Audiologic Outcomes After Cochlear Implantation in Prelingually Deaf Children Who Have Combined Anomalies or Disabilities

Abstract

Objective: To evaluate the audiologic outcomes after cochlear implantation (CI) in prelingually deaf children with comorbid disorders.

Study design: Retrospective study.

Setting: Tertiary care academic center.

Patients: We enrolled 85 deaf children (111 ears) with comorbid disorders who underwent CI surgery.

Main outcomes and measures: We selected the most common comorbid disorder and compared the categories of auditory performance (CAP) scores of each comorbidity group with those of the control group. Combined cochlear anomalies were also evaluated using temporal bone computed tomography.

Results: The most common comorbid disorder was CHARGE syndrome (n = 17), followed by developmental delay (n = 13), valvular heart disease (including septal defect) (n = 13), Waardenburg syndrome (n = 8), Down syndrome (n = 6), cerebral palsy (n = 5), and cryptorchidism (n = 5). The CAP scores differed significantly between all the groups and the control group after 6 months (p < 0.001). The differences in the CHARGE syndrome (p = 0.004), cerebral palsy (p = 0.001), and Down syndrome (p = 0.004) groups remained significant 2 years after CI. When comparing the presence or absence of anomalies within the syndromic and nonsyndromic groups, significantly poorer outcomes exist when cochlear anomalies with absent modiolus or cochlear nerve deficits were observed, regardless of the presence of syndromic disorders.

Conclusion: Compared with those in the control group, the CAP scores in the group with coexisting disabilities increased over time, but their final scores were relatively lower. Combined disabilities as well as the absent modiolus and cochlear nerve deficit may be clinical markers for poor CAP scores.