Usher Syndrome
On this page:
- What is Usher syndrome?
- Who is affected by Usher syndrome?
- What causes Usher syndrome?
- What are the characteristics of the three types of Usher syndrome?
- How is Usher syndrome diagnosed?
- How is Usher syndrome treated?
- What research is being conducted on Usher syndrome?
- Where can I get more information about Usher syndrome?
What is Usher syndrome?
Usher syndrome is the most common condition that affects both hearing and vision; sometimes it also affects balance. The major symptoms of Usher syndrome are deafness or hearing loss and an eye disease called retinitis pigmentosa (RP) [re-tin-EYE-tis pig-men-TOE-sa].
Deafness or hearing loss in Usher syndrome is caused by abnormal development of hair cells (sound receptor cells) in the inner ear. Most children with Usher syndrome are born with moderate to profound hearing loss, depending on the type. Less commonly, hearing loss from Usher syndrome appears during adolescence or later. Usher syndrome can also cause severe balance problems due to abnormal development of the vestibular hair cells, sensory cells that detect gravity and head movement.
RP initially causes night-blindness and a loss of peripheral (side) vision through the progressive degeneration of cells in the retina. The retina is the light-sensitive tissue at the back of the eye and is crucial for vision. As RP progresses, the field of vision narrows until only central vision remains, a condition called tunnel vision. Cysts in the macula [MAC-u-la] (the central part of the retina) and cataracts (clouding of the lens) can sometimes cause an early decline in central vision in people with Usher syndrome.
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