{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T23:40:45Z","timestamp":1782258045441,"version":"3.54.5"},"reference-count":13,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T00:00:00Z","timestamp":1769990400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T00:00:00Z","timestamp":1769990400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>\n                      The most common genes responsible for autosomal recessive nonsyndromic hearing loss (AR\u2010NSHL) are\n                      <jats:italic>GJB2<\/jats:italic>\n                      and\n                      <jats:italic>STRC<\/jats:italic>\n                      .\n                      <jats:italic>STRC<\/jats:italic>\n                      mutations are associated with mild\u2010to\u2010moderate sensorineural (SNHL)\u00a0hearing loss and a lack of progression. However, our institutional experience suggested otherwise, prompting this review.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      A 10\u2010year retrospective chart review was performed at a tertiary children's hospital after the University of Iowa added\n                      <jats:italic>STRC<\/jats:italic>\n                      to its OtoSCOPE\n                      <jats:sup>R<\/jats:sup>\n                      panel in 2013. Subjects with positive OtoSCOPE\n                      <jats:sup>R<\/jats:sup>\n                      results underwent audiologic review. Hearing progression was defined based on pure\u2010tone average changes, and mutation subtypes were categorized.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of 354 subjects undergoing OtoSCOPE\n                      <jats:sup>R<\/jats:sup>\n                      testing, 181 (51.1%) carried a pathogenic mutation;\n                      <jats:italic>GJB2<\/jats:italic>\n                      (28.7%) and\n                      <jats:italic>STRC<\/jats:italic>\n                      (16.6%) were most common. The\n                      <jats:italic>STRC<\/jats:italic>\n                      cohort included 30 subjects (21 males, 9 females) with hearing loss severity classifiable in 26 subjects and the highest proportion in the mild\u2010to\u2010moderate range (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200946 ears; 88.5%). Hearing progression was observed in 12\/24 subjects (20 ears: 8 bilateral, 4 unilateral). Median annual progression was 1.1\u2009dB (range \u22123.5 to 18.7\u2009dB). Two\n                      <jats:italic>STRC<\/jats:italic>\n                      subjects had substantial progression requiring cochlear implantation (one performed, one recommended). Genetic subtyping revealed seven categories, including six males with\n                      <jats:italic>STRC\/CATSPER2<\/jats:italic>\n                      deletions (deafness\u2010infertility syndrome). No association between subtype and severity or progression was identified.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>\n                      <jats:italic>STRC<\/jats:italic>\n                      is the second most common cause of childhood NSHL and the leading contributor to mild\u2010to\u2010moderate SNHL. Unlike most published literature, 50% of our STRC cohort exhibited progression, and 17.6% of progressing subjects had substantial unilateral loss. We recommend long\u2010term audiometric monitoring and standardized genomic reporting for this population.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Level of Evidence<\/jats:title>\n                    <jats:p>4.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/lary.70404","type":"journal-article","created":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T10:54:47Z","timestamp":1770029687000},"page":"2784-2791","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Natural History of Sensorineural Hearing Loss in Children With\n                    <scp>\n                      <i>STRC<\/i>\n                    <\/scp>\n                    Mutations"],"prefix":"10.1002","volume":"136","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9989-1322","authenticated-orcid":false,"given":"Kenny H.","family":"Chan","sequence":"first","affiliation":[{"name":"Department of Otolaryngology University of Colorado School of Medicine  Aurora Colorado USA"},{"name":"Department of Otolaryngology Children's Hospital Colorado  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily E.","family":"Nightengale","sequence":"additional","affiliation":[{"name":"Department of Audiology Children's Hospital Colorado  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Setareh","family":"Ekhteraei","sequence":"additional","affiliation":[{"name":"University of Colorado School of Medicine  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ericka","family":"Schicke","sequence":"additional","affiliation":[{"name":"Department of Audiology Children's Hospital Colorado  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suhong","family":"Tong","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, School of Medicine University of Colorado Anschutz Medical Campus  Aurora Colorado USA"},{"name":"Department of Biostatistics and Informatics, School of Public Health University of Colorado Anschutz Medical Campus  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1542-3113","authenticated-orcid":false,"given":"Austin","family":"Zhu","sequence":"additional","affiliation":[{"name":"McGovern Medical School at the University of Texas Health Science Center at Houston  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara K.","family":"Burton","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Northwestern University Feinberg School of Medicine  Chicago Illinois USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,2,2]]},"reference":[{"key":"e_1_2_8_2_1","volume-title":"GeneReviews [Internet]","author":"Shearer A. 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