Enlarged vestibular aqueduct syndrome is a clinical condition characterized for a progressive perceptive deafness linked to a broadening of vestibular aqueduct greater than 1.5 mm without other otic abnormal structures. We report the case of a 2-year-old child with such congenital malformation. We have reviewed the actual literature and point out its clinical and physiopathologic features as well treatment possibilities of these cases.

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