内耳畸形聋儿人工耳蜗植入术常见类型及手术并发症

目的 探讨内耳畸形聋儿实施人工耳蜗植入术时常见的类型及并发症。方法 回顾性分析电子耳蜗植入术病历资料170例，对其中的32例双侧内耳畸形患者加以畸形类型及手术并发症总结。结果 ①人工耳蜗植入患儿内耳畸形所占比例(32/170，18.8%)明显高于其他文献报道；②32例内耳畸形中，大前庭导水管23例(占全部畸形数71.3%)，大前庭导水管伴其他类型畸形者5例(并发Mondini畸形4例，并发外半规管未发育1例),Mondini畸形2例，Mondini畸形并发外半规管未发育前庭腔扩大1例，耳蜗CT影像疑似为“三叉”无法分类1例；③术中发生严重井喷3例(耳蜗CT影像疑似为“三叉”畸形、Mondini畸形并发外半规管未发育前庭腔扩大1例，及大前庭导水管并发Mondini畸形1例)；④耳蜗影像疑似为“三叉”患者，术中发生严重井喷，电极植入困难，4个电极不能植入，术后听力未改善，半年后行对侧耳植入成功；⑤Mondini畸形并发外半规管未发育前庭腔扩大患儿术后半年并发脑脊液耳鼻漏、反复脑膜炎发作，术后1年行手术探查，后治愈。结论 ①人工耳蜗植入常见的内耳畸形包括，大前庭导水管综合征及其相伴发或单发的各类内耳畸形；②内耳畸形非人工耳蜗植入术的绝对禁忌证，但术中严重井喷多见，电极植入不完全多见，术后脑脊液耳鼻漏并发脑膜炎也多发生于畸形耳蜗，术前详细的影像学检查可以对各类畸形进行详细分类，并在术前对手术难度有充分的准备，可以减少相关并发症的发生。

Analysis of the common types of inner ear malformations and the surgical complications in deaf children with cochlear implants

Objective To investigate common types of inner ear malformation and the surgical complicationsin deaf children with cochlear implants. Methods The clinical data of 170 cases of cochlear implantation were retrospectively analyzed. The type of 32 patients with bilateral inner ear malformation and the surgical complications were analyzed. Results ① The proportion of inner ear malformations is higher than other literature reports(32/170;18.8%)； ② In the 32 cases of inner ear malformations, there were 23 cases of large vestibular aqueduct(71.3% of the total number of malformations), 5 cases of large vestibular aqueduct with other innerear deformity (4 cases complicated by Mondini malformation and 1 case complicated by nondevelopment of lateral semicircular canal), 2 cases of Mondini deformity, and 1 case of mondini deformity complicated by lateral semicircular canal not developing and enlarged vestibular cavity, 1 case CT images of the cochlea was considered “trigeminal shape”; ③ 3 cases had cerebrospinal fluid(CSF)gusher during surgery. 1 case of the cochlea was considered “trigeminal shape” by CT imaging, 1 case of Mondini deformity complicated by lateral semicircular canal not developing and enlarged vestibular cavity, and 1 case of large vestibular aqueduct complicated by Mondini deformity; ④ The patient with cochlea imaging“trigeminalshape”occurred CSF gusher during surgery, and four pairs of electrodes could not be implanted. Hearing recovery was not ideal after surgery; ⑤The patient with Mondini deformity complicated by lateral semicircular canal not developing and enlarged vestibular cavity was followed cerebrospinal fluid leakage, repeated episodes after six months, and was cured after surgical exploration after 1 year. Conclusion ① The common deformity of cochlear includes large vestibular aqueduct syndrome and the inner ear malformation accompanies. ② Cochlear implantation in these inner ear deformities is not absolutely contraindicate, but serious CSF gusher is common during surgery. And totally electrodes implantation is difficult, cerebrospinal fluid leakage complicated after surgery is often occurred in deformity cochlears. Detailed imaging examination before surgery can reduce the incidence of complications.