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  2. Usher syndrome affects approximately 4 to 17 per 100,000 people, 1, 2 and accounts for about 50 percent of all hereditary deaf-blindness cases. 3 The condition is thought to account for 3 to 6 percent of all children who are deaf, and another 3 to 6 percent of children who are hard-of-hearing. 4

  3. Mar 4, 2024 · The diagnosis of Usher syndrome is ultimately established through genetic testing and identification of a pathologic genetic variant. Finally, there is currently no cure for Usher syndrome, therefore, treatment is largely aimed at improving the individual’s quality of life.

  4. Usher syndrome occurs when there are mutations in genes that are important for the function of the photoreceptors in the retina and the sound-sensing “hair cells” in the cochlea, or inner ear. So far, researchers have found 11 genes that are associated with the three main subtypes of the syndrome.

  5. It's estimated that over 400,000 people worldwide have Usher syndrome. However, since the data on the number of USH patients in Ireland is limited, it's our estimate there could be around 200-260 people living with Usher syndrome here.

  6. Aug 14, 2023 · Learn about Usher Syndrome, including symptoms, causes, and treatments. If you or a loved one is affected by this condition, visit NORD to find resources and Patients & Caregivers

  7. Usher syndrome causes vision loss and hearing loss. It may cause balance issues. There are three types of Usher syndrome. Each type causes varying symptoms that develop at different times. Usher syndrome is an inherited disorder. Its symptoms may get worse over time but don’t affect your life expectancy. Treatment focuses on managing symptoms.

  8. Jul 31, 2020 · Briefly, Usher syndrome type 1 is associated with the most severe early-life affection of the eye and the ear, whereas types 2 and 3 present with a less profound affection at early age.

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