CHARGE associationの1例—難聴および言語発達について

A Case of CHARGE Association:Hearing Loss and Language Development

　はじめに

　CHARGE associationは1981年にPagonら^1）によって提唱された疾患単位で，虹彩毛様帯欠損，心疾患，後鼻孔閉鎖，発育障害，生殖器の低形成，外耳奇形・難聴などを主要症候とするものである。今回われわれはCHARGE associationの1例を経験しその難聴および言語発達に関して考察を加えたので報告する。

CHARGE association is a recently described clus-ter of congenital defects including coloboma, heart disease, atresia choanae, retarded growthand/or retarded development and/or CNS anom-alies, genital hypoplasia, ear anomalies and/or deaf-ness. The authors reported a 4-year-old girl with CHARGE association and discussed especially on her hearing loss and language development. ABR and COR revealed a 40-50 dB hearing loss. CT scan showed middle ear anomalies which explained her hearing loss. We suspected developmental retarda-tion of her language was associated with mental retardation.