Ramsay Hunt Syndrome 

Updated: Jul 16, 2018
Author: Sombat Muengtaweepongsa, MD, MSc; Chief Editor: Niranjan N Singh, MBBS, MD, DM, FAHS, FAANEM 

Overview

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 Man 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. Diminished level of VZV-specific cell-mediated immunity may lead to reactivation of this virus.[4]

Epidemiology

Frequency

Ramsay Hunt syndrome is a rare complication of latent VZV infection.[5] 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.[6] 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.[5]

Ramsay Hunt syndrome is thought to be the cause of as many as 20% of clinically diagnosed cases of Bell palsy.[6]

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%.

 

Presentation

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 VIII)

  • 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.[7, 8]

Ipsilateral hearing loss has been reported in as many as 50% of cases. Vertigo is usually present in those with hearing loss.[9]

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.[10]

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/or vertigo. Hearing impairment is usually more severe in patients with vertigo than in those without vertigo.[11]

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]).

Complications

Rare complications include:

  • Brainstem encephalitis [12]
  • Cranial nerve XII involvement [13]
  • Meningitis [14]
 

DDx

 

Workup

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%.[15]

  • Antibody determinations on paired sera may be helpful in establishing the diagnosis by comparing titers at time of presentation and a few weeks later.

  • VZV DNA in cerebrospinal fluid (CSF) may help to predict degree of nerve damage, however, it is not correlated with clinical outcomes.[16]

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 [3D-FLAIR]) allow the evaluation of subtle alterations at the level of the blood-labyrinthine barrier.[17]  Precontrast hyperintesity and postcontrast enhancement 3D-FLAIR in facial nerve, vestibulococlea nerve and inner ear are correlated with clinical presentation.[18]

Transcranial magnetic stimulation (TMS) may be clinically useful in gaining additional information about the prognostic status of the facial nerve.[19]

Other Tests

Audiometry usually reveals sensorineural hearing loss in high frequency ranges.[11]

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.[6, 20, 21]  

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.[6]  However, the Yanagihara facial nerve grading scale is also clinically helpful.[22]

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

 

The Yanagihara facial nerve grading scale is as follows:

  • At rest 0 - 2 - 4
  • Wrinkle forehead 0 - 2 - 4
  • Close eyes normally 0 - 2 - 4
  • Close eyes forcefully 0 - 2 - 4
  • Close eye on involved side 0 - 2 - 4
  • Wrinkle nose 0 - 2 - 4
  • Blow out cheek 0 - 2 - 4
  • Wristle 0 - 2 - 4
  • Grin 0 - 2 - 4
  • Depress lower lip 0 - 2 - 4

Each function is scored 0 (complete palsy), 2 (partial palsy), or 4 (nearly normal). 

 

Treatment

Approach Considerations

Treatment goals of Ramsay Hunt syndrome are to minimize disability and relieve symptoms. Clinical evidence shows that earlier start of treatment is correlated with better outcomes.[10]

Medical Care

Oral 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.[23]

Evidence from clnical studies indicates that starting treatment in the first week is correlated with the highest rate of improvement, although treatment started later still has some benefit.[10]

Intravenous high-dose methylprednisolone is not commonly used in the treatment of Ramsay Hunt syndrome, however, it may provide some clinical benefit even if administered late.[24]

Another study concluded that controlled-release oxycodone was safe and generally well tolerated in patients experiencing acute pain due to herpes zoster.[25]

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

Medication Summary

Oral 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)

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)

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)

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)

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)

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)

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.

 

Follow-up

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.

Synkinesia, abnormal movements that accompany intended voluntary facial movements, may develop during recovery following Ramsay Hunt syndrome. The occurence rate of synkinesia is higher in Ramsay Hunt syndrome than in Bell's palsy. Up to 45% of patients with Ramsay Hunt syndrome experience synkinesia. Severe facial weakness documented by electroneurography (ENoG) is a predictor for development of synkinesia.[26]

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.

 

Questions & Answers

Overview

What is Ramsay Hunt syndrome (herpes zoster oticus)?

What can be observed in Ramsay Hunt syndrome (herpes zoster oticus)?

Which cranial nerves are involved in the pathogenesis of Ramsay Hunt syndrome?

What syndromes are caused by varicella-zoster virus (VZV)?

What is the main pathophysiological mechanism of Ramsay Hunt syndrome?

What is the prevalence of Ramsay Hunt syndrome?

What disease is thought to be caused by Ramsay Hunt syndrome?

What is the incidence of Ramsay Hunt syndrome among patients with HIV infection?

What is the prognosis of Ramsay Hunt syndrome?

Presentation

What is the presentation of Ramsay Hunt syndrome?

What are the signs and symptoms of Ramsay Hunt syndrome?

What are prognostic factors for poor recovery in Ramsay Hunt syndrome?

What are physical findings characteristic of Ramsay Hunt syndrome?

Where do rashes typically occur in Ramsay Hunt syndrome?

When is hearing impairment more severe in Ramsay-Hunt syndrome?

Which physical exams should be performed in suspected Ramsay Hunt syndrome?

What causes Ramsay Hunt syndrome?

What are complications of Ramsay Hunt syndrome?

DDX

What are the differential diagnoses for Ramsay Hunt Syndrome?

Workup

What is the role of lab testing in the diagnosis of Ramsay Hunt syndrome?

Which viral studies are performed in the diagnosis of Ramsay Hunt syndrome?

What is the role of imaging studies in the diagnosis of Ramsay Hunt syndrome?

What is the role of audiometry, ENG and EDX studies in the diagnosis of Ramsay Hunt syndrome?

What is the role of CSF analysis in the diagnosis of Ramsay Hunt syndrome?

How is otalgia managed in Ramsay Hunt syndrome?

Which histologic findings are characteristic of Ramsay Hunt syndrome?

Which scales are used to assess facial muscle weakness in Ramsay Hunt syndrome?

What are the classifications of facial muscle weakness in Ramsay Hunt syndrome?

Treatment

What are the treatment goals for Ramsay Hunt syndrome?

What are the treatment options for Ramsay Hunt syndrome?

Which medications are used in the treatment of Ramsay Hunt syndrome?

Which specialists should be consulted in the treatment of Ramsay Hunt syndrome?

Medications

Which medications are used for treatment of Ramsay Hunt syndrome?

Which medications in the drug class Anticholinergics are used in the treatment of Ramsay Hunt Syndrome?

Which medications in the drug class Antihistamines are used in the treatment of Ramsay Hunt Syndrome?

Which medications in the drug class Anticonvulsants are used in the treatment of Ramsay Hunt Syndrome?

Which medications in the drug class Antivirals are used in the treatment of Ramsay Hunt Syndrome?

Which medications in the drug class Corticosteroids are used in the treatment of Ramsay Hunt Syndrome?

Follow-up

Following initial medical treatment of Ramsay Hunt syndrome, what monitoring is required?

How frequently does synkinesia develop in Ramsay Hunt syndrome?

What is the prognosis of Ramsay Hunt syndrome?

What information about Ramsay Hunt syndrome should patients receive?