内耳畸形在人工耳蜗植入患者中的构成及临床分析

Inner Ear Malformation Among Cochlear Implant Recipients and Relevant Issues

目的分析先天性内耳畸形在人工耳蜗植入患者人群中的构成、分类、电极的选择、术中可能出现的风险及术后并发症。方法回顾性分析2012-2017年行人工耳蜗植入术的604例患者的听力学及影像学资料,根据senuaroglu 2010标准对内耳畸形进行分类,分析不同种类的内耳畸形在人工耳蜗植入患者中的分布、术中可能出现的风险及术后并发症。结果604例人工耳蜗植入患者中,内耳畸形共170例(330耳),占27.32%(330/1208)。其中前庭水管扩大132例(254耳),占先天性内耳畸形的76.97%(254/330),是最常见的先天性内耳畸形。在170例(330耳,单耳畸形10例)内耳畸形患者中,共218耳行人工耳蜗植入手术(其中41例行双侧人工耳蜗植入),4耳伴有面神经走行异常,4耳圆窗龛形态异常,16耳在术中发生“镫井喷”,发生率为7.34%,3耳电极植入困难(其中2耳部分植入,1耳全部植入)。1例大前庭水管综合征患者术后出现对侧听力下降,1例不完全分隔II型患者术后出现切口感染,所有患者术后均未出现脑脊液耳漏,面瘫等其它并发症。结论内耳畸形是先天性感音神经性聋的重要病因之一,大多数内耳畸形患者接受人工耳蜗植入手术具有肯定的疗效。手术前需要进行全面的评估,选择合适的手术入路及植入电极,谨慎处理术中意外,预防并发症。

Objective To report findings of congenital inner ear malformations among cochlear implant patients and relevant issues including electrode selection,intraoperative risks and postoperative complications.Methods Audiological and imaging data of 604 cochlear implantation patients from 2012 to 2017 were retrospectively analyzed.Inner ear malformations were classified based on the 2010 Senuaroglu standard.Distribution of different types of inner ear malformation and related intraoperative risks and postoperative complications were analyzed.Result Of the 604 patients(1208 ears),170(330 ears,27.32%)were found to have inner ear malformation,with enlarged vestibular aqueduct(n=132,254 ears,76.97%)being the most common.Cochlear devices were implanted in 218 of the 330 ears with inner ear malformation(bilateral in 41 cases).Intraoperative findings included facial nerve anomalies(n=4),abnormal round window anatomy(n=4),cerebrospinal fluid"gusher"(n=16,7.34%)and difficulty with electrode insertion(n=3)resulting in partial implantation in 2 ears and complete implantation in 1 ear.In 1 patient with large vestibular aqueduct syndrome,hearing in the contralateral ear deteriorated postoperatively,and postoperative infection occurred in 1 patient with type II incomplete partition.There was no cerebrospinal fluid otorrhea,facial paralysis or complications other than described above.Conclusion Inner ear malformation is one of the most important causes of congenital sensorineural deafness.Preoperative assessment is of critical importance for selecting the appropriate operative approach and electrode.Abnormal intraoperative findings must be carefully managed to prevent complications.