突然聴力悪化をきたしたPendred症候群の1例

A Case of Pendred's Syndrome with Sudden Hearing Aggravation

　I．緒言

　Pendred症候群は，1896年pendrcd^16）が2人の姉妹にlarge goiterとcongenital deafnegssが発生した症例を初めて報告したことに始まっている。それ以来，本症候群の報告は今までに海外では約300例と多数みられているが^15），本邦では意外に少なく，筆者らが調べたところでは約32例しかみあたらない。また本症候群において，突然聴力悪化をきたした例は極めて稀で，岡本ら^14）とBergstrom^2）の2例しか報告されていない。最近筆者らは，このような稀な症例を経験したので，文献的考察を加えて報告する。

　A 12-year-old girl, who had congenital hearing loss of moderate degree in both ears, complained of sudden total hearing loss in both sides with vertigo. She was diagnosed as Pendred's syndrome because of her congenital nerve deafness, goiter and positive perchlorate test. Her left hearing was gradually improved but no improvement in the right ear. The audiometric findings and vestibular examinations revealed the inner ear lesion.

　Sudden hearing aggravation in Pendred's syndrome is very rare and there were only two cases ever reported. The cause of sudden hearing aggravation was suspected as the result of physical stress.