10代半ばに聾となった両側性進行性感音難聴の3兄弟例

Three Brothers with Bilateral Non-syndromal Pro-gressive Sensorineural Hearing Loss

　はじめに

　感音難聴の発症に関して遺伝的要因の関与は少なくないと推定される。

　今回われわれは，血族結婚による常染色体劣性遺伝の関与が疑われる，両側性進行性感音難聴の結果，早期に失聴した3兄弟例を経験した。Prom-ontory testの結果などより人工内耳の適応があると判断されたので報告する。

Three brothers presented with bilateral and non-syndromal progressive deafness in early teenage. Their parents with normal hearing were cousins, and have four children including 3 sons and a daugh-ter with normal hearing. Three sons had hearing impairment, which were first noticed at their early teenage, aggravated to total deafness after a few years. Etiology of their hearing impairment was suspected due to autosomal recessive transmission. Promontory testing revealed a sensory deafness in the cochlea because a sound sensation was obtained. Then, cochlea implantation surgery will be indicat-ed in these brothers.