Background: Deaf children are heavily reliant on the sense of vision in order to develop efficient communication skills and explore the world around them. Any ophthalmic disorder may thus negatively impact on this process, especially if it is unrecognised in the early years of life. These disorders may be correctable (such as myopia) or treatable (such as cataract), and their early identification is of the utmost importance to optimise language development (spoken or sign, or both) and develop social cognition. Those children with non-correctable and non-treatable visual disorders, like retinitis pigmentosa in Usher syndrome, require multiple environmental adaptations and appropriate support services and information.
Aim: : To review the accumulated scientific knowledge on ophthalmic disorders in deaf children and assess the quality of evidence published in the literature in order to contribute to better diagnosis and management of these conditions.
Material and methods: The project reviewed more than 1000 published papers and other sources. 191 papers complied with the aims of the study and were used in the project. From these studies, 95% were based on type III or IV evidence (mainly descriptive studies or case reports). Only 3% were based on type II evidence and 2% on type I evidence.
Results-conclusions: The main conclusions of this project are: a) the overall quality of evidence in the literature concerning deaf children and their ophthalmic problems is very low, b) the prevalence of ophthalmic problems in deaf children is very high (approximately 40% to 60%) and these problems may remain undetected for years although they may have a serious impact on children's acquisition of communication skills, c) screening for ophthalmic problems in deaf children should be encouraged and specialist ophthalmic examination should be carried out as soon as the diagnosis of deafness is confirmed irrespective of age, and may need to be repeated at intervals following diagnosis, d) families should be informed about the nature of the screening process in discussion with the relevant professionals and appropriate information should be available in a range of formats and in different community languages, e) professionals administering the tests should be familiar with the needs of deaf children with ophthalmic problems and should be sensitive to the communication needs of the child, especially undertaking behavioural testing where their collaboration is needed, f) while orthoptists can perform the majority of psychophysical tests (visual and stereo acuity tests, ocular motility tests, etc.) a comprehensive opthalmologic assessment by slit lamp biomicroscopy, streak retinoscopy, direct and indirect ophthalmoscopy, intraocular pressure measurement etc is required. Electrophysiologic testing to help identification of Usher syndrome may also be required, and finally g) serial hearing assessments of children with dual sensory deficits are needed to monitor hearing thresholds, to optimise hearing aid use and to ensure timely referral for cochlear implantation for those who need it.