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A Large Vestibular Aqueduct Syndrome Associated With Otosclerosis

Large vestibular aqueduct syndrome was first described as a morphologic and clinical entity in 1978 by Valvassori and Clemis (1). The classic radiographic description of large vestibular aqueduct is that the diameter exceeds 1.5 mm in an anteroposterior dimension measured halfway between the common crus and the external aperture. Characteristically, the enlarged vestibular aqueduct (VA) is found bilaterally and is often associated with other anatomic anomalies, including an enlarged vestibule and semicircular canals and a hypoplastic cochlea (2). Large VA syndrome has been reported to be one of the most common anomalies associated with hearing loss in childhood, and in general, the hearing loss may vary from near normal to profound. The