大前庭水管综合征患儿人工耳蜗植入术8例临床分析

目的评价大前庭水管综合征患者人工耳蜗植入术的安全性和效果。方法大前庭水管综合征患者8例为内耳畸形组，其中语前聋6例，语后聋2例，以8例耳蜗发育正常的植入者作为对照组，进行手术及术后效果对比。结果8例患者人工耳蜗电极植入顺利，7例耳蜗底回开窗时发生井喷，迅速用颞肌筋膜牢固封闭开窗处制止井喷。全部患者术后未出现脑脊液漏、颅内感染、面瘫、中耳炎等并发症，畸形组与对照组术中和术后并发症差异无显著性意义。术后听阈畸形组多数患者达到30～45dBHL，与内耳正常对照组相似，经统计学分析差异无显著性（P〉0．05），听觉语言康复效果畸形组多数与对照组接近。结论大前庭水管综合征患者在人工耳蜗植入术中易发生井喷，术中可控，术后未出现并发症，所观察患者术后听力及语言康复效果满意，因此，大前庭水管综合征患者行人工耳蜗植入术是安全有效的。

Clinical analysis of cochlear implantation in 8 patients with large vestibular aqueduct syndrome

Objective To evaluate the intraoperative safe and post-operative effect of cochlear implantation in children with large vestibular aqueduct syndrome.Methods 8 patients with large vestibular aqueduct syndrome(cochlear malformation group)received cochlear implantation.The postoperative effect of 8 cases with cochlear malformation were compared with 8 cases with norma lcochlea( control group).Results Via cochleostomy pulsatile clear fluid gusher occurred in 7 cases,which were easily controlled by quickly inserting the electrode array and sealing the cochleostomy with muscle tissue in each instance.The implant was inserted without difficulty in all patients.There have been no post-operatively complications,Post-operative hearing threshold of most cases were 30 ～45 dBHL,which similar with normal cochlea.There was no statistically significan difference between the group of malformation and the control group( P ＞ 0.05),most of them received the similar hearing and speech recovering benefit from cochlear implantation opatients with normal inner ears.Conclusion Despite pulsatile clear fluid gusher in cochlear implantation,no post-operatively complications have been found,and the outcome of these 8 patients with cochlear implantation was excellent.Cochlear implantation can be safely and effectively performed in patients with profound sensorineural hearing loss associated with large vestibular aqueduct syndrome