Diseases

Usher Syndrome

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Usher syndrome, also known as hallgren syndrome, usher-hallgren syndrome, retinitis pigmentosa-dysacusis syndrome, or dystrophia retinae dysacusis syndrome, is an extremely rare genetic disorder caused by a mutation in any one of at least 11 genes resulting in a combination of hearing loss and visual impairment.

SYMPTOMS

The major symptoms of usher syndrome are hearing loss and an eye disorder called retinitis pigmentosa, or rp. Retinitis Pigmentosa causes night-blindness and a loss of peripheral vision (side vision) through the progressive degeneration of the retina.

CAUSES

The retina is a tissue at the back of the eye that is needed for vision. The gene mutation in usher syndrome affects the retina's light-sensing cells called rods and cones. This condition is called retinitis pigmentosa or rp.