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

Abstract

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.

Figure 1

Audiometry with sensorineural hearing loss on the left and mixed loss on the right.

Figure 2

CT scan showing bilateral large vestibular aqueduct.

Figure 3

CT scan showing bilateral large vestibular aqueduct, especially on the right.

Figure 4

Audiometry showing bilateral mixed hearing loss.

Figure 5

Audiometry showing mixed hearing loss on the left.

Figure 6

CT showing enlargement of left vestibular aqueduct.