Acute Profound Deafness in Ramsay Hunt Syndrome: Two Case Reports

Abstract

Two patients with sudden progressive profound hearing loss resulting from Ramsay Hunt syndrome are reported. Case 1: A 63-year-old woman was admitted to Jichi Medical School Hospital with sudden, progressing deafness of the left ear, vertigo, sore throat, and hoarseness. An otoscopic examination revealed the external ear and the tympanic membrane to be normal. Pure-tone audiometry revealed profound deafness in the left ear. A horizontal nystagmus in the non-affected direction was observed by gaze nystagmus test. An endoscopic examination revealed herpetic vesicles and shallow ulcers on the left side of the pharynx and the larynx. There was complete paralysis of the left recurrent nerve. Hearing acuity of the left ear did not recover at all with steroid hormone therapy. Case 2: A 75-year-old man was referred to the ENT Clinic by a dermatologist for hearing evaluation in Ramsay Hunt syndrome. The man had noticed severe otalgia and sudden progressive deafness of the right ear approximately 2 weeks prior to admission. Physical examination revealed herpetic vesicles and ulcers in the right external ear and lateral neck. Complete paralysis of the right facial nerve was noted. Profound hearing loss in the affected ear was observed by pure-tone audiometry. A gaze nystagmus test revealed a horizontal nystagmus in the non-affected direction. No recovery of the cochlear function was noted following administration of antiviral drug. The pertinent literature is briefly reviewed.

Key Words:

• recurrent nerve palsy
• facial nerve palsy
• sudden deafness
• Ramsay Hunt syndrome