{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,30]],"date-time":"2025-12-30T07:10:37Z","timestamp":1767078637598,"version":"3.48.0"},"reference-count":43,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,12,9]],"date-time":"2025-12-09T00:00:00Z","timestamp":1765238400000},"content-version":"vor","delay-in-days":69,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["aao-hnsfjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["OTO Open"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>While the etiology of Meniere's disease (MD) is likely multifactorial, genetics are thought to play a role. Several previous studies have yielded inconclusive results, potentially due to phenotypic uncertainty and variable diagnostic criteria. To explore potential genetic bases in a more rigorous context, we assessed the clinical predictors and diagnostic yield of current hearing loss panels in a highly curated cohort of patients with bilateral and\/or early\u2010onset MD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Study Design<\/jats:title>\n                    <jats:p>Retrospective cohort study.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Setting<\/jats:title>\n                    <jats:p>Multidisciplinary tertiary care hearing loss genetics clinic.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Data from clinical notes, audiograms, and genetic reports of adult patients diagnosed with bilateral and\/or early\u2010onset (&lt;40 years) MD from October 2019 to June 2025 were analyzed with logistic regression and summary statistics to determine predictive factors and diagnostic yields of existing genetic panels.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of the 37 patients analyzed (mean age 47.7\u2009+\u200914.5 years, 54% male), 24 (64.8%) had early\u2010onset MD, 22 (59.5%) had bilateral MD, and 9 (24.3%) had both. Moderately severe to profound hearing loss prior to 65 was significantly associated with pathogenic or likely pathogenic variants (PLPV) (OR 8.98 [1.17, 101];\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u2009.046). No significant predictors were found for definitive diagnosis, plausible diagnosis, or negative panels. Eight (22%) patients had a PLPV detected on their hearing loss panel, with 0 definitive diagnoses, 3 (8.1%) plausible diagnoses (\n                      <jats:italic>MYO15A<\/jats:italic>\n                      ,\n                      <jats:italic>SLC17A8<\/jats:italic>\n                      ,\n                      <jats:italic>P2RX2<\/jats:italic>\n                      ), and 6 (16%) completely negative panels.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Current hearing loss panels show limited diagnostic utility for MD. Future research should prioritize whole genome sequencing to identify novel MD\u2010associated loci and provide guidance to patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/oto2.70180","type":"journal-article","created":{"date-parts":[[2025,12,9]],"date-time":"2025-12-09T10:46:35Z","timestamp":1765277195000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Limited Utility of Existing Hearing Loss Panels in the Assessment of Early\u2010Onset, Bilateral Meniere's Disease"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3315-0665","authenticated-orcid":false,"given":"Keshav V.","family":"Shah","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery University of Pennsylvania Philadelphia Pennsylvania USA"},{"name":"Department of Genetics University of Pennsylvania Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian","family":"Jung","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery University of Pennsylvania Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel","family":"Talian","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery University of Pennsylvania Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sherrie","family":"Davis","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery University of Pennsylvania Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Douglas J.","family":"Epstein","sequence":"additional","affiliation":[{"name":"Department of Genetics University of Pennsylvania Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael J.","family":"Ruckenstein","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery University of Pennsylvania Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tiffany P.","family":"Hwa","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery University of Pennsylvania Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,12,9]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0039-3402065"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.g6544"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrdp.2016.28"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0030-6665(02)00019-1"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/00129492-200501000-00013"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaoto.2021.4282"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.heares.2019.107872"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11033-022-08149-8"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.12703\/r\/12-11"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.3233\/VES-150549"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-022-11532-x"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2024.1496384"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.3233\/VES-201634"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10162-023-00896-0"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.otc.2010.05.010"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.gim.0000227935.26763.c6"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.3390\/medicina56090471"},{"key":"e_1_2_8_19_1","first-page":"91","volume-title":"Body Recomposition","author":"Saranya K","year":"2025"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.3390\/genes11121414"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12864-024-10552-3"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/AUD.0000000000000878"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.3389\/fgene.2019.00076"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddu524"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-031-78100-1_6"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/AUD.0000000000001574"},{"key":"e_1_2_8_27_1","unstructured":"ShearerAE HildebrandMS OdellAM SmithRJH.Genetic hearing loss overview. 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