婴幼儿听神经病的诊断干预原则  

Diagnosis and Management for Infants with Auditory Neuropathy

作　　者：黄治物[1,2] 吴皓[1,2] 

机构地区：[1]上海交通大学医学院附属新华医院耳鼻咽喉-头颈外科,上海200092 [2]上海交通大学医学院耳科学研究所听力中心,上海200092

出　　处：《中国听力语言康复科学杂志》2016年第6期401-404,共4页Chinese Scientific Journal of Hearing and Speech Rehabilitation

基　　金：卫生行业科研专项项目(项目编号:201202005);十二五国家科技支撑计划项目(项目编号:2012BAI12B00)

摘　　要：听神经病（auditory neuropathy，AN）是一种近年来逐渐被人们认识的听觉疾病，临床表现特点是听性脑干反应（ABR）缺失或严重异常，耳声发射（OAE）和／或耳蜗微音电位（CM）正常或轻度异常，语言理解困难的特殊听力障碍。本文从AN流行病学。诊断和干预方面进行综述，并集中于婴幼儿以下几方面的诊断干预：①阐明婴幼儿AN诊断有时是很困难的；②许多患者受益于人工耳蜗植入，而助听器的受益较少，③对于听觉输入不足，视觉信息的利用对听觉交流和言语／语言发展是必要的；④患儿应密切随访，随着时间的推移听觉功能可能会发生变化。由于AN通常对婴幼儿有潜在的影响，是言语和语言发展的延迟。因此，婴幼儿尽早明确AN诊断和提供咨询帮助是非常重要的。未来有望对AN提供更精细的评估手段，将有利于干预的实施并带来更好的预期效果。Auditory neuropathy （AN）, known gradually for recent decades,is a disorder characterized by absent or highly abnormal auditory brainstem responses （ABRs）, shown by the presence of otoacoustic emissions （OAEs） and/or cochlear microphonics （CMs）, and deficient speech understanding. This review describes the epidemiology, diagnosis and management of AN. We have focused on the diagnostic pathway and management for infants and young children, because （1）Diagnosis is sometimes difficult, （2）Many patients benefit from cochlear implants; fewer benefit from hearing aids, （3） Without clear auditory input, visual information is needed for auditory communication and speech/language development. （4） Patients should be followed closely, as changes in auditory function may occur over time. It is very important to recognize AN and provide patient counseling and support as early as possible, due to the potentially major influence of infants and children, most often involves delays in speech and language development.This review is a guide to audiologists, speech- language pathologists, and early interventionists who work with individuals diagnosed with AN and/or their families. It highlights the need for more precise tools to describe the disorder in order to facilitate decisions about interventions and lead to better predictions of outcome.

关 键 词：听神经病 听神经病谱系障碍 诊断 干预 听性脑干反应 人工耳蜗植入 

分 类 号：R764.4[医药卫生—耳鼻咽喉科]