{"id":1013,"date":"2023-06-22T13:14:19","date_gmt":"2023-06-22T13:14:19","guid":{"rendered":"https:\/\/www.eikholt.no\/?post_type=knowledge&#038;p=1013"},"modified":"2026-02-01T15:18:25","modified_gmt":"2026-02-01T15:18:25","slug":"usher-syndrom-typer-och-arftlighet","status":"publish","type":"knowledge","link":"https:\/\/www.eikholt.no\/sv\/kunskapsvalvet\/usher-syndrom-typer-och-arftlighet\/","title":{"rendered":"Usher syndrom, typer och \u00e4rftlighet"},"content":{"rendered":"<h2 class=\"wp-block-heading\"><strong>Inledning\u00a0<\/strong><\/h2>\n\n\n\n<p>Ushers syndrom \u00e4r en s\u00e4llsynt \u00e4rftlig genetisk sjukdom som leder till kombinerad syn- och h\u00f6rselneds\u00e4ttning\/d\u00f6vblindhet. Ushers syndrom \u00e4r den vanligaste enskilda orsaken till d\u00f6vblindhet hos personer under 65 \u00e5r. 50% av alla d\u00f6vblinda personer under 65 \u00e5r har Ushers syndrom. Ushers syndrom \u00e4r inte kopplat till andra neurologiska tillst\u00e5nd, utan endast till syn- och h\u00f6rselneds\u00e4ttning.<\/p>\n\n\n\n<p>Baserat p\u00e5 kliniska observationer delas Usher syndrom in i tre typer, Usher 1, 2 och 3.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><strong>Usher syndrom typ 1 (35-40%)<\/strong><\/h2>\n\n\n\n<p>Ushers syndrom typ 1 k\u00e4nnetecknas av medf\u00f6dd d\u00f6vhet eller medf\u00f6dd sv\u00e5r neurogen h\u00f6rselneds\u00e4ttning. \u00d6gonsjukdomen Retinitis Pigmentosa debuterar tidigt i livet. Varierande grad av balansproblem, fr\u00e5n normal till avsaknad av balansfunktion.&nbsp;<\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><strong>Usher syndrom typ 2 (60-65%)<\/strong><\/h2>\n\n\n\n<p>Ushers syndrom typ 2 k\u00e4nnetecknas av medf\u00f6dd eller tidigt f\u00f6rv\u00e4rvad m\u00e5ttlig till sv\u00e5r stabil neurogen h\u00f6rselneds\u00e4ttning. \u00d6gonsjukdomen Retinitis Pigmentosa debuterar vanligen i ung vuxen \u00e5lder. Normal balansfunktion.&nbsp;<\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><strong>Usher syndrom typ 3 (0-5%)<\/strong><\/h2>\n\n\n\n<p>Ushers syndrom typ 3 k\u00e4nnetecknas av m\u00e5ttlig till sv\u00e5r h\u00f6rselneds\u00e4ttning med varierande debuttid. H\u00f6rselneds\u00e4ttningen kan vara snabbt progressiv. \u00d6gonsjukdomen Retinitis Pigmentosa debuterar vid olika tidpunkter, ofta i ung vuxen \u00e5lder. Varierande grad av balansproblem.&nbsp;<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Arvsanlag och Ushers syndrom<\/h2>\n\n\n\n<p>Alla identifierade Usher-gener har autosomal recessiv ned\u00e4rvning.&nbsp;<\/p>\n\n\n\n<p>F\u00f6r att f\u00e5 Ushers syndrom m\u00e5ste man ha tv\u00e5 alleler med samma genetiska defekt. Med andra ord m\u00e5ste man \u00e4rva genfelet fr\u00e5n b\u00e5de sin far och sin mor.&nbsp;<\/p>\n\n\n\n<p>Du har tv\u00e5 varianter av samma gen. Den ena \u00e4rvs fr\u00e5n fadern och den andra fr\u00e5n modern. Om du har tv\u00e5 friska gener \u00e4r du frisk. Om du har en Usher-gen \u00e4r du b\u00e4rare av syndromet, men du f\u00e5r inte sj\u00e4lv Usher. Om du har tv\u00e5 Ushergener f\u00e5r du Ushers syndrom.&nbsp;<\/p>\n\n\n\n<h3 class=\"wp-block-heading\">Exempel p\u00e5 detta:\u00a0<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Om en person med Ushers syndrom f\u00e5r barn med en person som inte har Ushers syndrom och som inte \u00e4r b\u00e4rare av syndromet, kommer inget av barnen att ha Usher. Alla barnen kommer att vara friska b\u00e4rare.<\/li>\n\n\n\n<li>Om tv\u00e5 friska anlagsb\u00e4rare f\u00e5r barn \u00e4r det 25% risk att barnet f\u00e5r Ushers syndrom, 25% risk att barnet f\u00e5r tv\u00e5 friska gener och 50% risk att barnet blir en frisk anlagsb\u00e4rare.<\/li>\n<\/ul>\n\n\n\n<details class=\"wp-block-sail-accordion\"><summary class=\"--title\">K\u00e4lla<\/summary><main class=\"--content\">\n<p>Informationen \u00e4r h\u00e4mtad fr\u00e5n artikeln Usher skriven av Castligone &amp; Muller, 2021 och kan l\u00e4sas i sin helhet h\u00e4r: <a href=\"https:\/\/www.mdpi.com\/2039-4349\/12\/1\/5\">Audiologisk forskning | Gratis fulltext | Usher syndrom (mdpi.com)<\/a><\/p>\n<\/main><\/details>\n\n\n\n<h2 class=\"wp-block-heading\"><strong>Andra anv\u00e4ndbara resurser:<\/strong><\/h2>\n\n\n\n<p><a href=\"https:\/\/www.eikholt.no\/sv\/kunskapsvalvet\/ushers-syndrom-och-sjuklighet-i-norge\/\">Ushers syndrom och orapporterade fall i Norge - Eikholt<\/a><\/p>\n\n\n\n<p><a href=\"https:\/\/www.eikholt.no\/sv\/kunskapsvalvet\/dovblindhet-definition\/\">D\u00f6vblindhet, nordisk definition reviderad<\/a><\/p>\n\n\n\n<p><a href=\"https:\/\/www.eikholt.no\/sv\/kunskapsvalvet\/dovblindhet\/\">D\u00f6vblindhet - Eikholt<\/a><\/p>\n\n\n\n<h2 class=\"wp-block-heading\">H<strong>Har du fr\u00e5gor om kombinerad syn- och h\u00f6rselneds\u00e4ttning?<\/strong><\/h2>\n\n\n\n<p><a href=\"https:\/\/www.eikholt.no\/sv\/kontakta-oss\/\">Kontakta oss - Eikholt<\/a><\/p>\n\n\n\n<p><strong>Denna artikel skrevs den 22 juni 2023<\/strong><\/p>\n\n\n\n<p><\/p>","protected":false},"excerpt":{"rendered":"<p>Ushers syndrom \u00e4r en s\u00e4llsynt \u00e4rftlig genetisk sjukdom som leder till kombinerad syn- och h\u00f6rselneds\u00e4ttning\/d\u00f6vblindhet. Ushers syndrom \u00e4r den vanligaste enskilda orsaken till d\u00f6vblindhet hos personer under 65 \u00e5r. 50% av alla d\u00f6vblinda personer under 65 \u00e5r har Ushers syndrom. Ushers syndrom \u00e4r inte kopplat till andra neurologiska tillst\u00e5nd, utan endast till syn- och h\u00f6rselneds\u00e4ttning.<\/p>","protected":false},"author":2,"parent":0,"template":"","class_list":["post-1013","knowledge","type-knowledge","status-publish","hentry","cat-knowledge-dovblindhet","cat-knowledge-syn","cat-knowledge-horsel"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v25.8 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Usher syndrom, typer og arv - Eikholt<\/title>\n<meta name=\"description\" content=\"Les om Usher syndrom type 1, 2 og 3 og arvelighet, den vanligste enkelt\u00e5rsaken til d\u00f8vblindhet hos personer under 65 \u00e5r\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.eikholt.no\/sv\/kunskapsvalvet\/usher-syndrom-typer-och-arftlighet\/\" \/>\n<meta property=\"og:locale\" content=\"sv_SE\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Usher syndrom, typer og arv - 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