婴幼儿单侧听神经瘤

Study of unilateral auditory neuropathy in infants

目的探讨婴幼儿单侧听神经病(AN)神经生理学特点。方法对3例单侧AN患儿在采集病史和耳科检查的基础上,行系统的听力学检查,包括声阻抗、镫骨肌反射、听性脑干反应(ABR)、耳蜗微音电位(CM)、诱发耳声发射(EOAE)、中潜伏期反应(MLR)和事件相关电位(ERP)以及CT和(或)MRI及周围神经系统检查。结果3例患儿鼓室图均呈“A”型,健耳的同侧和交叉镫骨肌反射可正常引出,而患耳的同侧和交叉镫骨肌反射未引出。3例患儿双侧EOAE有效引出,健耳的ABR正常引出,患耳的ABR未引出,但CM均正常。3例患儿双耳均记录到MLR及ERP。影像学和周围神经系统未见异常。结论单侧AN与双侧AN有相似的神经生理学特点。对婴幼儿单侧AN的诊断应着重分析其神经生理学特点,建议联合应用EOAE、ABR、CM和影像学检查。应对AN患儿定期随访。

Objective：To study neurophysiologieal characteristics in infants with unilateral auditory neuropathy （UAN）. Method： Audiological measurements （ acoustic immittanee, stapedial reflex, EOAE, ABR, CM, MLR and ERP） and peripheral neurological tests were conducted and evaluated in 3 infants with UAN. All patients received CT scan and/or MRI examination. Result：Three patients had type ＂A＂ tympanogram. The ipsilaterd and crossed stapediel reflerses were elicited in healthy ears,while those were absent in affected ears. Affected ears in 3 cases showed no ABR but normal CM and EOAE. All of them had normal MLR and ERP （ P300 and MMN ） recording. Peripheral neurological tests and CT and/or MRI showed no abnormality. Conclusion.. Neurophysiologieal characteristics in infants with UAN were similar with bilateral AN. The diagnosis of UAN in infants should focus on analyzing their neurophysiological characteristics and imaging examinations. Combining use of EOAEs,ABR and CM was recommended. It was necessary to fouow up the infants with AN.