听神经病听力学特征及病损部位分析  

Differential Audiological Assessment of Patients with Auditory Neuropathy

作　　者：王锦玲[1] 石力[1] 薛飞[1] 孙伟[1] 高磊[1] 谢娟[1] 韩丽萍[1] 

机构地区：[1]第四军医大学西京医院耳鼻咽喉头颈外科,西安710032

出　　处：《听力学及言语疾病杂志》2007年第2期89-97,共9页Journal of Audiology and Speech Pathology

摘　　要：目的总结286例听神经病(auditory neuropathy,AN)患者的临床表现和听力学特征,分析听力学检测在AN诊断的意义并探讨其病损部位。方法回顾性分析5年来诊治的286例AN患者的临床表现及听力学特征,分析患者年龄、病程和听力损失程度与纯音听阈、畸变产物耳声发射(DPOAE)之间的关系;讨论AV的诊断和病损部位。结果我院诊治的AN发病率占听力减退患者的1.37%,占感音神经性聋患者的2.49%。大多来自农村和山区。286例AN中单发性148例,并发于周围性、遗传性神经病138例,其中并发前庭神经病124例。男女性别之比为1:1.4。就诊者多为青少年(55.5%),平均20.2岁;发病年龄平均16.9岁;病程平均3.3年。其中277例(96.8%)表现为双耳渐进性听力减退,9例(3.1%)表现为单耳听力减退(单侧听神经病,UAN)。突出主诉为辨不清言语,尤其在嘈杂环境中;部分伴耳鸣、轻度头晕。并发性AN依病因不同可出现下肢无力、行走困难等多种症状。16例有家族遗传史。纯音听力图型以低频减退为主(76.2%),听力损失程度与患者年龄和病程具有相关性;言语识别率不成比例的差于纯音听阈;镫骨肌声反射多引不出,223耳(41%)单个或多个频率可引出阈值升高的非交叉和交叉声反射;530耳(92.8%)ABR自波Ⅰ起未引出,35耳引出Ⅴ波、6耳引出Ⅰ波,部分波幅低或潜伏期延长;DPOAE全部可引出,各频率DP-gram幅值与患者纯音听阈损失程度和病程具有相关性;DPOAE对侧声抑制减弱或消失。9例UAN患者的听功能特征与双侧的基本相同,但单侧6耳的听力图为下降型,以重度及极重度聋多见,DP-gram幅值全频降低,DPOAE健耳对侧声抑制存在,患耳对侧声抑制减弱或消失。结论AN多单独发病,也可并发于外周性、遗传性神经病。多发病于青少年。以辨不清言语,尤其在嘈杂环境中为突出主诉。本组听力学检测符合第Ⅷ颅神经的听支受损引起的听力学特�Objective To conduct differential audiological assessment of 286 auditory neuropathy （AN） patients for the purpose of the evaluation of the diagnosis of AN and the site of lesion. Methods In retrospective, the audiological data of 286 AN patients were carefully reviewed and various parameters analyzed for the identification of important characteristics in relation to AN. The application of the clinical battery of auditory threshold, acoustic immittance, ABR and DPOAE was studied for its role in the diagnosis of the site of lesion. Results Incidence of AN patients was 1.37% in hearing impairment patients and 2.49% in sensorineural hearing loss patients from our hospital. 138 patients were noted to have peripheral and hereditary nerve disorder, and 124 patients were accompanied with vestibular neuropathy in 286 patients identified to have auditory neuropathy. In all the patients, the ratio of both genders was 1 ： 1.40. Most of them were younger（55.5% ）. 277 patients（96.9% ） complained of bilateral gradual hearing loss and 9 patients（3.1% ）complained of unilateral hearing loss（unilateral auditory neumpathy, UAN）. Patients could not discriminate speech correctly, especially in the environment of noise. According to etiological factor, the patients accompartied with other nervous system disease showed varied symptoms. The various finding of the review of the date were presented in this paper. Conclusion A large number of the subjects were noted to suffer from auditory neumpathy alone while some were accompanied with other peripheral and hereditary nerve disorders. By the analyzable studies of the most important audiological characteristic of AN patients, we considered about the main site of lesion was in the cochlear afferent system with the possible involvement of demyelination of auditory nerve. We should be eautious to interpret the markedly abnormal findings of ABR in order to make appropriate diagnosis of the disorder.

关 键 词：听神经病 听力学检测 病变部位 听觉脑干反射弧 

分 类 号：R764.4[医药卫生—耳鼻咽喉科]