What is Usher Syndrome
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Usher Syndrome Definition
Usher syndrome is a rare inherited disorder that involves loss of both hearing and sight. Hearing Loss is usually present at birth or soon thereafter. It is due to an impaired ability of the auditory nerves to transmit sensory input to the brain. It is called sensorineural hearing loss.
The vision loss, called Retinitis Pigmentosa (RP), begins later in childhood, usually after age ten. It slowly gets worse over time. During the teen years, loss of vision is characterized by night blindness and loss of peripheral vision. RP is a deterioration of the retina. The retina is a layer of light-sensitive tissue that lines the back of the eye. It converts visual images into nerve impulses in the brain that allow us to see.
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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 distinguish the different types.
Approximately 4 out of 100,000 infants born in the United States have Usher syndrome. Usher syndrome accounts for 3%-6% of all deaf children and 3%-6% of all hard-of-hearing children.
