Usher Syndrome in Adult Nigerians: Report of Two Cases

Abstract

This is a report of Usher syndrome in adult Nigerians. Two adults aged 28 and 33 years had retinal degeneration, optic atrophy and visual fields defects consistent with retinitis pigmentosa. Each patient also had a hearing impairment and one patient had ataxia. These are features of Usher syndrome which usually manifests in the first and second decades of life. The late presentation of our patients reflects the inadequacy of the relevant services that could have facilitated early detection and management of the cases. Routine screening of pre-school children for visual and hearing loss is recommended for early detection of these cases.