The

Neurosurgical Atlas by Aaron Cohen-Gadol, M.D.

Hemifacial spasm
Last Updated: October 15, 2017

Hemifacial spasm (HFS) is a cranial nerve hyperactivity disorder

most likely caused by neurovascular conflict (compression) as one of
its underlying etiological phenomenon. It is typically characterized by
unilateral involuntary intermittent twitching of the muscles of the face.
The spasms usually start around the eye, involving the orbicularis
oculi muscle, and later spread to other muscles of the face that are
innervated by the facial nerve, including the platysma. The spasms
are bilateral in about 2% of the patients with this disorder.

Hemifacial spasm has an estimated prevalence of 11 cases per

100000 individuals and is twice as common in females as males.
Onset is mostly during the 4th and 5th decades of life. On average,
patients suffer from HFS for about 8 years before definitive treatment
is found. Familial clustering is rare.

Clinically, HFS presents as progressive, involuntary, irregular clonic

or tonic movements of the muscles innervated by the facial nerve.
The symptoms usually persist during sleep. Some patients complain
of a “ticking” sound on the affected side, which is caused by
contractions of the stapedius muscle.

Although HFS is not life threatening, patients may suffer severe

psychological stress because of cosmetic concerns, and their
binocular vision may be compromised by prolonged tonic spasms of
the orbicularis oculi. The symptoms are often exacerbated by
psychological stress and speaking.

Differentiating HFS from other movement disorders involving the face

can sometimes be challenging. Some common mimickers of HFS are
blepharospasm, facial nerve tics, and synkinesis after facial nerve
paralysis. A careful history and physical examination can greatly help
the clinician reach the correct diagnosis.

In most patients with HFS, the underlying cause is usually a

neurovascular conflict caused by an ectatic or aberrant vessel loop of
the posterior inferior cerebellar artery (PICA), the anterior inferior
cerebellar artery (AICA), or the vertebral artery (VA), causing
compression at the root exit zone of the facial nerve near the
brainstem. The root exit zone is particularly sensitive to compression
because the nerve is covered only by arachnoid in this location and
epineurium is absent. Also, no connective tissue septa traverse the
individual fascicles in this region that is the transition zone between
central and peripheral myelin.

Two theories have been proposed to explain the pathogenesis of

HFS. According to the peripheral hypothesis, ephaptic excitations
occur at the root exit zone altered by the offending vascular loop. In
contrast, the central hypothesis assumes that hyperexcitability of the
facial motor nucleus within the brainstem is the underlying cause. I
believe a combination of these two hypotheses most likely accounts
for the pathogenesis of HFS. Vascular compression is a contributory
and not necessarily the primary cause of this disorder.
Figure 1: One of Cushing’s patients (Circa 1920). Cushing is
triggering the spasms by pinching the right side of the patient’s
face.

Diagnosis and Evaluation

The clinical features at presentation are crucial for making the correct
diagnosis of HFS. No imaging or testing modality has been found to
reliably reach the diagnosis. Patients usually present with a fairly
long history of involuntary spasms of the facial muscles innervated by
CN VII, mostly originating in the periorbital region, involving the
orbicularis oculi, and spreading to other facial muscles as the
disease progresses.
They may complain of a “ticking” sound on the symptomatic side
caused by stapedius muscle contractions. Patients are generally
concerned about cosmesis, and some are significantly burdened by
visual impairment (caused by prolonged tonic spasms of the
orbicularis oculi) hindering their abilities to read and drive.

A detailed history and physical examination are important to reach

the correct diagnosis. The patient should be asked about the onset of
spasms, location, progression, exacerbating and relieving factors,
and previous chemodenervating treatments with botulinum toxin
injections. The patient should also be asked about a recent
occurrence of Bell’s palsy because postparalytic facial synkinesis is
an alternative diagnosis, although rare.

It is not unusual to find mild facial weakness caused by prior

botulinum toxin injections, facial neuropathy related to vascular
conflict, or facial muscle weakness caused by ongoing repetitive
spasms. Any additional findings on exam, such as hearing loss,
should raise concerns for an underlying structural lesion within the
cerebellopontine angle such as schwannoma, meningioma,
epidermoid tumor, or arachnoidal cyst. Certain disorders intrinsic to
the brainstem, such as gliomas, multiple sclerosis, and brainstem
stroke, can also give rise to similar symptoms and should be ruled
out.

Some movement disorders that can mimic hemifacial spasm include

blepharospasm (bilateral and synchronous symmetrical contractions
of the eyelids), oromandibular dystonia (involuntary and sustained
muscle spasms of the mouth and lower face), facial nerve tic
(complex, coordinated, multifocal movement patterns, and switches
between the right and the left sides of the face), hemimasticatory
spasm, focal seizures, and synkinesis after facial nerve paralysis
following acoustic neuroma surgery.
Before considering a microvascular decompression (MVD)
procedure, the patient should undergo a CT scan or preferably a
high-resolution dedicated posterior fossa MRI to rule out any
structural pathology. The MRI will determine the extent of the tumor
(epidermoid, meningioma, etc) and necessary preoperative
considerations to resect the tumor. A high resolution T2–weighted
sequence may display an aberrant vascular loop at the root exit zone
of the nerve.

Even if a high-resolution MRI does not identify an offending vascular

loop, I offer posterior fossa exploration to the patient if I am relatively
certain of the diagnosis. The MR sequences can also evaluate the
presence of a large tortuous vertebral artery that may not be safely or
effectively mobilized intraoperatively. In this situation, the surgeon
should discuss the lower rate of spasm freedom after surgery with the
patient.

Figure 2: Axial T2-weighted MRI demonstrates a vascular loop

(arrow) around the root exit zone of CN VII.


Figure 3: The only presentation of this large left-sided petroclival

epidermoid tumor (note the CP angle extension around the facial
nerve *) was hemifacial spasms. This tumor was resected
through staged retrosigmoid and pterional craniotomies.

Medical Therapy
The drugs used to treat hemifacial spasm include carbamazepine,
clonazepam, baclofen, and gabapentin, but success rates with these
medications have been disappointing. The adverse effects of these
medications notably affect some patients: fatigue, exhaustion, and
poor performance. Botulinum toxin (Botox) targeted chemical
denervation injections may help reduce spasms, but this is merely
symptomatic treatment.

Since these injections do not treat the cause of the problem, spasms
gradually return at the end of each 3 to 6-month Botox cycle,
necessitating repeat treatment. Botox injections may also injure some
of the motor nerve terminals and partially account for some residual
facial weakness after MVD surgery, despite successful relief of
spams.
Most importantly, the gradual return of spasms at the end of each
Botox cycle often leads the patient to seek a more lasting definitive
treatment. Microvascular decompression surgery has a reported
lasting success rate of 80-90% in experienced hands and
appropriately selected patients, and is the only durable therapeutic
option.

Indications for Surgery

Most patients elect to proceed with surgery because of the disfiguring
nature of HFS spasms and a desire for an improved cosmetic
outcome. Although Botox injections can provide considerable albeit
short-lasting relief, the need to undergo these injections every 3 to 6
months and the cosmetic deformity associated with HFS and Botox
injections are the main reasons to consider MVD surgery.

Many surgeons prefer to see the patient in his or her “off” period to
assess the validity of the spasms before offering surgery, but this has
not been the routine in my practice. Patients suffering from HFS are
generally reliable and know much about their disorder. They
frequently have videotaped their spasms during their “off” period.

Since HFS is usually not a disabling disease, surgical intervention

must be conducted with very minimal risk to justify the risk-to-benefit
ratio. As with MVD for trigeminal neuralgia, the operator’s experience
is especially important in achieving a favorable and safe outcome.

The patient’s history and severity of spasms are very important

because spasms are sometimes self-limiting and resolve
spontaneously. For this reason, I offer MVD surgery only if the patient
has experienced severe HFS symptoms for longer than 1-2 years.

Contralateral hearing loss is also a contraindication to MVD surgery

for HFS. Functional hearing loss is a real complication from this
procedure (<5%) and should be candidly discussed with patients
preoperatively.

Preoperative Considerations
I advocate using brainstem auditory evoked response (BAER)
monitoring during MVD to treat HFS (in contrast with MVD for
trigeminal neuralgia, when I do not use BAER monitoring). Latency of
Peak V is considered the best electrophysiologic indicator for
signaling cochlear nerve damage. I also look for intraoperative
disappearance of lateral spread reflex (LSR), a measure of
hyperactivity of the facial nerve/nucleus. This reflex is produced by
electrical stimulation of the temporal or zygomatic branch of the facial
nerve, which leads to a response recorded from the mentalis muscle.

The disappearance of LSR after surgery informs the surgeon that the
appropriate pathology was adequately discovered and addressed.
However, while the disappearance of LSR is reassuring, its
persistence is not inconsistent with complete postoperative relief of
spasms as long as a convincing offensive vascular loop was
identified intraoperatively and handled appropriately.

Operative Anatomy
Figure 4: Exposure of the cranial nerves in the cerebellopontine
angle after a right-sided retrosigmoid approach. Note the
relationship of the AICA and PICA to the CN VII/VIII complex (A).
The facial nerve is located anterior to the superior vestibular
nerve. The AICA or its labyrinthine branch transecting the CN
VII/VIII complex are often not the main offending vessels and
must be carefully handled during surgery to avoid hearing loss
(B)(Images courtesy of AL Rhoton, Jr).
Figure 5: An enlarged view of the left cerebellopontine angle and
its contents when viewed from a retrosigmoid approach. Note
the relationship of AICA to CN VIII (A). The subarcuate artery
must be preserved during operative maneuvers. The
vestibulocochlear nerve and flocculus have been elevated to
expose the junction of the facial nerve at the brainstem (B).
It is important to remember that the retrosigmoid approach
exposes the root exit zone of the facial nerve at the brainstem
below the root entry zone of the vestibulocochlear
nerve (Images courtesy of AL Rhoton, Jr).

MVD for HEMIFACIAL SPASM

I routinely use the infraflocculus route through a small retromastoid
craniotomy to reach the root exit zone of the facial nerve. The
general technical nuances for an extended retromastoid craniotomy
are presented in the Cranial Approaches Volume. In the following
section, I review the specific steps of exposure during MVD for HFS.
Figure 6: Modified reverse “U” skin incision whose summit is
marked 1cm below the presumed junction of the transverse and
sigmoid sinuses. Note the position of the head in the skull
clamp.
Figure 7: The operative corridors and trajectories for accessing
the cerebellopontine angle: The surgical corridors for
microvascular decompression for trigeminal neuralgia
(supralateral cerebellar approach-blue arrow) and hemifacial
spasm, and glossopharyngeal neuralgia (infralateral cerebellar
or infrafloccular approach-green arrow) are illustrated.

Mobilization of the cerebellum in a purely medial direction must

be avoided since this vector of retraction will be parallel to the
sensitive CN VIII, increasing the risk of hearing loss. I use the
infralateral cerebellar corridor through the infraflocculus route to
access the root exit zone of CN VII.
Figure 8: Burr hole and craniotomy (HS: hemifacial spasm, GN:
glossopharyngeal neuralgia). A single burr hole is created at the
edge of the sigmoid sinus and 1 centimeter below the transverse
sinus. The size of the craniotomy or craniectomy is often small,
about 1.0-1.5 times the size of a quarter coin.

A craniotomy should be avoided in older patients with adherent

venous dural surface. The last osteotomy (2) is near the sigmoid
sinus. The bone over the sigmoid sinus is removed during the
next step using an air drill. For additional nuances, please refer
to the extended retromastoid craniotomy chapter.
Figure 9: The method for dural opening (top image). A magnified
operative photo also demonstrates the extent of craniotomy and
dural opening (bottom image). There is no need to expose the
transverse sinus. Unlike the linear skin incision, the curvilinear
incision mobilizes the retracted myocutaneous flap inferiorly
and prevents it from increasing the operator’s working distance.

INTRADURAL PROCEDURE
Figure 10: Supramedial cerebellar retraction: A piece of glove
(cut slightly larger than the cottonoid patty) acts as a rubber
dam. It protects the cerebellar hemisphere against the rough
surface of the cottonoid as the rubber dam slides over the
cerebellum while dissection is continued to expose the
cerebellopontine angle (top image). I identify the junction of the
petrous bone and the floor of posterior fossa (P, bottom
intraoperative image) and advance the cottonoid over the rubber
dam near the turn of the petrous bone toward the lower cranial
nerves.

Medial retraction of the cerebellum parallel to CN VII/VIII is avoided

to prevent direct transmission of retraction to these nerves. The
vector of retraction is parallel to CN IX. Note that I do not apply
fixed retractors, but instead use the suction apparatus to mobilize the
cerebellar hemisphere in a dynamic fashion during dissection. Along
with generous opening of the regional arachnoid membranes over
the cranial nerves, this maneuver minimizes the risk of hearing loss.
Dynamic retraction of the suction apparatus allows intermittent
exposure only where needed. Aggressive retraction of fixed retractors
often provides exposure at places that may not be necessary.

Figure 11: I sharply dissect the arachnoid membranes over the

lower cranial nerves and identify CN IX. Supramedial cerebellar
mobilization allows me to follow the path of this nerve to the exit
zone of CN VII at the level of the brainstem. Intraoperative BAER
monitoring guides the surgeon to adjust retraction to minimize
undue traction on CN VIII.

I continue irrigating the field periodically during intradural dissection

because the intense light of the microscope can cause heat injury to
CN VIII. I also cover the surface of CN VIII with a small piece of
papaverine-soaked gelfoam to relieve vasospasm. If the BAERs
change at anytime during the intradual procedure, I perform the
following steps:

1. Stop dissection and relieve all retraction while irrigating the

operative field.
2. Allow a few minutes for the BAERs to return to normal. It may
be necessary to increase the blood pressure. Before
reapplying dynamic retraction, I further dissect the arachnoid
membranes over CN VII/VIII to relieve any traction on these
nerves while mobilizing the medial cerebellum. I also cover
these nerves with a small piece of papaverine-soaked sponge
to relieve any vasospasm caused by traction and the heat of the
microscope.
3. I then attempt more superiorly directed retraction and use the
infrafloccular corridor while minimizing any traction parallel to
the CN VII/VIII complex.
Figure 12: The arachnoid membranes over the CN VII/VIII
complex are split sharply, close to the brainstem, to prevent
undue traction on these nerves during mobilization of the
flocculus.


Figure 13: Microscissors are used to sharply open the arachnoid

layers between the CN VII/VIII complex and
cerebellum/flocculus.
Figure 14: In rare circumstances, I coagulate and shrink a small
portion of the cerebellar flocculus overlying the root exit zone of
CN VII to facilitate exposure of the root exit zone while
minimizing the required force of retraction.
Figure 15: Neurovascular conflict leads to gray discoloration of
the root exit zone of CN VII. This root exit zone is anterior and
slightly inferior to the root entry zone of CN VIII (more whitish in
color) and may be directly seen upon gentle elevation of CN VIII
using a fine dissector. The offending vessel (most commonly the
posterior or anterior inferior cerebellar artery) is hidden in the
axilla of CN VII and may be mobilized and padded away from the
nerve with pieces of shredded Teflon.

The perforators originating from the compressing vessel should

be protected during this mobilization. Note that the site of the
neurovascular conflict is along the root exit zone of CN VII near
the brainstem. This area must be thoroughly examined using the
following techniques: Wide opening of the arachnoid
membranes and strategic use of dynamic retraction using
suction apparatus at the appropriate operative angles.

Figure 16: An intraoperative photo (top image) demonstrates the

area of discoloration (black arrow) at the root exit zone of the left
facial nerve caused by the adjacent vascular loop. The lower
image shows the final result after mobilization of the offending
artery and implantation of Teflon. Please note the piece of
papaverine-soaked gelfoam (yellow arrow) covering CN VIII. This
piece of gelfoam relieves vasospasm of intraneural vessels and
protects the nerve from the intense heat of the microscope.

Figure 17: I believe that the offending vessel’s entire length

along the root exit zone of CN VII and the area over the
brainstem should be padded with Teflon. The lateral spread
reflex, present in most patients, may disappear upon
mobilization of the artery. Although this test is not an absolute,
the disappearance of the reflex is reassuring that the pathologic
entity has been found and managed appropriately.

I place the shredded Teflon implant piecemeal between the

artery and brainstem. An intact (unshredded) Teflon patch may
be easily dislodged postoperatively, so its use is discouraged.
These pieces of prosthesis are pushed superiorly and inferiorly
(inset) to mobilize the artery along its entire length and away
from the brainstem.

Figure 18: The implant has mobilized the artery along its entire
length away from the root exit zone and the brainstem. It is
important to remember that the presumed site of neurovascular
conflict is at the root exit zone of the nerve near the brainstem
and not only along the nerve’s cisternal segment.

Inadequate exposure of the root exit zone is the primary reason

for inadequate decompression. The offending vessel(s) may be
hidden deep in the arachnoid membranes or at the depth of the
cleft formed by the cerebellum and brainstem (cerebellopontine
fissure).

The surgeon’s lack of experience or fear of excessive retraction (due

to limited familiarity with the use of dynamic retraction) does not
justify inadequate exposure of the deeply located CN VII root exit
zone and ultimately inadequate arterial mobilization. More often than
realized, the surgeon mistakenly mobilizes the vessel along the
nerve instead of along its hard-to-reach root exit zone and brainstem,
leading to suboptimal operative outcome.

Closure
Figure 19: The dura is approximated primarily. I do not persist on
performing a watertight dural closure and have experienced a
very low rate of cerebrospinal fluid leak through the incision or
the nose. Mastoid air cells are rewaxed thoroughly (“wax in, wax
out”) and the bone flap is replaced or a methyl
methacrylate cranioplasty is performed. The muscle and scalp
are closed in anatomic layers.

Repeat MVD for Hemifacial Spasm

Factors that contribute to the failure of the first surgery include
restricted and inadequate retromastoid craniectomies that did not
extend laterally to the level of the sigmoid sinus or inferiorly to the
level of the posterior fossa floor.

In addition, although the cisternal portion of the facial nerve should

be inspected to relieve any neurovascular conflict there, meticulous
examination of the root exit zone near the brainstem is mandatory in
every case. I have operated on a number of patients who underwent
an unsuccessful attempt elsewhere and I have routinely found a
convincing offending vessel near the facial nerve root exit zone. In
these cases, the initial surgeon presumably only handled the conflict
along the brainstem.

Postoperative Considerations
Patients are usually admitted to the neuro intensive care unit for
overnight observation and then transferred to the regular ward for a
couple of days before they can be discharged home. Special
attention should be paid to hemodynamic parameters, the neurologic
examination, and wound care. Steroids are administered to prevent
aseptic meningitis and minimize postoperative nausea and
headaches.

Delayed resolution of spasms is not uncommon, and patients should

be assured of this if a convincing arterial loop was identified
intraoperatively. We do not routinely perform a head CT scan
postoperatively. Occasionally, delayed facial palsy may occur after
MVD for HFS. This palsy is temporary and responds well to a
dexamathasone taper of 1 week in duration.

Pearls and Pitfalls

Hemifacial spasm is an uncommon cranial nerve hyperactivity
disorder characterized by unilateral, involuntary, intermittent
twitching of the facial muscles. Spasms typically start around
the eye, involving the orbicularis oculi muscle, followed by
 involvement of other muscles of the face that are innervated by
the facial nerve, including the platysma.
Differential diagnosis of HFS, particularly postparalytic facial
synkinesis, facial tics, and blepharospasm, should be kept in
mind and ruled out with the aid of an extensive history and a
detailed neurologic examination.
The cerebellum should be retracted only parallel to the direction
of CN IX to avoid direct traction on CN VIII. Sharp arachnoid
dissection and strategic dynamic cerebellar retraction will
facilitate cerebellar mobilization without placing the cranial
nerves at risk. These maneuvers allow adequate exposure to
permit safe mobilization of the offending vascular loop and
Teflon implantation.
While mobilizing the offending vessel, it is important that the
vessel be moved away from the brainstem at the root exit zone
and padded with shredded Teflon; failure to do so will lead to
suboptimal results.

Contributor: Aqueel Pabaney, MD

DOI: https://doi.org/10.18791/nsatlas.v6.ch02

References
Barker FG 2nd, Jannetta PJ, Bissonette DJ, Shields PT, Larkins MV,
Jho HD. Microvascular decompression for hemifacial spasm. J
Neurosurg. 1995;82:201-210.

Cohen-Gadol AA. Microvascular decompression surgery for

trigeminal neuralgia and hemifacial spasm: Naunces of the
technique based on experiences with 100 patients and review
of the literature. Clin Neurol Neurosurg. 2011;113:844-853.

McLaughlin MR, Jannetta PJ, Clyde BL, Subach BR, Comey CH,
 Resnick DK. Microvascular decompression of cranial nerves:
lessons learned after 4400 operations. J Neurosurg.
1999;90:1-8.

Polo G, Fischer C, Sindou MP, Marneffe V. Brainstem auditory

evoked potential monitoring during microvascular
decompression for hemifacial spasm: intraoperative brainstem
auditory evoked potential changes and warning values to
prevent hearing loss--prospective study in a consecutive series
of 84 patients. Neurosurgery. 2004;54:97-104.

Rhoton AL Jr. The cerebellopontine angle and posterior fossa cranial

nerves by the retrosigmoid approach. Neurosurgery.
2000;47:S93-129.

Rosenstengel C, Matthes M, Baldauf J, Fleck S, Schroeder H.

Hemifacial spasm—conservative and surgical treatment
options. Dtsch Arztebl Int. 2012;109: 667−673.

Sindou MP. Microvascular decompression for primary hemifacial

spasm. Importance of intraoperative neurophysiological
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