耳蜗植入在常见类型遗传性耳聋中的应用效果  

Effecacy of Cochlear Implants in Genetic Deafness

作　　者：卿洁[1,2] 彭安全[2] 谢鼎华[1] 刘学忠[1,2] 

机构地区：[1]中南大学湘雅二医院耳鼻咽喉头颈外科,耳科研究所,湖南410011 [2]迈阿密大学米勒医学院耳鼻喉科,美国迈阿密33136

出　　处：《中华耳科学杂志》2013年第2期175-180,共6页Chinese Journal of Otology

基　　金：湖南省"十一五"耳聋患儿人工耳蜗植入项目;国家重点基础研究发展计划(973):2012CB967900和2012CB967904

摘　　要：遗传因素是重度及极重度耳聋的主要病因之一,与人工耳蜗植入后听力言语康复效果密切相关。多种致聋基因常见致聋基因型成功克隆后,致聋基因的致病机理以及其与耳蝸植入效果间的相关性已成为研究热点。目前研究认为耳聋表型可能与致聋病因,螺旋神经节细胞残留数量等有关。通过对耳蜗植入患者的致聋基因突变位点和基因突变方式可阐明影响耳蜗植入效果的相关因素。本文探讨各类遗传性耳聋耳蜗植入效果的当前进展,并着重于耳聋临床表型-基因型的相关性。Genetic factor is one of the most important etiologies in severe to profound sensorineural hearing loss and plays an important role in cochlear implantation outcomes. As pathogenic mutation types of deafness genes have been cloned, the pathogenesis and the relationship between it and the outcomes of cochlear implantation have become a hot research area. The auditory performance is considered to be effected by the number of etiologies of hearing loss, surviving spiral ganglion cells and others. The current research development of cochlear implantation for hereditary deafness, especially regarding the relationship among clinical types, genotypes and outcomes of cochlear implantation, will be discussed in this review article.

关 键 词：遗传性耳聋 耳蜗植入 常见类型 螺旋神经节细胞 基因突变位点 临床表型 基因型 重度耳聋 

分 类 号：R764.43[医药卫生—耳鼻咽喉科] R764.431[医药卫生—临床医学]