Large vestibular aqueduct syndrome with mixed hearing loss: a case report

A case report of a large vestibular aqueduct syndrome (LVAS) as the sole radiographically detectable inner ear anomaly with a mixed hearing loss is presented. Hearing loss in LVAS is sensorineural with a conductive component in 15-27% of the cases. The hearing loss fluctuates and is progressive, resulting in the deterioration of the hearing after minor head trauma. The pathophysiology of the sensorineural hearing loss and the conductive component remains unknown. Endolymphatic sac surgery has been abandoned because of possible adverse results. In our case a complex anomaly was found in the stapes region without noticeable hearing gain after correction. In case of a progressive mixed hearing loss in children with a normal tympanic membrane, a CT-scan is mandatory to rule out LVAS before attempting middle ear surgery that is to no avail.