Onset of Ocular Abnormalities in Children with Hearing Loss

Children with hearing loss commonly are referred for eye examinations, but the optimal timing of asymptomatic ophthalmologic screening examinations is unclear. We sought to determine the prevalence and onset of abnormal ocular findings in children with hearing loss.

Retrospective cohort study.

Children with hearing loss seen for any indication by the pediatric ophthalmology practice at Children's Hospital of Philadelphia over a 15-year period.

Ophthalmology and other medical records, including slit-lamp and fundus examination findings and genetic testing results, were reviewed.

Prevalence and onset of ocular abnormalities and sensitivity and yield of continuing screening eye examinations by age.

Of 11 437 children with hearing loss, 2729 children showed sensorineural, 3399 children showed conductive, 1601 children showed mixed, and 3708 children showed unspecified types of loss. Ocular abnormalities were identified in 2199 children (19.2%), including 1,339 children (12.7%) with possible syndromic association (e.g., retinal degeneration or signs of a genetic syndrome). Among the latter, the cumulative sensitivity of continued screening was 48% to 72% by 5 years of age, 71% to 87% by 10 years of age, and 90% to 96% by 15 years of age. The estimated yield of continued screening beyond 5 years of age was 3.7%, beyond 10 years of age was 1.9%, and beyond 15 years of age was 0.6%.

Ocular abnormalities in children with hearing loss may present throughout childhood and adolescence. We recommend that asymptomatic children undergo an ophthalmology examination on diagnosis of hearing loss, 2 years later, and again at 5, 10, and 15 years of age, or earlier if visual symptoms develop or pediatrician vision screening identifies a concern. When genetic testing is possible, an identified genetic cause may help to tailor the timing of ophthalmology examinations.

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