面神经后入路人工耳蜗植入术治疗内耳畸形患儿：一例临床胶囊报告

文献信息

中文摘要

目的： 报告一例内耳畸形且圆窗暴露受限的患儿采用面神经后入路（RFA）行人工耳蜗植入术的技术可行性和早期结果。
方法： 一名3岁患儿，双侧极重度感音神经性听力损失，表现为不完全分隔I型（IP-I）畸形，特征为囊性耳蜗腔、前庭扩大以及外半规管和后 半规管发育不全。术前影像学提示经面神经隐窝圆窗暴露有限。术中评估表明，传统后鼓室切开术对圆窗的暴露有限且呈切线方向。因此，采用RFA行人工耳蜗植入术，并处理了脑脊液井喷。
主要结局指标： 电极插入、术中安全性、术后影像学发现以及早期听觉康复结果。
结果： 实现了电极完全插入。术中遥测反应从若干电极获得，表明神经反应性。术后影像学证实耳蜗内放置。未发生面神经功能障碍或手术并发症。观察到早期听觉反应。
结论： 对于经面神经隐窝圆窗暴露受限的特定IP-I畸形儿科患者，RFA可能是一种有用的替代方法。仔细的影像学分析、术中评估和手术规划仍然至关重要。

英文摘要

OBJECTIVE: To report the technical feasibility and early outcome of cochlear implantation using a retrofacial approach (RFA) in a child with an inner ear malformation and limited access to the round window.
METHODS: A 3-year-old child with bilateral profound sensorineural hearing loss presented with an incomplete partition type I (IP-I) malformation characterized by a cystic cochlear cavity, enlarged vestibule, and hypoplasia of the lateral and posterior semicircular canals. Preoperative imaging suggested limited exposure of the round window through the facial recess. Intraoperative assessment indicated that conventional posterior tympanotomy would provide limited and tangential access to the round window. Cochlear implantation was therefore performed using a RFA with management of a cerebrospinal fluid gusher.
MAIN OUTCOME MEASURES: Electrode insertion, intraoperative safety, postoperative imaging findings, and early auditory rehabilitation outcomes.
RESULTS: Complete electrode insertion was achieved. Intraoperative telemetry responses were obtained from several electrodes, indicating neural responsiveness. Postoperative imaging confirmed intracochlear placement. No facial nerve dysfunction or surgical complications occurred. Early auditory responses were observed.
CONCLUSIONS: The RFA may represent a useful alternative in selected pediatric patients with IP-I malformation when access to the round window through the facial recess is limited. Careful imaging analysis, intraoperative assessment, and surgical planning remain essential.