{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T04:52:59Z","timestamp":1784523179575,"version":"3.55.0"},"reference-count":24,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2023,2,10]],"date-time":"2023-02-10T00:00:00Z","timestamp":1675987200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:sec><jats:title>Objectives<\/jats:title><jats:p>Enlarged vestibular aqueduct (EVA) is the most common anatomic abnormality contributing to permanent hearing loss (HL) in children. Although the association between EVA and HL is well\u2010documented, the pass rate for the newborn hearing screening (NBHS) for patients with EVA\u2010related HL is not. Our objective was to investigate the association between NBHS results and audiologic and clinical outcomes in a large cohort of pediatric patients with EVA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This was a retrospective chart review of patients seen in the Boston Children's Hospital (BCH) Department of Otolaryngology and Communication Enhancement with confirmed HL, known NBHS results, and confirmed EVA. Demographic, clinical, audiologic, and imaging data were collected from the medical record. Frequency\u2010specific data points from pure\u2010tone audiograms and\/or automated auditory brainstem response tests were recorded, and four\u2010frequency pure tone average was calculated using air conduction thresholds at 500, 1000, 2000, and 4000\u2009Hz.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of the 183 patients included in the study, 84 (45.9%) passed their NBHS, whereas 99 (54.1%) did not pass. Compared with patients who did not pass, patients who passed were more likely to have unilateral EVA and unilateral HL, whereas they were less likely to undergo cochlear implantation and to have causative <jats:italic>SLC26A4<\/jats:italic> variants.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>EVA\u2010associated HL may be identified at birth or during childhood, with nearly half the patients in this cohort passing their NBHS. Our results provide prognostic information for patients with EVA who pass their NBHS and highlight the importance of regular hearing monitoring for children not initially suspected of having HL.<\/jats:p><\/jats:sec><jats:sec><jats:title>Level of Evidence<\/jats:title><jats:p>4 <jats:italic>Laryngoscope<\/jats:italic>, 133:2786\u20132791, 2023<\/jats:p><\/jats:sec>","DOI":"10.1002\/lary.30605","type":"journal-article","created":{"date-parts":[[2023,2,10]],"date-time":"2023-02-10T08:50:36Z","timestamp":1676019036000},"page":"2786-2791","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Newborn Hearing Screening Results in Patients with Enlarged Vestibular Aqueduct"],"prefix":"10.1002","volume":"133","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4960-9752","authenticated-orcid":false,"given":"Julia","family":"Perry","sequence":"first","affiliation":[{"name":"Department of Otolaryngology and Communication Enhancement Boston Children's Hospital  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2950-8433","authenticated-orcid":false,"given":"Erica","family":"Sher","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology and Communication Enhancement Boston Children's Hospital  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0908-5296","authenticated-orcid":false,"given":"Kosuke","family":"Kawai","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology and Communication Enhancement Boston Children's Hospital  Boston Massachusetts USA"},{"name":"Department of Otolaryngology\u2010Head and Neck Surgery Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shelby","family":"Redfield","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology and Communication Enhancement Boston Children's Hospital  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tieqi","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology and Communication Enhancement Boston Children's Hospital  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0851-9272","authenticated-orcid":false,"given":"Margaret","family":"Kenna","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology and Communication Enhancement Boston Children's Hospital  Boston Massachusetts USA"},{"name":"Department of Otolaryngology\u2010Head and Neck Surgery Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,2,10]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra050700"},{"key":"e_1_2_7_3_1","unstructured":"Centers for Disease Control and Prevention.2019Summary of Hearing Screening Among Total Occurrent Births. Accessed May 23 2022.https:\/\/www.cdc.gov\/ncbddd\/hearingloss\/2019-data\/02-screen.html"},{"key":"e_1_2_7_4_1","unstructured":"National Conference of State Legislatures. Newborn Hearing Screening State Laws.2022. Accessed May 23 2022.https:\/\/www.ncsl.org\/research\/health\/newborn-hearing-screening-state-laws.aspx"},{"key":"e_1_2_7_5_1","unstructured":"American Academy of Audiology.American Academy of Audiology Childhood Hearing Screening Guidelines.2011. Accessed May 23 2022.https:\/\/www.cdc.gov\/ncbddd\/hearingloss\/documents\/AAA_Childhood-Hearing-Guidelines_2011.pdf."},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.159.10.949"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.1978.88.5.723"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1297-411"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.124.5.501"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.22083"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/MLG.0b013e31818208ad"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.130.10.1169"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.21278"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-020-06333-9"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1989.01860250056026"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2020.109862"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2013.02.023"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/00129492-200307000-00016"},{"key":"e_1_2_7_19_1","unstructured":"Commonwealth of Massachusetts.Universal Newborn Hearing Screening Program.2022. Accessed May 23 2022.https:\/\/www.mass.gov\/universal-newborn-hearing-screening-program."},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1001\/archoto.2009.202"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2004.024208"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.23889"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2013.08.038"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41436-019-0563-5"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A7339"}],"container-title":["The Laryngoscope"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lary.30605","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/lary.30605","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lary.30605","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,19]],"date-time":"2023-09-19T18:59:47Z","timestamp":1695149987000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/lary.30605"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,10]]},"references-count":24,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2023,10]]}},"alternative-id":["10.1002\/lary.30605"],"URL":"https:\/\/doi.org\/10.1002\/lary.30605","archive":["Portico"],"relation":{},"ISSN":["0023-852X","1531-4995"],"issn-type":[{"value":"0023-852X","type":"print"},{"value":"1531-4995","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,2,10]]},"assertion":[{"value":"2022-07-03","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-01-02","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-02-10","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}