The large vestibular aqueduct syndrome: the role of cochlear implantation in its management.

In recent years the large vestibular aqueduct syndrome has become an increasingly recognized cause of a progressive sensorineural hearing loss. Cochlear implantation, although not correcting the bony abnormality, does offer an avenue for rehabilitation for affected individuals and initial results are encouraging. Of 15 patients identified with the large vestibular aqueduct syndrome we have implanted seven (five adults and two children). All patients underwent an uneventful electrode insertion with uncomplicated postoperative periods. Three of our adult patients are showing very good initial results with BKB speech recognition scores of 100%, 74% and 66% with the implant alone. It is still early days with the children, but initial results are encouraging. A full review of the large vestibular aqueduct syndrome is given along with our results.