耳蜗神经发育不良听神经病谱系障碍患者听力学分析

发布时间：2018-04-21 21:41

本文选题：听神经病谱系障碍 + 听神经病　；参考：《中华耳科学杂志》2015年02期

【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。
[Abstract]:Objective to analyze the development of cochlear nerve in Chinese patients with auditory neuropathy pedigree disorders and to explore the imaging features of Chinese patients with auditory neuropathy spectrum disorders. Methods in this study, 19 patients with auditory neuropathy spectrum disorders in oblique sagittal position of internal auditory canal were studied. The history of the disease was collected and audiological examination was carried out. Pure tone audiometry, acoustic impedance test, auditory brainstem response DPOAE test, speech recognition rate test, etc. The imaging features of cochlear nerve in patients with spectrum disorders of auditory neuropathy were analyzed retrospectively, and the audiological characteristics of patients with dysplasia of cochlear nerve were discussed. Results among the 19 patients with auditory neuropathy spectrum disorder, 4 cases were found to have abnormal cochlear nerve development. The proportion of them was 21.05% 4 / 19.4 cases showed that the bilateral cochlear nerves were fine on oblique sagittal position of the auditory canal. Two of them had a family history of deafness, one patient with ataxia, four patients with dysplasia of the cochlear nerve, the hearing curves were of different types, two patients showed a flat hearing curve with moderate complete frequency decline, and one patient showed a flat hearing curve. Medium and severe low frequency ascending hearing curve, The speech recognition rate of the other 7 ears was lower than that of 40 ears except 1 ear. Conclusion for the patients with auditory neuropathy spectrum disorders, we should perform high-resolution magnetic resonance imaging in oblique sagittal position of auditory canal to evaluate the thickness and development of cochlear nerve. To understand the location and etiology of auditory neuropathy pedigree disorders.
【作者单位】：解放军总医院解放军医学院耳鼻咽喉头颈外科解放军耳鼻咽喉研究所;
【基金】：国家重大科学研究计划项目(2014CB943001) 国家自然基金重大国际合作项目(81120108009) 全军“十二五”重点项目(BWS11J026)
【分类号】：R764

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