Cochlear implantation in children with large vestibular aqueduct syndrome.

OBJECTIVE

This study describes the effectiveness of a multielectrode cochlear implant prosthesis (Cochlear; Cochlear Pty., Lane Cove, Australia) for providing hearing to children with deafness caused by large vestibular aqueduct syndrome (LVAS).

STUDY

DESIGN

The study design was a retrospective study.

SETTING

All the children attended The Children's Cochlear Implant Center (NSW), which is a specialist center that provides audiologic testing, speech therapy, habilitation, and medical assistance for children with cochlear implants.

PATIENTS: Ten children were studied who had profound hearing loss and radiologic evidence of a vestibular aqueduct larger than 2 mm in width in its intraosseous portion.

INTERVENTION: The children received a multielectrode (Cochlear) cochlear implant prosthesis, and the associated programming of the device and habitation were performed postoperatively. No significant problems were encountered at any of the surgeries, although there was an initial gush of perilymph when the otic capsule was opened in 7 ears.

MAIN OUTCOME MEASURES: Postoperative audiologic performance at six monthly intervals and school performance were assessed.

RESULTS

The postoperative auditory performance was improved in all children. At 6 months, their average BKB score had increased from 31% to 79%; average word score, from 8% to 43%; and average phoneme score, from 38% to 70%. The older children were able to continue their education in their usual setting with less reliance on hearing support staff.

CONCLUSION

Children with a deteriorating hearing loss caused by LVAS can derive considerable benefit from a cochlear implant.