前庭水管扩大综合征患者的人工耳蜗植入术

目的 评价前庭水管扩大综合征患者人工耳蜗植入术的安全性和可行性。方法 1995年5月1日～2002年6月1日因双耳重-极重度感音神经性聋在北京协和医院接受人工耳蜗植入术的患者312例中诊断为双耳前庭水管扩大者10例(3．2％)，其中语前聋7例，语后聋3例。回顾性分析这10例患者的临床资料。结果 10例患者人工耳蜗电极植入顺利，8例耳蜗底回开窗时发生轻度井喷，迅速用颞肌筋膜牢固封闭圆窗制止井喷。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。开机6个月时8例患者具有开放性言语识别力，已进入普通学校(幼儿园、小学、大学)就读。另2例语前聋的幼儿视觉强化测听听阈达40dB HL，与其他无内耳畸形的全聋儿童术后效果差异无显著性。全聋前语言能力较好的5例患者术后语言能力明显好于语前聋的患者，语言交流基本听不出聋人特有的语音特征。另5例患者语言均有不同程度的进步。结论 尽管前庭水管扩大患者在人工耳蜗植入术中可能出现井喷，但术后未出现并发症且听力-言语康复效果好，因此重-极重度聋的前庭水管扩大综合征患者行人工耳蜗植入术是安全可行的。

Implication of cochlear implantation in patients with enlarged vestibular aqueduct syndrome]

OBJECTIVE: To evaluate the intraoperative obstacles and post-operative outcomes of cochlear implantation in children with enlarged vestibular aqueduct syndrome. METHODS: Between May 1995 and June 2002, 10 patients with enlarged vestibular aqueduct syndrome received cochlear implantation in Pekin Union Medical College Hospital. The clinical records of 10 cases of enlarged vestibular aqueduct syndrome were analyzed retrospectively. RESULTS: Via cochleostomy pulsatile clear fluid gusher occurred in 8 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, and audiologic outcome of the 10 patients with cochlear implantation were almost the same as the other patients without inner ear malformations. Five cases of them can even speak better than the other prelingual deafness that may be attributed in part to postlingual deafness. CONCLUSIONS: Despite pulsatile clear fluid gusher in cochlear implantation, no post-operatively complications have been found, and the outcome of these 10 patients with cochlear implantation was excellent. Cochlear implantation can be safely and effectively performed in patients with profound sensorineural hearing loss (SNHL) associated with enlarged vestibular aqueduct syndrome.