![]() ![]() Higher than average numbers of people with Usher syndrome have been found among Jewish people in Israel, Berlin, Germany French Canadians of Louisiana Argentineans of Spanish descent and Nigerian Africans. More than 400,000 people are affected by Usher syndrome worldwide. Usher syndrome types 1 and 2 account for approximately 10 percent of all cases of moderate to profound deafness in children. Usher syndrome is the most common genetic disorder involving both hearing and vision abnormalities. Usher syndrome is the most common genetic cause of combined deafness and blindness. However, many people with retinitis pigmentosa retain some central vision throughout their lives. In some cases, vision is further impaired by clouding of the lens of the eye (cataracts). Over time, these blind spots enlarge and merge to produce tunnel vision (loss of all peripheral vision). Night vision loss begins first, followed by blind spots that develop in the side (peripheral) vision. Vision loss occurs as the light-sensing cells of the retina gradually deteriorate. The loss of vision is caused by an eye disease called retinitis pigmentosa, which affects the layer of light-sensitive tissue at the back of the eye (the retina). The hearing loss is classified as sensorineural hearing loss, which means that it is caused by abnormalities of the inner ear. Usher syndrome is a rare genetic disorder characterized by partial or total hearing loss and vision loss that worsens over time and sometimes it also affects balance 1. Usher syndrome treatment What is Usher syndrome
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