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Usher syndrome cause8/12/2023 If you inherit a changed copy of a specific Usher gene, for example MYO7A, from one parent, and also a changed copy of the same gene from your other parent, you will develop Usher syndrome. What are the chances of inheriting Usher syndrome? It affects between 4 and 17 people in 100,000 worldwide. This affects as many as 50% of people with retinitis pigmentosa. Leakage of blood vessels in the retina causes swelling of the macula.Some people may also get the following eye conditions, which can cause further sight deterioration: Balance problemsįor some people, Usher syndrome can cause problems with balance because of damage to the inner ears. Others lose much more of their sight in early adulthood.Įventually, most people with retinitis pigmentosa lose most of their sight. Some people retain their central vision and a restricted visual field into their 50s. Some people also notice that they cannot make out different colours as clearly as before. Sight loss happens over time until you’re left with a small area of central vision. Retinitis pigmentosa makes it hard to see at night or when it’s dark or dim, and causes loss of peripheral (side) vision. Usher syndrome causes sight loss through a condition called retinitis pigmentosa. Some people can manage their hearing loss with hearing aids or cochlear implants. Usher syndrome causes sensorineural hearing loss, which damages the inner ears. What are the symptoms of Usher syndrome? Hearing loss It can take years for symptoms to appear and is usually diagnosed in older children or teenagers. Genes are sets of information that instruct the growth and development of every cell in every part of your body.Ĭhanges in specific genes disrupt the healthy growth and development of the hair cells of the inner ear and the cells in the retina of the eyes, causing Usher syndrome. Inherited conditions are caused by changes in specific genes you inherit from your parents. Usher syndrome is an inherited condition, meaning it is present from birth. Read more about how the Sense Usher Service can help you.Ĭontact us at for more information. With the right support, you can overcome the challenges presented by Usher syndrome, and live a full independent life.
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