遺伝性一側難聴

Hereditary Unilateral Deafness

I．緒言

　いわゆる若年性一側感音難聴の原因については明らかでない部分が多いが，過去のいくつかの報告によってその少なくとも一部分は家族性ないし遺伝性であることが示されている。しかしこの遺伝性の一側性難聴の臨床像についてはあまり明らかにされていない。著者らは本疾患の家族例について自験例および文献例を合わせて検討した。

　In the cases of unilateral sensorineural deafness, there were 4 familial cases (8.4%). In these 4 families and the families reported in the literature, autosomal dominant inheritance was presumed. Assuming the penetrance rate of 51%, segregation ratio calculated by the singles method agreed with the hypothesis.

　Audiograms of family members of probands suggested that the phenotype varied remarkably, namely in the one extreme it was unilateral mild hearing impairment and in the other extreme it was bilateral profound deafness.