Usher syndrome
Background
Usher syndrome is a genetic condition characterised by sensory neural hearing loss with Retinitis Pigmentosa. The hearing loss is usually congenital and may be total or partial. Retinitis pigmentosa, a progressive deterioration of the retina which causes night blindness, tunnel vision and finally severely reduced central vision, may not occur until late childhood or early adulthood. In some forms, poor balance is an associated problem.