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[显性遗传性蜗后低频听力损失]

[Phanerogenetic retrocochlear low frequency hearing loss].

作者信息

Han Wei-ju, Gu Rui, Yu Li-ming, Zheng Jie-fu, Zhou Na, Cao Ju-yang, Wang Li

机构信息

Department of Otorhinolaryngology and Head & Neck Surgery, General Hospital of PLA, Beijing 100853, China.

出版信息

Zhonghua Er Bi Yan Hou Ke Za Zhi. 2003 Dec;38(6):459-64.

PMID:15040112
Abstract

OBJECTIVE

To study the pathogenisis of retrocochlear low frequency hearing loss.

METHODS

Clinical and audiologic findings [auditory brainstem response (ABR), evoked otoacoustic emission (EOAE), et al] of 29 cases with retrocochlear low frequency hearing loss were studied.

RESULTS

The head injury, acoustic neuroma, peripheral neurophathy, hereditary hear loss, multiple sclerosis and brainstem disease can cause retrocochlear low frequency hearing loss. The typical clinical manifestations of retrocochlear low frequency hearing loss were normal EOAE which cannot be suppressed by contralateral white noise, but with abnormal ABR as well as with no acoustic reflex and -SP/AP > 0.44.

CONCLUSIONS

The results suggest that phanerogenetic retrocochlear low frequency hearing loss should be a syndrome rather than a disease called "auditory neuropathy". The main lesions of the disease are brainstem, cochlear nuclei and auditory nerve.

摘要

目的

研究蜗后性低频听力损失的发病机制。

方法

对29例蜗后性低频听力损失患者的临床及听力学检查结果[听性脑干反应(ABR)、诱发性耳声发射(EOAE)等]进行研究。

结果

头部损伤、听神经瘤、周围神经病、遗传性听力损失、多发性硬化及脑干疾病均可导致蜗后性低频听力损失。蜗后性低频听力损失的典型临床表现为EOAE正常,对侧白噪声不能抑制,但ABR异常,且无听觉反射,-SP/AP>0.44。

结论

结果提示,蜗后性低频听力损失应为一种综合征,而非一种名为“听觉神经病”的疾病。该病主要病变部位在脑干、蜗神经核及听神经。

相似文献

1
[Phanerogenetic retrocochlear low frequency hearing loss].[显性遗传性蜗后低频听力损失]
Zhonghua Er Bi Yan Hou Ke Za Zhi. 2003 Dec;38(6):459-64.
2
[Central low frequency hearing loss].[中枢性低频听力损失]
Zhonghua Er Bi Yan Hou Ke Za Zhi. 2000 Dec;35(6):441-5.
3
[Otoacoustic emission and auditory efferent function testing in normal subjects and patients with sensori-neural hearing loss].[正常受试者及感音神经性听力损失患者的耳声发射及听觉传出功能测试]
Zhonghua Yi Xue Za Zhi. 1996 Oct;76(10):763-6.
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Am J Otol. 1996 Mar;17(2):317-20.
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Acta Otorrinolaringol Esp. 1998 Nov-Dec;49(8):603-8.
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9
[Retrocochlear hearing disorders].[蜗后听觉障碍]
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10
[Diagnosis of sensorineural hearing loss by ipsilateral masking of brain stem auditory evoked potentials].[通过脑干听觉诱发电位的同侧掩蔽诊断感音神经性听力损失]
Acta Otorhinolaryngol Ital. 1991 Jul-Aug;11(4):367-77.