{"id":1754,"date":"2022-08-16T19:59:57","date_gmt":"2022-08-16T19:59:57","guid":{"rendered":"https:\/\/blogs.bmj.com\/jmg\/?p=1754"},"modified":"2026-02-23T00:45:57","modified_gmt":"2026-02-23T00:45:57","slug":"tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation","status":"publish","type":"post","link":"https:\/\/blogs.bmj.com\/jmg\/2022\/08\/16\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\/","title":{"rendered":"TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation (Contributed by Dr. Rick F Nelson)"},"content":{"rendered":"<p>Bi-allelic pathogenic variants in the <em>TMPRSS3<\/em> gene result in non-syndromic deafness at the DFNB8\/10 locus. <em>TMPRSS3<\/em>-realted hearing loss is a common form of congenital deafness in humans. Although two decades have passed since <em>TMPRSS3<\/em> was casually linked to deafness, its expression in the inner ear and the best course of clinical intervention for individuals with <em>TMPRSS3<\/em>-realted deafness has been controversial. In this study, we show that <em>TMPRSS3<\/em> is primarily expressed in the cochlea sensory epithelium and not in the sound transducing neurons of the cochlea. We also show that patients with TMPRSS3-related deafness have excellent speech understanding with cochlear implants. These discoveries improve our understanding of <em>TMPRSS3<\/em>-related deafness and improve patient care. (<a href=\"https:\/\/jmg.bmj.com\/content\/early\/2022\/08\/11\/jmg-2022-108654\">https:\/\/jmg.bmj.com\/content\/early\/2022\/08\/11\/jmg-2022-108654<\/a> )<\/p>\n<p><a href=\"https:\/\/blogs.bmj.com\/jmg\/2022\/08\/16\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\/img_0447_crop\/\" rel=\"attachment wp-att-1755\"><img loading=\"lazy\" decoding=\"async\" class=\"alignnone size-medium wp-image-1755\" src=\"https:\/\/blogs.bmj.com\/jmg\/files\/2022\/08\/IMG_0447_crop-300x300.jpg\" alt=\"\" width=\"300\" height=\"300\" srcset=\"https:\/\/blogs.bmj.com\/jmg\/files\/2022\/08\/IMG_0447_crop-300x300.jpg 300w, https:\/\/blogs.bmj.com\/jmg\/files\/2022\/08\/IMG_0447_crop-150x150.jpg 150w, https:\/\/blogs.bmj.com\/jmg\/files\/2022\/08\/IMG_0447_crop-250x250.jpg 250w, https:\/\/blogs.bmj.com\/jmg\/files\/2022\/08\/IMG_0447_crop.jpg 506w\" sizes=\"auto, (max-width: 300px) 100vw, 300px\" \/><\/a><\/p>\n<p>This 3 years old girl with TMPRSS3-hearing loss has bilateral cochlear implants.<\/p>\n<p>&nbsp;<!--TrendMD v2.4.8--><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Bi-allelic pathogenic variants in the TMPRSS3 gene result in non-syndromic deafness at the DFNB8\/10 locus. TMPRSS3-realted hearing loss is a common form of congenital deafness in humans. Although two decades have passed since TMPRSS3 was casually linked to deafness, its expression in the inner ear and the best course of clinical intervention for individuals with [&#8230;]<\/p>\n<p><a class=\"btn btn-secondary understrap-read-more-link\" href=\"https:\/\/blogs.bmj.com\/jmg\/2022\/08\/16\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\/\">Read More&#8230;<\/a><\/p>\n","protected":false},"author":123,"featured_media":0,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[1],"tags":[],"class_list":["post-1754","post","type-post","status-publish","format-standard","hentry","category-uncategorized"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.5 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation (Contributed by Dr. Rick F Nelson) - JMG Contact blog<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/blogs.bmj.com\/jmg\/2022\/08\/16\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation (Contributed by Dr. Rick F Nelson) - JMG Contact blog\" \/>\n<meta property=\"og:description\" content=\"Bi-allelic pathogenic variants in the TMPRSS3 gene result in non-syndromic deafness at the DFNB8\/10 locus. TMPRSS3-realted hearing loss is a common form of congenital deafness in humans. Although two decades have passed since TMPRSS3 was casually linked to deafness, its expression in the inner ear and the best course of clinical intervention for individuals with [...]Read More...\" \/>\n<meta property=\"og:url\" content=\"https:\/\/blogs.bmj.com\/jmg\/2022\/08\/16\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\/\" \/>\n<meta property=\"og:site_name\" content=\"JMG Contact blog\" \/>\n<meta property=\"article:published_time\" content=\"2022-08-16T19:59:57+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2026-02-23T00:45:57+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/blogs.bmj.com\/jmg\/files\/2022\/08\/IMG_0447_crop.jpg\" \/>\n\t<meta property=\"og:image:width\" content=\"506\" \/>\n\t<meta property=\"og:image:height\" content=\"506\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"author\" content=\"hqqu\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:creator\" content=\"@HuiQiQu\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"hqqu\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"Article\",\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2022\\\/08\\\/16\\\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\\\/#article\",\"isPartOf\":{\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2022\\\/08\\\/16\\\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\\\/\"},\"author\":{\"name\":\"hqqu\",\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/#\\\/schema\\\/person\\\/be0250f8d5b52412c3e7c222dabd591b\"},\"headline\":\"TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation (Contributed by Dr. Rick F Nelson)\",\"datePublished\":\"2022-08-16T19:59:57+00:00\",\"dateModified\":\"2026-02-23T00:45:57+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2022\\\/08\\\/16\\\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\\\/\"},\"wordCount\":156,\"commentCount\":0,\"publisher\":{\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/#organization\"},\"image\":{\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2022\\\/08\\\/16\\\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\\\/#primaryimage\"},\"thumbnailUrl\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/files\\\/2022\\\/08\\\/IMG_0447_crop-300x300.jpg\",\"inLanguage\":\"en-US\",\"potentialAction\":[{\"@type\":\"CommentAction\",\"name\":\"Comment\",\"target\":[\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2022\\\/08\\\/16\\\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\\\/#respond\"]}]},{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2022\\\/08\\\/16\\\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\\\/\",\"url\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2022\\\/08\\\/16\\\/tmprss3-expression-is-limited-in-spiral-ganglion-neurons-implication-for-successful-cochlear-implantation\\\/\",\"name\":\"TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation (Contributed by Dr. Rick F Nelson) - 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