{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T11:03:30Z","timestamp":1790247810991,"version":"4.1.0"},"reference-count":59,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,8,4]],"date-time":"2025-08-04T00:00:00Z","timestamp":1754265600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,8,4]],"date-time":"2025-08-04T00:00:00Z","timestamp":1754265600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["aao-hnsfjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Otolaryngol.--head neck surg."],"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>The study aimed to describe the auditory phenotype of patients with bilateral enlarged vestibular aqueduct, including benefits of cochlear implantation, and to look for genotype\u2010phenotype correlation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Study Design<\/jats:title>\n                    <jats:p>Retrospective single\u2010center study.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Setting<\/jats:title>\n                    <jats:p>Tertiary adult reference center.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Fifty\u2010eight patients were included. Evolution of pure tone and speech audiometry was analyzed. Cochlear size, vestibular aqueduct width, and endolymphatic duct and sac volume were assessed on both computed tomography scan and magnetic resonance imaging. Genetic explorations were documented, and genotype\u2010phenotype correlations were explored. Outcomes after cochlear implant (CI) were analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Hearing loss was diagnosed at 4\u2009\u00b1\u20094.0 years, being initially severe\u2010to\u2010profound in 74% of the ears. Sudden sensorineural hearing loss concerned 54% of patients. Both vestibular aqueduct and endolymphatic sac showed symmetric measurements (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u2009.001). Patients with\n                      <jats:italic>SLC26A4<\/jats:italic>\n                      biallelic alterations (n\u2009=\u200922) had more associated thyroid disorders (14\/22 vs 3\/31,\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u2009.001), earlier (2\u2009\u00b1\u20091.6 years old [yo] vs 5\u2009\u00b1\u20094.5 yo,\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u2009.001) and lower age\u2010adjusted pure\u2010tone average (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u2009.001, two\u2010way analysis of variance), and greater endolymphatic sac volume (369\u2009\u00b1\u2009118.1\u2009mm\n                      <jats:sup>3<\/jats:sup>\n                      vs 194\u2009\u00b1\u2009208.7\u2009mm\n                      <jats:sup>3<\/jats:sup>\n                      ,\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u2009.042), compared with patients with unconclusive genetic results. Consequently, more patients with\n                      <jats:italic>SLC26A4<\/jats:italic>\n                      biallelic alterations received a CI (19\/22 vs 17\/31,\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u2009.015) and at an earlier age (18\u2009\u00b1\u200912.5 yo vs 34\u2009\u00b1\u200918.9 yo,\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u2009.009). Finally, 12 months after cochlear implantation, speech intelligibility for words improved from 23%\u2009\u00b1\u200927.6% to 61%\u2009\u00b1\u200934.7% in silence (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u2009.0012), and from 30%\u2009\u00b1\u200931.3% to 87%\u2009\u00b1\u200910.6% in noisy conditions.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      The auditory phenotype of patients with bilateral enlarged vestibular aqueduct is variable but seems to be more severe in case of biallelic alterations of\n                      <jats:italic>SLC26A4<\/jats:italic>\n                      . However, cochlear implantation benefits to these patients, regardless of genotype.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ohn.1375","type":"journal-article","created":{"date-parts":[[2025,8,5]],"date-time":"2025-08-05T06:26:46Z","timestamp":1754375206000},"page":"1215-1227","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Bilateral Enlarged Vestibular Aqueduct: Auditory, Genetic and Radiological Characterization, and Benefits of Cochlear Implants"],"prefix":"10.1002","volume":"173","author":[{"given":"Augustin","family":"Vigouroux","sequence":"first","affiliation":[{"name":"APHP, Sorbonne Universit\u00e9, GHU Piti\u00e9\u2010Salp\u00eatri\u00e8re, Service d'ORL et de chirurgie cervico\u2010faciale, UF Implants Auditifs Paris 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