Ophthalmological screening of a paediatric cochlear implant population: a retrospective analysis and 12-year follow-up

Abstract

To determine the nature and prevalence of ophthalmological findings for a cohort of children in a paediatric cochlear implant program and to assist the clinician in devising an investigative plan for this population. Retrospective medical record review of children who underwent multichannel cochlear implantation at a tertiary care hospital between February 1996 and July 2008. In all, 141 children (mean age 28 months, range 16 months to 9 years) had complete medical record documentation consisting of orthoptic and opthalmological examination, including cycloplegic refraction. A total of 59 children (41.8%) had ocular abnormalities with refractive errors being the most common abnormality. Hypermetropia was the most common refractive error and was found in 21 children (14.8%). Strabismus was found in six patients, with constant esotropia being the most common. Ocular pathology (excluding refractive or muscle abnormalities) were found in nine patients (6.3%). Three patients had syndromes associated with ocular findings including Waardenburg and Usher syndrome. During the follow-up period, 14 children were fitted with prescription lenses, 3 had strabismus surgery, and 2 underwent ptosis. Routine orthoptic and ophthalmologic examination can be beneficial in the initial evaluation of children assessed for cochlear implants. Electroretinography is useful in evaluating children with unexplained congenital sensorineural hearing loss, suggestive symptoms including night blindness, unexplained reduction in visual acuity, or delayed motor milestones. Routine yearly follow-up may aid in the detection of changing refractive errors and the possibility of later-onset retinal degeneration.