Cochlear implants in aplasia and hypoplasia of the cochleovestibular nerve

Objective: To report on the outcome of four patients with aplasia or hypoplasia of the cochleovestibular nerve who have received a cochlear implant. Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Four patients with: 1) type I aplasia; 2) type IIa aplasia: 3) type IIa hypoplasia: and 4) type IIb aplasia received a cochlear implant. All patients had corner audiograms even with hearing aids. Intervention: Three patients received a LAURA multichannel implant, and one patient received a Nucleus 24 implant. Main Outcome Measure: Auditory performance, educational setting. Results: The patients with type I and type IIb aplasia did not have auditive perception with their implant and became non-users. Both are now in a total communication educational setting. The patients with type IIa aplasia and hypoplasia had moderate audiological results with the implant with audiometrical thresholds of approximately 40-60 dB HL (pure tone average), moderate phoneme discrimination, and poor word discrimination. One child is in a total communication educational setting and the other in an oral educational setting, but the preferred mode of communication remains total communication for both. Both appear to benefit from the implant nonetheless. Conclusion: Patients with aplasia/hypoplasia of the cochleovestibular nerve should be counseled with caution with respect to cochlear implantation, but particular circumstances may justify the intervention. At present these circumstances seem to be a type IIa aplasia or hypoplasia in which the end organ (cochlea or common cavity) still connects to a neural structure on MRI.