Objectives. To evaluate children with inner ear malformations following cochlear implantation (CI) in a tertiary pediatric hospital in Singapore to identify factors influencing outcomes after CI. Methods. This is a retrospective cohort study of children aged 0 to 18 years, who had CI between 2000 and 2013. Demographic information, data on risk factors, type of inner ear malformation (IEM), age at implantation, speech pre- and postimplantation, and duration of follow-up were collected from clinical records. Operative details and audiological outcomes were also analyzed. Results. A total of 70 children underwent 83 CI surgeries. The mean age of the patients was 4.05 ± 3.17 years (range 1–18 years). Twenty patients (28.57%) had abnormal CT scan findings. CSF gusher occurred in 15 out of 26 CI (57.69%) in the group with IEM. Nine out of twenty patients (45.00%) had poor IT-MAIS scores prior to implantation. The average preoperative IT-MAIS score for children with anomalous inner ear anatomy was 14.1. The older CI patients, 3/20 (15.00%), mean age 8.33 years (range 7–10 years), were mostly referred for persistently unclear speech following hearing aids. Eleven patients (55.00%) had good speech and aided hearing threshold within speech limits after CI and were eligible for reintegration into mainstream schools. Five patients (25.00%) had improvement in speech but continued to receive education in special schools. Four patients (20.00%) had poor progress after surgery. Conclusion. The presence of absent cochlear nerve, electrode folding, and underlying neurological disorders seemed to be associated with poorer outcomes.
Cochlear Implantation in Children with Inner Ear Malformation and Postoperative Performance.
