内耳发育畸形的人工耳蜗植入术

The inner ear malformations of cochlear implantation

目的探讨对内耳发育畸形患儿行人工耳蜗植入的手术疗效及可行性。方法回顾性分析武汉大学人民医院于2000年1月~2014年1月期间行人工耳蜗植入术的43例(43耳)内耳发育畸形患儿的临床资料,包括大前庭水管综合征(LVAS)37例(37耳),Modini畸形6例(6耳)。随机选择临床资料相匹配43例(43耳)耳蜗结构正常的人工耳蜗植入患儿作为内耳发育正常组。分析并比较两组患儿的术前听力、术中情况以及术后听觉言语能力恢复情况。结果所有患儿人工耳蜗植入术后均获得有意义的听觉。5例(5耳)LVAS患儿术中出现严重井喷现象,20例(20耳)LVAS患儿耳蜗钻孔后出现外淋巴液不同程度波动,2例(2耳)Modini畸形患儿出现严重井喷现象,其余病例(36耳)及内耳发育正常组(43耳)术中及术后均未出现严重并发症。在同一康复阶段,两组患儿的听觉言语能力比较,差异无统计学意义(P>0.05)。同组内,术后3、6、9、12、24个月的听觉言语能力较术前均有明显提高(P<0.05)。结论内耳发育畸形增加了人工耳蜗植入的手术难度,且术中易出现井喷,但内耳发育畸形患儿并不是人工耳蜗植入的绝对禁忌证,对LVAS、Modini畸形患儿,人工耳蜗是治疗的重要手段。

Objective To explore the surgical efficacy and the operation feasibility to the cochlear implantation in children with abnormal inner ear structure. Methods The clinical data of 43 children with abnormal inner ear structure were analyzed retrospectively, who had cochlear implantation in Renmin Hospital of Wuhan University from January 2000 to January 2014 and were selected as abnormal inner ear structure group, including 37 cases （37 ears） with large vestibular aqueduct syndrome （LVAS） and 6 cases （6 ears） with Modini deformity. And 43 cases with no inner ear malformation were randomly selected as normal inner ear structure group. The preoperative hearing, intraoperative condition and postoperative auditory verbal ability of the two groups were compared. Results All patients received meaningful auditory after cochlear implantation. Serious gusher were happened in 5 cases with LVAS and 2 cases with Modini deformity. Perilymph fluids were fluctuated at different levels in 20 （20 ears） cases with LVAS when drilling the cochlear. The remaining cases in the abnormal inner ear structure group and all cases in the abnormal inner ear structure group did not appear serious intraoperative and postoperative complications. At the same recovery time, there was no differ- ence in the auditory verbal ability of the two groups （P 〉 0.05）. Postoperative 3, 6, 9, 12, 24 months, the auditory ver- bal ability was higher than that before operation at the same group （P 〈 0.05）. Conclusion The inner ear malformations increase the difficulty of operation, with the high risk of gushers. While the inner ear malformations are not absolute contraindieation, cochlear implantation is an important means of treatment to patients with LVAS and Modini deformity.