Understanding auditory neuropathy spectrum disorder： a system- atic review in transgenic mouse models  

作　　者：Li Wang Jing Guan Hongyang Wang Lan Lan Qiujing Zhang Liang Zong Wan Du Wenping Xiong Fengjiao Li Kaiwen Wu Dayong Wang Qiuju Wang 

机构地区：[1]Institute of Otolaryngology Chinese PLA General Hospital, Medical School of Chinese PLA, Beijing 100853, China [2]Medical College, Nankai University, Tianjin 300071, China

出　　处：《Science China(Life Sciences)》2016年第5期480-486,共7页中国科学（生命科学英文版）

基　　金：supported by the National Key Basic Research Program of China (2014CB943001);the National Natural Science Foundation of China (81120108009, 81530032)

摘　　要：Auditory neuropathy spectrum disorder is a unique group of hearing dysfunctions characterized by preserved outer hair cell function and abnormal neural conduction of the auditory pathway. However, the pathogenic mechanism underlying this disorder is not clear. We therefore performed a systematic review of genetic mouse models with different gene mutations to provide a valuable tool for better understanding of the process and the possible molecular mechanisms. Of the 18 articles retrieved, nine met the required criteria. All biochemical, histological, and electrophysiological results were recorded for each of the mouse models, as was the transgenic technology. This review provides a summary of different mouse models that may play an important role in the diagnosis and management of auditory neuropathy spectrum disorder in the future.Auditory neuropathy spectrum disorder is a unique group of hearing dysfunctions characterized by preserved outer hair cell function and abnormal neural conduction of the auditory pathway. However, the pathogenic mechanism underlying this disor- der is not clear. We therefore performed a systematic review of genetic mouse models with different gene mutations to provide a valuable tool for better understanding of the process and the possible molecular mechanisms. Of the 18 articles retrieved, nine met the required criteria. All biochemical, histological, and electrophysiological results were recorded for each of the mouse models, as was the transgenic technology. This review provides a summary of different mouse models that may play an important role in the diagnosis and management of auditory neuropathy spectrum disorder in the future.

关 键 词：auditory neuropathy spectrum disorder transgenic mouse models PATHOGENESIS systematic review 

分 类 号：R764.4[医药卫生—耳鼻咽喉科] R-332[医药卫生—临床医学]