Abstract
We report two cases of large vestibular aqueduct syndrome, which was first described by Valvassori and Clemis in 1978. A 12-year-old female and a one-year-old male presented with bilateral profound hearing loss. Auditory evoked brainstem response was performed in the latter case, but no reproducible wave was recorded bilaterally. High-resolution axial CT of the temporal bone revealed markedly enlarged vestibular aqueducts bilaterally in the former case and unilaterally in the latter. No significant anomaly was found in any other part of the temporal bone on C T scans.The possible etiology of sensorineural hearing loss in this syndrome is discussed, and the available literature is reviewed.
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