{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T12:28:59Z","timestamp":1784291339323,"version":"3.55.0"},"reference-count":15,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2015,11,24]],"date-time":"2015-11-24T00:00:00Z","timestamp":1448323200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2016,3]]},"abstract":"<jats:sec><jats:title>Objectives\/Hypothesis<\/jats:title><jats:p>To analyze the clinical manifestations and genetic features of patients with hearing loss (HL) and incomplete partition (IP) type III malformation, and to evaluate speech performance after cochlear implantation (CI) in these patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design<\/jats:title><jats:p>Individual retrospective cohort study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Of 206 probands with inner ear malformations (IEMs), we constructed a homogeneous cohort of 11 genetically documented IP type III (DFNX2). Mutations affecting <jats:italic>POU3F4<\/jats:italic> were classified as extension (n = 2), truncation (n = 3), large genomic deletion (n = 2), or missense substitution (n = 4). Postoperative outcomes were rigorously assessed with focus on <jats:italic>POU3F4<\/jats:italic> genotypes and compared with 80 age\u2010matched implantees without IEMs.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>HL in our cohort was prelingual in onset irrespective of degree. Serviceable hearing was obtained by wearing conventional hearing aids in three, and eight subjects required CI. No correlation was found between mutation types and initial auditory phenotype. As for the eight cochlear implantees, average Categories of Auditory Perception score was &lt;1 preoperatively and 3.9 at 2 years post\u2010CI. Speech performances improved over the first 3 months postoperatively in a manner comparable to implantees without IEMs. However, it then tended to slow down until 1 year postoperatively, leading to worse scores at 2 years than implantees without IEMs. Furthermore, this was more evident in those with a truncation or deletion mutation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>CI surgeons should be aware that postoperative auditory performance may be not as good in IP type III patients as in patients without IEMs. In particular, our study implies that certain types of <jats:italic>POU3F4<\/jats:italic> mutations may have poorer prognoses than other types after CI.<\/jats:p><\/jats:sec><jats:sec><jats:title>Level of Evidence<\/jats:title><jats:p>4. <jats:italic>Laryngoscope<\/jats:italic>, 126:E123\u2013E128, 2016<\/jats:p><\/jats:sec>","DOI":"10.1002\/lary.25573","type":"journal-article","created":{"date-parts":[[2015,11,24]],"date-time":"2015-11-24T14:08:07Z","timestamp":1448374087000},"source":"Crossref","is-referenced-by-count":32,"title":["Clinical observations and molecular variables of patients with hearing loss and incomplete partition type <scp>III<\/scp>"],"prefix":"10.1002","volume":"126","author":[{"given":"Byung Yoon","family":"Choi","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery Seoul National University Bundang Hospital Seongnam South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yong\u2010Hwi","family":"An","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery Eulji University School of Medicine Seoul South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jae\u2010Jin","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery Seoul National University Bundang Hospital Seongnam South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ja\u2010Won","family":"Koo","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery Seoul National University Bundang Hospital Seongnam South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun Ho","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery Seoul National University Hospital Seoul South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seung Ha","family":"Oh","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery Seoul National University Hospital Seoul South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sun O.","family":"Chang","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery Seoul National University Hospital Seoul South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chong Sun","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery Seoul National University Hospital Seoul South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joo Hyun","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery Dongguk University Ilsan Hospital Goyang South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,11,24]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"64","article-title":"X\u2010linked mixed deafness with congenital fixation of the stapedial footplate and perilymphatic gusher","volume":"7","author":"Nance WE","year":"1971","journal-title":"Birth Defects Orig Artic 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inner ear anomaly in East Asian populations and implications for genetic counseling","volume":"36","author":"Choi JW","year":"2015","journal-title":"Otol Neurotol"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1177\/0194599811429244"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/MAO.0b013e3182839864"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005537-200501001-00001"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mlg.0000240286.43289.87"}],"container-title":["The 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