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

Enlarged 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, moderat...

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Bibliographic Details
Published in:Brazilian journal of otorhinolaryngology 2005-05, Vol.71 (3), p.386-391
Main Authors: Pinto, José A., Mello, Carlos Fernando, Marqui, Ana Carla S., Perfeito, Delmer J., Ferreira, Roberto D.P., Silva, Rubens H.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age Factors
Case Report
Child
Cochlear Implantation
Diagnosis, Differential
enlarged vestibular aqueduct
Female
floating sensorineural hearing loss
Hearing Loss, Sensorineural - diagnosis
Hearing Loss, Sensorineural - genetics
Hearing Loss, Sensorineural - therapy
Humans
Male
mixed hearing loss
Syndrome
Tomography, X-Ray Computed
Vestibular Aqueduct - diagnostic imaging
Vestibular Diseases - diagnosis
Vestibular Diseases - genetics
Vestibular Diseases - therapy
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Summary:Enlarged 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.
ISSN:1808-8694
1808-8686
DOI:10.1016/S1808-8694(15)31342-2