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We led a retrospective monocentric study in the national referral center for ECD. One hundred and sixty\u2010two patients with ECD and ENT data were included between January 1, 1980 and December 31, 2020. Ear and nose clinical and radiological findings were noted. We described and studied the prevalence of ENT involvement in ECD population. The association between sinonasal and ear involvement, other organ involvement, and BRAF mutations was calculated. The prevalence of ENT manifestations is around 45%. No clinical rhinologic or otologic signs were specific to ECD. Sinus imaging was abnormal in 70% of cases. A bilateral maxillary sinus frame osteosclerosis was highly specific of ECD. Associations were found between the sinus MRI imaging type and BRAF status, central nervous system involvement, cerebellum involvement and xanthelasma. Sinonasal or ear involvement is frequent in ECD and has specific imaging features for sinuses.<\/jats:p><jats:p>Trial registration: #2011\u2010A00447\u201034.<\/jats:p>","DOI":"10.1111\/bjh.18963","type":"journal-article","created":{"date-parts":[[2023,7,3]],"date-time":"2023-07-03T05:29:25Z","timestamp":1688362165000},"page":"194-201","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Sinonasal and ear manifestations of <scp>Erdheim\u2010Chester<\/scp> disease"],"prefix":"10.1111","volume":"203","author":[{"given":"Myl\u00e8ne","family":"Drouillard","sequence":"first","affiliation":[{"name":"ENT Department, Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, APHP Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phanie","family":"Trunet","sequence":"additional","affiliation":[{"name":"Neuroradiology Department, Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, APHP Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1569-3578","authenticated-orcid":false,"given":"R\u00e9mi","family":"Hervochon","sequence":"additional","affiliation":[{"name":"ENT Department, Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, APHP Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Levi\u2010Dan","family":"Azoulay","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine 2, National Reference Center for Histiocytosis, Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, APHP Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zahir","family":"Amoura","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine 2, National Reference Center for Histiocytosis, Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, APHP Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fleur","family":"Cohen\u2010Aubart","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine 2, National Reference Center for Histiocytosis, Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, APHP Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6073-4466","authenticated-orcid":false,"given":"Jean\u2010Fran\u00e7ois","family":"Emile","sequence":"additional","affiliation":[{"name":"Anatomopathology Laboratory, Ambroise Par\u00e9 Hospital, APHP Paris\u2010Saclay University  Boulogne\u2010Billancourt France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fr\u00e9d\u00e9ric","family":"Tank\u00e9r\u00e9","sequence":"additional","affiliation":[{"name":"ENT Department, Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, APHP Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien","family":"Haroche","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine 2, National Reference Center for Histiocytosis, Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, APHP Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,7,2]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01942684"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102016\u201001\u2010690636"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis\u20102013\u2010204924"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11926\u2010014\u20100412\u20100"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102014\u201003\u2010561381"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000145368.17934.91"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-199605000-00005"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.revmed.2014.04.007"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.1991.01080060114037"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1179\/1754762814Y.0000000106"},{"issue":"2","key":"e_1_2_12_12_1","first-page":"138","article-title":"Central diabetes insipidus in adult patients\u2014the first sign of Langerhans cell histiocytosis and Erdheim\u2010Chester disease. 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