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[儿童肾上腺脑白质营养不良患者双耳分听测试的异常结果]

[Abnormal findings of dichotic listening test in patients with childhood adrenoleukodystrophy].

作者信息

Shirane Seiko, Inagaki Masumi, Horiguchi Toshihiro, Nakamura Masako, Sasaki Kyoko, Kaga Makiko

机构信息

Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Ichikawa, Chiba.

出版信息

No To Hattatsu. 2004 Jul;36(4):311-7.

Abstract

Ten Japanese boys with childhood-onset adrenoleukoqdystrophy (ALD) were evaluated with dichotic listening test (DLT). Six cases showed abnormal findings especially of laterality index (L.I.) calculated from the score of each ear. Some of them showed no abnormal findings with other auditory examinations containing auditory brainstem responses (ABR). One patient showed abnormal L.I. of DLT at an early stage. The abnormality of laterality index was similar to the so-called "strong left-ear suppression" in patients who underwent callosotomy. Although all of these six patients had a high signal lesion at the splenium of the corpus callosum in a T3-weighted MRI sequence, it was difficult to evaluate the width of demyelinated area. DLT could detect the early damage of connecting fibers mediating inter-hemispheric transfer of auditory information, and might be a useful method for evaluating the cerebral function of auditory processing in patients with childhood ALD.

摘要

对10名患有儿童期肾上腺脑白质营养不良(ALD)的日本男孩进行了双耳分听测试(DLT)。6例显示出异常结果,尤其是根据每只耳朵的得分计算出的偏侧指数(L.I.)。其中一些患者在包括听性脑干反应(ABR)在内的其他听觉检查中未显示异常结果。一名患者在早期显示出DLT的偏侧指数异常。偏侧指数的异常类似于接受胼胝体切开术患者中所谓的“左耳强烈抑制”。尽管这6名患者在T3加权MRI序列中胼胝体压部均有高信号病变,但难以评估脱髓鞘区域的宽度。DLT可以检测到介导听觉信息半球间传递的连接纤维的早期损伤,可能是评估儿童ALD患者听觉处理脑功能的一种有用方法。

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