Cysts in the macula (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.Ĭhances of inheriting a recessive disorder ![]() As RP progresses, the field of vision narrows until only central vision remains, a condition called tunnel vision. The retina is the light-sensitive tissue at the back of the eye and is crucial for vision. RP initially causes night-blindness and a loss of peripheral (side) vision through the progressive degeneration of cells in the retina. 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. ![]() Less commonly, hearing loss from Usher syndrome appears during adolescence or later. Most children with Usher syndrome are born with moderate to profound hearing loss, depending on the type. ĭeafness or hearing loss in Usher syndrome is caused by abnormal development of hair cells (sound receptor cells) in the inner ear. The major symptoms of Usher syndrome are deafness or hearing loss and an eye disease called retinitis pigmentosa (RP). Usher syndrome is the most common condition that affects both hearing and vision sometimes it also affects balance.
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