Shimizu H, Moser H W, Naidu S
Department of Otolaryngology--Head and Neck Surgery, Johns Hopkins Medical Institutions, Baltimore, MD 21205.
Otolaryngol Head Neck Surg. 1988 Mar;98(3):215-20. doi: 10.1177/019459988809800307.
Auditory brain stem response, auditory sensitivity, speech discrimination function, acoustic reflex threshold, and reflex decay were studied in seven children with adrenoleukodystrophy (ALD), nine adults with adrenomyeloneuropathy (AMN), one child with neonatal ALD, and two carriers of ALD. Significant auditory dysfunctions were found in only two patients; a retrocochlear type auditory disorder in one child with ALD, and a profound deafness in a child with neonatal ALD. None of the patients showed significant abnormality in acoustic reflex threshold and reflex decay except for one patient with ALD who showed a slightly elevated reflex threshold. All but two ALD patients showed significantly prolonged wave latencies and interwave intervals. The latency values revealed the highest incidence of abnormality in the I to III interval; this was followed by the III to V interval, then by wave I. Significantly, one child with asymptomatic ALD and both heterozygous female relatives showed abnormal ABRs, demonstrating the high sensitivity of ABR in detection of the existence of pathophysiological condition in subclinical or presymptomatic ALD.
对7例肾上腺脑白质营养不良（ALD）患儿、9例肾上腺脊髓神经病（AMN）成人患者、1例新生儿ALD患儿以及2例ALD携带者进行了听觉脑干反应、听觉敏感性、言语辨别功能、听觉反射阈值和反射衰减的研究。仅在2例患者中发现明显的听觉功能障碍；1例ALD患儿存在蜗后型听觉障碍，1例新生儿ALD患儿出现重度耳聋。除1例ALD患者听觉反射阈值略有升高外，其他患者的听觉反射阈值和反射衰减均无明显异常。除2例ALD患者外，其余患者的波潜伏期和波间期均显著延长。潜伏期值显示I至III间期异常发生率最高；其次是III至V间期，然后是波I。值得注意的是，1例无症状ALD患儿和2名杂合子女性亲属的听觉脑干反应均异常，这表明听觉脑干反应在检测亚临床或症状前ALD病理生理状况的存在方面具有很高的敏感性。
