Introduction

Background

Facial paralysis is a disfiguring disorder that has a great impact on the patient. Facial
nerve paralysis may be congenital, neoplastic, or result from infection, trauma, toxic
exposures, or iatrogenic causes. The most common cause of unilateral facial paralysis is
Bell palsy, also known as idiopathic facial paralysis. Bell palsy is thought to account for
approximately 60-75% of cases of acute unilateral facial paralysis.

In 1550, Fallopius noted the narrow lumen in the temporal bone through which a part of
the seventh cranial nerve passes. In 1828, Charles Bell made the distinction between the
fifth and seventh cranial nerves; he noted that the seventh nerve was involved mainly in
the motor function of the face and the fifth nerve was concerned mainly with the sensory
perception of the face.

Even today, controversy still surrounds the etiology and treatment of Bell palsy. Clinical
features of Bell palsy that may help distinguish it from other causes of facial paralysis
include sudden onset of unilateral facial paralysis (less than 48 hours), absence of signs
and symptoms of CNS disease, and absence of signs and symptoms of ear or posterior
fossa disease.

Pathophysiology

The course of the facial nerve is tortuous, both centrally and peripherally (see Media file
1).

The facial nerve nucleus lies within the reticular formation of the pons, adjacent to the
fourth ventricle. The facial nerve roots include fibers from the motor, solitary, and
salivatory nuclei. The nervus intermedius comprises fibers from salivatory and solitary
nuclei (it contains sensory fibers from the tongue, mucosa, and postauricular skin as well
as parasympathetic fibers to the salivary and lacrimal glands). The fibers of the facial
nerve then course around the sixth cranial nerve nucleus and exit the pons at the
cerebellopontine angle. The fibers go through the internal auditory canal along with the
vestibular portion of the eighth cranial nerve. The narrowest portion of the internal
auditory canal is the labyrinthine segment. This is the location that is thought to be the
most common site of compression of the facial nerve in Bell palsy.

The seventh cranial nerve contains parasympathetic fibers to the nose, palate, and
lacrimal glands. The preganglionic parasympathetic fibers that originate in the salivatory
nucleus join the fibers from nucleus solitarius to form the nervus intermedius. These
fibers then synapse with the submandibular ganglion, which has fibers that supply the
sublingual and submandibular glands. The fibers from the nervus intermedius also supply
the pterygopalatine ganglion, which has parasympathetic fibers that supply the nose,
palate, and lacrimal glands.

The facial nerve passes through the stylomastoid foramen in the skull and terminates into
the zygomatic, buccal, mandibular, and cervical branches. These nerves serve the
muscles of facial expression, which include frontalis, orbicularis oculi, orbicularis oris,
buccinator, and platysma. Other muscles innervated by the facial nerve include stapedius,
stylohyoid, posterior belly of the digastric, occipitalis, and anterior and posterior auricular
muscles. All muscles of the facial nerve are derived from the second brachial arch.

The location of injury of the facial nerve in Bell palsy is peripheral to the seventh nerve
nucleus. The injury is thought to occur near or at the geniculate ganglion. If the lesion is
proximal to the geniculate ganglion, the motor paralysis is accompanied by gustatory and
autonomic abnormalities. Lesions between the geniculate ganglion and the origin of the
chorda tympani produce the same effect except that they spare lacrimation. If the lesion is
at the stylomastoid foramen, it may result in facial paralysis only.

Bell palsy is thought to be caused by edema and ischemia resulting in compression of the
facial nerve in its course through the bony canal. The cause of the edema and ischemia is
still being debated. In the past, cold exposure (eg, chilly wind, cold air conditioning, or
driving with the car window down) were considered the only triggers to Bell
palsy. Several authors believe that the herpes simplex virus (HSV) is a common cause of
Bell palsy. However, studying the causal relationship between HSV and Bell palsy is
difficult because of the ubiquitous nature of HSV.

In 1972, McCormick first suggested that HSV is responsible for idiopathic facial
paralysis.1 This was based on the analogy that HSV was found in cold sores, and he
hypothesized that HSV may remain latent in the geniculate ganglion. Since then, autopsy
studies have shown HSV in the geniculate ganglion of patients with Bell palsy.
Murakami et al performed polymerase chain reaction (PCR) testing for HSV in the
endoneural fluid of the seventh nerve of patients who underwent surgery for Bell palsy.
Of the 14 patients, 11 were found to have HSV in the endoneural fluid.2 Assuming that
HSV is the etiologic agent in Bell palsy is reasonable. If this is true, then the virus is most
likely to travel up the axons of the sensory nerves and reside in the ganglion cells. At
times of stress, the virus will reactivate, causing local damage to the myelin.

Thus, Bell palsy may be secondary to viral, and/or autoimmune reactions causing the
facial nerve to demyelinate, resulting in unilateral facial paralysis.

Frequency

United States

The annual incidence of Bell palsy is approximately 23 cases per 100,000 persons. The
right side is affected 63% of the time. Persons with diabetes have a 29% higher risk of
being affected by Bell palsy than persons without diabetes. Thus, measuring blood
glucose levels at the time of diagnosis of Bell palsy may detect undiagnosed diabetes.

International

The highest incidence was found in a study in Seckori, Japan, in 1986 and the lowest
incidence was found in Sweden in 1971. Most population studies generally show an
annual incidence of 15-30 cases per 100,000 population.

Mortality/Morbidity

• Most patients who suffer from Bell palsy have neurapraxia or local nerve
conduction block. These patients are likely to have a prompt and complete
recovery of the nerve. Patients with axonotmesis, with disruption of the axons,
have a fairly good recovery but it is usually not complete. The risk factors thought
to be associated with a poor outcome in patients with Bell palsy include (1) age
greater than 60 years, (2) complete paralysis, and (3) decreased taste or salivary
flow on the side of paralysis (usually 10-25% compared to the patient's normal
side). Other factors thought to be associated with poor outcome include pain in
the posterior auricular area and decreased lacrimation.
• Patients generally have a good prognosis; approximately 80-90% of patients
recover without noticeable disfigurement within 6 weeks to 3 months. Patients
aged 60 years or older have an approximately 40% chance of complete recovery
and have a higher rate of sequelae. Patients younger than 30 years have only a 10-
15% chance of less than complete recovery and sequelae. If no recovery occurs by
4 months, then the patient is more likely to have sequelae from the disease, which
include synkinesis, crocodile tears, and rarely hemifacial spasm.
o Synkinesis is an abnormal contracture of the facial muscles while smiling
or closing the eyes. It may be mild and result in slight movement of the
chin when the patient blinks, eye closure with smiling, or contracture
around the mouth while blinking. Crocodile tears are observed; patients
shed tears while they eat.
o Facial spasm is a very rare complication of Bell palsy. It occurs as tonic
contraction of one side of the face. Spasms are more likely to occur during
times of stress or fatigue and may occur during sleep. This condition may
occur secondary to compression of the root of the seventh nerve by an
aberrant blood vessel, tumor, or demyelination of the nerve root. It occurs
most commonly in the fifth and sixth decades of life, and sometimes the
etiology is not found. The presence of progressive facial hemispasm with
other cranial nerve findings indicates a possibility of a brainstem lesion.
o Diabetic patients are 30% more likely than nondiabetic patients to have
only partial recovery; recurrence of Bell palsy is also more common
among diabetic patients.
• Bell palsy accounts for only 23% of bilateral facial paralysis. The majority of
patients with bilateral facial palsy have Guillain-Barré syndrome (GBS),
 sarcoidosis, Lyme disease, meningitis (neoplastic or infectious), or bilateral
neurofibromas (in patients with neurofibromatosis type 2).
• Bell palsy recurs in 10-15% of patients. It may recur on the ipsilateral or
contralateral side of the initial palsy. Recurrence usually is associated with a
family history of recurrent Bell palsy. Approximately 30% of patients with
recurrent ipsilateral facial palsy were found to have tumors of the seventh nerve
or parotid gland. Patients with recurrent ipsilateral facial palsy should undergo
MRI or high-resolution CT scan to rule out neoplastic or inflammatory (eg,
multiple sclerosis, sarcoidosis) cause of recurrence.

Sex

• Bell palsy appears to affect the sexes equally. However, young women aged 10-
19 years are more likely to be affected than men in the same age group.
• Pregnant women have a 3.3 times higher risk of being affected by Bell palsy than
nonpregnant women; Bell palsy occurs most frequently in the third trimester.

Age

The lowest incidence is found in persons younger than 10 years and the highest incidence
in persons aged 60 years or older.

Clinical
History

Bell palsy is a diagnosis of exclusion. The diagnosis must be made on the basis of a
thorough history and physical examination and use of diagnostic testing when necessary.

• Symptoms of Bell palsy

o Acute onset of unilateral upper and lower facial paralysis (over a 48-h
period)
o Posterior auricular pain
o Decreased tearing
o Hyperacusis
o Taste disturbances
• The paralysis must include the forehead and lower aspect of the face. The patient
may report inability to close the eye or to smile on the affected side. He or she
also may report increased saliva on the side of the paralysis. If the paralysis
involves only the lower portion of the face, a central cause should be suspected
(ie, supranuclear). If the patient complains of contralateral weakness or diplopia
in conjunction with the supranuclear facial palsy, a stroke or intracerebral lesion
should be strongly suspected.
 o Half of the patients affected with Bell palsy may complain of posterior
auricular pain. Ask the patient if he or she has experienced trauma, which
may account for the pain and facial paralysis.
o One third of patients may experience hyperacusis in the ear ipsilateral to
the paralysis, which is secondary to weakness of the stapedius muscle.
o One sixth of patients experience decreased lacrimation.
o Many patients report numbness on the side of the paralysis. Some authors
believe that this is secondary to involvement of the trigeminal nerve,
whereas other authors argue that this symptom is probably due to lack of
mobility of the facial muscles and not lack of sensation.
• If a patient has gradual onset of facial paralysis, weakness of the contralateral
side, or history of trauma or infection, other causes of facial paralysis must be
strongly considered. Patients who have bilateral facial palsy must be evaluated for
GBS, Lyme disease, and meningitis.
• If a patient is from the Northeast, Lyme disease should be considered as a cause
of facial paralysis, and serologic testing should be performed. Approximately 5-
10% of untreated Lyme patients may have a peripheral seventh nerve palsy. For
related information, see Medscape's Lyme Disease Resource Center.
• Recurrent ipsilateral facial paralysis must raise the suspicion of a tumor of the
seventh nerve or parotid gland. If the patient reports sudden onset of hearing loss
and severe pain with the onset of facial paralysis, Ramsay Hunt syndrome must be
considered.
• Symptoms associated with seventh nerve neoplasm include slowly progressive
paralysis, facial hyperkinesis, severe pain, recurrent palsy, and other cranial nerve
involvement. Cerebellopontine tumors may affect the seventh, eighth, and fifth
cranial nerves simultaneously. Patients with a progressive paralysis of the facial
nerve lasting longer than 3 weeks should be evaluated for neoplasm.

Physical

• Initial inspection of the patient demonstrates flattening of the forehead and

nasolabial fold on the side affected with the palsy.
• When the patient is asked to raise the eyebrows, the side of the forehead with the
palsy will remain flat.
• When the patient is asked to smile, the face becomes distorted and lateralizes to
the side opposite the palsy.
• The patient is not able to close the eye completely on the affected side. On
attempted eye closure, the eye rolls upward and inward on the affected side. This
is known as Bell phenomenon and is considered a normal response to eye closure.
• A careful examination of the head, ears, eyes, nose, and throat (HEENT) must be
carried out in all patients with facial paralysis.
o The external auditory canal must be inspected for vesicles, injection,
infection, or trauma.
o The patient may have decreased sensation to pinprick in the posterior
auricular area.
 o The patient who has paralysis of the stapedius muscle will report
hyperacusis.
o Bell phenomenon is observed on attempted eye closure.
o With weakness/paralysis of the orbicularis oculi muscle (facial nerve
innervation) and normal function of the levator muscle (oculomotor nerve
innervation) and Mueller muscle (sympathetic innervation), eye closure
may be partial or absent. The tear reflex may also be absent in many cases
of Bell palsy. For these reasons the patient may have decreased tearing
and susceptibility to corneal abrasion and dryness of the eye. The patient
may appear to have loss of corneal reflex on the affected side; however,
the contralateral eye blinks when testing the corneal reflex on the affected
side.
• A careful oral examination must be performed.
o Taste and salivation are affected in many patients with Bell palsy.
o Taste may be assessed by holding the tongue with gauze and testing each
side of the tongue independently with salt, sugar, and vinegar. The mouth
must be washed after testing with different substances. The affected side
has decreased taste as compared to the normal side.
• Careful neurologic examination is necessary in patients with facial paralysis. A
neurologic abnormality warrants neurologic referral and further testing such as
MRI of the brain, lumbar puncture, and electromyography (EMG) where
appropriate.