婴幼儿听神经病谱系障碍与人工耳蜗植入研究进展

Research progress on auditory neuropathy spectrum disorder and cochlear implantation in infants and young children

听神经病谱系障碍（auditory neuropathy spectrum disorder,ANSD）,于20世纪90年代由Starr等^（[1]）首次报道了这种言语识别率与纯音听阈不成比例的听力障碍的表现形式,Starr等称之为听神经病（auditory neuropathy,AN）。感音神经性聋（sensorineural hearing loss,SNHL）患者通常表现为听力损失程度与言语识别率成比例下降。

Auditory neuropathy is a disorder characterized by absent or severely abnormal auditory brainstem response（ABR）with intact outer hair cell function,as evidenced by the presence of evoked otoacoustic emissions and/or cochlear microphonics.Unlike patients with sensory hearing loss who show clinical evidence of impaired outer hair cell function.For ANSD patients,clinical rehabilitation is mainly limited to hearing aid wearing and cochlear implantation.However,the effect of cochlear implantation for ANSD patients may be difference.The outcome of cochlear implantation is related to the lesion location,the age of implantation and the diameter of cochlear nerve.