A Case of Sensory-Neural Hearing Loss due to Kearns-Shy Syndrome
1984 Volume 27 Issue 1 Pages 57-63
Details
1984 Volume 27 Issue 1 Pages 57-63
A 35-year-old woman with Kearns-Shy syndrome first noted a hearing loss at the age of 32, and the first hearing test was perfomed at age of 35.
A pure tone audiometry showed a moderate sensorineural hearing loss in the right ear and a severe sensorineural hearing loss in the left. Békésy audiogram showed Jerger's type II pattern. Auditory brainstem responses were difficult to detct in the right ear because of involuntary movement, but latency and threshold of wave V showed fairly good relationship with the pure tone audiogram in the right. CM, SP and AP findings obtained by E. Co. G. showed the same relationship with the results of pure tone audiometry.
After 10 months, her right hearing loss suddenly progressed and E. CoG. findings showed severe cochlear damage. It was determimed that the right cochlea might be damaged by Kearns-Shy syndrome.
