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治 疗
目前对听神经病尚缺乏十分有效的药物治疗，对于是否适合佩带助听器和人工
耳蜗植入尚存有争议。Starr等［5］认为佩带助听器对改善听神经病患者的听力无
益，在某些情况下甚至产生负面效果。Rance等［9］的报道则积极得多，在15例佩
带助听器的患儿中7例在使用1年后无改善，另8例患儿的言语识别和一般听觉反应
都有明显的改善，这至少表明听神经病患儿存在通过佩带助听器改善言语识别技巧
的可能。因此Rance推荐所有行为听阈异常的外周神经病患儿都应该佩带助听器，
但这是否适用于成年人，尚待证实。
人工耳蜗植入可以明显改善重度感音神经性聋患者的言语识别能力，但听神经
病大多数只表现为轻或中度感音神经性聋，其预后较难预料。在听神经纤维脱髓鞘
的情况下人工耳蜗植入的效果如何？Shepherd等［22］报道电刺激有髓鞘缺失的听
神经，产生了可传导的动作电位；电刺激有髓鞘缺失的听神经纤维也可引出ABR［
23］。但髓鞘缺失与不均匀脱髓鞘是不同的。Rance等［9］报道1例听神经病患儿
人工耳蜗植入1年后，其言语识别能力没有任何提高。该患儿未引出电刺激诱导的
ABR，提示听觉脑干水平存在病变，但这种情况可能并不出现于每位听神经病患者
，所以目前还不能说听神经病是人工耳蜗植入的禁忌证。
值得注意的问题
听神经病的出现提示对于一些听觉检查结果矛盾的感音神经性聋患者及在高危
新生儿听力筛选中，有必要同时进行ABR、TEOAE和DPOAE（和/或耳蜗微音电位）检
查，以免漏诊。
听神经病的表现具有明显的个体差异，这种个体差异的产生是由于病变不同还
是由于同一病变而程度不同，目前还无法确定。但这提醒我们在临床中对于不同的
听神经病患者应根据其各自独特的表现综合分析，并采取不同的处理。
听神经病的命名是否恰当，其病因、病变部位、治疗及预防等都是尚待解决的
问题。
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