摘要
目的:报道1组小儿类听神经病(ANSD)的临床特征,以加深对该病的认识。方法:回顾性分析84例(151耳)ANSD患儿病史、影像学、听力学表现及人工耳蜗植入效果。结果:①高胆红素血症11例,家族史2例,缺氧3例,早产1例,基底核病变1例,先天性脑发育落后2例,脑瘫1例,蜗神经发育不全13例;②79.8%(67/84)为双侧发病,20.2%(17/84)为单侧发病;③短声ABR:70.2%(106/151)在最大输出(100dB nHL)无反应波形引出,29.8%(45/151)在非常高的刺激强度仅有分化不良的Ⅴ波;④行为听力测试:23例有测试结果者中,1例为轻度感音神经性聋,2例重度,20例为极重度;⑤影像学:29例有内耳MRI者中,外、中、内耳结构正常者16例,蜗神经发育不良12例,蜗神经发育不良伴双脑室周围白质髓鞘形成不良1例;⑥人工耳蜗植入效果:5例患耳植入人工耳蜗,其中3例术后1年婴幼儿有意义听觉整合量表得分与蜗性聋儿相当,1例稍差于蜗性聋儿,另1例无效。结论:ANSD是听力学表现相同的一大类疾病,在病史、听力学、影像学及人工耳蜗植入效果方面存在多样性,因此对此类患儿应进行全面、详细的临床检查,以便制定个体化的干预方案。
Objective：In order to extend the knowledge of auditory neuropathy spectrum disorder（ANSD）,the clinical data of a group of infants and young children with this disorder was reported.Method：This retrospective study involved 84 cases（151 ears）.The subjects aged from 2 months to 6 years old.Descriptive analysis was carried out on case history,audiological and imaging results and the outcome of cochlear implantation.Result：There were a variety of case history,including 11 of hyperbillirubinium,2 of family history of deafness,3 of hypoxia,1 of prematurity,1 of nuclei basales pathology,2 of congenital atelencephaila,and 13 of cochlear nerve deficiency（CND）.The audiological results varied too.Click ABR ranged from no response at the maximum output level to Wave V thresholds as low as 70 dB nHL.Behavioral thresholds were mild in 1,moderate in 2 and profound in 20.The inner ear MRI were normal in 16 cases,CND in 12 and CND accompanied by periventricular leukomalacia in 1.5 cases were implanted in the affected ear.The results in 3 were comparable with that of cochlear loss,1 were less effective than that of cochlear loss,1 became none user of the device.Conclusion：ANSD is a heterogeneous group with a wide variety on case history,audiological and imaging results and the outcome of cochlear implant.Therefore,comprehensive clinical examinations are necessary so that individualized intervention program can be made.
出处
《临床耳鼻咽喉头颈外科杂志》
CAS
CSCD
北大核心
2011年第22期1012-1014,1018,共4页
Journal of Clinical Otorhinolaryngology Head And Neck Surgery
基金
北京市卫生系统高层次卫生技术人才培养基金(No:2011-3-042)
科技部国际合作课题资助(No:2009DFA32200)
关键词
类听神经病
听神经病
auditory neuropathy spectrum disorder
auditory neuropathy
