el Khateeb I, Abdul Razzak B, Moosa A
Department of Paediatrics, Mubarak Al Kabeer Hospital, Kuwait.
Brain Dev. 1988;10(4):243-8. doi: 10.1016/s0387-7604(88)80005-6.
Auditory brainstem responses (ABR) were studied in 35 children with neurological disorders and 24 controls. Abnormal results were obtained in 16 patients. All 5 of the patients with metachromatic leukodystrophy had evidence of peripheral and/or central delay in transmission in keeping with evidence of demyelination of both peripheral (i.e. auditory nerve) and central (i.e. brainstem) pathways as occurs in this disorder. Two children with lead poisoning had delayed conduction in the peripheral pathways only and in these there was good correlation between the degree of delay and the ulnar nerve conduction velocity; both improved after chelation therapy. One infant with lead poisoning had central delay only. One infant with osteopetrosis manifested progressive damage to the auditory nerves. Delayed conduction was also found in one patient each with hydrocephalus, spinal muscular atrophy, and in 2 infants with cerebral palsy. No responses were obtained in one infant with congenital rubella, one deaf-mute and one child with an undiagnosed degenerative neurological disease. Auditory brainstem responses are of value in detecting disturbances of the auditory nerve or brainstem in children with various neurological disorders.
对35名患有神经疾病的儿童和24名对照儿童进行了听觉脑干反应（ABR）研究。16名患者获得了异常结果。所有5名患有异染性脑白质营养不良的患者都有外周和/或中枢传导延迟的证据，这与该疾病中外周（即听神经）和中枢（即脑干）通路的脱髓鞘证据一致。两名铅中毒儿童仅在外周通路存在传导延迟，且延迟程度与尺神经传导速度之间存在良好的相关性；螯合治疗后两者均有改善。一名铅中毒婴儿仅存在中枢延迟。一名患有骨硬化症的婴儿表现出听神经的进行性损伤。在一名脑积水患者、一名脊髓性肌萎缩患者以及两名脑瘫婴儿中也发现了传导延迟。一名先天性风疹婴儿、一名聋哑人和一名患有未确诊退行性神经疾病的儿童未获得反应。听觉脑干反应在检测患有各种神经疾病儿童的听神经或脑干障碍方面具有价值。
