ABR and temporal bone pathology in Hurler's disease
ABR and temporal bone pathology in Hurler's disease
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DOI：
10.1016/s0165-5876(97)00176-6
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发表时间：
1998-03-01
影响因子：
1.5
通讯作者：
Suzuki, JI
中科院分区：
文献类型：
--
作者：
Komura, Y;Kaga, K;Suzuki, JI
这被认为是第一篇基于ABR与颞骨病理之间的相关性来估计赫勒氏病听力损失的报道。ABR结果显示，由于周围混合性损伤，听力损失约为70 dB或更多。颞骨的组织学研究显示，除内耳道内蛛网膜增生外，传导系统几乎所有病理结果。中耳腔内可见中耳炎、间质残留、听骨畸形。我们解释传导成分是由于中耳炎和听骨连接不良。然而，感觉神经成分的病因仍然是推测性的。在赫勒氏病中，听力丧失伴智力迟钝常发生在婴儿时期。因此，准确地评估听力损失的程度是困难的，例如，严重程度。感觉神经损伤的病因和发病率。我们强调不仅需要良好的病理研究，而且需要更客观的功能调查，至少是ABR。(C) 1998爱思唯尔科学爱尔兰有限公司版权所有。
This is believed to be the first report on estimating hearing loss in Hurler's disease, based on the correlation between ABR and temporal bone pathology. ABR findings revealed hearing loss to be about 70 dB or more as result of peripheral mixed impairment. A histological study of the temporal bones revealed almost all pathological findings in the conductive system, except for the hyperplastic arachnoid in the internal auditory canal. In the middle ear cavity, otitis media, residual mesenchyme and deformity of ossicles were found. We explain the conductive component to be due to otitis media and poor ossicular connection. However, the etiology of the sensorineural component remains speculative. In Hurler's disease, hearing loss with mental retardation is often found at infantile age. Therefore, assessing the extent of hearing loss exactly was difficult, for example, the severity. the etiology and incidence of sensorineural impairment. We emphasize the need for not only well-described pathological studies but also for more objective functional investigations, at least ABR. (C) 1998 Elsevier Science Ireland Ltd. All rights reserved.