併有鐙骨足板缺損的Mondini氏內耳發育不良—病例報告

Mondini Dysplasia Associated with Defects of the Stapedial Footplate —Case Report

顏士爵(Shih-Chen Yen) ； 許權振(Chuan-Jen Hsu) ； 林凱南(Kai-Nan Lin)

《中華民國耳鼻喉科醫學會雜誌》 29卷6期 (1994/12) Pp. 532-536

https://doi.org/10.6286/1994.29.6.532

摘要

Mondini氏內耳發育不良，常併有骨迷路外壁及內壁婁管，其外壁婁管以鐙骨足板的缺損最常見，而內壁婁管則常位於內聽道底部與前庭之間，或因果常開放的耳蝸導水管所致。鐙骨足板缺損的原因可能是足板發育不良，或迷路增大的壓力侵蝕鐙骨足板而引起。本院耳鼻喉部最近經歷2例Mondini氏內耳發育不良併腦脊髓液外漏之病童，於手術時移除其鐙骨，發現鐙骨足板均有缺損。手術時，利用脂肪組織填塞前庭或中耳，以阻止腦髓外流，對於處理腦脊髓液外漏，可避免經由顱內探查之危險，為有效且較安全之方法。

關鍵字

Mondini氏內耳發育不良 ； 腦脊髓液耳漏

並列摘要

Incomplete development of the bony and membranous labyrinth has been noted in Mondini dysplasia. In this anomaly, fistula of the outer wall of the bony labyrinth may be found at the stapedial footplate, and fistula of the inner wall may be at the fundus of the internal auditory canal. The pathogenesis of the defect of stapedial footplate may be due to developmental abnormality and/or the effect of erosion caused by increased inner ear pressure. The CSF leakage may be stopped by obliterating the vestibule with fat. Two cases of Mondini dysplasia with such defects were reported.

並列關鍵字

Mondini dysplasia ； CSF otorrhea