AUTHOR AND EDITOR INFORMATION

Section 1 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

INTRODUCTION

Section 2 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Background

Acute facial paralysis that occurs in association with herpetic blisters of the skin of the ear canal, auricle, or both is referred to as the Ramsay Hunt syndrome, or herpes zoster oticus. This syndrome also is known as geniculate neuralgia or nervus intermedius neuralgia. Ramsay Hunt syndrome was described first in 1907 by J. Ramsay Hunt in patients 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]).

Pathophysiology

The primary pathophysiology of Ramsay Hunt syndrome is located in the geniculate ganglion of the seventh cranial nerve (CN VII). Classically, Ramsay Hunt syndrome has been associated with VZV infection. Interestingly, VZV has been detected by polymerase chain reaction (PCR) in the tear fluid of patients with Bell palsy (prevalence, 25-35%). Thus, VZV infection may be seen in patients with CN VII paresis without skin lesions (ie, Bell palsy).

Besides deep otalgia on the affected side, the classic Ramsay Hunt syndrome, which always develops after a herpetic infection, also can be associated with vertigo, ipsilateral hearing loss, tinnitus, and facial paresis. These symptoms are due to involvement of the geniculate ganglion, which is located near the petrous pyramid portion of the temporal bone where the ear apparatus is located, and to that of the ganglia of Corti and Scarpa, which are located in the inner ear and involved with hearing and balance. The facial paresis is caused by inflammation of the facial nerve (ie, CN VII), which courses through the inner and middle ear.

The location of the rash varies from patient to patient, as does the area innervated by the nervus intermedius (ie, sensory portion of CN VII). This area may include the anterior two thirds of the tongue, the soft palate, the external auditory canal, and the pinna.

Frequency

United States

Rare (much less common than Bell palsy)

International

Rare

Mortality/Morbidity

Ramsay Hunt syndrome usually is not 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%.

CLINICAL

Section 3 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

History

A careful history must be obtained.

• 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.
• Classic Ramsay Hunt syndrome, which always develops after a herpetic infection, also can be associated with the following:
• • Vertigo and ipsilateral hearing loss
  • Tinnitus
  • Facial paresis
• The patient also complains of rash or blisters in the distribution of the nervus intermedius.
• These herpetic blisters of the skin of the ear canal, auricle, or both may become infected secondarily, causing cellulitis.

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.
• 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 (VZV).

DIFFERENTIALS

Section 4 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Other Problems to be Considered

Adenocystic carcinoma
Carcinoma of the nasopharynx
External otitis
Otitis media
Referred pain
Dental infection or abscess
Labyrinthitis
Vertigo

WORKUP

Section 5 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Lab Studies

• WBC count, erythrocyte sedimentation rate (ESR), and serum electrolytes are helpful in distinguishing the infectious and inflammatory nature of this syndrome.
• Viral studies
• • VZV, the virus responsible for this syndrome, is the causative agent for chickenpox. This agent can be cultured in human cells, where it tends to remain bound to cells.
  • Serologic tests also can reveal VZV, although prior history of chickenpox may lead to a positive result.
  • The diagnosis of VZV usually is made without difficulty when the characteristic rash is present as well as vesicular eruption. If necessary, VZV may be isolated from vesicle fluid and inoculated into susceptible human or monkey cells for identification by serologic means.
  • 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 can be detected by PCR on samples of tear fluid from affected individuals.

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.

Other Tests

• Audiometry usually demonstrates sensorineural hearing loss.
• Unilateral caloric weakness may be present on electronystagmography (ENG).

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

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 also may be present. Some of the cells of the ganglia are swollen and others degenerated.

TREATMENT

Section 6 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Medical Care

• Corticosteroids and oral acyclovir are utilized commonly.
• 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.

Surgical Care

Surgical decompression of the facial nerve has no role in this syndrome.

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

Section 7 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

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

Drug Category: Corticosteroids

These agents reduce the inflammation of the cranial nerves and help alleviate the pain and neurologic symptoms.

Drug Category: Antivirals

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

Drug Category: Anticonvulsants

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.

Drug Category: Antihistamines

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

Drug Name	Dimenhydrinate (Dimetabs, Dramamine)
Description	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.
Adult Dose	50 mg PO/IM q4-6h; 100 mg supp q8h
Pediatric Dose	<2 years: Not established 2-6 years: Up to 12.5-25 mg PO q6-8h; not to exceed 75 mg/24h 6-12 years: 25-50 mg PO q6-8h; not to exceed 150 mg/24h >12 years: Administer as in adults
Contraindications	Documented hypersensitivity; neonates (IV products may contain benzyl alcohol, which has been associated with fatal "gasping syndrome" in premature infants and low-birth-weight infants)
Interactions	Alcohol or other CNS depressants may have additive effect on dimenhydrinate; may mask ototoxic symptoms caused by certain antibiotics, resulting in irreversible damage (use cautiously with these antibiotics)
Pregnancy	B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions	Do not treat severe emesis with antiemetic drugs alone; may contain either sulfites or tartrazine, which may cause allergic-type reactions in susceptible persons; may impede diagnosis of conditions such as brain tumors, intestinal obstruction, and appendicitis; may obscure signs of toxicity from overdosage of other drugs

Drug Category: Anticholinergics

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

FOLLOW-UP

Section 8 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Further Outpatient Care

• After initiation of medical therapy, the patient 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.

REFERENCES

Section 9 of 9

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

• Aizawa H, Ohtani F, Furuta Y, et al. Variable patterns of varicella-zoster virus reactivation in Ramsay Hunt syndrome. J Med Virol. Oct 2004;74(2):355-60. [Medline].
• Aviel A, Marshak G. Ramsay Hunt syndrome: a cranial polyneuropathy. Am J Otolaryngol. Jan-Feb 1982;3(1):61-6. [Medline].
• Byl FM, Adour KK. Auditory symptoms associated with herpes zoster or idiopathic facial paralysis. Laryngoscope. 1976;86:372-379.
• Furuta Y, Ohtani F, Kawabata H, et al. High prevalence of varicella-zoster virus reactivation in herpes simplex virus-seronegative patients with acute peripheral facial palsy. Clin Infect Dis. Mar 2000;30(3):529-33. [Medline].
• Furuta Y, Aizawa H, Ohtani F, et al. Varicella-zoster virus DNA level and facial paralysis in Ramsay Hunt syndrome. Ann Otol Rhinol Laryngol. Sep 2004;113(9):700-5. [Medline].
• Hunt JR. On herpetic inflammations of the geniculate ganglion: a new syndrome of its complications. J Nerv Ment Dis. 1907;34:73-96.
• Kohler A, Chofflon M, Sztajzel R, Magistris MR. Cerebrospinal fluid in acute peripheral facial palsy. J Neurol. Mar 1999;246(3):165-9. [Medline].
• McKenna MJ, Rauch SD. Practical Neurotology. The Practice of Neurosurgery. 1996;Volume I:199.
• Pitkaranta A, Piiparinen H, Mannonen L, et al. Detection of human herpesvirus 6 and varicella-zoster virus in tear fluid of patients with Bell's palsy by PCR. J Clin Microbiol. Jul 2000;38(7):2753-5. [Medline].
• Robillard RB, Hilsinger RL, Adour KK. Ramsay Hunt facial paralysis: clinical analyses of 185 patients. Otolaryngol Head Neck Surg. Oct 1986;95(3 Pt 1):292-7. [Medline].

Article Last Updated: Feb 2, 2007