Favorable outcome of cochlear implant in VIIIth nerve deficiency.

Objective: To report on the outcomes of cochlear implantation (CI) in a child with cochleovestibular nerves (CVN) hypoplasia.
Study Design: Retrospective case review.
Setting: Tertiary referral center, University hospital.
Patients: An 18-month-old child with profound bilateral congenital hearing loss and bilateral hypoplasia of the CVN at imaging.
Intervention: Left CI at age 29 months with a Nucleus Contour device (Cochlear Ltd., Lane Cove, New South Wales, Australia) after unsatisfactory results of hearing aid use for 10 months.
Main Outcome Measures: Speech perception tests, behavioral observation, electrophysiologic tests, and cognitive evaluation.
Results: Although the child scores poorly in every perceptive category with the CI alone, the device greatly enhances his speech understanding with the hearing aid in the opposite ear. In the bimodal condition, his words and sentences identification, recognition, and comprehension far exceed the monaural figures. The Meaningful Auditory Integration Scale (MAIS) tests reaches a score of 26/40, and the MacArthur's questionnaires confirm the improvement of language production and comprehension. These results became noticeable after 5 to 6 months and continued to improve up to the 10th month. The child's cognitive scores and overall performance competences greatly benefit from the CI, with the mental age overcoming the chronological age.
Conclusion: We can confirm the chance of achieving satisfactory results by CI even when the imaging of CVN is doubtful and the electrophysiological tests are disappointing. In our experience, a CI in Type IIb dysplasia of the CVN is a feasible option, provided that the candidate shows some responses at aided audiogram and at least minimal signs of language development. Adequate counseling is necessary for these children because the expected outcome is somewhat lower than that of their deaf peers with normal appearance of the nerves.