![]() Hearing loss is due to a defect in the inner ear hair cells. It has been shown that Type III occurs more frequently in the Finnish population. Type III accounts for only about 2% of all cases. It has been found that Type I Usher Syndrome is more common among people of Ashkenazi Jewish or French Acadient descent. In the United States it occurs in approximately 1 in 23,000 people. It is significantly more prevalent the genetically deaf accounting for approximately 5-10% of those with the handicap. Usher syndrome is a rare disease in the general population with a prevalence of 3 cases per 100,000 in the population. In general, genes associated with Usher syndrome provide instructions for the synthesis of proteins involved in normal hearing, balance, and vision. Usher Syndrome II Loci: USH2A, USH2C, USH2D Usher Syndrome I Loci: USH1B, USH1C, USH1D, USH1E, USH1F, USH1G, USH1H, USH1J, USH1K Usher Syndrome III Genes: CLRN1 and PDZD7 Usher Syndrome II Genes: USH2A, GPR8, and DFNB31. Usher Syndrome I Genes: CDH23, MYO7A, PCDH15, USH1C, CIB2 and USH1G 13 genes and 16 loci have been found to contribute to Usher Syndrome. The mode of inheritance for Usher Syndrome is autosomal recessive. These patients also tend to have better vision than the other subtypes. 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. Patients with this subtype have moderate-to-severe congenital hearing loss, RP, and normal vestibular function. It is characterized by profound congenital hearing loss, RP, and absent vestibular function. Usher Syndrome Type I is the most severe subtype. While all three types involve progressive vision loss due to retinitis pigmentosa (RP), they are categorized according to the genes responsible and the onset and severity of the signs and symptoms. Usher syndrome has been classified into three major subtypes: I, II, and III. Usher syndrome, also known as Hallgren syndrome, is a rare genetic condition that is characterized by progressive vision and hearing loss. 2018 ICD-10 Code: H35.53: Other dystrophies primarily involving the sensory retina.
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