中耳奇形を伴うKlippel-Feil症候群の1例のアブミ骨手術

Stapes Surgery in a Case of Klippel-Feil Syndrome with Middle Ear Malformation

　はじめに

　全身の骨系統疾患で中耳奇形を合併するものにKlippel-Feil syndrome，oto-palato-digital synd-rome，van der Hoeve's syndrome，Goldenhar'ssyndromeなどが知られている。今回われわれは，中耳奇形を伴うKlippel-Feil症候群の1例に対するアブミ骨手術を経験したので報告する。

Klippel-Feil syndrome is a clinical triad consist-ing of short neck, decreased head mobility, and low occipital hair line. Some patients of this syndromehave been known to have deafness which is mostly sensorineural or mixed. We experienced a case of Klippel-Feil syndrome with conductive hearing loss. The absence of the malleus, the malformation of the incus and the stapes, and the fixation of the foot-plate were observed during surgery of his left ear. We performed stapedotomy with a wire Teflon piston hanging over the chorda tympani nerve. The hearing improvement of 24 dB was achieved. There was no perilymph gusher.