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与鳄鱼泪和耳部畸形相关的杜安综合征。一例镫骨异常及其重建病例。

Duane's syndrome associated with crocodile tear and ear malformation. A case of stapes anomaly and its reconstruction.

作者信息

Tachibana M, Hoshino A, Oshima W, Nishimura H, Mizukoshi O

出版信息

Arch Otolaryngol. 1984 Nov;110(11):761-2. doi: 10.1001/archotol.1984.00800370063017.

Abstract

Duane's syndrome is a congenital eye movement disorder characterized by marked limitation or absence of abduction, variable limitation of adduction, and narrowing of the palpebral fissure with retraction of the globe on attempted adduction. We treated a patient with bilateral Duane's syndrome associated with crocodile tear and external, middle, and inner ear anomalies. Exploratory tympanotomy showed an absence of the oval window and a rudimentary superior structure of the stapes that was connected to the abbreviated incudal lenticular process with connective tissue. A small hole was drilled into the vestibulum and the ossicular chain was reconstructed by inserting a Teflon wire piston to minimize the air-bone gap. Pertinent literature is reviewed, and the pathogenesis of Duane's syndrome and the aspect of concomitant anomalies are discussed.

摘要

杜安综合征是一种先天性眼球运动障碍,其特征为外展明显受限或缺失、内收可变受限,以及在内收尝试时睑裂变窄并伴有眼球后缩。我们治疗了一名患有双侧杜安综合征并伴有鳄鱼泪以及外耳、中耳和内耳异常的患者。探查性鼓室切开术显示卵圆窗缺失,镫骨上结构发育不全,该结构通过结缔组织与缩短的砧骨豆状突相连。在前庭钻一个小孔,并通过插入一根特氟龙丝活塞重建听骨链,以尽量减小气骨间隙。回顾了相关文献,并讨论了杜安综合征的发病机制及伴随异常情况。

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