A clinical evaluation of ophthalmic assessment in children with sensori-neural deafness.

BACKGROUND: Since 1991, children with sensori-neural deafness and their families within Southern Derbyshire have been supported by a multiagency approach to their diagnosis and management.
RESULTS: One hundred and twenty-two children were seen at the Child Development Centre for an holistic assessment, and 110 of these children have had an ophthalmic assessment, of whom 48 (43.6%) were found to have ophthalmic abnormalities. These included 43 children (39.1%) with refractive errors, ranging from mild to severe. Of 82 children with uncomplicated deafness, 26 (31.7%) had refractive errors. The prevalence of ophthalmic problems was higher than the prevalence in hearing school children in the literature and in local pre-school children. There were six cases of Usher syndrome. Twenty-one children had more than one eye defect. The findings indicate that deaf and hearing impaired children are two to three times more likely to develop ophthalmic abnormalities than their hearing peers, which makes early detection of paramount importance. An important local outcome has been the establishment of guidelines for ophthalmic assessment to include assessment of vision and screening for Usher syndrome by electro-physiological testing at aged 7 years and above. Screening has been completed to date in 78 children with congenital sensori-neural deafness.
CONCLUSIONS: Sensitive and efficient ophthalmic assessment and management are essential for all deaf and hearing impaired children and national guidelines need to be established for both assessment of vision and screening for Usher syndrome, based on further evaluated research.