Long-term outcomes of cochlear implantation in children with cochlear nerve deficiency: auditory speech performance and predictive factors over 5 years.

PURPOSE: While cochlear implantation (CI) is increasingly offered to children with cochlear nerve deficiency (CND), long-term outcomes and their predictors remain understudied. We evaluated 5-year auditory-speech performance and identified factors influencing outcomes.

METHODS

A retrospective review of the UKM Cochlear Implant Program (2007-2024) included 12 children with CND (CI aged 14-52 months). Primary outcomes measured were unaided/aided hearing thresholds, Categories of Auditory Performance II (CAP-II), Speech Intelligibility Rating (SIR) scores preoperatively and at 1/3/5 years post-CI.

RESULTS

11/12 children achieved CAP-II ≥ 5 (understanding phrases without lipreading); 7/12 attained SIR ≥ 3 (intelligible speech with lipreading) 5 years post-CI. Earlier implantation correlated with better hearing thresholds (p < 0.05). Higher baseline CAP-II/SIR scores predicted superior outcomes (p < 0.05). Visible cochlear nerves (vs. absent) were associated with higher 5-year CAP-II/SIR scores (p < 0.05).

CONCLUSION

CI provides significant long-term benefits for children with CND, with outcomes influenced by age at implantation, preoperative function, and nerve visibility. These factors should guide candidacy and parental counselling, reinforcing CI's viability even in radiologically severe CND.