A Ten-Year Review of Audiological Performance in Children with Inner Ear Abnormalities after Cochlear Implantation in Singapore.

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.