小儿蜗神经发育不良  

Cochlear Nerve Deficiency in Children

作　　者：莫玲燕[1] 燕飞[2] 刘辉[1] 陈静[1] 陈雪清[1] 黄丽辉[1] 韩德民[1] 

机构地区：[1]首都医科大学附属北京同仁医院北京市耳鼻咽喉科学研究所耳鼻咽喉头颈科学教育部重点实验室(首都医科大学),北京100005 [2]首都医科大学附属北京同仁医院放射科

出　　处：《听力学及言语疾病杂志》2011年第5期402-405,共4页Journal of Audiology and Speech Pathology

基　　金：北京市卫生系统高层次卫生技术人才培养基金(2009-3-29);国家"十一五"科技支撑计划(2007BAI18B12);科技部国际合作课题(2009DFA32200);"十一五"国家科技支撑计划重点项目(2008BAI50B08)资助

摘　　要：目的报道一组蜗神经发育不良(cochlear nerve deficiency,CND)小儿的临床特征,以提高对本病的认识。方法回顾性分析2007年1月到2008年4月就诊的、内耳MRI及听力学资料完整的20例(37耳)CND小儿(其中男13例,女7例,年龄10个月到4岁)的影像学、听力学表现及人工耳蜗植入效果。结果 85%(17/20)的患儿为双侧发病,15%(3/20)为单侧发病;16.22%(6/37)合并前庭神经发育异常,8.11%(3/37)合并面神经发育异常。根据是否伴有内耳畸形分为三组:32.43%(12/37)伴耳蜗畸形或同时伴有前庭畸形(第一组);13.51%(5/37)仅伴前庭畸形(第二组);54.05%(20/37)独立发病,不伴内耳畸形(第三组)。听力学测试结果:86.49%(32/37)ABR最大输出(100 dB nHL)无反应,13.51%(5/37)在非常高的刺激强度仅有分化不良的波Ⅴ;9耳有行为听阈结果者均为极重度感音神经性聋;第一组12耳DPOAE和CM均未引出,第二组60%(3/5)DPOAE和CM均未引出,40%(2/5)DPOAE和/或CM引出;第三组45%(9/20耳)DPOAE和CM均未引出,55%(11/20耳)DPOAE和/或CM引出。人工耳蜗植入效果:2例术前、术后资料完整者中1例术后开机一年婴幼儿有意义听觉整合量表(infant and toddler meaningful auditory integration scale,IT-MAIS)评估得分与蜗性聋儿相当,另1例无效。结论 CND患儿多无特殊病史,根据是否伴有内耳畸形及内耳畸形的种类,其听力学表现多样,诊断主要依据影像学检查,应注意鉴别诊断,以便制定合理有效的干预措施。Objective To review the clinical results of a group of children with cochlear nerve deficieney （CND）. Methods 20 eases（37 ears） confirmed CND by inner ear magnetic resonance imaging （MRI） were included in this study. The subjects aged from 10 months to 4 years old. Their case history, imaging results and audiological data were retrospectively analyzed. Results Most of the cases had no particular history. The inner ear MRI ranged from isolated CND to accompanied cochlear and/or vestibular malformation. Click ABR varied from no response at the maximum output level to the lowest threshhold of 80 dB nHL. DPOAE and/or CM were present in 45% of the ears with CND only while they were absent in all of the ears with cochlear malformation. 2 were implanted with cochlear implants on the affected ear and with detailed results of infant and toddler meaningful auditory integration scale （IT--MAIS） measurement up to 1 year after the switch on. The results in one of them were comparable to that of the children with cochlear loss while other was nonuser of the device. Conclusion Depending on the presence or absence of inner ear malformation and other factors, there is variability in imaging and audiological results in children with CND.

关 键 词：蜗神经发育不全 听神经病 内耳畸形 

分 类 号：R764.4[医药卫生—耳鼻咽喉科]