The age of onset and the severity of visual symptoms among patients are highly variable, but symptoms of RP tend to begin earliest in Usher syndrome type 1, usually before the age of 10 years, while it is more variable in type 2, ranging from adolescence up to early 20s. Later on, they will notice issues with their central vision (things appearing blurry/not able to read) and colour vision as the cones start to degenerate as well. Patients tend to notice difficulties seeing in dim light/at night (night blindness) initially along with “blind spots” in their peripheral vision, causing them to bump into things. It causes a gradual but permanent visual impairment in both eyes. Usher syndrome type 3 (very rare compared to types 1 and 2)- the severity of hearing loss is variable but affected children tend to be born with normal hearing but start deteriorating gradually by puberty Around half of the patients also experience some degree of difficulties with balanceĪll Usher syndrome patients are affected by RP, a type of inherited retinal dystrophy that primarily affects the normal function of rod photoreceptor cells in the retina.Usher syndrome type 2- moderate to severe hearing loss from birth and normal balance.Usher syndrome type 1- severe to profound hearing loss and difficulties with balance from birth.The condition is categorised into three subtypes depending on the severity of hearing loss and whether there are any difficulties with balancing. Usher syndrome is a rare condition that affects around 4 – 17 in 100,000 people worldwide. Usher syndrome is an inherited condition that involves both hearing loss and visual loss caused by retinitis pigmentosa (RP). A proportion of patients also experience difficulties with their balance.
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