A person with Usher syndrome type 3 is born with normal hearing and close to normal balance, however hearing gradually deteriorates with age. Usher syndrome (USH) is an autosomal recessive (AR) disorder characterized by sensorineural hearing loss (SNHL), vision loss due to retinitis pigmentosa (RP).Usher syndrome type 3 is very rare compared to the other Usher syndrome types and only one subtype has been discovered so far, with the majority of people with USH3 living in Finland.Vision loss due to the RP does not usually develop until late adolescence or in some cases until the late twenties.Those with Usher syndrome type 2 do not have a dysfunctional vestibular system. In Usher syndrome type 2, the severity of hearing loss from birth can range from very mild to severe.Usher syndrome type 2 can be grouped into 3 subtypes, 2A, 2C and 2D.The severity and onset of RP varies between individuals but commonly develops before the age of 10. Vision problems are initially characterised by night blindness or tunnel vision but this also varies between individuals. Gradual vision loss in the child occurs due to Retinitis Pigmentosa (RP).Healthcare providers usually diagnose it during childhood. Usher syndrome is a disease you are born with. Some people also experience balance problems. Although considered a rare disease, it is the most. Due to the vestibular dysfunction, a child with Usher syndrome type 1 will usually take longer to sit up and develop walking at a later stage than a typically developing child. Overview What is Usher syndrome Usher syndrome is a disease that leads to hearing loss and vision loss. Usher syndrome is a genetic condition involving sensorineural hearing loss and retinitis pigmentosa (RP).Children diagnosed with Usher syndrome type 1 are profoundly deaf at birth and have a dysfunctional vestibular system.Type 1 is comprised of 5 different subtypes 1B, 1C, 1D, 1F and 1G depending on the specific gene mutation.Each of these types has a number of different genes responsible for causing the syndrome. There are three main types of Usher syndrome - Type 1, Type 2 and Type 3.
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