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[大前庭导水管综合征:3例报告及文献复习]

[Enlarged vestibular aqueduct syndrome: report of 3 cases and literature review].

作者信息

Pinto José A, Mello Carlos Fernando, Marqui Ana Carla S, Perfeito Delmer J, Ferreira Roberto D P, Silva Rubens H

机构信息

Núcleo de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço de São Paulo.

出版信息

Braz J Otorhinolaryngol. 2005 May-Jun;71(3):386-91. doi: 10.1016/s1808-8694(15)31342-2. Epub 2005 Dec 14.

Abstract

nlarged Vestibular Aqueduct Syndrome is characterized by a widening of the vestibular aqueduct, associated with sensorineural hearing loss, or sometimes with mixed hearing loss, which may be congenital or acquired during childhood. The sensorineural hearing loss may be classified into mild, moderate and severe, associated with sudden periods of improvement or aggravation. The enlargement of the vestibular aqueduct is the most common inner ear anomaly. This syndrome is admitted as a result of a genetic abnormality of the vestibular aqueduct development, previous to the fifth week of gestation. The incidence of this syndrome ranges from 1% to 1.3%, with the possibility of getting up to 7%, depending on the examined population. The aim of this study was to analyze three cases of LVAS seen at the Otorhinolaryngology and Radiology Department of Sao Camilo Hospital - Sao Paulo. Two of these three cases were of brothers, from the same mother but from different fathers. Two were male and one was female and the ages ranged from 9 to 30 years old. The diagnostic method of election was CT - Computerized Tomography of the temporal bones. The recommended management of the cases was conservative, except for those of cranial trauma, barotrauma and, if necessary, the use of hearing aids.

摘要

大前庭导水管综合征的特征是前庭导水管增宽,伴有感音神经性听力损失,有时伴有混合性听力损失,可先天性或在儿童期获得。感音神经性听力损失可分为轻度、中度和重度,伴有突然的改善或加重期。前庭导水管扩大是最常见的内耳异常。该综合征被认为是由于妊娠第五周之前前庭导水管发育的基因异常所致。该综合征的发病率在1%至1.3%之间,根据所检查的人群不同,可能高达7%。本研究的目的是分析圣保罗圣卡米洛医院耳鼻喉科和放射科所见的三例大前庭导水管综合征病例。这三例病例中有两例是兄弟,来自同一母亲但不同父亲。两例为男性,一例为女性,年龄在9至30岁之间。首选的诊断方法是颞骨CT(计算机断层扫描)。除颅外伤、气压伤病例以及必要时使用助听器外,建议对这些病例采取保守治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/667c/9450622/7275956be563/gr1.jpg

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