurgical reanastomosis or nerve grafting is possible. Grafting donor sites include the sural, great auricular, and median antebrachial cutaneous nerves. For Bells palsy, a wait of 12 months is advocated to monitor for spontaneous return of function before surgical i n t e r ~ e n t i o n . ~ ~ Electromyography may be helpful in assessing early return. If no return is present, nerve substitution is possible in the form of a hypoglossal-to-facial nerve crossover. This procedure preserves muscle tone should other techniques be in consideration. Attention has been paid to the cross-facial nerve grafting

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technique," which is most successful when performed early after injury3* For patients in whom facial paralysis is long-standing with either congenital paralysis or fibrosis of the musculature, regional muscle transfers (temporalis or masseter muscle) or microneurovascular muscle transfer is applicable.'" Improvements in microsurgical technique and instrumentation have yielded increasing success in restoring symmetric facial movement using microneurovascular muscle transfer from such muscles as the gracilis and latissimus dorsi. These muscles are typically grafted to the upper lip and oral commissure. In a large study by O'Brien and Morrison7* with 59 such transfers, 88% of patients have had return of function, and 48% have independent function of the two sides of the face. For all such rehabilitation strategies, reasonable expectation, for grafting too, is House-Brackmann grade 111-V.
CONCLUSION

The facial nerve has many functions, perhaps the most physically obvious of which are the conveyance of emotion, eye closure, and assistance with speech and mastication. Because it is frequently paralyzed relative to other cranial nerves, much attention is paid to it. Future areas of interest will be more detailed microbiologic study of the nerve and larger DNA PCR studies of patients with Bell's palsy, controlled studies of decompression for paralysis, better monitoring and care for the nerve during surgery of the temporal bone, more uniform classification and treatment of paralysis, better electrical and radiologic testing, and better surgical restoration of function. It is hoped that the causes of paralysis will become less controversial and that all medical practitioners will become more enlightened in their effective and rapid treatment.
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