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Chen-Chi Wu, MD, MMS; Yi-Chin Lee, MD, MPH; Pei-Jer Chen, MD, PhD; Chuan-Jen Hsu, MD, PhD

Arch Pediatr Adolesc Med. 2008;162(3):269-276.

Objective  To investigate the roles of genetic diagnosis and imaging studies, as well as other prognostic factors, in predicting outcomes in children with cochlear implant.

Design  Prospective cohort study.

Setting  Tertiary referral center.

Participants  Sixty-seven consecutive children with sensorineural hearing impairment who had at least 3 years of experience with cochlear implant.

Interventions  Imaging of the inner ear was done with high-resolution computed tomography, and mutations were screened in 3 genes commonly associated with pediatric hearing impairment: GJB2, SLC26A4, and the mitochondrial 12S ribosomal RNA gene. Speech perception performance was compared according to genetic diagnosis and imaging data. A general linear model was constructed to demonstrate the predictive values of specific genetic and imaging results after adjusting for other factors.

Main Outcome Measure  Recognition scores on speech perception tests.

Results  Twenty-two children (33%) showed genetic mutations: 18 with SLC26A4 and 4 with GJB2 mutations. According to imaging findings, 33 children (49%) showed inner ear malformations: 9 with a narrow internal auditory canal and 24 with other malformations. All children with SLC26A4 or GJB2 mutations exhibited excellent speech recognition scores, whereas a narrow internal auditory canal was associated with poorer outcomes (P < .001 in all recognition scores). The general linear model confirmed that both a narrow internal auditory canal (P < .001) and SLC26A4 mutations (P = .04) correlated with speech perception outcome.

Conclusions  Genetic diagnosis and imaging results are the 2 predominant factors determining the outcome in children with cochlear implants. In pediatric candidates for cochlear implantation, both genetic examination and imaging studies should be included in the battery of preoperative evaluations.

Author Affiliations: Division of Pediatric Otolaryngology, Departments of Otolaryngology (Drs Wu and Hsu) and Medical Genetics (Dr Wu), National Taiwan University Hospital and Graduate Institute of Clinical Medicine, College of Medicine (Drs Wu and Chen) and Division of Biostatistics, Graduate Institute of Epidemiology, College of Public Health (Dr Lee), National Taiwan University, Taipei.

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