Lessons from an Analysis of Newborn Hearing Screening Data for Children with Cochlear Implants

Objectives: The aims of the study are to identify the limitations of the current newborn hearing screening (NHS) programs and provide recommendations for better protocols. Study Design: Retrospective analysis. Setting: Tertiary referral center. Patients: The study participants were 185 children who received cochlear implants (CIs) at ≤5 years of age. Interventions: Therapeutic and rehabilitative. Main Outcome Measures: The results of NHS, screening tools used, age, and hearing thresholds at which hearing loss was confirmed, causes of the hearing loss, age of CI insertion, aided pure-tone audiogram findings, and language development level were analyzed. Results: NHS data was available for 109 children, and 24 patients (22.0%) had passed NHS for both ears. Hearing loss was confirmed considerably later in children who had passed NHS than in children who were referred for further evaluation (p < 0.01). The most common cause for hearing loss in the NHS-pass group was SLC26A4 mutations (41.7%). Patients in the NHS-pass group received CIs considerably later than those in the NHS-referred group (p < 0.01). Among patients with SLC26A4 mutations, the language development level was significantly lower in the NHS-pass group than in the NHS-referred group (p < 0.01). Conclusions: Careful counseling regarding NHS results is necessary for parents to understand that the absence of hearing loss at birth does not mean that the child will not develop hearing loss later in life. Genetic testing for SLC26A4 mutations may be necessary in regions with a high incidence of these mutations, such as East Asia.