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Onset for type III is typically within the second to fourth decades of life. Usher Syndrome Type III involves progressive hearing loss, RP, and varying degrees of vestibular dysfunction. Usher syndrome is an autosomal recessive disorder manifesting hearing loss, retinitis pigmentosa and vestibular dysfunction, and having three clinical. In USH type 2, USH2A is believed to represent up to 85% of all USH type 2 cases. Patients with this subtype have moderate-to-severe congenital hearing loss, RP, and normal vestibular function. Patients with Usher syndrome type 2 caused by USH2A have been found to show more visual field loss than patients with non-syndromic USH2A-related RP. 52 has mutations in the USH 2A gene 208,000. The spread of the three clinical types is as follows: 80 suffers from USH 2 320,000 people, of whom. 1 out of 20,000 people becomes both deaf and blind caused by Usher Syndrome. In USH type one, USH1B is the most common subtype representing between 33 – 50% of all USH type 1 cases, followed by USH1D. About 1000 people in the Netherlands suffer from Usher Syndrome (400,000 worldwide). USH type one and two are the most common types of Usher syndrome with USH type three representing only about 2-5% of overall USH cases. There are also cases of non-syndromic Usher syndrome, where a person has been genetically tested and has an identified USH gene, but has only one of the symptoms associated with this condition. However, these symptoms are not representative of all those with Usher syndrome as the symptoms can vary in onset and severity. The chart above represents the typical onset of hearing, sight, and balance loss experienced in each USH type. De fleste danske patienter med USH2 har undertype 2A med fejl i USH2A -genet Disponerende faktorer Beslægtede forældre (konsangvinitet) Sygdomstegn 2 Retinitis pigmentosa er fælles for alle typer af USH. Patients with type 2 disease generally are hearing.

Usher syndrome is catergorised into four major types, 1,2,3, and 4. Patients with type 1 Usher syndrome usually are deaf from birth and have speech and balance problems.