【摘要】 目的　综合评估伴中枢神经系统病变的感音神经性聋婴儿临床听力学特点及与中枢性神经系统病变的关系。方法　选取我院神经康复科住院患儿 ,听性脑干反应 (auditorybrainstemresponses ,ABR)异常并排除中耳功能异常者 ,月龄不超过 12个月 ,共 12 1例 2 2 3耳。根据神经康复科的专科评估 ,分为核黄疸 -脑瘫、外部性脑积水和精神运动发育迟缓三组。设立同年龄段正常对照组。每一组组员同时检测ABR和畸变产物耳声发射 (distor tionproductotoacousticemissions,DPOAE) ,对比观察不同组别间ABR波V阈值及DPOAE异常耳的比率、同一组间不同ABR波V阈值耳和DPOAE异常耳比率的变化特征。结果　核黄疸 -脑瘫常导致严重的听力损失 ,其波V阈值≥ 80dBnHL以上耳达 83.91% ;外部性脑积水主要表现为轻中度的听力损害 ,波V阈值在 4 0～ 80dBnHL区间耳为77.91% ;精神运动发育迟缓组波V阈值的分布则无明显的极端性。当ABR波V阈值在 4 0～ 80dBnHL区间内时 ,三组受试者主要表现以蜗后听神经系统损害为主 ;当ABR波V阈值≥ 80dBnHL时 ,测试耳DPOAE异常的比率明显增加 ,三组分别达 5 8.90 %、4 2 .11%和 5 2 .38%。结论　伴中枢性神经系统病变感音神经性聋婴儿 ,其听力障碍特征表现出多样性 ,临床上需进行跨学科多手段的综合评估更多还原

【Abstract】 Objective To explore the clinical and audiological characteristics of sensorineural hearing loss (SHL) in infants with diseases in central nervous system (CNS);To evaluate the relationship between SHL and CNS.Methods 121 cases (223 ears) of SHL,ageing under 12 months, were studed. Accroding the evaluation of function of CNS by pediatric neurologist,121 cases of SHL were divided into three groups: ⑴group of SHL with kernicterus-cerebral palsy, ⑵group of SHL with external hydrocephalus and ⑶group of SHL with psycho-motor delay. Some normal hearing children with the same age were accepted as control group. All cases were tested using auditory brainstem responses (ABR) and distortion product otoacoustic emissions (DPOAE) at the same time.Results ⑴ Patients with kernicterus-cerebral palsy usually had serious hearing loss, The rate of the ears suffering serious hearing loss (threshold of wave Ⅴ of ABR ≥80 dB nHL ) was 83.91%; In the group of SHL with external hydrocephalus, the rate of the ears suffering hearing loss (threshold of wave Ⅴ of ABR<80 dB nHL) was 77.91%; ⑵The hearing loss mostly caused by acoustic nerve lesion at retrocochlear was usually occured when the thresholds of wave Ⅴ of ABR were under 80 dB nHL in all groups. When the threshold of wave Ⅴ of ABR was up to above 80 dB nHL, The rate of the ears with abnormal DPOAE obviously increased.Conclusion Patients with SHL are usually accompanied by CNS diseases in infants, Their hearing loss appears different in audiological characteristics. We suggest that ABR and DPOAE testing at the same time in these patients is necessary.A team including pediatric neurologist and audiologist is necessary too.更多还原