Ushers Syndrome

What is Ushers Syndrome?

Ushers syndrome is a form of retinitis pigmentosa (RP), with associated deafness. Ushers syndrome is the cause of approximately 10% of all hereditary deafness, with deafness existing at birth or developing soon afterward. The eye symptoms of RP appear later, usually by the age of 10, and start with night blindness. Visual acuity continues to decrease as the child grows older, and may result in complete blindness by midlife.

People with RP may develop other treatable eye diseases, such as glaucoma and cataract.

Symptoms

  • Hearing loss
  • Night blindness
  • Loss of peripheral vision 

The symptoms described above may not necessarily mean that you have ushers syndrome or retinitis pigmentosa. However, if you experience one or more of these symptoms, contact your eye doctor for a complete exam.

Treatment

Currently, there is no specific treatment for persons with Ushers syndrome or RP. Genetic studies of RP are a significant factor in finding a cure or prevention for this disease. The U-M is performing research on genetic factors of RP.

Research

Researchers at the University of Michigan Department of Ophthalmology are not only conducting cell-biological research on these eye diseases, they are vigorously working on molecular genetic aspects of X-chromosomal retinal/macular dystrophies. The molecular information obtained in testing has become critical, in some cases, for refining diagnosis. The investigators at the University of Michigan Department of Ophthalmology have broad experience with the molecular genetics of a number of retinal conditions. In addition to research relating to Ushers syndrome, other research areas include X-linked blue cone monochromacy, X-linked cone dystrophy, Best's macular degeneration, Stargardt's macular degeneration, Sorsby's hemorrhagic macular dystrophy, and choroideremia.

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