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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
Objective To comprehensively evaluate the clinical audiological features of sensorineural deaf infants with central nervous system diseases and its relationship with central nervous system diseases. Methods A total of 121 children aged 22 months (12 months) were enrolled in this study. All children hospitalized in neuro-rehabilitation department of our hospital were enrolled and their auditory brainstem response (ABR) was abnormal. The patients with abnormal middle ear were excluded. According to neurological rehabilitation specialist evaluation, divided into nuclear jaundice - cerebral palsy, external hydrocephalus and mental retardation three groups. Set up the same age normal control group. In each group, ABR and DPOAE were measured at the same time. The ABR V threshold and the ratio of DPOAE abnormal ear were compared among different groups. The V threshold ear and DPOAE of different ABR waves in the same group were abnormal Ear ratio changes. The results of nuclear jaundice - cerebral palsy often lead to severe hearing loss, the wave V threshold ≥ 80dBnHL above the ear reached 83.91%; external hydrocephalus mainly mild to moderate hearing loss, wave V threshold in the 40 ~ 80dBnHL range ear 77.91%. There was no obvious extremity in the distribution of wave V threshold in psychomotor retardation group. When the ABR wave V threshold was in the range of 40 ~ 80dBnHL, the three groups of subjects mainly showed auditory nocturnal auditory system damage. When the ABR threshold V ≥ 80dBnHL, the ratio of DPOAE abnormalities in the test ear increased significantly. Three groups Respectively up to 5 8.90%, 42.11% and 52.38%. Conclusions Infants with sensorineural deafness of central nervous system diseases show diversified features of hearing impairment, so it is necessary to conduct a comprehensive interdisciplinary evaluation