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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 investigate the neurophysiological characteristics of unilateral auditory neuropathy (AN) in infants and young children. Methods A total of 3 unilateral children with AN were enrolled in the study of the history and the otology, and the systematic audiological examination including acoustic impedance, stapedius reflex, auditory brainstem response (ABR), cochlear microphony potential CM), evoked otoacoustic emissions (EOAE), mid-latency response (MLR) and event-related potentials (ERPs) as well as CT and / or MRI and peripheral neurological examinations. Results The tympanograms of the three children showed “A” type. The ipsilateral and traulectomytic muscles of the ear were elicited normally, while those of the ipsilateral and cross stapes muscles of the affected ears were not elicited. Three cases of bilateral EOAE elicited effectively, and ABR of eardrum elicited normally. However, ABR of eared ears did not lead to ectopic pregnancy, but CM was normal. MLR and ERP were recorded in both ears of 3 children. No abnormalities in imaging and peripheral nervous system. Conclusion Unilateral AN and bilateral AN have similar neurophysiological characteristics. The diagnosis of unilateral AN in infants and young children should focus on the analysis of its neurophysiological characteristics, it is recommended that the joint application of EOAE, ABR, CM and imaging studies. Should be regular follow-up AN children.