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Ramsay-Hunt syndrome - Skin
See also in: External and Internal Eye
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Ramsay-Hunt syndrome - Skin

See also in: External and Internal Eye
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Contributors: Benjamin R. Bohaty MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
Other Resources UpToDate PubMed

Synopsis

Herpes zoster oticus is a viral infection characterized by herpetic eruption of the auricula and external ear canal and, when associated with facial palsy, is known as Ramsay-Hunt syndrome. The incidence of Ramsay-Hunt syndrome is about 5 cases per 100,000 of the US population annually, with a significantly increased incidence in those older than 60 years of age. It rarely occurs in children. The syndrome is caused by the reactivation of latent varicella-zoster virus (VZV) in the geniculate ganglion (the sensory ganglion of the facial nerve), which affects the seventh and eighth cranial nerves. Reactivation may be triggered by immunosuppression, certain medications, other infections, or different forms of physical or emotional stress. 

Ramsay-Hunt syndrome generally starts with a 1- to 3-day prodrome of otalgia and ipsilateral facial paralysis followed by development of herpetic vesicles on the face just anterior to the tragus and various parts of the ear including the auricle, external ear canal, and tympanic membrane. Vesicles may also appear on the mouth and anterior two-thirds of the tongue. Evolution of disease may differ between age groups; in children, the vesicles may appear several days after the facial paralysis. Over the course of a week, the vesicles become pustules, and then ulcerate to form crusts. The lesions heal within weeks and may result in scarring. Other symptoms can include tinnitus, hearing loss, vertigo, hyperacusis, nystagmus, nausea, vomiting, taste impairment, lesions of the oral mucosa, dry mouth, and dry eyes. Cerebrovascular accidents have been associated with zoster.

Diagnosis is primarily clinical and is based on a combination of facial palsy, otalgia, and vesicles on the pinna or oral mucosa. Treatment is empiric and involves the use of high-dose corticosteroids and antiviral therapy. Ramsay-Hunt syndrome is less common than Bell's palsy (idiopathic facial palsy), tends to cause more severe paralysis, and has a worse prognosis for facial nerve recovery (especially in children). Only 10% of patients with complete facial paralysis are totally cured. Prognosis for associated hearing loss is very good, with only around 5% of patients having residual impairment.

Codes

ICD10CM:
B02.21 – Postherpetic geniculate ganglionitis

SNOMEDCT:
21954000 – Herpes zoster auricularis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 09/04/2014
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Ramsay-Hunt syndrome - Skin
See also in: External and Internal Eye
Print 7 Images
View all Images (7)
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Ramsay-Hunt syndrome : Ear, Face, Hearing loss, Neck pain, Pain out of proportion to exam findings, Tinnitus, Pruritus, Umbilicated vesicles
Clinical image of Ramsay-Hunt syndrome
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