蜗后性听力减退29例病因分析

目的 探讨蜗后性低频听力减退的发病原因。方法 复习1988～2002年29例原因明确的蜗后性低频听力减退患者的临床特点和听力学检查结果。结果 头部外伤、听神经瘤、周围神经病、遗传性聋、多发性硬化和脑干疾病均可表现为蜗后性低频听力减退,其共同的特点是：低频听力减退,诱发性耳声发射正常或至少在部分频率正常,不受对侧噪声抑制,引不出镫骨肌反射,听性脑干反应(auditory能超群brainstem response,ABR)异常。结论 以耳声发射正常和ABR异常为主要特征的听功能障碍是一组症状群,可为不同病因引起的听神经和(或)听性脑干病变的表现,建议对这一组听力学表现称之为蜗后性低频听力减退。

Phanerogenetic retrocochlear low frequency hearing loss]

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.