Sennaroglu Levent
Department of Otolaryngology, Hacettepe University Medical Faculty, Sihhiye, Ankara, Turkey.
Cochlear Implants Int. 2010 Mar;11(1):4-41. doi: 10.1002/cii.416. Epub 2009 Apr 8.
Inner ear malformations constitute about 20% of congenital sensorineural hearing loss. In this review article an updated classification of cochlear malformations is provided. Incomplete partition and cochlear hypoplasia cases are each divided further into three groups. There are two main difficulties in the surgery of inner ear malformations; gusher and facial nerve abnormalities. Radiological features of malformations necessary to identify these problems preoperatively are discussed. Facial nerve abnormalities that may occur are described. Two different types of cerebrospinal fluid leakage are defined and necessary measures to prevent leakage are described. Standard and modified surgical approaches to overcome the described problems are described with literature findings. Finally meningitis which may occur with and without cochlear implantation in this special group of patients is emphasized. This is common in incomplete partition type I patients and is usually due to a fistula in one of the windows (usually oval window) which occurs as a result of cerebrospinal fluid pressure. This is a medical emergency leading to potential meningitis and measures that should be taken to stop the leak as soon as possible are described.
内耳畸形约占先天性感音神经性听力损失的20%。在这篇综述文章中，提供了一种更新的耳蜗畸形分类方法。不完全分隔和耳蜗发育不全病例各自进一步分为三组。内耳畸形手术存在两个主要难点：脑脊液喷射和面神经异常。讨论了术前识别这些问题所需的畸形的放射学特征。描述了可能出现的面神经异常。定义了两种不同类型的脑脊液漏，并描述了预防漏液的必要措施。结合文献研究结果，描述了克服上述问题的标准和改良手术方法。最后强调了在这一特殊患者群体中，无论是否进行人工耳蜗植入都可能发生的脑膜炎。这在I型不完全分隔患者中很常见，通常是由于脑脊液压力导致的一个窗（通常是椭圆窗）出现瘘管所致。这是一种可能导致潜在脑膜炎的医疗急症，并描述了应尽快采取的阻止漏液的措施。
