Ramsay Hunt Syndrome

For 2 days, an 80-year-old woman had left ear pain and a rash around the ear canal and pinna. She also complained of pain and small papules in the back of her pharynx on the left side. She had no fever or chills and no rash elsewhere on her body. She denied drainage from the ear and previous ear infections or skin eruptions. Several years earlier, she had undergone surgery and chemotherapy for lymphoma but had been well since then.

Rebecca E. Galante, MD, of Hammond, Ind, noted pustules on the pinna and canal of the patient's left ear and blisters in the pharynx characteristic of herpes zoster (A), and she prescribed an oral antiviral agent. Two days later, the patient returned with significant muscle weakness on the left side of her face, involving her forehead, cheek, and chin muscles (B and C). She was unable to close her left eye. Because of a previous ptosis, she had difficulty in seeing without lifting her own eyelid.

Acute facial paralysis that is associated with herpetic blisters of the skin of the ear canal, auricle, or both is referred to as Ramsay Hunt syndrome (or herpes zoster oticus). This condition was first described in 1907 by J. Ramsay Hunt. The primary pathophysiology of this syndrome involves the geniculate ganglion of the seventh cranial nerve.

Symptoms may include otalgia, vertigo, ipsilateral hearing loss, tinnitus, and facial paresis. The characteristic rash may appear on the anterior two thirds of the tongue, the soft palate, the external auditory canal, or the pinna.

Ramsay Hunt syndrome has a complete recovery rate of less than 50%, as opposed to 85% for Bell palsy.

The patient's course was complicated by severe vertigo and vision problems as a result of her ptosis, despite tapering of corticosteroid and antiviral treatment. She underwent facial nerve stimulation and balance retraining. After 5 months, she had nearly fully recovered.