TreatmentĬurrent treatment focuses on helping an individual to adapt to hearing and vision loss and to maximising the vision and hearing that they do have. Usher syndrome occurs equally in both genders and is always inherited in a recessive pattern, meaning that both a person’s copies of the gene must be faulty for the condition to occur. So far, researchers have found 11 genes that are associated with the three main subtypes of the syndrome. 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. The prevalence of Usher syndrome varies from country to country, but it is a rare condition affecting approximately 1 in 10,000 people. Hearing at birth is usually normal but is lost during late childhood and becomes worse over time, with sight beginning to deteriorate during adolescence. Usher type 3 is very rare and is generally found in people with their family origins in Finland. Sight loss usually becomes apparent during the teenage years. People with Usher type 2 also experience early onset hearing loss but this is less severe than in type 1. Balance is often affected in those with type 1 and can slow the development of sitting or walking. The deafness is generally so early in onset and so severe that hearing aids may not be of value, although cochlear implants may be beneficial. People with Usher type 1 develop profound deafness from birth and sight loss becomes evident in early childhood. Sight loss follows a similar pattern to non-syndromic retinitis pigmentosa early symptoms include night blindness and reduced peripheral vision, with central vision loss occurring later. ![]() Hearing loss is the first symptom to become apparent, usually from birth. There are three different clinical types of Usher syndrome, type 1, type 2, and type 3. Recordings and resources Expand dropdown.Joining the research effort Expand dropdown. ![]() Information and support services Expand dropdown.About inherited sight loss Expand dropdown.The UK Inherited Retinal Dystrophy Consortium.
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