Cochlear implantation with large vestibular aqueduct syndrome

Objectives: In this investigation, we report the outcomes of 14 adults (age > 18 y) and 9 children (age < 18 y) with radiographically proven large vestibular aqueduct syndrome (LVAS) who received cochlear implants at Indiana University School of Medicine. Study Design: This is a retrospective case-control study detailing the outcomes of 23 patients with LVAS and 46 control patients implanted with Nucleus (Cochlear Corp., Englewood, CO), Clarion (Advanced Bionics Corp., Sylmar, CA), or Med-El (MED-EL Corp., Innsbruck, Austria) cochlear implants. Methods: Performance data on pure-tone averages, speech detection thresholds, and a variety of auditory and speech recognition tasks from these patients with LVAS were compared with performance data obtained from a matched group of 46 cochlear implant users who did not have LVAS. Specific patient characteristics used in matching included the age of the patient, the age at implant of the patient, and whether the patient was pre- or postlingually deafened. Data for the adult group was analyzed using the Student t test, and data for the pediatric patient group was compared using a χ² test. Results: The results indicated positive outcomes for both pediatric and adult groups. With both adult and pediatric groups, auditory and speech recognition performance did not differ significantly between those patients with LVAS and control subjects. Conclusions: This study adds further support for the use of cochlear implantation in patients with LVAS. Cochlear implantation is beneficial and can be offered as an eventual treatment of LVAS if hearing loss progresses to profound levels in these patients.