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We hypothesized that children with enlarged vestibular aqueduct (EVA) exhibit more favorable speech development compared to those with other genetic or structural causes of hearing loss, and explored the potential presence of a \u201chidden\u201d air-bone gap associated with EVA.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      We retrospectively reviewed 36 children under five years of age diagnosed with bilateral severe sensorineural hearing loss and ascertained before age two at Seoul National University Bundang Hospital. Patients were classified into EVA (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200916) and non-EVA (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200920) groups based on radiologic and genetic data. All participants underwent one year of bilateral hearing aid rehabilitation. Speech and language outcomes were assessed using the Categories of Auditory Perception (CAP), Sequenced Language Scale for Infants (SELSI), and Receptive and Expressive Vocabulary Test (REVT), and were compared pre- and post-treatment.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Both groups showed improved CAP scores after one year. However, the EVA group exhibited significantly better expressive language percentile scores (mean 41.8\u2009\u00b1\u200930.9) compared to the non-EVA group, despite progressive threshold deterioration. Receptive language also improved more in the EVA group, although not statistically significant.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Children with EVA may achieve superior short-term language outcomes with hearing aids, potentially due to a third window\u2013related hidden air-bone gap. However, given the progressive nature of EVA, long-term follow-up is required to assess articulation development and determine optimal timing for cochlear implantation.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00405-025-09649-6","type":"journal-article","created":{"date-parts":[[2025,9,18]],"date-time":"2025-09-18T19:47:36Z","timestamp":1758224856000},"page":"149-156","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Comparison of One-Year auditory rehabilitation outcomes by etiology in pediatric patients with bilateral severe hearing loss (70\u201390 dB): enlarged vestibular aqueduct vs. Other causes"],"prefix":"10.1007","volume":"283","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0390-8415","authenticated-orcid":false,"given":"Seung Jae","family":"Lee","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-2183-5924","authenticated-orcid":false,"given":"Junhyung","family":"Bae","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-7486-2054","authenticated-orcid":false,"given":"Do Hyun","family":"Chung","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2596-4140","authenticated-orcid":false,"given":"Heonjeong","family":"Oh","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5810-5161","authenticated-orcid":false,"given":"Jin Hee","family":"Han","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3056-198X","authenticated-orcid":false,"given":"Yehree","family":"Kim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5125-2118","authenticated-orcid":false,"given":"Byung Yoon","family":"Choi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,9,18]]},"reference":[{"issue":"3","key":"9649_CR1","doi-asserted-by":"publisher","first-page":"262","DOI":"10.1093\/deafed\/enac009","volume":"27","author":"AM Rudge","year":"2022","unstructured":"Rudge AM, Coto J, Oster MM, Brooks BM, Soman U, Rufsvold R et al (2022) Vocabulary outcomes for 5-year-old children who are deaf or hard of hearing: impact of age at enrollment in specialized early intervention. 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This study was reviewed and approved by the Institutional Review Board of Seoul National University Bundang Hospital (IRB No. B-2406-908-103).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Approval"}},{"value":"This study contains researches involving human participants and are confined within retrospective chart reviews.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"<!--Emphasis Type='Bold' removed-->Research involving human participants and\/or animals"}},{"value":"The informed patient consent was waived due to the retrospective design of the chart review.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"The authors declare\u00a0no conflicts of interest.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Disclosure of potential conflicts of interest"}}]}}