{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T00:43:56Z","timestamp":1761007436743,"version":"build-2065373602"},"reference-count":15,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,3,7]],"date-time":"2025-03-07T00:00:00Z","timestamp":1741305600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["aao-hnsfjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Otolaryngol.--head neck surg."],"published-print":{"date-parts":[[2025,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>This study aims to investigate the characteristics of hearing loss associated with <jats:italic>MYO15A<\/jats:italic> mutations and to analyze the longitudinal prognosis over a 4\u2010year period using different treatment modalities, including cochlear implants (CIs), hearing aids (HAs), and conservative management.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design<\/jats:title><jats:p>A retrospective case review.<\/jats:p><\/jats:sec><jats:sec><jats:title>Setting<\/jats:title><jats:p>A tertiary referral center.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Sequencing was performed to recruit patients with potentially pathogenic <jats:italic>MYO15A<\/jats:italic> mutation\u2010induced hearing loss. Audiological data, radiological imaging, and assessment of hearing and speech performance before and after different treatments were analyzed in combination with patients' genotypes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixteen patients with <jats:italic>MYO15A<\/jats:italic> mutation\u2010induced deafness from 14 unrelated pedigrees were enrolled, carrying 5 previously unreported mutations: c.3660_3666delinsAA (p.Glu1221fs), c.4635delG (p.Val1545fs), c.6664A&gt;G (p.Met2222Val), c.8215delG (p.Ala2739fs), and c.8897delG (p.Gly2966fs). Inner ear malformations were observed in 3 patients. CI recipients exhibited significant improvements in hearing and speech abilities 1\u2010year post\u2010implantation, while individuals using HAs showed a gradual improvement trend over a 4\u2010year period. Notably, even those with bilateral cochlear aperture atresia achieved satisfactory hearing and speech outcomes following early CIs.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Patients with <jats:italic>MYO15A<\/jats:italic> mutations who underwent CIs generally demonstrated earlier improvements in hearing and speech development compared to those using HAs. Early genetic detection and timely implementation of assistive acoustic stimulation are recommended for optimal outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ohn.1200","type":"journal-article","created":{"date-parts":[[2025,3,11]],"date-time":"2025-03-11T11:41:43Z","timestamp":1741693303000},"page":"2051-2057","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Characteristics and Prognosis of Patients With Non\u2010Syndromic Sensorineural Hearing Loss Associated With <i>Myo15a<\/i> Mutations"],"prefix":"10.1002","volume":"172","author":[{"given":"Yueying","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology The First Affiliated Hospital, Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"},{"name":"Institute of Otorhinolaryngology Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shubin","family":"Fang","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology The First Affiliated Hospital, Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"},{"name":"Institute of Otorhinolaryngology Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaoqing","family":"Cen","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology The First Affiliated Hospital, Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"},{"name":"Institute of Otorhinolaryngology Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yue","family":"Liang","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology The First Affiliated Hospital, Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"},{"name":"Institute of Otorhinolaryngology Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anhai","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology The First Affiliated Hospital, Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"},{"name":"Institute of Otorhinolaryngology Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lusha","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology The First Affiliated Hospital, Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of China"},{"name":"Institute of Otorhinolaryngology Sun Yat\u2010sen University, Guangzhou Guangdong People's Republic of 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