摘要
目的
听神经病(auditory neuropathy，AN)是1996年Starr首次命名，由于第Ⅷ脑神经的听神经支受损而引起的一种临床表现特殊的感音神经性听力损失。听性脑干反应(auditory brainstem responses，ABR)缺失或严重异常，而诱发性耳声发射(evoked otoacoustic emissions，EOAE)正常，是听神经病最重要的听力学特征。这一组不同于一般感音神经性聋、具有特征的听功能障碍，近年来已引起越来越多的关注。目前对其命名、病变部位意见不一，病因及发病机制尚不清楚，治疗无十分有效措施。本课题旨在总结听神经病的临床表现特点和听力学特征，探讨纯音听阈测试、声导抗测试、ABR和畸变产物耳声发射(distortion product otoacoustic emissions，DPOAE)在听神经病诊断中的意义，调查听神经病并发前庭功能障碍及其他神经系统疾病的情况，探讨听神经病与其他神经系统疾病之间的关系。
方法
回顾性调查分析2000-12～2003-04我科确诊为听神经病的患者134例，其中包括我院神经内科诊断为神经系统遗传病且同时合并听神经病的
第四军医大学项士学位论义
患者4例。总结这些患者的临床资料、听力学测试及相关电生理检查结果，
并与一般感音神经性聋患者 30例（49耳）、听力正常青年志愿者 30例（60
耳）的听力学测试结果比较。
结果
一、对象及临床表现 134例听神经病患者男48例，女86例，男女
性别比为1：1．79。患者大多来自农村，来自山区者占34．3％。就诊者大多
为青少年，首次就诊年龄1历～48岁，平均20刀士8．45岁；其中1历～10
岁 13例（9厂％），11～20岁 66例（49．3％），ZI～30岁 41例（30石％），
31～48岁 14例门．4％）。发病年龄自出生后～39岁，平均 17j士789
岁；病程1历～15年，平均3．7士4．87年，其中病程10～2年者59例（叩．0％）。
133例o9．2％）表现为双耳渐进性听力减退，听不清言语，尤其在嘈杂环
境中，其中10例由父母发现其自幼听力差。61例（45．5％）伴间断性或持
续性耳鸣；15例伴头晕，11例伴四肢乏力、走路不稳；11例不明原因视
力减弱。9例有家族遗传史，所有患者均无噪声接触史及耳毒性药物使用
史。
二、纯音听阈测试与声导抗测试 听神经病组 120例（240耳）纯音
听力图上升型 167耳（69．6％），覆盆型 45耳（18．8％），平坦型 23耳（9．6％），
下降型 5耳（2．l％）。按低频 0．125～0．5 kHZ处的平均气导纯音听阈进行
听力损失程度分级，26～叩dB HL轻度听力障碍门例门 阮八41～55
dB HL中度听力障碍35例（29二％）；56～70 dB HL中重度听力障碍42
例（35．0呢）；71～90 dB HL重度听力障碍20例（16．6％）；大于gO dB HL
极重度听力障碍 6例（5刀％）。听神经病组的气导纯音听阈升高程度在低
频 0．125及 0．25 kHZ处分别高于感音神经性聋组 6．9土 6．17及 7．8土 6．37 dB
HL，两组间的差异有显著性意义（P＜0刀5）；在中频 1刀、2刀 kHZ和高频
4．0、8．0 kHZ处分别低于感音神经性聋组 12．5土7．13、27．2上7．72、42．6士
．4．
第四草巴大学项士学位论文
一
7．63、39．7士 7．84 dB HL，两组间的差异有显著性意义（P＜0．05）。听神经
病组纯音听力图左、右耳对称，在 0．125～4刀 kHZ两耳间的差异无显著性
意义（P＞0．IO）。20例听神经病患者的言语识别率6例为0，14例为14％～
60％，言语接受阈在40％～80％，而纯音听阈轻度听力障碍6例，中度听
力障碍9例，中重度听力障碍4例，重度听力障碍1例。镜骨肌声反射63
例同侧及交叉声反射均未引出，57例橙骨肌声反射单项或多项频率可引
出，但阈值升高。听神经病组所有患耳均无响度重振现象，感音神经性聋
组9耳重振现象阳性。
5、ABR与DPOAE 134例听神经病患者14例19耳ABR可引出V
波，其余患耳 ABR自波 1起全部未引出（叫 dB SPL）。DPOAE全部
可引出。DP－gram巾值在低频 0．5及 0．7 kHz处分别高于正常对照组 2．且土
l．51及 4．l上 l．48 dB SPL，两组间的差异有显著性意义（P＜0．05）：在中
频 l。0～2．0 kHZ、高频 3．0～8．0 kHZ处两组间的差异无显著性意义
（P叩刀5八在有和无对侧声刺激两种条件下，20例听神经病患者 DP唱ram
幅值间的差异无显著性意义o为二 10）
四、并发前庭功能障碍的情况40例行前庭功能检查的听神经病患
者，除3例伴走路不稳、10例伴头晕外，均无视物旋转、恶心、呕吐等典
型眩晕发作症状。冷热试验诱发性眼震电图反应有以下三种类型：双侧正
常30例（7刀％），左侧半规管麻痹1例门．5％），双侧半规管麻痹9例
（二二．5％）
五、并发其他神经系统疾病的发病情况4例神经内科患者的临床诊
断分别为：进行性脓骨肌萎缩症1例、Friedreich共济失调2例和Refsum
病1例，听力学检测结果表明他们均同时合并听神经病。我科门诊伴其他
神经系统障碍者，3例为视神经萎缩，7例为下肢神
Aim
Auditory neuropathy (AN) first named by Starr in 1996, which has the specific clinical characteristics, is one kind of sensorineural hearing loss caused by the injury of acoustic nerve branch of the eighth cranial nerve. The most important audiological characteristic of AN patients is that auditory brainstem responses (ABR) are absent or abnormal severely, but evoked otoacoustic emissions (EOAE) are normally recorded. AN is different from other common sensorineural deafness, which has the specific hearing impairment. AN has attracted attention more and more recently. The name of AN and site of AN lesion are still need discuss. The pathogeny and pathogenesis of AN are unknown. The effect treatment of AN is still absent. In this research, we summarized the clinical and audiological characteristics of
auditory neuropathy and discussed the significance of pure tone auditory threshold, acoustic immittance, ABR and distortion product otoacoustic emissions (DPOAE) in the diagnosis of AN. We also investigated the vestibular function disorder in AN patients and complicated AN in other nervous system diseases. Finally, we analysised the relationship between nervous system diseases and AN.
Method
By the retrospective studies of 134 AN patients including 4 complicated AN patients with nervous system hereditary disease from the department of neurology, we summarized their clinical dates, results of audiometry and other electrophysiological examinations. These results were compared with the results of audiometry of 30 common sensorineural deafness patients (49 ears) and 30 young subjects (60 ears) with normal hearing.
Results
1. Objects and clinical characteristics In all of the 134 AN patients, 48 patients were male and 86 were female. The ratio of both genders was 1:1.79. They mostly came from countryside, and 34.3% of them came from mountain area. Most of them were younger, and the ages of these first clients were from 1/6 to 48 years old, averaged at (20.0+8.45) years old. 13 patients (9.7%) were from 1/6 to 10 years old, 66 patients (49.3%) were from 11 to 20 years old, 41 patients (30.6%) were from 21 to 30 years old, and 14 patients (10.4%) were from 31 to 48 years old. The episode ages of them were from neonate to 39 years old, averaged at (17.1+7.89) years old. Their courses of diseases were from 1/6 to 15 years, averaged at (3.7+4.87) years. The courses of diseases of 59 patients (44.0%) were from half to 2 years. 133 patients (99.2%)
complained of bilateral gradual hearing loss, and can't discriminate speech correctly, especially in the environment of noise. 10 children were found by their parents from a child. 61 patients (45.5%) complained of last or discontinuous tinnitus. 15 patients had the experience of dizziness, and 11 patients of inertia and walking lability. 11 patients had weak sight for unknown reason. 9 patients had the family history of deafness. All of them didn't have the exposure history of noise or ototoxic drug.
2. The pure tone auditory threshold and acoustic immittance audiometry In 120 AN patients (240 ears), the pure tone audiogram of 167 ears (69.6%) were ascending curve, 45 ears (18.8%) were updown basin curve, 23 ears (9.6%) were flat curve, and other 5 ears (2.1%) were descending curve. Classified by the average pure tone threshold at low frequencies (0.125, 0.25 and 0.5 kHz), 17 patients (14.2%) were mild hearing loss (26-40 dB HL), 35 patients (29.2%) were moderate hearing loss (41-55 dB HL), 42 patients (35.0%) were moderate severe hearing loss (56-70 dB HL), 20 patients (16.6%) were severe hearing loss (71-90 dB HL), and 6 patients (5.0%) were profound hearing loss (>90 dB HL). The pure tone auditory threshold of the AN group was higher (6.9+6.17 and 7.8+6.37 dB HL separately) obviously (P<0.05) than the sensorineural deafness group at low frequencies (0.125 and 0.25 kHz), but lower (12.5+7.13, 27.2+7.72, 42.6+7.63 and 39.7+7.84 dB HL separately) obviously (P<0.05) at intermediate frequencies (1.0 and 2.0 kHz) and high frequencies (4.0 and 8.0 kHz). The
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