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SynopsisHerpes 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. 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 on various parts of the ear, including the auricle, external ear canal, and tympanic membrane. However, some patients may experience vesicle formation preceding development of ipsilateral otalgia and facial paralysis. 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. However, facial paralysis may be chronic unless antiviral with or without simultaneous corticosteroid treatment is initiated within 72 hours of disease presentation. Other symptoms and signs 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. Ramsay-Hunt syndrome is the second most common cause of nontraumatic peripheral facial paralysis, behind Bell palsy (idiopathic facial palsy), and tends to cause more severe paralysis. It has a worse prognosis for facial nerve recovery than Bell palsy, 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.
Related topics: herpes zoster, varicella
B02.21 – Postherpetic geniculate ganglionitis
21954000 – Herpes zoster auricularis
Differential Diagnosis & Pitfalls
- Allergic contact dermatitis
- Bullous fixed drug eruption
- Eczema herpeticum
- Herpes simplex virus infection
- Insect bites
- Irritant contact dermatitis
- Poxviruses (cowpox, monkeypox)
- Pyoderma gangrenosum
- Varicella infection (primary or disseminated)
- Zoster sine herpete (in patients without rash)
- During the prodromal phase, Ramsay-Hunt syndrome may present as Bell palsy with otalgia.
Drug Reaction DataBelow 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.