先天性顔面神経麻痺に同側高度感音難聴，前庭機能廃絶，内耳道狭窄を伴った1症例

Congenital Facial Palsy with Inner Ear Malformation: A Case Report

I．はじめに

　先天性顔面神経麻痺に同側高度感音難聴，半規管機能障害を伴い，X線学的に中耳，蝸牛，前庭，半規管に形態学的異常なく，内耳道が高度に狭窄していた症例を経験し，文献考察よりこの奇形の発生に母体の糖尿病がなんらかの関係があると考えられたので報告する。

　A boy, aged 17, has been recognized facial palsy on the right side at birth. EMG showed electrical silence, and the facial muscles were not responded to maximal stimulation.

　Facial nerve function test revealed absence of lacrimation, taste sensation and stapedial reflex on the ipsilateral side. Audiogram showed profound deafness on the ipsilateral ear and cold caloric stimulation by ice water did not produce any nystagmus.

　Eye movement was full in all directions. Roentgenogram of the temporal bone showed a narrowed internal auditory meatus.

　Maternal diabetes mellitus may be the causative factor of these abnormalities.