听性脑干反应严重异常和耳声发射正常的小儿听力障碍的临床特征分析

Analyze of the clinical characteristics in c hildren with the severe abnormal auditory brainstem response and normal distorti on product otoacoustic emission

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

Objective To explore the clinic and audiological characteristics of auditory neuropathy in children. Methods Between 2002 and 2004, ninety-eight cases with the severe abnormal results of A BR and the normal results of DPOAE were enrolled in the study group. The mean p atient age was 9 months, with a range of 1 month to 5 years. According to the e valuation of pediatric neurologist, the high-risk factors of psychomotor develo pment retardation were statistically analyzed whether the cases were accompanied by psychomotor development retardation or not. The cases with abnormal results of ABR and DPOAE and without abnormal conductive function were selected as the cochlea lesion group. Some same age children without hearing loss were subjecte d as normal control group. Results The psychomotor development retardation was performed in 83.67% of cases. Hype rcholesterolemia of newborn was the common high-risk factor of psychomotor deve lopment retardation (43.88%). Severe abnormal results of ABR were occurred in 66.07% of ears. Prolonged latency of wave Ⅰ～Ⅴ ABR and acoustic stapedius r eflex were observed in few cases. The DPOAE amplitudes at intermediate and high frequencies were normal or slightly descending. Occuring rate of DPOAE at inte rmediate and high frequencies was the same as in normal control group. Conclusions Hypercholesterolemia of newborn is the most c ommon cause of auditory neuropathy. The auditory test results are various in ch ildren with auditory neuropathy. This character is different from it in adults .