Kearns-Shy Syndrome: Report of a Case

A 52-year-old woman with Kearns-Shy syndrome was reported in regard to audiometric findings in her clinical course of five years' duration. The patient first noted a hearing loss at the age of 41, and it was slowly progressive. The first hearing test was performed at the age of 47, and a pure tone audiogram showed a moderate sensorineural hearing loss bilaterally, which was formed rather prominent above 1000Hz. At the age of 49, a pure tone audiometry revealed deterioration of bilateral hearing only at 250Hz and 500Hz. For the last three years, her hearing remained unchanged. Speech discrimination was extremely poor. Békésy audiogram showed type II tracing according to the Jerger's classification. The contralateral stapedial reflexes could not be elicited for the pure tone signal (125dB HTL) at 500, 1000, 2000, 4000Hz. Although the lesion of the hearing loss was not determined, the author suggested that the hearing loss of this syndrome might result from cochleosaccular degeneration.