The large vestibular aqueduct syndrome: A cause of neurosensory dysacusia

Background: The large vestibular aqueduct syndrome (LVAS) is characterized by the enlargement of the vestibular aqueduct associated with sensorioneural hearing loss. The level of hearing loss varies and may be fluctuant, progressive or sudden. Vestibular symptoms may be present. The diagnosis is reached by imaging methods. Aim: To report an LVAS case. Method: A female infant was submitted to a computerized tomography of the ears and to audiologic tests. Results: Enlargement of the vestibular aqueduct of more than 1.5mm and sensorioneural hearing loss in the right ear were observed. Conclusion: With an early hearing evaluation it is possible to diagnose hearing loss, even in children were this loss is unilateral. Although the literature indicates that the diagnosis of LVAS occurs at a later age, in this case time etiologic diagnosis was enabled by computerized tomography.