27例听神经病患者的临床特征分析

目的总结探讨听神经病（auditory neuropathy,AN）患者的临床特点和听力学特征。方法回顾性分析27例听神经病患者,并总结其临床资料、纯音听阈、声导抗测试、听性脑干反应与畸变产物耳声发射结果。结果 27例患者中,25例表现为双耳渐进性听力减退,1例表现为渐进性左侧听力下降,1例为查体发现。其中23例伴间断性或持续性耳鸣,3例伴头晕。纯音听力图示上升型感音神经性听力损失。21例患者镫骨肌反射同侧及交叉声反射均未引出,6例镫骨肌反射阈值提高。27例听神经病患者听性脑干反应53耳自波Ⅰ起全部未引出（〉100 dB SPL）。畸变产物耳声发射全部可引出。结论听神经病是一临床表现特殊的感音神经性聋,其病变部位可能位于听神经传入通路,或伴有脑干内侧橄榄耳蜗系统的传出神经通路病变;听神经病的病因及发病机制需进一步探讨研究。

Analysis on clinical features of auditory neuropathy in 27 patients from Nanjing area

Objective To study and summarize the clinical and auditory characteristics of auditory neuropathy（ AN） in Nanjing area. Methods 27 patients were diagnosed as AN. Their clinical data,results of pure tone auditory threshold, acoustic immittance audiometry, auditory brainstem responses（ ABR） and distortion product otoacoustic emissions（ DPOAE） were studied retrospectively. Results 11 patients were male and 16 were female with a male-to-female sex ratio of 1：1. 45. Most of them were adolescents,and their average age was（ 19. 8 ±8. 07） years. The age of onset ranged from 12 to 35 years,averaged at（ 17. 3 ± 7. 41）. The disease duration ranged from 4 months to 5 years,averaged at（ 2. 9 ± 3. 93） years. 25 patients complained of bilateral progressive hearing loss and poor speech perception. One complained of left gradual hearing loss. And one was diagnosed with medical examination. 23 patients complained of intermittent or continuous tinnitus,and 3 patients of dizziness. The pure tone audiograms of most patients showed ascending curve. Ipsilateral and contralateral stapedial reflexes were absent in 21 patients. Stapedial reflexes of the other 6 patients were present at some frequencies,but the reflex thresholds got raised. In all 27 AN patients（ 53 ears）,auditory brainstem responses were unrecorded from wave Ⅰ.DPOAEs were evoked in all patients. Conclusion Auditory neuropathy is a kind of sensorineural hearing loss with special clinical characteristics. It seemed that the lesions of AN were focused on the afferent nerve pathway of acoustic nerve,or involved brain stem medial olivocochlear system and its efferent nerve pathway. The etiology and pathogenesis of AN should be explored and studied further.