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Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient\u2019s hearing was rehabilitated with bilateral cochlear implantation.<\/jats:p>","DOI":"10.1177\/0145561321996839","type":"journal-article","created":{"date-parts":[[2021,3,8]],"date-time":"2021-03-08T13:24:52Z","timestamp":1615209892000},"page":"343S-346S","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":3,"title":["Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes"],"prefix":"10.1177","volume":"100","author":[{"given":"Wenqi","family":"Liang","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, People\u2019s Republic of China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Line","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, People\u2019s Republic of China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xinyu","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, People\u2019s Republic of China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fenqi","family":"Gao","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, People\u2019s Republic of China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pan","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2013Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, People\u2019s Republic of China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tiffany H.","family":"Lee","sequence":"additional","affiliation":[{"name":"LAC+USC Medical Center\t\t, Los Angeles, CA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9166-0869","authenticated-orcid":false,"given":"Kevin A.","family":"Peng","sequence":"additional","affiliation":[{"name":"House Clinic and House Ear Institute, Los Angeles, CA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2021,3,8]]},"reference":[{"key":"bibr1-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2012.11.001"},{"key":"bibr2-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.215.1.r00ap36243"},{"key":"bibr3-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1097\/01.aud.0000224100.30525.ab"},{"key":"bibr4-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2018.01.015"},{"key":"bibr5-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1155\/2018\/5675848"},{"key":"bibr6-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1177\/0194599815592365"},{"key":"bibr7-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1016\/j.gene.2016.07.011"},{"key":"bibr8-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.131.6.481"},{"key":"bibr9-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gks539"},{"key":"bibr10-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1097\/00005537-200212000-00019"},{"key":"bibr11-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215100141349"},{"key":"bibr12-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1177\/0194599817718798"},{"key":"bibr13-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1016\/j.otc.2015.07.007"},{"key":"bibr14-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2016.03.020"},{"key":"bibr15-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1007\/PL00000673"},{"key":"bibr16-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1086\/383285"},{"key":"bibr17-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1038\/79063"},{"key":"bibr18-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.20065"},{"key":"bibr19-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1155\/2016\/6720420"},{"key":"bibr20-0145561321996839","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-1809.2008.00499.x"}],"container-title":["Ear, Nose &amp; 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