{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T01:29:00Z","timestamp":1777426140447,"version":"3.51.4"},"reference-count":32,"publisher":"SAGE Publications","issue":"5","license":[{"start":{"date-parts":[[2024,2,15]],"date-time":"2024-02-15T00:00:00Z","timestamp":1707955200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Ann Otol Rhinol Laryngol"],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:sec>\n                    <jats:title>Objectives:<\/jats:title>\n                    <jats:p>There is a limited understanding of the impact of cochlear implantation (CI) in patients with Charcot-Marie-Tooth disease (CMT), given the scarcity of reported cases. We aim to evaluate the audiological outcomes and quality of life (QoL) after CI in CMT.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>Multi-institutional, university-affiliated, tertiary-referral centers, retrospective chart review. Our cohort includes 5 patients with CMT. Patients\u2019 charts were reviewed for demographic characteristics, operation notes, and pre- and post-implantation audiology evaluation. Patients completed the Cochlear Implant Quality of Life-10 (CIQOL-10) Global questionnaire.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>Pre-implantation, the mean pure tone average was 84.1\u2009\u00b1\u20097.2\u2009dB, and the mean word recognition score was 2.4% in the implanted ear. AzBio sentence test was performed in quiet, revealing a mean of 4\u2009\u00b1\u20091.4% in the implanted ear. Post-implantation, PTA results were all within the mild hearing loss range (mean 33.0\u2009\u00b1\u20095.9\u2009dB). Post-CI, AZ-Bio test results were 5%, 65%, and 74% (for 3 patients), and HINT scores were 55% and 58% (for 2 patients). The mean score of the CIQOL-10 questionnaire was 42.7\u2009\u00b1\u200910.47 (range 1-100). Patients were most satisfied with their ability to listen to the television or radio, have conversations in a quiet environment, and feel comfortable being themselves.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>To the best of our knowledge, this is the most extensive series of CI in CMT-associated sensorineural hearing loss and auditory neuropathy. Our cohort suggests that CI is a safe and reliable method for hearing rehabilitation that can achieve good speech performance and improve QoL in CMT patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/00034894241232206","type":"journal-article","created":{"date-parts":[[2024,2,16]],"date-time":"2024-02-16T00:15:35Z","timestamp":1708042535000},"page":"469-475","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Cochlear Implantation in Charcot-Marie-Tooth Patients: Speech Perception and Quality of Life"],"prefix":"10.1177","volume":"133","author":[{"given":"Nicole I.","family":"Farber","sequence":"first","affiliation":[{"name":"Department of Otolaryngology\u2014Head and Neck Surgery, University of California-Davis, Sacramento, CA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oliver Y.","family":"Chin","sequence":"additional","affiliation":[{"name":"Department of 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