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Ramsay-Hunt Syndrome

Ramsay-Hunt Syndrome

 

A 24-year-old man, who recently received a diagnosis of HIV infection, presents to the ED with a 24-hour history of left ear pain. He saw his doctor the day before the ED visit and was told that everything looked okay and there was no suggestion of ear infection. Today his pain is worse and his ear is now red and swollen. He also feels dizzy and vomited twice as a result. He describes the dizziness as a “spinning” feeling.

The patient denies fever or feeling faint and also denies any recent scratching or other trauma to the ear. He has not had a recent cold or flu that would lead to a middle ear infection and has not been swimming in over a month. He reports his T-cell count was 240/µL when it was last tested 3 weeks ago and is improving with antiretroviral therapy. He is otherwise healthy.
 
In the ED, the patient is alert, calm, and cooperative, with an obviously red and swollen left ear. His temperature is 97.9°F; blood pressure, 132/87 mm Hg; pulse, initially 124 beats/min, but 83 beats/min after administration of 1 L of saline and 2 Vicodin tabs; respirations, 18 breaths/min. The head and neck examination is remarkable for unilateral nystagmus on rightward gaze and subtle weakness of the left side of the face. Cranial nerves are otherwise intact. There is 1+ erythema and swelling of the entire left auricle with multiple areas of superficial vesicles (Figure). The oropharynx is clear without thrush.

The rest of the physical exam, including his neurologic exam, is unremarkable except for an appendectomy scar, which is well-healed.
 
The diagnosis is Ramsay-Hunt syndrome, a herpes zoster reactivation that affects cranial nerve 7 often with additional involvement of cranial nerve 8, 9, 5, or 6. The treatment is IV acyclovir.

Ramsay-Hunt Syndrome
Ramsay Hunt syndrome is a peripheral facial neuropathy that causes unilateral lower motor neuron pattern facial weakness. It is associated with a painful erythematous vesicular rash of the ear or nearby areas and/or oropharynx. Pain may begin up to 1 week before the onset of the rash. It is caused by reactivation of a dormant varicella (human herpes virus 3) infection in the geniculate ganglion. When cranial nerve 8 is affected, there may also be tinnitus, hearing loss, vertigo, nystagmus, and/or ataxia. Less frequently, cranial nerve 9, 5, or 6 may also be involved.

Treatment is with acyclovir or other appropriate antivirals plus corticosteroids. To prevent corneal damage from exposure, the eye should be taped shut at night and during the day if it does not close completely. An infectious disease specialist should be consulted along with an otolaryngologist if the patient has vertigo or hearing problems.

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