Clinical Disorders of the Facial

Nerve
Book Chapter

Douglas E. Mattox
and Esther X. Vivas

Cummings Otolaryngology: Head and Neck Surgery, 172, 2587-

2597.e4

Key Points
•

The term B e ll palsy should be reserved for abrupt-onset idiopathic

facial paralysis without any other identifiable cause.

Progressive facial paralysis is not Bell palsy and should prompt imaging
studies to investigate neoplasm as the etiology of the paralysis.

The etiology of Bell palsy is reactivation of herpes simplex virus.

The mainstay of treatment for Bell palsy is corticosteroids, although

studies conflict concerning the value of adding antivirals.
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 •

Ramsay Hunt syndrome (herpes zoster oticus) is caused by varicella

zoster virus.

Bell palsy is common during pregnancy and is managed in the same

way as any other facial palsy but in collaboration with the patient's
obstetrician.

Bilateral facial paralysis suggests the possibility of metabolic,

autoimmune, or infectious disease, including Lyme disease.

When facial paralysis occurs in the setting of chronic otitis media

(suppurative or cholesteatoma), the first line of treatment is to resolve
the ear disease; subsequent recovery of the facial paralysis is the usual
outcome in this setting.

This chapter describes clinical disorders of the facial nerve. Most of the
discussion is devoted to idiopathic paralysis (Bell palsy); however,
disorders of the facial nerve associated with other diseases are also
presented. The usefulness of electrodiagnostic testing and medical and
surgical management of facial palsy are discussed.

Bell Palsy: Spontaneous Idiopathic Facial

Paralysis
The term Bell palsy should be reserved for cases of facial paralysis that
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 have signs and symptoms consistent with the disease and in which a
diligent search for another cause is negative. Facial paralysis can have
multiple etiologies, including cutaneous or metastatic malignancy, facial
neuroma, intracranial tumor, infection, or neurologic problems ( Fig.
172.1 ). These disorders require different management than Bell palsy.
The dictum that “all that palsies are not Bell” cannot be
overemphasized.

Fig. 172.1

Management algorithm for facial paralysis.

CT , Computed tomography; degen , degeneration; ENoG , electroneuronography; Gd ,
gadolinium enhanced; MRI , magnetic resonance imaging.

Although older literature relegated Bell palsy to a diagnosis of

exclusion, May and others emphasized the diagnosis on the basis of
specific clinical features. Taverner outlined the minimum diagnostic
criteria for Bell palsy: (1) paralysis or paresis of all muscle groups of one
side of the face; (2) sudden onset; (3) absence of signs of central
nervous system (CNS) disease; and (4) absence of signs of ear or
cerebellopontine angle disease. The differential diagnosis of facial
palsy is shown in Table 172.1 .

TABLE 172.1

Differential Diagnosis of Facial Paralysis

Modified from Adour KK, Hilsinger RL Jr, Callan EJ: Facial paralysis and
Bell's palsy: a protocol for differential diagnosis, Am J Otol
Nov(Suppl):68, 1985.

Chronic or Progressive
Acute Paralysis
Paralysis
:
 Polyneuritis

Bell palsy

Herpes zoster

Guillain-Barré syndrome

Autoimmune disease

Lyme disease

HIV infection
Malignancies
•
•
Kawasaki disease
Primary parotid tumor
Trauma
•
•
Metastatic tumor
Temporal bone fracture
Benign tumors
•
•
Barotrauma
Schwannoma
•
•
Birth trauma
:
 Birth trauma
Glomus tumor
Otitis media
Cholesteatoma
•

Acute bacterial

Chronic bacterial

Cholesteatoma

Sarcoidosis

Melkersson-Rosenthal syndrome

Neurologic disorders

HIV infection

Cerebrovascular disorders, central or

peripheral

HIV , Human immunodeficiency virus.

Incidence
The annual estimated incidence of Bell palsy is 23 to 37 per 100,000
population. The incidence is greater in patients older than 65 years (59
of 100,000) and lower in children younger than 13 years (13 of
100,000). A primary care database study out of the United Kingdom
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 suggested a peak in people over age 70. The male/female ratio for Bell
palsy is approximately equal except for a predominance in women
younger than 20 years and a slight predominance in men older than 40
years. The left and right sides of the face are equally involved. Around
30% of patients have incomplete paralysis on presentation and 70%
have a complete paralysis. Bilateral paralysis occurs in 0.3% of
patients, and 9% have a history of previous paralysis. A family history
of Bell palsy exists in 8% of patients.

Etiology
Proposed causes of Bell palsy include microcirculatory failure of the
vasa nervorum, viral infection, ischemic neuropathy, and autoimmune
reactions. Of these, the viral hypothesis has been the most widely
accepted, although no virus has ever been consistently isolated from
the serum of patients with Bell palsy. Thus the evidence for the viral
hypothesis is indirect and relies on clinical observations and changes in
viral antibody titers. Furthermore, although the underlying cause may
be viral, the immediate cause of the paralysis itself is debated; it may
be viral neuropathy alone or ischemic neuropathy secondary to viral
infection.

Acute facial paralysis can occur as a part of many viral illnesses

including mumps, rubella, herpes simplex virus (HSV), and Epstein-Barr
virus. Bell palsy is often a part of a cranial nerve polyneuritis, of which
facial paralysis the most obvious finding, supporting a viral cause for
Bell palsy. A careful neurologic examination will reveal other cranial
weaknesses in more than half of patients with Bell palsy. Adour found
several affected cranial nerves in most patients ( Table 172.2 ).

TABLE 172.2
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 Polyneuropathy in Bell Palsy

From Adour KK: Current concepts in neurology: diagnosis and

management of facial paralysis, N Engl J Med 307:348, 1982.

Incidence
Symptom
(%)
Hypesthesia or dysesthesia of the glossopharyngeal or
80
trigeminal nerve
Hypesthesia of C2 20
Vagal motor weakness 20
Trigeminal motor weakness 3
Facial or retroauricular pain 60
Dysgeusia 57
Hyperacusis 30
Decreased tearing 17

Although a direct link between Bell palsy and viral infection has been
difficult to establish, the best current evidence suggests that
reactivation of latent HSV is the most likely cause. Serologic studies
have examined both seroconversion and prevalence of antigens to
suspected etiologic agents in patients with Bell palsy, especially HSV
and varicella zoster virus (VZV). The results have been conflicting and
have been dependent on the techniques used and the populations
studied. One reason for failure of these studies is that they sought
signs of an acute infection immediately preceding the onset of the Bell
palsy. However, considering that there is no seasonal predilection or
epidemic clustering, it is much more likely that Bell palsy is caused by
reactivation of a latent virus rather than by direct communicable viral
infection. Morgan and colleagues and Vahlne and associates support
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 the hypothesis that Bell palsy is a reactivation of VZV that may be
initiated by several stresses, such as from heterotrophic viruses
(viruses other than herpesviruses) or physical or metabolic stressors.

The best evidence for reactivation of HSV as the mechanism for Bell
palsy comes from several studies of the geniculate ganglion and facial
nerve. Furuta and others and Takasu and others demonstrated HSV-1
DNA in both the trigeminal and geniculate ganglia of unselected
autopsy specimens. Burgess and others reported a single case of a
patient who died shortly after Bell palsy developed and in whom HSV-1
genomic DNA was identified in the geniculate ganglion of the facial
nerve; archival control tissue was negative for viral DNA. In the most
important study so far, Murakami and others looked at endoneurial fluid
and postauricular muscle from patients with Bell palsy, Ramsay Hunt
syndrome, and controls. The polymerase chain reaction was used to
test for HSV-1 DNA and VZV DNA, and the results were confirmed with
Southern blot analysis. HSV DNA was detected in 11 of 14 patients with
Bell palsy, whereas VZV DNA was not recovered from any. Conversely,
VZV DNA was recovered from all the patients with Ramsay Hunt
syndrome, whereas none had HSV-1 DNA. Unaffected controls did not
have DNA for either virus. This study suggests a clean distinction
between Bell palsy and Ramsay Hunt syndrome; however, other studies
suggest more overlap.

Furuta and coworkers studied 142 patients with both clinical Ramsay
Hunt syndrome and acute facial palsy; of these, 13 were diagnosed as
having Ramsay Hunt syndrome on the first visit, and 8 more had
vesicular lesions develop subsequent to their initial evaluation. Of the
121 with nonvesicular acute facial paralysis, 35 had VZV DNA detected
in their saliva or had serologic evidence of VZV reactivation. Yamakawa
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 and colleagues demonstrated how technique sensitive these studies
are. Using two different polymerase chain reaction techniques, they
found high but differing levels of VZV DNA in patients with Ramsay
Hunt syndrome.

Adding to the confusion about the distinction between Bell palsy and
Ramsay Hunt syndrome is the increasing recognition of zoster sine
herpete (ZSH, zoster without vesicles) among the Bell palsy population.
Lee and colleagues emphasized the clinical distinction between the
two. The diagnosis of ZSH was based on subjects having deep
muscular pain (sclerotomal) and/or superficial prickling pain
(dermatomal) at the time of presentation. Lee and colleagues studied
patients diagnosed with Bell palsy, ZSH, and typical Ramsay Hunt
syndrome and evaluated normal unaffected controls for anti-HSV and
anti-VZV immunoglobulin G (IgG) and IgM. Anti-VZV IgG and anti-HSV
IgG were present among all groups, including controls; however, anti-
VZV at levels four times normal was present in the zoster and the ZSH
patients but not in the Bell palsy or control groups. Further supporting
the clinical diagnoses of ZSH, these patients were randomized to those
treated with prednisone alone and those treated with prednisone and
antiviral medication. The latter group had better recovery than those
treated with prednisone alone.

Although it is likely that the underlying disease in Bell palsy is viral

polyneuropathy, the question remains as to why there is such a
profound effect on the facial nerve, whereas the changes in the other
cranial nerves are relatively minor and transient. The major anatomic
difference between the facial nerve and other cranial nerves is its long
bony canal. Fisch measured the diameter of the fallopian canal
throughout the temporal bone and found that the narrowest point was
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 at the junction of the internal auditory canal and the labyrinthine
portion, which he called the meatal foramen . This foramen averaged
0.68 mm in diameter, whereas the remainder of the fallopian canal
measured 1.02 to 1.53 mm. Fisch reasoned that edema in this narrow
segment of the fallopian canal could cause damming of the axoplasmic
flow within the facial nerve. This site of lesion in Bell palsy has been
confirmed by clinical observation and by intraoperative neuronography.
A similar site of lesion has been identified electrophysiologically in
herpes zoster oticus.

Histopathology
Fowler reported autopsy findings in a patient who died shortly after Bell
palsy developed. The entire intratemporal nerve contained dilated and
engorged veins and venules. Fresh hemorrhage was found within the
internal auditory canal surrounding the facial nerve and extended as far
as the geniculate ganglion. Fowler theorized that the ischemia resulted
from microthrombi rather than vasospasm.

Reddy and others examined a facial nerve 17 days after the onset of
spontaneous paralysis. The nerve showed scattered degeneration of
the myelin sheaths and axons; 10% to 30% of facial nerve fibers were
surrounded by phagocytic cells, and the perivascular areas also
showed inflammatory reaction and hemorrhage.

Proctor and others examined a patient who died from Bell palsy 13 days
after the onset of paralysis. The facial nerve showed lymphocytic
infiltration and phagocytosis of myelin by macrophages throughout the
infratemporal course of the nerve. Initially these observations were
interpreted as evidence of a viral cause. However, when McKeever and
others reexamined the case, they emphasized that the inflammatory
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 cells were most prominent where the facial nerve was surrounded by
bone, especially in the labyrinthine portion of the fallopian canal, but
not within the internal auditory canal. They theorized that the
histopathology suggested a compression-type injury with no evidence
of vascular occlusion.

O'Donoghue and Michaels found degeneration of myelin sheaths and

axon fibers throughout the intratemporal course of the facial nerve. The
nerve was constricted at the meatal foramen, and an osteoclastic giant
cell reaction caused resorption of bone around the geniculate ganglion.
These authors interpreted their findings as consistent with a viral
cause, but—as pointed out by Jenkins and others —these findings do
not preclude edema and constriction of the facial nerve as the final
event leading to the paralysis.

Podvinec found inflammatory infiltrates in the intratemporal portion of

the facial nerve 6 months after the onset of the palsy; he also found
signs of wallerian degeneration and regeneration. He suggested that
the persistence of infiltrates for this extended time might result from
compromised circulation within the fallopian canal. Jackson and others
reported the histopathologic findings from the labyrinthine segment of
the facial nerve in a patient 1 year after the onset of total facial paralysis
secondary to herpes zoster infection. A portion of the nerve within the
auditory canal was essentially healthy; however, at the meatal foramen,
the nerve became a mixed fibrotic and necrotic acellular mass.

Michaels described the histopathologic findings from an autopsy

performed 11 days after the onset of Bell palsy. Similar to other authors,
he found demyelination, compression, bone resorption, and
lymphocytic infiltration in the proximal labyrinthine segment of the
facial nerve. He cautioned against overinterpretation of bulging of the
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 nerve in the distal internal auditory canal because it was found in both
the healthy and pathologic sides.

Another approach to the histologic evaluation of Bell palsy is biopsy

specimens obtained intraoperatively, including biopsies of the chorda
tympani and greater petrosal nerve. Biopsy specimens of the chorda
tympani in acute Bell palsy have shown degeneration of myelinated
fibers but no inflammatory response. Fisch and Felix examined biopsy
specimens of the greater petrosal nerve obtained during
decompression of the middle cranial fossa facial nerve. Their findings
included degeneration and demyelination of large axons and
lymphocytic infiltration. They found these results consistent with
wallerian degeneration starting proximal to the geniculate ganglion,
probably from the meatal foramen.

In summary, most histologic studies show diffuse demyelination of the

facial nerve throughout its intratemporal course, with the most severe
findings in the labyrinthine segment and at the meatal foramen.

Central Nervous System Changes

Auditory symptoms, usually in the form of hyperacusis, may be present
in up to 30% of patients with Bell palsy. The cause of these auditory
symptoms is unknown. McCandless and Schumacher did not find
evidence of a lesion affecting the cochlear nerve in patients with facial
paralysis. Many authors have attributed the hyperacusis to decreased
damping of sound secondary to dysfunction of the stapedius muscle.
However, most authors have not found reduced contralateral stapedius
reflex thresholds, which suggests that an absence of stapedial
damping is not the cause.
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 On the basis of these observations, many authors have concluded that
the auditory dysfunction results from CNS involvement. A few patients
with Bell palsy have abnormalities of the auditory brainstem response
as compared with age-matched controls. These abnormalities include
an increase in the wave I-to-V interval and the interaural difference for
wave V. These changes are usually present bilaterally and resolve with
recovery of the facial paralysis. The findings also suggest a brainstem
abnormality associated with Bell palsy, which these authors
hypothesized is related to the reactivation of HSV. This hypothesis
should be considered with caution, as other authors did not confirm
such brainstem auditory findings, and the findings have not been
corroborated by somatosensory or visual evoked potentials.

Several investigators have looked for CNS changes in acute facial

paralysis by studying the cerebrospinal fluid (CSF). Again, the results
are contradictory. The finding of elevated levels of myelin basic
proteins and pleocytosis in the CSF support CNS involvement in Bell
palsy. Weber and others found a normal total cell count, total protein
concentration, blood-CSF permeability, and CSF/serum
immunoglobulin ratios in most patients with Bell palsy. Only
approximately 10% of these patients had some pathologic feature of
the CSF, including a mild increase in blood-CSF permeability or
pleocytosis. Weber and others concluded that their findings did not
support the hypothesis that Bell palsy was part of a cranial
polyneuropathy.

CNS changes in Bell palsy have also been suggested by MRI. Jonsson
and others found brain or brainstem changes in 5 of 19 patients with
Bell palsy. These areas of increased signal did not correlate with the
facial nerve brainstem nucleus or the supranuclear pathways and were
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 interpreted as indicative of unrelated vascular disease.

Electrophysiology and Testing

The multiple branches of the facial nerve—including the greater
petrosal nerve, stapedial nerve, chorda tympani, and multiple muscular
branches—led to the development of topodiagnostic testing to localize
the site of a facial nerve lesion. The Schirmer test evaluates greater
petrosal nerve function by measuring the lacrimal secretions that
accumulate on a piece of filter paper placed under the eyelid at the
medial canthus. Stapedial nerve function can be evaluated with
stapedial reflex testing. The chorda tympani nerve can be evaluated
with taste testing or with the submandibular salivary flow test
described by Magielski and Blatt. Unfortunately the accuracy of
localization with topodiagnostic testing has been disappointing. This is
not a surprising finding in Bell palsy, in which the lesion is a diffuse
demyelination throughout the nerve. However, topodiagnostic testing
has also been disappointing in tumors in which a sharply defined site of
lesion was expected.

Electrodiagnostic tests on the motor branches of the facial nerve have

also been used to assess function and predict outcome. All of these
tests have a similar shortcoming: stimulation and recording are
performed distal to the lesion rather than on opposite sides of the site
of injury. Therefore the clinician should wait until nerve degeneration
has reached the site of stimulation; it usually takes 4 to 5 days before
electrodiagnostic tests become abnormal.

Nerve Excitability Threshold

The use of electricity as a neurodiagnostic test was described by
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 Duchenne in 1892 and was adapted to the facial nerve by Laumans and
Jongkees. The Hilger nerve stimulator is the method of facial nerve
evaluation most commonly used by otolaryngologists. The
extratemporal portion of the nerve is stimulated with a small pulsed DC
current. The face is observed for the lowest current to produce a visible
twitch. Although a threshold difference of more than 3.5 mA between
the two sides has been considered suggestive of nerve degeneration,
Gates found that if testing was carefully performed, low thresholds
were obtained with percutaneous stimulation. The thresholds increased
slowly with age and body weight, but a nerve excitability threshold of
greater than 1.25 mA in the upper division and 2 mA in the lower division
was statistically abnormal.

Maximal Stimulation Test

A modification of the minimal excitability test, the maximal stimulation
test attempted to determine the difference between the strength and
amount of contraction of the facial musculature caused by a
supramaximal electrical stimulus. However, the maximal stimulation
test is difficult to quantitate and is more subject to interobserver
variation than is the minimal stimulation test.

Electroneurography
Electroneurography, or electroneuronography (ENoG), adds recording
of the facial muscle action potential with surface or needle electrodes
to the stimulation tests. Esslen introduced the use of bipolar surface
electrodes for stimulation and the recording of responses. The two
electrodes are moved independently to produce the maximum
amplitude of response, which is evaluated by comparing the peak-to-
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 peak amplitude of the maximum response obtained for the two sides of
the face.

Electromyography
Electromyography (EMG) measures muscle action potentials generated
by spontaneous and voluntary activity. It is distinct from other
electrodiagnostic tests, in which the activity is generated by active
stimulation of the nerve. Denervation potentials are seen 10 or more
days after the onset of the palsy; therefore they are of limited value in
determining early prognosis of facial paralysis; however, the loss of
voluntary motor units within the first 3 to 4 days of paralysis suggests a
poor prognosis. Conversely, retention of voluntary motor activity past
the seventh day suggests that complete degeneration will not occur.
Sittel and Stennert found that the detection of spontaneous fibrillation
by needle EMG had an 80% accuracy in predicting a poor outcome 10
to 14 days after onset of paralysis. Unfortunately this is too late to be
useful in surgical decision making.

Nerve Conduction Velocity

Nerve conduction velocity can be measured between the stylomastoid
foramen and the mandibular branch of the facial nerve. There is a
strong correlation between a decrease in nerve conduction velocity and
a decrease in the compound nerve action potential measured by ENoG
during the first 2 weeks after the onset of paralysis. Normal facial nerve
conduction velocity ranges between 37 and 58 m/s, and conduction
velocities in this range uniformly have good outcomes. Conduction
velocities between 20 and 30 m/s have a 50% chance of significant
residual paresis or synkinesis, and those less than 10 m/s had poor
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 outcomes.

Interpretation of Electrical Tests

All electrical stimulation tests have the same fundamental weakness—
that is, inferences are made on the basis of tests on the nerve distal to
the site of injury within the temporal bone. Therefore a delay is inherent
between the onset of the injury and the development of sufficient
degeneration of the distal segment of the nerve to be discovered by
the tests. Only intraoperative monitoring with stimulation proximal to
the lesion provides direct information about the state of the nerve at
the site of the lesion. New experimental techniques that include
antidromic stimulation and magnetic stimulation hold some promise for
circumventing this limitation.

Excellent recovery of facial function always occurs when the decline in

the compound action potential (CAP) as measured by ENoG does not
reach 90%, and one half of patients who do reach this level of
degeneration also have excellent outcomes. Ryu and others
investigated prognostic factors associated with Bell palsy and Ramsay
Hunt syndrome and performed ENoG 4 days after the onset of
paralysis and found that responses less than 10% of those of the
contralateral side predicted a poorer response in both groups
regardless of treatment. The problem is how to identify patients who
will not have good recovery, and at least a partial answer lies in the
combination of ENoG with standard EMG. Patients who maintain
recordable voluntary motor potentials despite a severe depression of
the CAP have excellent prognoses. This may be explained by severe
desynchronization of the motor units, which causes a reduced or
absent CAP.
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 Electromagnetic Stimulation of the Facial Nerve
Facial nerve testing with electromagnetic stimulation has also been
explored. Benecke and others could stimulate the nerve with cortical
(supranuclear) electromagnetic discharges, demonstrating that the
nerve could be stimulated transsynaptically. However, it is not yet clear
whether magnetic stimulation will be a useful predictive test because
there are significant discrepancies between the response to magnetic
stimulation and clinical findings. Nowak and colleagues tested 65
patients with facial palsy with both transcranial magnetic stimulation
and electrical stimulation of the facial nerve. A severe reduction in
muscle action potential response in both Bell palsy and herpes zoster
infection did not correlate with the clinical grade of the palsy. A similar
discrepancy between clinical grade of the palsy and evoked action
potentials was reported by Hur and colleagues. Based on current
clinical information, electrical stimulation seems to add little to the
diagnostic armamentarium in facial palsy.

Antidromic Stimulation of the Facial Nerve

Although antidromic stimulation of the facial nerve is still predominantly
an investigative modality, Lee and colleagues tested 20 patients with
Bell palsy and suggested that this modality was more sensitive than
EnoG in detecting nerve degeneration.

Imaging of the Facial Nerve

Gadolinium-enhanced MRI has been used to study the peripheral facial
nerve in Bell palsy. Enhancement of the facial nerve is a common
finding in healthy persons, making evaluation of facial nerve findings on
MRI difficult. The most consistent areas of enhancement in Bell palsy
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 and herpetic palsy are the distal meatal and labyrinthine segments.
However, the location or degree of enhancement does not correlate
with the recovery of facial movement. Imaging for facial paralysis may
be improved as techniques evolve ; however, overall, the literature
would support Jun and colleagues in their conclusion that MRI imaging
is not essential in the diagnosis of patients with facial palsy.

Prognosis and Statistics

The prognosis for most patients with Bell palsy is excellent: 80% to
90% recover completely. Peitersen reported that 0 of 1505 patients
followed without management had permanent complete paralysis and
17 had moderate to severe sequelae (contracture, synkinesis, palsy).
Many large series of patients with Bell palsy have been analyzed to
identify prognostic factors that have a significant impact on outcome.
The most important is whether the paralysis is incomplete or complete.
The prognosis for those who never develop complete facial paralysis is
excellent: 95% to 100% recover with no identifiable sequelae.

The many reports available in the literature are difficult to compare

because they used different criteria for inclusion and evaluation of
outcome. Stankiewicz collated the outcome from nine reports,
including the large series of Park and Watkins and Peitersen. The
overall results from these series was 53% complete recovery, 44%
partial recovery, and only 3% no recovery. The results in the general
population are probably better than those in these studies because
patients with early spontaneous recovery do not seek treatment.

Other factors associated with poor outcome include hyperacusis;

decreased tearing; age above 60 years; diabetes mellitus;
hypertension; and severe aural, anterior facial, or radicular pain.
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 However, Abraham-Inpijn and others compared outcomes in 200
patients by use of several potential prognostic factors that included
severity of the facial paralysis, mean arterial pressure, age, clinical or
chemical diabetes mellitus, and history of hypertension. Only the
severity of the paralysis at its maximum extent and the mean arterial
pressure at presentation proved to be statistically significant. Peitersen
noted a correlation between the sequelae (synkinesis, contracture) and
the interval between paralysis and the onset of recovery.

Management
The management of Bell palsy has had a long, complex evolution that is
not yet complete. Treatment methods currently advocated include
observation, corticosteroids, antiviral agents, and surgical
decompression.

The use of corticosteroids in Bell palsy was first proposed by

Rothendler. Since then, corticosteroids have become the most
common agents used for the management for Bell palsy. Numerous
prospective and retrospective studies have been done on the
effectiveness of corticosteroids in Bell palsy. In a meta-analysis,
Ramsey and others found two studies with sufficient numbers of
patients and rigor for analysis and concluded that treated patients had
a 17% better chance of complete recovery than patients who were
given placebo or were untreated. Their analysis of a larger number of
studies concluded that the odds of recovery with steroid treatment
ranged between 49% and 97% versus 23% and 64% for untreated
patients. No consensus has been reached concerning the dose or
duration of steroid treatment. A multi-institutional prospective double-
blind study of 829 patients by Axelsson and colleagues suggests
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 higher recovery rates and less synkinesis if steroids are started within
48 hours of onset of paralysis. A Cochrane review of 2280 participants
from randomized controlled trials suggested that there was benefit
from combination therapy using antivirals with corticosteroids—as
compared with corticosteroids alone—in treating patients with severe
Bell palsy, albeit this was low-quality evidence. The same report also
found moderate-quality evidence suggesting a reduction in synkinesis
and excessive tearing with combination therapy—antivirals and
corticosteroids—as compared with corticosteroids alone.

The case for steroid treatment in children is less clear. Neither Unüvar
and others nor Pavlou and others found a beneficial effect of
corticosteroids for Bell palsy in children.

Patients with Bell palsy are commonly treated with antiviral agents in
addition to prednisone, although, once again, definitive proof of
efficacy is lacking. Although initial enthusiasm surrounded the use of a
combination of steroids and antivirals, more recent studies have shown
negative results. Sullivan and colleagues conducted a four-armed
multi-institutional trial of prednisolone, acyclovir, a combination of both
agents, and placebo. Significant improvement of outcome was
observed in both arms that contained prednisolone, but no additional
benefit was found from antiviral treatment. A similar outcome occurred
in a study for Japan by Kawaguchi and colleagues. Several other large
randomized trials have come to the same conclusion. A 2012 report of
the Guideline Development Subcommittee of the American Academy of
Neurology found that “the addition of antiviral agents does not increase
the probability of facial functional recovery by more than 7%. Patients
offered antivirals should be counseled that a benefit from antivirals has
not been established, and, if there is a benefit, it is likely that it is
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 modest at best.” Some of the discrepancy between studies may result
from the inclusion/exclusion of patients with ZSH among the Bell
patients. Future studies need to include serologic studies to dissect the
effects of antivirals on this subpopulation of facial palsy patients.

Surgical therapy is more controversial, because unlike corticosteroid

therapy, additional injury can occur with surgery. Early enthusiasm for
transmastoid decompression of the tympanic and mastoid segments of
the facial nerve has waned, and the procedure has been abandoned,
because randomized trials showed no benefit and because of evidence
that the site of the lesion is in the proximal labyrinthine portion of the
facial nerve, which is inaccessible through the mastoid.

The efficacy of surgical decompression of the meatal foramen and

labyrinthine segment of the facial nerve in patients with a poor
prognosis as shown by ENoG testing is promising, but it has been
difficult to assemble a large enough series to definitively establish its
value in randomized trials. Fisch compared 14 surgical patients with
90% or more degeneration as shown by ENoG within 3 weeks of the
onset of paralysis with 13 similar patients who refused surgery. He
found a subtle but statistically significant improvement in long-term
facial recovery in the operative group. Sillman and colleagues found a
shift toward Grades I and II recovery in patients with greater than 90%
degeneration who underwent surgical decompression versus those
managed with corticosteroids alone. In the best-controlled study of
middle fossa decompression of the facial nerve in Bell palsy, Gantz and
others evaluated the outcome when ENoG showed greater than 90%
degeneration and there were no voluntary motor unit potentials on EMG
within 2 weeks of the onset of paralysis. Of the surgical patients, 91%,
achieved House-Brackmann (HB) Grade I or II recovery versus a 42%
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 chance of these same results with steroids alone. Graham and Kartush
reported six patients with recurrent facial paralysis, one of whom had
Melkersson-Rosenthal syndrome. None of these patients had recurrent
facial paralysis after total seventh nerve decompression from the
stylomastoid foramen to the internal auditory canal. Thus, surgical
decompression remains in the armamentarium for the management of
facial paralysis, but timing and appropriate patient identification are still
difficult.

Rehabilitation of Facial Paralysis

Botulinum Toxin
Persistent synkinesis can be a problem for many patients recovering
from severe degeneration of the facial nerve regardless of the cause.
Chua and colleagues performed a dose-escalation study to determine
the best treatment for reducing eyelid synkinesis and found the lowest
dose, 40 units injected into the orbicularis oculi, to give the best
reduction in synkinesis while avoiding ptosis. Borodic and associates
found similar results in a multiinstitutional study.

Physical Therapy
A large number of physical rehabilitation measures have been applied
to improve recovery and function in patients with facial palsy, which
includes various forms of electrostimulation, mime therapy,
biofeedback, and neuromuscular reeducation. In two recent meta-
analyses, one found no evidence for the benefit of physical therapy and
the other found Level C support for mime therapy.

Facial Reanimation
:
 Permanent facial palsy can be rehabilitated with reanimation surgery,
classified as either static or dynamic. Examples of static procedures
include facial slings with xenograft or muscle transfers, whereas
dynamic reconstruction typically involves the use of nerve grafts. The
details of these procedures are beyond the scope of this chapter.

Special Cases of Facial Paralysis

Ramsay Hunt Syndrome
Ramsay Hunt syndrome, or herpes zoster oticus, differs from Bell palsy
because it is associated with VZV (as shown by rising titers of
antibodies to VZV) and the presence of skin vesicles on the pinna,
retroauricular area, face, or mouth. Compared with Bell palsy, Ramsay
Hunt syndrome generally causes more severe symptoms and patients
have a higher risk of complete nerve degeneration developing. Ramsay
Hunt syndrome can be underdiagnosed on the basis of initial
presentation. In approximately 10% of patients, the vesicular rash
appears well after the initial facial paralysis, and many patients have a
rise in antibody to the VZV without ever having cutaneous or mucous
membrane vesicles develop, so-called zoster sine herpete , as
previously discussed.

Varicella, or chickenpox, is the manifestation of the primary infection by

VZV (HSV, varicella) in a nonimmune host. Herpes zoster infection is
the manifestation of this same virus in a partially immune host.
Serologic and epidemiologic data strongly suggest that VZV represents
the reactivation of a latent virus rather than reinfection. After the
primary infection, the virus probably travels to the dorsal root to
extramedullary cranial nerve ganglia, where it remains dormant until it is
reactivated. Reactivation generally occurs during a period of decreased
:
 cell-mediated immunity. VZV is the second most common cause of
facial paralysis. The incidence of herpes zoster virus in patients with
peripheral facial palsy is 4.5% to 9%. A Mayo Clinic study estimated the
annual incidence of herpes zoster virus at 130 cases per 100,000. The
incidence increases dramatically in patients older than age 60; 10% of
this population had identifiable risk factors for decreased cell-mediated
immunity that included carcinoma, trauma, radiotherapy, or
chemotherapy. The increased incidence in the elderly is explained by
an age-related decrease in cellular immune response to VZV.

Compared with Bell palsy, the severity of the paralysis is worse and the
prognosis poorer in herpes zoster oticus. Peitersen reported full
recovery in only 22% of patients, and Devriese found complete
recovery in only 16%. As in Bell palsy, the recovery is in part predicted
by the severity of the paralysis. Complete recovery occurred in only
10% of patients after complete loss of facial function and in about 66%
after incomplete loss.

The timing of the appearance of the vestibular eruption may have

prognostic significance. In most cases, eruption and paralysis occur
simultaneously. In approximately 25% of cases, the eruption precedes
the paralysis; these patients have a higher likelihood of recovery.
Patients with Ramsay Hunt syndrome are also more likely than patients
with Bell palsy to have associated cranial nerve symptoms, including
hyperacusis, hearing loss, and pain.

Severe ocular complications can occur with herpes zoster

ophthalmicus. These complications include uveitis, keratoconjunctivitis,
optic neuritis, and glaucoma; they are almost always associated with
involvement of the ophthalmic division of the trigeminal nerve. Herpes
zoster ophthalmicus may be difficult to differentiate from the localized
:
 skin rash associated with HSV. Although both conditions may cause
keratitis, differentiation between them is extremely important because
topical corticosteroids are used to manage herpes zoster infection but
are contraindicated in HSV. Ophthalmologic consultation for
biomicroscopy, staining, cytologic studies, and viral isolation studies
may differentiate these two conditions. Adour stated that aside from
concerns about ophthalmic involvement, the development of skin
vesicles before or after initiation of prednisone does not contraindicate
corticosteroid use.

Management of patients with herpes zoster, including cephalic zoster,

is with systemic corticosteroids. A specific benefit of corticosteroid
therapy is a reduction of postherpetic neuralgia. The usefulness of
corticosteroids in fostering the recovery of facial paralysis is
controversial; however, early institution of corticosteroids seems to
relieve acute pain, reduce vertigo, and decrease the incidence of
postherpetic neuralgia.

The antiviral agent acyclovir is also recommended to treat herpes

zoster facial paralysis. Acyclovir is a nucleotide analogue that interferes
with herpes virus DNA polymerase and inhibits DNA replication. The
drug is preferentially taken up by HSV-infected cells, making it nearly
nontoxic to noninfected cells. There is now consensus that herpes
zoster–associated facial paralysis should be treated with both steroids
and antivirals.

Congenital Facial Paralysis

The incidence of facial paralysis in newborns has been estimated at
0.23% of live births. The first dilemma in managing facial palsy in an
infant or young child is differentiating between true congenital paralysis
:
 and birth trauma. The differential diagnosis of congenital facial palsy is
shown in Box 172.1 . Birth trauma usually causes isolated facial
paralysis and other signs of injury, including facial swelling,
ecchymosis, or hemotympanum. Abnormalities of other cranial nerves
or on brainstem audiometry (prolongation of the I to III or I to V interval)
suggest that the facial paralysis is congenital and not traumatic.

Box 172.1

Modified from Harris JP, Davidson TM, May M, Fria T: Evaluation and
treatment of congenital facial paralysis, Arch Otolaryngol 109:145,
1983.

Congenital Versus Acquired Facial Paralysis

Congenital
Mononeural agenesis

Congenital facial paralysis

Congenital unilateral lower lip palsy

Facial paralysis with other deficits

Möbius syndrome (cranial nerves VI and VII; bilateral)

Hemifacial microsomia

Oculoauriculovertebral dysplasia

Poland syndrome (agenesis of pectoralis major muscle)

Secondary to teratogens
:
 Thalidomide

Rubella

Acquired
Birth trauma

Forceps injury

Pressure from maternal sacrum

Pressure from fetal shoulder

Intracranial hemorrhage

Idiopathic

Bell palsy

Systemic or infectious disease

Melkersson-Rosenthal syndrome

Poliomyelitis

Infectious mononucleosis

Varicella

Acute otitis media

Meningitis

Of facial paralysis cases in infants, 78% are related to birth trauma.

:
 These cases are equally divided between forceps deliveries and vaginal
deliveries plus cesarean sections, which suggests that intrauterine
facial nerve injury can occur from pressure on the infant's face by the
sacral prominence during birth. Supranuclear palsy secondary to
intracranial hemorrhage has also been reported.

The mildest form of congenital facial dysfunction is congenital

unilateral lower lip palsy, in which the defect is limited to an absence of
activity in the depressor labii inferioris muscle. This is associated with a
lesion of the brain stem.

Möbius syndrome represents a broad spectrum of clinical and

pathologic findings that range from isolated unilateral facial paralysis,
usually associated with sixth cranial nerve paralysis, to bilateral
absence of facial and abducens nerve function. Multiple other cranial
nerves—including the glossopharyngeal, vagus, hypoglossal, and other
extraocular motor nerves—may also be affected. Of children with
Möbius syndrome, 43% have a history of some significant event in
utero. The majority of patients with Möbius syndrome have sparing of
the lower face compared with the upper face. Verzijl and associates
performed electrophysiologic testing on 11 patients with Möbius
syndrome and concluded that the syndrome is not a primary
developmental disorder of the facial musculature but rather a disorder
of the rhombencephalon, which includes both motor nuclei and
traversing long tracts. MRI evidence suggests absence of the seventh
cranial nerve in the internal auditory canal, implying that the lower face
receives aberrant innervation from other nerves, including the
trigeminal, glossopharyngeal, and hypoglossal nerves.

Multiple cranial nerve palsies, including facial palsy, are seen in

association with CHARGE syndrome ( c olobomata, h eart disease, a
:
 tresia of choanae, r etarded growth, g enital hypoplasia, and e ar
anomalies and deafness). Of cases associated with the syndrome, 75%
have at least one cranial nerve involved and 58% have two or more
cranial nerves involved. The most frequently involved nerves are the
auditory nerve in 60%, the facial nerve in 43%, and the
glossopharyngeal vagus nerve in 30%.

Spontaneous Facial Paralysis in Children

Bell palsy is uncommon in children; 8% of patients in the series by
Peitersen and only 2% in the series by Adour and others were younger
than 10 years old. Manning and Adour and Prescott found a female
preponderance in this group.

The prognosis for children with Bell palsy is uncertain. Peitersen,

Taverner, and Prescott reported a high rate of spontaneous recovery in
children with Bell palsy in part related to the high frequency of
incomplete paralysis. Inamura and others found that 97% of 58
patients recovered completely and concluded that corticosteroids had
no impact on recovery. However, Alberti and Biagioni and Manning and
Adour reported that a significant percentage of children did not recover
from their facial paralysis. Jenkins and others and Prescott argued that
the prognosis was determined by the amount of nerve degeneration
revealed by electrophysiologic testing and that children and adults with
similar electrical testing results had similar outcomes. The role of
corticosteroids in treating idiopathic paralysis in children is not as well
established as it is in adults.

Familial Facial Paralysis

Adour and others reported that 8% of patients with Bell palsy had a
:
 positive family history, and Willbrand and others reported a 6%
incidence. In addition, there are sporadic reports of families with
several affected members, usually each having experienced several
attacks. Cawthorne and Haynes reported on two brothers, one had five
episodes of Bell palsy and the other had three. DeSanto and Schubert
reported 10 cases of Bell palsy in a family over 83 years; Willbrand and
others reported a family with 29 cases of Bell palsy over 40 years.
Samuel reported a child who experienced four episodes of Bell palsy
that involved both sides of the face; the child's father had six episodes
of unilateral Bell palsy. Amit described a family tree in which only
women—the patient, her mother, a maternal aunt, and her maternal
grandmother—had Bell palsy. In three of the four, onset occurred during
puberty, which suggests that hormonal influence may have been
important. These sporadic cases are insufficient to conclusively
determine a genetic influence. However, these cases often have an
early age of onset, are recurrent, and have an excellent prognosis.

Recurrent Paralysis
Adour and others found that 9.3% of patients with Bell palsy had a
history of previous paralysis. Similarly, Hallmo and associates reported
that 10.9% of patients had a history of a previous facial paralysis, about
equally on the ipsilateral side and the contralateral side. Prescott
reported that 20 of 228 children (11.4%) younger than 18 years of age
with Bell palsy had a history of previous paralysis on the same side, and
8 had contralateral paralysis. The interval between the two attacks was
usually more than a year. Perhaps the record for recurrent facial
paralysis was a woman who had more than 50 episodes of unilateral
lower facial paralysis after exposure to the disinfectant chlorocresol.
The age distribution of patients with recurrent facial palsy was the
:
 same as that of the overall population with Bell palsy except for those
with ipsilateral recurrent palsies, which may be associated with a
younger age of onset. Several reports noted a slight female
predominance in recurrent facial palsy. Diabetes mellitus was present in
39% of patients with recurrent paralysis in one study but in only 4% in
another.

Controversy continues as to whether the prognosis for recurrent facial

palsy is better or worse on subsequent attacks. Several authors have
stated that the second attack has a poorer prognosis and thus
constitutes a stronger indication for surgical decompression. Most
other authors, however, found no prognostic difference between the
primary and subsequent attacks of facial paralysis and found no
difference regarding whether the second attack occurred on the
ipsilateral side or contralateral side.

Recurrence of facial palsy should prompt a careful investigation of the

patient. May and others found a tumor in 8 of 40 patients (20%) with a
second palsy on the same side.

Bilateral Facial Paralysis

Bilateral idiopathic facial paralysis is much less common than unilateral
paralysis and occurs in only 0.3% to 2% of patients. Bilateral facial
paralysis has a much higher incidence of systemic causes than
unilateral palsy and should spur a diligent search for an underlying
cause. The diseases most commonly associated with bilateral facial
paralysis are Guillain-Barré syndrome, Bell palsy, multiple idiopathic
cranial neuropathies, brainstem encephalitis, benign intracranial
hypertension, syphilis, leukemia, sarcoidosis, Lyme disease, and
bacterial meningitis. The possibility of intrapontine and prepontine
:
 tumor also should be excluded. Most of these conditions are
associated with other systemic or neurologic signs that suggest the
appropriate diagnosis.

Guillain-Barré syndrome is a progressive ascending motor paralysis

after a viral infection that usually affects the lower limbs. However, rare
bulbar, myelitic, and cerebral forms of Guillain-Barré do not affect the
limbs. Of the cranial nerves, the facial nerve is the third most commonly
affected after the glossopharyngeal and vagus nerves. The diagnosis is
based on the typical clinical picture and the presence of elevated spinal
fluid protein but a normal cell count. Other conditions associated with
facial diplegia include influenza, infectious mononucleosis, other
viruses, granulomatosis with polyangiitis, and diabetes. Heerfordt
syndrome—parotid enlargement, iridocyclitis, and cranial nerve palsy—
associated with sarcoidosis can cause bilateral facial paralysis.

The workup for a patient with bilateral facial paralysis should include a
careful neurologic examination to detect other cranial neuropathies, a
lumbar puncture for cytologic studies, blood chemistry, culture,
autoantibody titers, a Venereal Disease Research Laboratory (VDRL)
test for syphilis, and an MRI to exclude space-occupying lesions.

The disability caused by bilateral facial paralysis is dramatically more

severe than that caused by unilateral paralysis. Aggressive eye care
with ointment, taping, or patching is usually required, but this approach
significantly interferes with visual function. Bilateral paralysis of the
lower lip leads to a characteristic speech impairment, oral
incompetence, and drooling. In severe cases, dental sequelae can
occur from inadequate circulation of saliva. The psychologic impact
can be devastating because these patients are incapable of voluntary
or emotional expression. Recovery in bilateral Bell palsy is similar to that
:
 in unilateral palsy, although one side may recover before the other.

Progressive Paralysis
Slowly progressive facial paralysis is not Bell palsy. The differential
diagnosis includes primary neuromas of the facial nerve ( Fig. 172.2 );
metastases from squamous cell carcinomas and melanomas of the
face and scalp; and occasionally distant metastases from the kidney,
breast, lung, and prostate. Progressive facial paralysis can also occur
from other primary temporal bone and cerebellopontine angle lesions
and from carotid artery aneurysms. A tumor should be excluded in all of
these cases, beginning with physical examination for neuroma of the
peripheral branches of the facial nerve and continuing with CT and
gadolinium-enhanced MRI of the infratemporal and intracranial portions
of the nerve. If these studies are negative, these patients require
careful serial observation and imaging in an attempt to find a cause.

Fig. 172.2

Axial CT scan of the right temporal bone shows enlargement of the mastoid segment of the
fallopian canal (arrow) in a child with progressive facial paralysis. The lesion was a neuroma
of the facial nerve.

Traumatic Facial Paralysis

The most common causes of intratemporal facial nerve injury are
temporal bone fractures, penetrating injuries (e.g., by gunshot), and
iatrogenic injuries. Injury of the facial nerve in temporal bone fractures
can result from compression from bone fragments, intraneural
hematomas, entrapment from compression, and loss of continuity. The
distal labyrinthine segment and geniculate ganglion are the areas of the
facial nerve most susceptible to injury ( Fig. 172.3 ). This portion of the
facial nerve is particularly at risk because of its small size and lack of
:
 fibrous supporting tissue and the traction between the greater petrosal
nerve and the geniculate ganglion caused by temporary distraction of
the anterior and posterior portions of the temporal bone at the time of
the injury. In addition to direct injury to the nerve, Grobman and others
presented a case of severe compression, demyelination, and edema of
the facial nerve proximal to the site of the injury, with the most severe
changes at the meatal foramen. The authors pointed out that the
histologic changes in the facial nerve were similar to those in Bell palsy
despite the fact that the initial insult to the nerve was different. High-
resolution CT of the temporal bone is the most effective way to identify
potential sites of injury of the facial nerve ( Fig. 172.4 ).

Fig. 172.3

An axial CT scan of the temporal bone shows a fracture of the temporal bone through the
vestibule and geniculate ganglion.

Fig. 172.4

A coronal CT scan of the temporal bone shows a fracture of the temporal bone through the
cochlea; it traverses the fallopian canal in the tympanic portion of the facial nerve.

Management of the facial nerve in temporal bone fractures has been

controversial. Some authors recommend observation and symptomatic
care only. Maiman and others followed 45 patients nonoperatively and
found that 29 had severe enough injuries to be visible on
polytomography. Of these 45 patients, 44 had satisfactory recovery
and 65% had complete recovery. McKennan and Chole reported
excellent recovery in patients with delayed-onset paralysis managed
nonsurgically.

In making the decision about surgical decompression in specific cases,

patients have traditionally been divided between immediate-onset
versus delayed-onset paralysis after the injury; immediate-onset
:
 paralysis is associated with severe injuries and poorer outcomes.
Conversely, Adegbite and others found that the outcome was predicted
by the severity of the paralysis but not the manner of onset. Fisch,
however, advocated surgical intervention on the basis of ENoG results
rather than on the manner of onset of the paralysis. He established a
criterion of more than 90% degeneration within 6 days of onset. The
timing of surgery, however, does not have to be within 6 days; in fact,
some advantage may be gained by delaying surgery up to 3 weeks
after an immediate paralysis to allow resolution of edema and
hematoma and to make the surgical field more discernible. Chang and
Cass carefully reviewed the literature on surgical versus expectant
management of facial paralysis after temporal bone fracture and
concluded that the natural history, appropriate timing, and reporting
criteria should be considered in judging the true efficacy of facial nerve
decompression in nonpenetrating injury of the temporal bone.

Long-standing paralysis is also a management problem because

electrical testing is not valid. In these cases, unless CT shows gross
disruption of the fallopian canal, it is advisable to wait 12 months and
explore the nerve if no clinical or EMG signs of recovery are apparent.

Penetrating injuries, such as gunshot wounds, are often accompanied

by severe injuries that include dural tears, CSF liquorrhea, damage to
the otic capsule, and vascular injury. Evaluation includes high-
resolution CT, carotid arteriography, and facial nerve electrical testing.
Telischi and Palete emphasize that extratemporal facial nerve blast-
type injuries may not cause transection of the facial nerve; they
recommend continued conservative management even if the nerve is
severely hemorrhagic and contused.

Iatrogenic facial paralysis is a feared complication of ear surgery. It can

:
 be grouped into cases in which there is concern about the integrity of
the facial nerve intraoperatively and those in which the paralysis is a
surprise. In the former group, the nerve should be positively identified
by the use of landmarks distant from the site of presumed injury. The
nerve is traced to the site of injury, the local area is decompressed, and
disrupted nerve fibers are reapposed. If loss of nerve tissue is serious,
an onlay or interpositional facial graft can be used. If the landmarks are
not discernible, the dissection should be terminated before further
injury occurs and an appropriate colleague consulted. In the
emotionally and medicolegally charged realm of iatrogenic injury,
consultation is often beneficial to all involved. If the facial paralysis is
unexpected, it is appropriate for the original surgeon to manage the
patient. Tight dressings and packing should be released immediately,
and time should be allowed for any local anesthetic effects to dissipate.
If the face has not recovered, urgent reexploration and decompression
of the nerve should be performed.

Surgical exploration of the facial nerve after trauma requires that the
surgeon be familiar with the entire temporal bone and be prepared for
mastoid, translabyrinthine, and middle fossa exploration and repair of
the nerve. Caution is necessary for middle fossa exploration of patients
with temporal bone trauma. Jones and others reported that 14 of 15
patients who had CT evidence of temporal bone fracture also had MRI
evidence of significant ipsilateral temporal lobe contusion.

Facial Paralysis Associated With Other

Conditions
Pregnancy
The possible role of hormonal and fluid changes in the pathogenesis of
:
 Bell palsy has been debated since Bell first suggested an association
between idiopathic paralysis and pregnancy in 1830. Bell palsy occurs
3.3 times more frequently in pregnant women than in similarly aged
nonpregnant women and most commonly occurs in the third trimester
or soon after delivery. Recurrent palsy with repeated pregnancies and
bilateral facial palsies during pregnancy have also been reported.

In a series of 18 patients, Falco and Eriksson reported that

preeclampsia was six times more common among patients with facial
palsy than in the general pregnant population. Facial palsy in pregnant
women cannot be correlated with preterm labor, low birth weight, fetal
congenital abnormality, or other prenatal abnormality. Statistics
regarding the prognosis and outcome compared with age-matched
nonpregnant women are uncertain; some studies indicate that the
prognosis and outcome are no different ; others suggest that they are
decidedly worse for those who are pregnant. Gillman and colleagues
compared 77 women who developed Bell palsy during pregnancy or
soon after delivery with age-matched nonpregnant women and age-
matched men. The pregnant patients had a statistically significant
poorer result than either of the comparison groups. Similar findings
were reported by Phillips and colleagues. Treatment with prednisone is
the mainstay of management in pregnant women with Bell palsy,
although a significant difference in outcome was not found with or
without steroids in the study by Gillman and others. Prednisone
apparently poses minimal risk to the developing fetus, especially in the
third trimester.

Melkersson-Rosenthal Syndrome
Melkersson-Rosenthal syndrome is a triad of symptoms: recurrent
:
 orofacial edema, recurrent facial palsy, and lingua plicata (fissured
tongue). Orofacial edema is the defining feature; lingua plicata and
peripheral facial paralysis each occur in one half of the patients. The
complete triad is present in only one-fourth of the patients. The
condition generally begins in the second decade of life, and the
manifestations usually occur sequentially and seldom appear
simultaneously.

The major diagnostic criterion is persistent or recurrent nonpitting facial

edema that cannot be explained by infection, malignancy, or
connective tissue disorder. The oral swelling usually involves the lips
and buccal area, but the gingiva, palate, and tongue can also be
affected. The swelling can extend to the supraorbital and infraorbital
tissues of the cheek and is usually transient, but it may recur at regular
intervals. The edematous lips assume a chapped, fissured, red-brown
appearance.

After numerous recurrences, the lips eventually become permanently

deformed. They may also have chronic fissuring that is painful and slow
to heal. The extent of the facial swelling varies from unilateral
involvement of the lower lip to bilateral total facial edema. Chronic
swelling causes a cosmetic problem and may interfere with speaking
and eating. The chronic and recurrent nature of the edema
distinguishes it from transient angioneurotic edema. Biopsy specimens
of the lip reveal noncaseating epithelioid cell granulomas surrounded
by histiocytes, plasma cells, and lymphocytes. Although the cause of
Melkersson-Rosenthal syndrome is unknown, the tissue edema is
attributed to lymphatic and vascular disruption by granulomas.

Facial paralysis is the least common symptom of the triad; it has an

abrupt onset that is identical to that in Bell palsy. A history of bilateral
:
 sequential paralysis and relapse of paralysis after initial recovery is
common, and the site of the paralysis usually corresponds to the area
of facial swelling. Symptomatic management of the facial paralysis is
indicated. Multiple treatments have been tried; they include steroids,
metronidazole, dapsone, acyclovir, and methotrexate—all without a
consistent response. No randomized trials of corticosteroids or surgery
have been undertaken, although cessation of the recurrent facial
paralysis has been reported in several series after facial nerve
decompression.

Human Immunodeficiency Virus

Acute facial palsy can occur at any stage of infection with HIV. The
palsy can be a direct result of infection, or it may occur secondary to
HIV, such as that resulting from a secondary infection by herpes zoster
virus. Most cases of facial palsy in early-stage HIV infection resemble
Bell palsy in that they have an abrupt onset and no other cause is
identifiable. These patients also are at risk for the craniocervical form of
Guillain-Barré syndrome.

HIV is neurotropic and has been isolated from the CSF and neural
tissues in all stages of HIV infection. Acute facial palsy in acute and
chronic HIV infection may be indistinguishable from Bell palsy in the
absence of HIV; however, secondary causes seen in late HIV, which
include lymphoma and infection, should also be considered.

The prognosis for idiopathic paralysis in patients with AIDS is similar to

that in the general population. The prognosis for facial paralysis from
other causes in patients with AIDS depends on the underlying
pathology.
:
 Lyme Disease
Lyme disease (Bannwarth syndrome in Europe) is caused by the tick-
borne spirochete Borrelia burgdorferi . The vectors for Lyme disease
are several species of Ixodes ticks, and the primary reservoirs of
infection are the white-footed mouse and the white-tailed deer. The
disease occurs in all parts of the United States and is the most
commonly reported vector-borne disease in the country.

Similar to syphilis, Lyme disease occurs in several stages. It begins with

erythema migrans, an influenza-like illness; regional lymphadenopathy;
and general malaise. Erythema migrans is an enlarging annular
erythematous skin lesion that may be multiple and is not limited to the
site of the bite. The second stage starts several weeks to months later,
when neurologic abnormalities develop that include meningitis and
cranial nerve and other peripheral neuropathies. Early symptoms
include headache, neck stiffness, and spinal pain. Stage three occurs
months to years later in the form of chronic arthritis, neurologic deficits,
recurrent meningitis, and subtle mental disorders. Chronic neurologic
complications occur in 18% of unmanaged patients with Lyme disease.

Facial paralysis is a relatively rare manifestation of tick-borne disease

and occurs in only 0.9% to 4.5% of patients; it may be unilateral or
bilateral and may be the only neurologic abnormality. Halperin and
Golightly emphasized that the facial paralysis may precede serologic
evidence of infection and that facial paralysis may occur in the absence
of either erythema migrans or an identified tick bite. Lyme disease is
recognized as an increasingly important cause of facial paralysis,
especially in children. In endemic areas, Lyme disease is responsible for
half of the cases of facial paralysis seen in children.
:
 The diagnosis of Lyme disease can be difficult. In one study, 89% of
patients had a history of erythema migrans, 5% had arthritis, and 3%
had early neurologic symptoms. Laboratory diagnosis is also
challenging. Serologic assays for antibodies are negative in early
stages and should be obtained only when convalescent serum levels
can be expected to be obtained. Atypical cases without erythema
migrans should be evaluated with the enzyme-linked immunosorbent
assay and confirmed by separate IgM and IgG immunoblots.

Lyme disease can be treated with multiple oral antibiotics, such as

doxycycline (100 mg twice daily) or amoxicillin (500 mg three times
daily) for 14 to 21 days.

Jowett and colleagues reported worse long-term facial function

outcomes in patients treated with antibiotics and corticosteroids versus
antibiotic monotherapy.

Kawasaki Disease
Kawasaki disease, also known as infantile acute febrile mucocutaneous
lymph node syndrome, is a multisystem disease that primarily occurs in
infants and young children. In addition to involvement of the mucous
membranes, skin, lymph nodes, and cardiac system (coronary artery
aneurysms), neurologic complications have been reported in up to 30%
of patients. Aseptic meningitis and irritability are the most common
neurologic complications; however, facial palsy has been reported by
several authors. It has been suggested that facial paralysis is a marker
for increased severity of Kawasaki disease.

Management of Kawasaki disease includes supportive care,

appropriate management of cardiac failure if present, and high-dose
:
 aspirin.

Sarcoidosis
Sarcoidosis is a chronic noncaseating granulomatous disease.
Systemic involvement usually includes the lungs (hilar or peripheral
adenopathy), polyarthralgias, anergy, hepatic dysfunction, and elevated
serum calcium levels. A variant of sarcoidosis, Heerfordt syndrome
(uveoparotid fever), is characterized by nonsuppurative parotitis,
uveitis, mild fever, and cranial nerve paralysis, most commonly of the
facial nerve. Although only 5% of patients with sarcoidosis have cranial
nerve involvement, the facial nerve is most commonly affected. In
contrast, 50% of patients with uveoparotid fever have facial paralysis,
which starts abruptly days to months after the onset of the parotitis.
Paralysis is thought to be caused by direct invasion of the nerve by the
granulomatous process and not by pressure from the swollen parotid
gland. Sarcoidosis should be included in the differential diagnosis when
paralysis is bilateral.

Angiotensin-converting enzyme levels are usually elevated in

sarcoidosis. After the sarcoidosis is managed by corticosteroids,
angiotensin-converting enzyme levels can be expected to return to
normal.

Otitis Media
Facial paralysis from otitis media is rare but can occur in acute or
chronic cases. In one series, otitis media accounted for only 3.1% of
acute facial palsies. Of these 50 cases, only 5 were children with acute
otitis media; the remaining adult cases were divided equally between
chronic purulent otitis media and cholesteatomas. Three additional
:
 cases were from tuberculous otitis. Most paralyses were incomplete.
Nearly all patients explored surgically had a dehiscence of the bony
canal of the facial nerve, usually in the tympanic segment, which
presumably allowed the spread of the inflammation from the middle ear
to the nerve. Facial paralysis associated with acute otitis media,
especially in infants and children, should be managed with parenteral
antibiotics and a wide myringotomy for drainage. Surgical manipulation
of the facial nerve in acute otitis media is not recommended. Adour also
recommended the addition of a 10-day course of corticosteroids in
conjunction with myringotomy and antibiotics.

Facial paralysis associated with chronic otitis media suggests a high

probability of cholesteatoma, and surgical intervention is appropriate.
The mechanism of facial paralysis associated with cholesteatoma could
be compression or inflammation. Djeric studied autopsy specimens
from patients who had chronic otitis media but no antemortem
evidence of facial paralysis. Two of 20 facial nerves had focal areas of
demyelination, which suggests that adjacent inflammation may be
more important than pressure. The most important hallmark of facial
paralysis from cholesteatoma is a gradual onset, which distinguishes it
from Bell palsy. Removal of the cholesteatoma and decompression of
the nerve without opening the perineurium allows recovery of the
paralysis in most cases.

Barotrauma
Several authors have described a curious phenomenon of recurrent
facial paralysis with changes in barometric pressure. Woodhead
reported a 50-year-old man who had repeated facial paralysis and
ipsilateral loss of taste on ascending to 8000 to 10,000 feet in a car or
:
 airplane. Symptoms reversed on descent. Similar cases have been
reported after commercial airline flights and after scuba diving. A brief
facial palsy has also been reported after forceful nose blowing.
Barometric facial paralysis seems to be related to pressure changes in
the middle ear transmitted directly to the facial nerve through natural
dehiscences in the fallopian canal. Symptoms are relieved by pressure-
equalization tubes or other means of improving eustachian tube
function.

Benign Intracranial Hypertension

The most common symptoms of benign intracranial hypertension are
headache and visual disturbances, but occasionally it is associated
with cranial nerve palsies, which falsely suggest a localized lesion. The
abducens nerve is most commonly involved (10% to 60% of cases).
Unilateral and occasionally bilateral facial paralysis has been reported
in a few cases of benign intracranial hypertension. These cranial nerve
palsies tend to reverse promptly after reestablishment of normal
intracranial pressure through medical means or by shunting.

Metabolic Disorders
Adour and others reported that 17% of patients older than age 40 years
with Bell palsy had abnormal glucose tolerance tests, leading them to
calculate that a person with diabetes is 4.5 times more likely to have
Bell palsy develop than a person without diabetes. However, a recent
case-controlled study found that only aging and not diabetes was a risk
factor for facial palsy. A reversible facial paralysis has also been
reported with hypovitaminosis A.

Central Facial Paralysis

:
 Central facial paralysis is caused by a lesion in the parietal motor cortex
or in the connections between the cortex and the facial nucleus.
Lesions in these areas are usually accompanied by other neurologic
symptoms referable to the CNS. A supranuclear palsy of the face most
severely affects the lower half of the face and spares the forehead.
Another important sign is that emotional facial reaction remains intact
even though voluntary motion may be severely affected.

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