Interoculo-irido-dermato-auditive Syndrome （waardenburg）の1症例

A Case of Interoculo-irido-dermato-auditive Syndrome (Waardenburg)

I．緒言

　1951年，オランダのP．J．　Waardenburg氏は第1表に示す如き先天異常から成る症候群を報告した。即ち，本症候群は，1）内眼角および涙点の側方偏位，2）鼻側部眉毛の増生，3）広大な鼻根部，4）虹彩異色症，5）限局性白皮症，殊に前頭部の限局性白毛および6）先天性難聴（聾唖）の6つから成り，従つてInteroculo-irido-dermato—auditive Syndromeと呼ばれている。著者らも最近その1例に遭遇したが，本邦における報告例は極めて少ないようなので，ここに報告したい。

A sporadic occurrence of Waardenburg's syndrome in a nine-year-old girl was reported.

With the exception of white forelock, the syndrome was fully developed : lateral displa-cement of the medial canthi, broad nasal root, hyperplasia of medial portions of the eyebrows,heterochromia of the iris and pigmentary trou-bles similar to those of an albino in the fun-dus of the left eye, deafness, probably conge-nital and perceptive, in the left ear. The inter-ocular dimensions were 40-54-79 mm for the medial canthi, pupils and the lateral canthi respectively.