To determine the nature and prevalence of ophthalmologic abnormalities in children with congenital or prelinguistic sensorineural deafness, we performed complete ophthalmologic examinations on 54 children aged 2 to 14 years from the University of Miami Ear Institute's Cochlear Implant Program. Of 54 children, 33 (61.1%) had some form of ophthalmologic abnormality, with the majority (24 patients; 44.4%) being refractive errors. Of 54 patients, two (3.7%) had strabismus and two (3.7%) had external adnexal anomalies. One child (1.8%) had cataracts. The cause of the deafness, which had previously been unknown, was determined in three of 54 cases (5.6%) as a result of ocular examinations documenting rubella retinopathy, tapetoretinal degeneration (Usher's syndrome), and iris heterochromia (Waardenburg's syndrome). Routine ophthalmologic examination must be mandated for this population, and the ophthalmologist should be encouraged to play a broader role in the care of children with deficiencies of both vision and audition.
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