Co-incidence of sensorineural hearing loss and ophthalmic abnormalities has a tremendous influence on development of children. This study was done to determine the nature and prevalence of ophthalmic disturbances in children with congenital sensorineural hearing loss. In a descriptive cross-sectional study, complete ophthalmologic examinations such as assessment of visual acuity, cycloplegic refraction, ocular motility examination, slit lamp examination and indirect funduscopy on 50 children with sensorineural hearing loss (determined by ABR), were performed. Thirty-two percent of cases had at least one kind of ophthalmic disturbances. Refractive errors were the most common abnormalities (28%), including astigmatism (12%), myopia and astigmatism (8%), and hyperopia (8%). Three (6%) cases had ocular motility disturbance including one case of esotropia, one case of exophoria and one case of exotropia. Twenty-four percent of cases had retinal abnormalities, including suspected Rubella retinopathy (6%), pigmentary changes suspect to retinitis pigmentosa (4%), optic nerve hypoplasia (4%), and finally poor fovea reflex (10%).