Usher syndrome is a rare inherited disorder that involves loss of hearing and sight. Hearing loss is usually present at birth or soon after. It is due to the inability of the auditory nerves to send sensory input to the brain. It is called sensorineural hearing loss.
The vision loss, called retinitis pigmentosa (RP), usually happens after after age ten. RP is a deterioration of the retina. The retina is a layer of light-sensitive tissue that lines the back of the eye. It changes visual images into nerve impulses in the brain that allow us to see. RP slowly gets worse over time.
Nerve and Retina of the Eye
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Three types of Usher syndrome have been identified: types I, II, and III. The age of onset and severity of symptoms separate the different types.
Last reviewedNovember 2012by Kari Kassir, MD
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