中文摘要：

目的 分析中国听神经病谱系障碍患者蜗神经的发育情况,进一步探讨中国听神经病谱系障碍患者的相关影像学特征。方法 本研究以19例行内听道斜矢状位高分辨核磁共振成像的听神经病谱系障碍患者为研究对象,进行详细的病史采集和听力学检测。纯音测听检测、声导抗检测、听性脑干反应、DPOAE检测、言语识别率检测等,回顾性分析听神经病谱系障碍患者的蜗神经影像学特征,并探讨蜗神经发育异常患者的听力学特征。结果 本研究的19例听神经病谱系障碍患者中,共发现4例蜗神经发育异常的患者,所占比例为21.05（4/19）。4例患者在内听道斜矢状位MRI上均表现为双侧蜗神经细小。其中,2例患者有耳聋家族史,1例患者伴有共济失调障碍。4例蜗神经发育异常的患者听力曲线类型各异：2例患者表现为全频中度下降的平坦型听力曲线,1例表现为中重度低频上升型听力曲线,另外1例表现为中重度的高频下降型听力曲线。4人（8耳）的言语识别率均较差,除1耳外,其余7耳的言语识别率均低于40%。结论 对于听神经病谱系障碍的患者,若条件允许的情况下,应常规行内听道斜矢状位高分辨核磁共振成像,以评价蜗神经粗细及发育情况,对于了解听神经病谱系障碍患者的病变部位和病因。

英文摘要：

Objective To identify the radiological characteristics of cochlear nerve in patients with auditory neuropathy spectrum disorder（ANSD）. Methods We enrolled 19 patients with auditory neuropathy spectrum disorder （ANSD） in this study who had undergone cochlear nerve high-resolution magnetic resonance imaging （HRMRI）. All the above subjects were studied by history collection, physical examination and audiological examination, including pure tone audiometry, acoustic impedance, ABR, DPOAE, speech perception testing, et al. This study analyzed the radiological characteristics of cochlear nerve in patients with ANSD, and also analyzed the audiometric features of cochlear nerve deficiency patients with ANSD. Results In this study, the 19 ANSD patients showed a high occurrence of cochlear nerve deficiency （21.05%, 4/19）. Within the 4 patients （8 ears）having small cochlear nerves, one patient had movement disorders, one patient had optic nerve atrophy, and two patients had family histories of hearing loss. These 4 patients presented different configuration and degree of hearing loss. Two patients presented fiat hearing curve with moderate hearing loss at all frequencies, another one showed low frequency ascending pattern with moderate to severe hearing loss, while and the other displayed high frequency sloping curve with moderate to severe hearing loss. This study also found that patients with cochlear nerve deficiency had bad speech discrimination scores, with 7 out of 8ears showed speech discrimination scores less than 40%. Conclusion HRMRI should be the initial imaging study of choice for children with ANSD to evaluate the radiological characteristics of cochlear nerve, and to analyse the possible site of lesion and one of the etiology of ANSD.