{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T09:55:59Z","timestamp":1792058159534,"version":"4.4.0"},"reference-count":14,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2005,3,1]],"date-time":"2005-03-01T00:00:00Z","timestamp":1109635200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Ann Otol Rhinol Laryngol"],"published-print":{"date-parts":[[2005,3]]},"abstract":"<jats:p>Proteus syndrome (PS) is a rare hamartomatous disorder characterized by mosaic overgrowth of multiple tissues that manifests early in life and is progressive. The presence of unilateral external auditory canal exostoses in a patient who is not a swimmer or surfer is suggestive of PS. However, hearing loss is not a typical feature. Here, we describe exostoses and ossicular discontinuity with conductive hearing loss in a patient with PS. The treatment consisted of canalplasty and ossicular chain reconstruction. A postoperative reduction was demonstrated in the patient's air-bone gap, from 21 dB to 13 dB for the pure tone average (four frequencies) and from 41 dB to 15 dB in the high-frequency range (6,000 to 8,000 Hz). Causes of ossicular discontinuity are discussed. Routine annual audiometric and otolaryngological evaluation should be considered in all patients with temporal bone inyolvement of PS.<\/jats:p>","DOI":"10.1177\/000348940511400314","type":"journal-article","created":{"date-parts":[[2014,10,3]],"date-time":"2014-10-03T14:40:52Z","timestamp":1412347252000},"page":"242-246","source":"Crossref","is-referenced-by-count":3,"title":["Ossicular Discontinuity and Exostoses in Proteus Syndrome: A Case Report"],"prefix":"10.1177","volume":"114","author":[{"given":"Joni K.","family":"Doherty","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dennis R.","family":"Maceri","sequence":"additional","affiliation":[{"name":"Los Angeles, California"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2005,3,1]]},"reference":[{"key":"bibr1-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1001\/jama.285.17.2240"},{"key":"bibr2-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1971.00770060901010"},{"key":"bibr3-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-198104000-00013"},{"key":"bibr4-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(87)80249-9"},{"key":"bibr5-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.39.12.937"},{"key":"bibr6-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1086\/340026"},{"key":"bibr7-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1016\/S1525-1578(10)60690-3"},{"key":"bibr8-000348940511400314","first-page":"291","volume":"15","author":"Cohen MM","year":"1979","journal-title":"Birth Defects Orig Artic Ser"},{"key":"bibr9-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1053\/jpsu.2002.33832"},{"key":"bibr10-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1311"},{"key":"bibr11-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1597\/1545-1569_2002_039_0233_cadmop_2.0.co_2"},{"key":"bibr12-000348940511400314","doi-asserted-by":"publisher","DOI":"10.1016\/S1010-5182(89)80088-5"},{"key":"bibr13-000348940511400314","doi-asserted-by":"publisher","DOI":"10.3109\/15513819309048266"},{"key":"bibr14-000348940511400314","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1097\/00003086-198109000-00008","volume":"159","author":"Bergstrom L","year":"1981","journal-title":"Clin Orthop"}],"container-title":["Annals of Otology, Rhinology &amp; Laryngology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/000348940511400314","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/000348940511400314","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T11:18:30Z","timestamp":1777375110000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/000348940511400314"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,3]]},"references-count":14,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2005,3]]}},"alternative-id":["10.1177\/000348940511400314"],"URL":"https:\/\/doi.org\/10.1177\/000348940511400314","relation":{},"ISSN":["0003-4894","1943-572X"],"issn-type":[{"value":"0003-4894","type":"print"},{"value":"1943-572X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,3]]}}}