The large vestibular aqueduct syndrome as a cause for sudden deafness in children

A case report and a review of the literature on the 'large vestibular aqueduct syndrome' are presented. This syndrome purports to be a large vestibular aqueduct with a sensorineural or combined sensorineural and conductive hearing loss. The vestibular aqueduct has retained its wide embryologic size. A though indications have been found supporting a possible genetic origin for the persistence of the large aqueduct, many questions remain on the development of the hearing loss. Once the diagnosis is suspected, an MRI or CT scan will reveal the large vestibular aqueduct. Operations proposed to stop the decline of the hearing or even to improve it, have not been successful to date. For this reason, these patients mostly rely on revalidation.