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蜗神经发育不良患者的听力学特征分析

Auditory Characteristics in Patients with Cochlear Nerve Deficiency

樊小勤;林颖;任寸寸;唐永强;查定军;

1:空军军医大学西京医院耳鼻咽喉头颈外科

2:空军军医大学西京医院放射科

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摘要

目的探讨蜗神经发育不良(cochlear nerve deficiency,CND)患者的听力学特征。方法以30例(54耳)(男14例,女16例,年龄6月～29岁,平均4.9岁)内听道斜矢状位磁共振成像示蜗神经发育不良的患者为研究对象,回顾性分析患者的纯音测听或行为测听、声导抗、耳声发射、听性脑干反应(ABR)、听性稳态反应等听力学检测结果及颞骨高分辨CT(HRCT)结果,分析蜗神经发育异常者的听力学特征。结果 30例54耳蜗神经发育不良患者中,双侧24例(80%),单侧6例(20%),其中蜗神经缺如33耳,蜗神经细小21耳。54耳中,共有25耳(46.29%)伴内耳畸形,而单侧蜗神经发育不良者的对侧6耳中,50%(3耳)伴内耳畸形。纯音测听或行为测听和听性稳态反应结果均提示蜗神经发育不良患耳呈重度—极重度听力损失,且蜗神经缺如耳较蜗神经细小耳的听力损失程度更重。11耳(20.37%,11/54)蜗神经发育不良耳可在80～100 dB nHL高强度声刺激下引出听性脑干反应;6例双侧蜗神经发育不良者(20.0%,6/30)中7耳(1例双耳,5例单耳)全部频率或部分频率引出DPOAE,但其中5耳未引出ABR,2耳分别于100和90 dB nHL刺激声强下引出ABR。结论蜗神经发育不良患者常表现为重度—极重度听力损失,近一半伴内耳畸形,部分患耳可引出DPOAE,而ABR严重异常或缺失。

关键词

蜗神经发育不良;畸变产物耳声发射;听性脑干反应

基金项目(Foundation):

作者

樊小勤;林颖;任寸寸;唐永强;查定军;

参考文献

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蜗神经发育不良畸变产物耳声发射听性脑干反应

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樊小勤林颖任寸寸唐永强查定军