新疆地区255例重度-极重度感音神经性耳聋患儿的内耳畸形临床分析

  目的  探讨重度-极重度感音神经性耳聋患儿的内耳畸形的影像及手术特点，从而更好地为人工耳蜗植入提供临床指导。   方法  调取新疆维吾尔自治区人民医院2020年1月—2021年12月筛查并行人工耳蜗植入术的内耳畸形患儿的颞骨高分辨率CT资料，根据Sennaroglu的分类方法对其进行分类，依次为Michel畸形、耳蜗未发育、共同腔畸形、耳蜗发育不良(Ⅰ型、Ⅱ型、Ⅲ型)、不完全分隔Ⅰ型(IP-Ⅰ)、Ⅱ型(IP-Ⅱ)、Ⅲ型(IP-Ⅲ)、前庭及半规管畸形、前庭导水管扩大及内听道狭窄等，并记录人工耳蜗植入径路、电极类型及并发症，分别分析各类畸形的影像特点及手术注意事项。   结果  255例重度-极重度感音神经性耳聋患者中有66例(125耳)内耳畸形患者，其中，IP-Ⅱ型占内耳畸形的30.30%(20例/66例)，单纯前庭导水管扩大占19.70%(13例/66例)，其他类型比例较低。共55例(57耳)内耳畸形患儿接受了人工耳蜗植入手术，双侧植入2例。所有畸形病例均鼓阶开窗或圆窗植入电极。耳蜗发育不良(Ⅱ型、Ⅲ型)、IP-Ⅰ和IP-Ⅲ型选择短的直电极，IP-Ⅱ型、前庭导水管扩大、内听道扩大者选择标准电极。1例IP-Ⅱ患者术后CT提示植入前庭，二次手术植入耳蜗。“井喷”发生率为29.82% (17耳/57耳)。无脑脊液耳漏、皮瓣坏死等并发症。   结论  内耳畸形中IP-Ⅱ和大前庭导水管(LVA)两型占主导，内耳畸形患儿人工耳蜗植入术中“井喷”发生率高，高分辨率CT与MRI互补，可清晰、全面地显示内耳结构，为人工耳蜗植入手术的顺利实施提供保障。 

Inner ear malformation among 255 cases with severe to extremely severe sensorineural hearing loss in Xinjiang and relevant issues

  Objective  To investigate the image and surgical characteristics of inner ear malformation in children with severe to extremely severe sensorineural hearing loss, so as to provide better clinical guidance for cochlear implantation.   Methods  From January 2020 to December 2021, children admitted to People's Hospital of Xinjiang Uygur Autonomous Region for screening and cochlear implantation were examined by high-resolution CT of temporal bone and the cases of inner ear malformation were screened out. According to Sennaroglu ' s classification method, they were classified as follows: Michel malformation, cochlea aplasia, common cavity, cochlear hypoplasia types Ⅰ, Ⅱ and Ⅲ, incomplete partition types Ⅰ-Ⅲ (IP-Ⅰ, IP-Ⅱ and IP-Ⅲ), vestibular and semicircular canal malformation, large vestibular aqueduct and internal auditory canal stenosis. Cochlear implantation approaches, electrode types and complications were recorded, and the image characteristics and surgical precautions of various malformations were analysed.   Results  Among 255 patients with severe to extremely severe sensorineural hearing loss, 66 cases (125 ears) had inner ear malformation, in which IP-Ⅱ accounted for 30.30% (20 cases/66 cases) of inner ear malformation cases, large vestibular aqueduct accounted for 19.70% (13 cases/66 cases) and other types were relatively low. A total of 55 children (57 ears) with inner ear malformation received cochlear implantation (CI), including two bilateral CI cases. All cases had been implanted with electrodes through a cochleotomy or round window. Short straight electrodes were implanted in patients with cochlear hypoplasia types Ⅱ and Ⅲ, IP-Ⅰ and IP-Ⅲ. Standard electrodes were implanted in patients with IP-Ⅱ, large vestibular aqueduct and enlarged internal auditory canal. Postoperative CT scans suggested one IP-Ⅱ case were implanted into vestibular cavity, so this case received another operation by cochlear implantation successfully. The incidence of cerebrospinal fluid "gusher" was 29.82% (17 ears/57 ears). There was no complications such as cerebrospinal fluid otorrhea and flap necrosis.   Conclusion  IP-Ⅱ and LVA are the predominant types of inner ear malformations. The incidence of cerebrospinal fluid "gusher" in CI is high in children with inner ear malformation. HRCT and MRI are complementary, which can clearly and comprehensively display the inner ear structure and provide guarantee for cochlear implementation successfully.