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耳硬化症的病因概述。

An overview of the etiology of otosclerosis.

作者信息

Markou Konstantinos, Goudakos John

机构信息

Otolaryngology Department, AHEPA University Hospital, Aristotle's University of Thessaloniki, 54006, Thessaloniki, Greece.

出版信息

Eur Arch Otorhinolaryngol. 2009 Jan;266(1):25-35. doi: 10.1007/s00405-008-0790-x. Epub 2008 Aug 13.

Abstract

Otosclerosis is the primary disease affecting the homeostasis of otic capsule and is among the most common causes of acquired hearing loss. Otosclerosis is considered as a multifactor disease, caused by both genetic and environmental factors. The aim of the present review is to summarize and analyze the bibliographic data, associated with the etiology of the disease. In some cases, the otosclerosis has an autosomal dominant mode of inheritance with incomplete penetrance. Genetic studies reveal the occurrence of at least nine chromosomal loci as candidate genes of the disease. The localized measles virus infection of the otic capsule has been postulated as a possible etiological theory. The role of hormonal factors, immune and bone-remodeling system in the etiopathogenesis of otosclerosis and the association of the disease with the disorders of the connective tissue are the issues of the present study. Despite the extensive research, many etiological factors and theories have been suggested and the process of development of the otosclerosis remains unclear.

摘要

耳硬化症是影响耳囊内环境稳定的主要疾病,也是后天性听力损失的最常见原因之一。耳硬化症被认为是一种多因素疾病,由遗传和环境因素共同导致。本综述的目的是总结和分析与该疾病病因相关的文献数据。在某些情况下,耳硬化症呈常染色体显性遗传模式,具有不完全外显率。遗传学研究揭示了至少九个染色体位点作为该疾病的候选基因。耳囊局部麻疹病毒感染被认为是一种可能的病因理论。激素因素、免疫和骨重塑系统在耳硬化症发病机制中的作用以及该疾病与结缔组织疾病的关联是本研究的课题。尽管进行了广泛的研究,但已经提出了许多病因因素和理论,耳硬化症的发展过程仍不清楚。

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