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Background

Ramsay Hunt syndrome is defined as an acute peripheral facial neuropathy associated with
erythematous vesicular rash of the skin of the ear canal, auricle (also termed herpes zoster
oticus), and/or mucous membrane of the oropharynx.

Herpes zoster oticus, day 6. Image


courtesy of Manolette Roque, MD, ROQUE Eye Clinic.

This syndrome is also known as geniculate neuralgia or nervus intermedius neuralgia. Ramsay
Hunt syndrome can also occur in the absence of a skin rash, condition known as zoster sine
herpete. [1]

Ramsay Hunt syndrome was first described in 1907 by James Ramsay Hunt in a patient who had
otalgia associated with cutaneous and mucosal rashes, which he ascribed to infection of the
geniculate ganglion by human herpesvirus 3 (ie, varicella-zoster virus [VZV]). [2]

The following may be observed:

VZV auricularis

VZV in any of the zoster zones of the head and neck (herpes auricularis, herpes facialis,
and herpes occipito-collairs) with facial palsy

VZV in any of the zoster zones with facial palsy and auditory symptoms (eg, tinnitus,
deafness, vertigo, nystagmus, ataxia)

Pathophysiology
Ramsay Hunt syndrome is defined as VZV infection of the head and neck that involves the facial
nerve, often the seventh cranial nerve (CN VII). Other cranial nerves (CN) might be also
involved, including CN VIII, IX, V, and VI (in order of frequency). This infection gives rise to
vesiculation and ulceration of the external ear and ipsilateral anterior two thirds of the tongue
and soft palate, as well as ipsilateral facial neuropathy (in CN VII), radiculoneuropathy, or
geniculate ganglionopathy.

VZV infection causes 2 distinct clinical syndromes. Primary infection, also known as varicella or
chickenpox, is a common pediatric erythematous disease characterized by a highly contagious
generalized vesicular rash. The annual incidence of varicella infection has significantly declined
after the introduction of mass vaccination programs in most countries of the world. [3]

After chickenpox, VZV remain latent in neurons of cranial nerve and dorsal root ganglia.
Subsequent reactivation of latent VZV can result in localized vesicular rash, known as herpes
zoster. VZV infection or reactivation involving the geniculate ganglion of CN VII within the
temporal bone is the main pathophysiological mechanism of Ramsay Hunt syndrome.

Epidemiology

Frequency
Ramsay Hunt syndrome is a rare complication of latent VZV infection. [4] As previously stated,
Ramsay Hunt syndrome might occur in the absence of cutaneous rash (zoster sine herpete).
Interestingly, VZV has been detected by polymerase chain reaction (PCR) in the tear fluid of
patients diagnosed with Bell palsy. [5] Ramsay Hunt syndrome is estimated to account for 16% of
all causes of unilateral facial palsies in children, and 18% of facial palsies in adults. Ramsay
Hunt syndrome is rare in children younger than 6 years. [4]

Ramsay Hunt syndrome is thought to be the cause of as many as 20% of clinically diagnosed
cases of Bell palsy. [5]

The incidence of Ramsay Hunt syndrome among patients with HIV infection is unknown.
However, it may occur at a higher rate than in the general population because individuals with
HIV infection have a higher risk of VZV infection. [2]

Mortality/Morbidity
Ramsay Hunt syndrome is not usually associated with mortality. It is a self-limiting disease; the
primary morbidity results from facial weakness. Unlike Bell palsy, this syndrome has a complete
recovery rate of less than 50%.

History
A careful history must be obtained in patients with suspected Ramsay Hunt syndrome.
Patients usually present with paroxysmal pain deep within the ear. The pain often radiates
outward into the pinna of the ear and may be associated with a more constant, diffuse, and dull
background pain. The onset of pain usually precedes the rash by several hours and even days.

Classic Ramsay Hunt syndrome can be associated with the following:

Vesicular rash of the ear or mouth (as many as 80% of cases)

The rash might precede the onset of facial paresis/palsy (involvement of the seventh
cranial nerve [CN VII])

Ipsilateral lower motor neuron facial paresis/palsy (CN VII)

Vertigo and ipsilateral hearing loss (CN VII)

Tinnitus

Otalgia

Headaches

Dysarthria

Gait ataxia

Fever

Cervical adenopathy

Facial weakness usually reaches maximum severity by 1 week after the onset of symptoms.

Other cranial neuropathies might be present and may involve cranial nerves (CNs) VIII, IX, X,
V, and VI.

Ipsilateral hearing loss has been reported in as many as 50% of cases.

Blisters of the skin of the ear canal, auricle, or both may become secondarily infected, causing
cellulitis.

Poor prognostic factors for good functional recovery include the following:

Age older than 50 years

Complete facial paralysis


Lack of CN VII nerve excitability

Physical
The primary physical findings in classic Ramsay Hunt syndrome include peripheral facial nerve
paresis with associated rash or herpetic blisters in the distribution of the nervus intermedius. [6]

The location of the accompanying rash varies from patient to patient, as does the area innervated
by the nervus intermedius. It may include the following:

Anterior two thirds of the tongue

Soft palate

External auditory canal

Pinna

The patient may have associated ipsilateral hearing loss and balance problems.

A thorough physical examination must be performed, including neuro-otologic and audiometric


assessment.

Causes

Classic Ramsay Hunt syndrome is ascribed to infection of the geniculate ganglion by herpesvirus
3 (varicella-zoster virus [VZV]).

Differential Diagnoses

Bell Palsy

Persistent Idiopathic Facial Pain

Postherpetic Neuralgia

Temporomandibular Disorders

Trigeminal Neuralgia

Laboratory Studies
The diagnosis of Ramsay Hunt syndrome is usually made without difficulty when the clinical
characteristics are present. If necessary, varicella zoster virus (VZV) may be isolated from
vesicle fluid and inoculated into susceptible human or monkey cells for identification by
serologic means.

WBC count, erythrocyte sedimentation rate (ESR), and serum electrolytes are helpful in
distinguishing the infectious and inflammatory nature of this syndrome.

When CNS complications are suspected (eg, meningitis, meningoencephalitis, myelitis, arteritis
[large and small vessel], and ventriculitis), spinal fluid analysis and CNS imaging studies are
recommended.

Viral studies include the following:

VZV isolation in conventional cell culture is considered the definite diagnostic test.
However, growing VZV in cell culture can be difficult and is usually too slow to be
clinically helpful.

The sensitivity of conventional cell culture is 30-40%, with a specificity of 100%.

Other tests, including Tzanck test, electron microscopy, and polymerase chain reaction
(PCR) are generally more rapid and sensitive. The sensitivity of conventional PCR
technique is estimated to be 60%.

VZV has been detected by PCR in the tear fluid of patients with Bell palsy (prevalence,
25-35%).

VZV antigen detection by direct immunofluorescence assay (DFA) is also possible, with
sensitivity of 90% and specificity close to 99%. [7]

Antibody determinations on paired sera may be helpful in establishing the diagnosis by


comparing titers at time of presentation and a few weeks later.

Imaging Studies

Structural lesions can be ruled out by CT scan, MRI, or magnetic resonance (MR) angiography.

Gadolinium enhancement of the vestibular and facial nerves on MRI has been described in
Ramsay Hunt syndrome.

Recent advances in clinical MRI images (eg, 3-Tesla MRI, multichannel phased array coil, 3-
dimensional fluid-attenuated inversion recovery [FLAIR]) allow the evaluation of subtle
alterations at the level of the blood-labyrinthine barrier. [8]

Other Tests

Audiometry usually reveals sensorineural hearing loss.


Unilateral caloric weakness may be present on electronystagmography (ENG).

Electrodiagnostic methods, such as facial motor nerve conductions studies (electroneurography),


electromyography of facial innervated muscles, the blink reflex, and nerve excitability testing,
could add information regarding the extent of seventh cranial nerve (CN VII) involvement, as
well as prognostic factors. [5]

Procedures

In the setting of a peripheral facial palsy, cerebrospinal fluid (CSF) rarely is analyzed. Although
lumbar puncture is not recommended in the diagnosis of this disease, CSF findings can be
helpful in confirming the diagnosis. In one study, CSF findings were abnormal in 11% of 239
patients with idiopathic peripheral facial palsy, in 60% of 17 patients with Ramsay Hunt
syndrome (abnormal finding was pleocytosis), in 25% of 8 patients with Lyme disease, and in all
8 patients with HIV infection. Thus, if the CSF is abnormal, a specific cause should be sought.

Temporary relief of otalgia in geniculate neuralgia may be achieved by applying a local


anesthetic or cocaine to the trigger point, if in the external auditory canal.

Histologic Findings
See the list below:

The affected ganglia of the cranial nerve roots are swollen and inflamed.

The inflammatory reaction is chiefly of a lymphocytic nature, but a few


polymorphonuclear leukocytes or plasma cells may also be present.

Some of the cells of the ganglia are swollen and others degenerated.

Staging
Several scales have been developed to quantify the degree of facial muscle weakness. Of those,
the House-Brackmann scale is most commonly used. [5]

The House-Brackmann facial neuropathy scale is as follows:

1- Normal

2 - Mild dysfunction (slight weakness only noticeable on close inspection)

3 - Moderate dysfunction (obvious weakness, but not disfiguring differences between


both sides)
4 - Moderately severe dysfunction (obvious weakness and disfigurement)

5 - Only barely perceptive motor function

6 - Complete paralysis

Medical Care

Corticosteroids and oral acyclovir are commonly used in the treatment of Ramsay Hunt
syndrome. In one review, combined therapy using corticosteroids plus intravenous acyclovir did
not show benefit over corticosteroids alone in promoting facial nerve recovery after 6 months.
However, randomized clinical trials evaluating both therapies are required. [9]

Another study concluded that controlled-release oxycodone was safe and generally well tolerated
in patients experiencing acute pain due to herpes zoster. [10]

Vestibular suppressants may be helpful if vestibular symptoms are severe.

As with Bell palsy, care must be taken to prevent corneal irritation and injury.

Temporary relief of otalgia may be achieved by applying a local anesthetic or cocaine to the
trigger point, if in the external auditory canal.

Carbamazepine may be helpful, especially in cases of idiopathic geniculate neuralgia.

Consultations

Consultation with an infectious disease specialist is recommended.

If a structural lesion is discovered on imaging, consultation with a neurosurgeon or


otolaryngologist is recommended.

Consultation with an ophthalmologist to assist with eye care, especially pertaining to the cornea,
may be appropriate.

Medication Summary

Corticosteroids and oral acyclovir are frequently prescribed in patients with Ramsay Hunt
syndrome. Vestibular suppressants may be helpful if vestibular symptoms are severe.
Carbamazepine may be helpful, especially in cases of idiopathic geniculate neuralgia.

Corticosteroids

Class Summary
These agents reduce the inflammation of the cranial nerves and help alleviate the pain and
neurologic symptoms.
Prednisone (Rayos, Deltasone)

View full drug information

May decrease inflammation by reversing increased capillary permeability and suppressing PMN
activity. May be taken during acute inflammatory period (1-2 wk) and then tapered slowly. As an
alternative, Dosepaks (ie, several prepackaged tablets with decreasing doses) can be taken.
Individualize dose based on response.

Antivirals

Class Summary
Acyclovir can be used to combat infection caused by herpesviruses such as VZV.

Acyclovir (Zovirax)

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Patients experience less pain and faster resolution of symptoms when used within 48 h from
onset of symptoms. May prevent recurrent outbreaks.

Anticonvulsants

Class Summary
Mechanism of action of antiepileptics in this syndrome is still unknown. Carbamazepine has
been shown to help the neuralgic pain associated with this syndrome, especially in cases of
idiopathic geniculate neuralgia.

Carbamazepine (Tegretol, Carbatrol, Epitol, Equetro)

View full drug information

DOC that may reduce polysynaptic responses and block posttetanic potentiation. Adjust dose
depending on response to treatment and blood levels.

Antihistamines

Class Summary
These agents prevent histamine responses in sensory nerve endings and blood vessels. They are
effective in treating vertigo.

Meclizine (Medi-Meclizine, Dramamine Less Drowsy, Motion-Time, Travel


Sickness)

View full drug information


Decreases excitability of middle ear labyrinth and blocks conduction in middle ear vestibular-
cerebellar pathways. Associated with therapeutic effects in relief of nausea and vomiting.

Dimenhydrinate (Driminate, Dramamine, Motion Sickness)

View full drug information

A 1:1 salt of 8-chlorotheophylline and diphenhydramine thought to be useful in treatment of


vertigo. Through central anticholinergic activity, diminishes vestibular stimulation and depresses
labyrinthine function.

Anticholinergics

Class Summary
These agents are thought to work centrally by suppressing conduction in the vestibular-cerebellar
pathways.

Scopolamine (Transderm-Scop)

View full drug information

Blocks action of acetylcholine at parasympathetic sites in smooth muscle, secretory glands, and
the CNS. Antagonizes histamine and serotonin action.

Transdermal scopolamine may be most effective agent for motion sickness. Its use in vestibular
neuronitis limited by its slow onset of action.

Further Outpatient Care

After initiation of medical therapy, the patient with Ramsay Hunt syndrome should be seen in
follow-up at 2 weeks, 6 weeks, and 3 months.

Prognosis

In general, prognosis is good for the resolution of symptoms. However, fewer than 50% of
patients have complete recovery of facial function.

Patient Education

The patient should be educated concerning care of the eyes to prevent corneal irritation or injury.

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