Retinitis pigmentosa and deafness.
Seventeen patients with retinitis pigmentosa (RP) have been investigated audiologically. Of 9 found to have a significant hearing loss, 6 were examples of Usher's syndrome; these patients had a cochlear pattern of hearing loss. The other 3 were examples of Senior's syndrome, Kearne-Sayre syndrome and Lawrence-Moon-Biedle syndrome respectively. Two of these patients had absent stapedius reflexes. It is suggested that patients with different RP-deafness syndromes may have lesions in different parts of the auditory system.
D000293:Adolescent; D000328:Adult; D000368:Aged; D001301:Audiometry, Pure-Tone; D002648:Child; D004423:Ear / Q000503:physiopathology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D012022:Reflex, Acoustic; D012174:Retinitis Pigmentosa / Q000150:complications*