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目的 探讨包含小儿听神经病在内的听性脑干反应 (auditorybrainstemresponse ,ABR)严重异常 ,畸变产物耳声发射 (distortionproductotoacousticemission ,DPOAE)正常为特征的听功能障碍症侯群的病因学特征、病损部位与临床听力学特点。方法 选取 2 0 0 2～ 2 0 0 4年听力专科中ABR严重异常、DPOAE正常 ,排除中耳传导功能异常的患儿 98例 (16 8耳 )入选为研究组。根据神经康复科医师的评估 ,作出是否伴有精神运动发育迟缓及其相关病因的诊断 ,统计伴有精神运动发育迟缓的研究组中听力损失高危因素的类型与比例 ;选择ABR严重异常、DPOAE异常、排除中耳病变的患儿4 6例 (82耳 )作为蜗性病变对照组 ,比较研究组与蜗性病变患儿ABR的异同点 ;选择健康同龄小儿4 0例 (72耳 )作为正常对照组 ,观察研究组与正常听力小儿ABR和DPOAE的异同点。结果 ①研究组 98例患儿中 ,83 6 7%患儿伴有精神运动发育迟缓的临床症状 ,其相关的致病因素中 ,以新生儿高胆红素血症所占的比例最高 ;② 10 3dB强度刺激声 (听力级 )刺激时 ,无波分化的为 5 3耳、仅能记录到波Ⅴ的 7耳 ,90dB强度刺激声仅能记录到Ⅴ波的 9耳 ,80dB仅能记录到Ⅴ波的 4耳 ,共 73耳(43 4 5 % ) ,病损部位主要在听神经远端 ;10 3dB强度刺激声仅能记录到波Ⅰ
Objective To investigate the etiological characteristics of hearing disorder patients with abnormal auditory brainstem response (ABR) and distortion product otoacoustic emissions (DPOAE), including auditory neuropathy in children. And clinical audiology features. Methods A total of 98 children (168 ears) with abnormal ABR, normal DPOAE, and abnormal conduction in the middle ear were selected as the study group from 2002 to 2004. According to the assessment of neuro-rehabilitative physicians, the diagnosis of mental retardation and its related etiology was made. The types and proportions of the risk factors of hearing loss in the study group with psychomotor retardation were statistically analyzed. The patients with severe ABR and DPOAE abnormalities , 46 cases (82 ears) of middle ear lesions were excluded as the control group of cochlear lesions, and the similarities and differences between the study group and the children with cochlear lesions were compared. 40 healthy children of the same age (72 ears) were selected as the normal control Group, observing the similarities and differences between ABR and DPOAE in study group and normal hearing children. Results ① Among the 98 infants in the study group, 83.67% of the children had clinical symptoms of mental retardation. Among the related risk factors, neonatal hyperbilirubinemia accounted for the highest proportion; ② 10 3dB intensity stimulus sound (hearing level) stimulation, no differentiation of 53 ears, only to the wave Ⅴ of the 7 ears, 90dB intensity stimulus can only record Ⅴ wave 9 ears, 80dB can only be recorded Ⅴ wave of 4 ears, a total of 73 ears (43 4 5%), the lesion mainly in the distal part of the auditory nerve; 10 3dB intensity stimuli can only record wave Ⅰ