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Bilateral large vestibular aqueduct syndrome in an 11-year-old boy. Radiological and clinical findings
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Abstract
Clinical observations supplemented with imaging examination show that the large vestibular aqueduct syndrome (LVAS) is a rare developmental anomaly of the inner ear, which leads to hearing loss. The authors present a case history, results of imaging examination (high resolution CT, MRI), results of hearing acuity examinations (tonal audiometry, otoacoustic emissions, brainstem auditory evoked potentials) and results of balance examinations (videonystagmography) in an 11-year-old boy suffering from deep mixed progressive hearing loss of the right ear due to head trauma. The aim of this paper is to specify the most typical clinical, radiological and anatomopathological manifestations of this pathology of the inner ear. The authors describe the diagnostic and identification difficulties associated with the mixed hearing loss observed in this case. The article also discusses the child’s activity limitations, which should be taken into account once diagnosis of this rare labyrinthine pathology is established.
Abstract
Clinical observations supplemented with imaging examination show that the large vestibular aqueduct syndrome (LVAS) is a rare developmental anomaly of the inner ear, which leads to hearing loss. The authors present a case history, results of imaging examination (high resolution CT, MRI), results of hearing acuity examinations (tonal audiometry, otoacoustic emissions, brainstem auditory evoked potentials) and results of balance examinations (videonystagmography) in an 11-year-old boy suffering from deep mixed progressive hearing loss of the right ear due to head trauma. The aim of this paper is to specify the most typical clinical, radiological and anatomopathological manifestations of this pathology of the inner ear. The authors describe the diagnostic and identification difficulties associated with the mixed hearing loss observed in this case. The article also discusses the child’s activity limitations, which should be taken into account once diagnosis of this rare labyrinthine pathology is established.
Keywords
large vestibular aqueduct syndrome, hearing loss, congenital inner ear defect
About this articleTitle
Bilateral large vestibular aqueduct syndrome in an 11-year-old boy. Radiological and clinical findings
Journal
Issue
Article type
Case report
Pages
265-271
Published online
2015-05-28
Page views
1416
Article views/downloads
3701
DOI
10.5603/FM.2015.0022
Pubmed
Bibliographic record
Folia Morphol 2015;74(2):265-271.
Keywords
large vestibular aqueduct syndrome
hearing loss
congenital inner ear defect
Authors
T. Przewoźny
K. Markiet
J. Piątkowski
