黏多糖贮积症的耳科表现。

The otologic manifestations of mucopolysaccharidoses.

作者信息

Hayes E, Babin R, Platz C

出版信息

Am J Otol. 1980 Oct;2(2):65-9.

Abstract

Patients with mucopolysaccharidoses are frequently hard of hearing. This disorder is often conductive in nature and considered the result of chronic serous otitis media. Such patients frequently undergo myringotomy. A coexisting progressive neurosensory hearing loss in some children is recognized far less often. Examination of temporal bones in such patients suggests arrested development including incomplete pneumatization. Hair cell dysfunction is implicated in the neurosensory component. Close communication among pediatrician, otolaryngologist, and speech and hearing personnel is essential to maximize the communicative skills of these patients.

摘要

黏多糖贮积症患者常伴有听力障碍。这种病症本质上多为传导性的，通常被认为是慢性浆液性中耳炎的结果。此类患者常接受鼓膜切开术。而一些儿童同时存在的进行性神经感觉性听力损失却很少被发现。对这些患者颞骨的检查显示发育停滞，包括气化不完全。毛细胞功能障碍与神经感觉成分有关。儿科医生、耳鼻喉科医生以及言语和听力专业人员之间的密切沟通对于最大限度地提高这些患者的沟通能力至关重要。