{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,20]],"date-time":"2026-06-20T07:25:38Z","timestamp":1781940338272,"version":"3.54.5"},"reference-count":23,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2022,8,31]],"date-time":"2022-08-31T00:00:00Z","timestamp":1661904000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Primary ciliary dyskinesia (PCD) is a rare genetical disease characterized by an abnormal structure or function of the cilia, causing sinusitis, otitis, and bronchiectasis. Hearing loss affects 60% of PCD patients, but data are lacking concerning hearing and temporal bone imaging in adults. Our aim was to describe clinical and radiological ear disease in adults with genetically confirmed PCD. Data were recorded from January 2018 to December 2019. PCD patients were compared with controls with bronchiectasis without PCD. Clinical examination included otomicroscopy and auditory tests. A temporal bone CT scan (TBCT) was systematically performed. Seventeen patients (34 ears) were included in each group. The eardrums were abnormal in 25 (74%) PCD ears versus 8 (24%) ears in the controls (p &lt; 0.05). Conductive hearing loss was more frequent in the PCD group (24% vs. 12% in controls). TBCT were abnormal in 94% PCD patients vs. 32% in the controls (p &lt; 0.05). The Main CT-scan images in PCD were middle ear inflammation (65%), mastoid condensation (62%), or ossicular anomalies (35%). With its excellent sensitivity, TBCT gives typical arguments for PCD diagnosis, adding otological signs to the usual sinus CT signs (hypoplasia, aplasia). Systematic TBCT could be useful in the initial evaluation of patients with suspicion of PCD.<\/jats:p>","DOI":"10.3390\/jcm11175163","type":"journal-article","created":{"date-parts":[[2022,9,1]],"date-time":"2022-09-01T01:50:51Z","timestamp":1661997051000},"page":"5163","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Otological Manifestations in Adults with Primary Ciliary Dyskinesia: A Controlled Radio-Clinical Study"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5134-7344","authenticated-orcid":false,"given":"Mihaela","family":"Alexandru","sequence":"first","affiliation":[{"name":"ENT Department, Bic\u00eatre Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris-Saclay University, 94270 Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"de Boissieu","sequence":"additional","affiliation":[{"name":"Epidemiology and Public Health Department, Bic\u00eatre Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris-Saclay University, 94270 Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Farida","family":"Benoudiba","sequence":"additional","affiliation":[{"name":"Diagnostic Neuroradiology Department, Bic\u00eatre Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris-Saclay University, 94270 Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malik","family":"Moustarhfir","sequence":"additional","affiliation":[{"name":"Diagnostic Neuroradiology Department, Bic\u00eatre Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris-Saclay University, 94270 Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sookyung","family":"Kim","sequence":"additional","affiliation":[{"name":"ENT Department, Bic\u00eatre Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris-Saclay University, 94270 Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5193-5776","authenticated-orcid":false,"given":"\u00c9milie","family":"Bequignon","sequence":"additional","affiliation":[{"name":"ENT Department, Henri Mondor Hospital, Intercommuncal Hospital of Cr\u00e9teil, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris Est University, 94010 Cr\u00e9teil, France"},{"name":"Mondor Institute of Biomedical Research INSERM-UPEC UMR 955, CNRS ERL7000, 94010 Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Honor\u00e9","sequence":"additional","affiliation":[{"name":"Pneumology Department, Cochin Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris Cit\u00e9 University, 75014 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gilles","family":"Garcia","sequence":"additional","affiliation":[{"name":"Pneumology Department, Bic\u00eatre Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris-Saclay University, 94270 Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rana","family":"Mitri-Frangieh","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cytology Pathology, Intercommuncal Hospital of Cr\u00e9teil, 94010 Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Legendre","sequence":"additional","affiliation":[{"name":"Molecular Genetics Functional Unit, Armand-Trousseau Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Sorbonne University, 75012 Paris, France"},{"name":"INSERM, UMR-S933 Genetic Diseases of Pediatric Expression, Sorbonne University, 75005 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Crestani","sequence":"additional","affiliation":[{"name":"Rare Pulmonary Diseases (FHU APOLLO) Pneumology A Department, Bichat Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris Cit\u00e9 University, 75877 Paris, France"},{"name":"Laboratory of Excellence Inflamex, 75013 Paris, France"},{"name":"INSERM U1152, Paris Cit\u00e9 University, 75018 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camille","family":"Taill\u00e9","sequence":"additional","affiliation":[{"name":"Rare Pulmonary Diseases (FHU APOLLO) Pneumology A Department, Bichat Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris Cit\u00e9 University, 75877 Paris, France"},{"name":"INSERM U1152, Paris Cit\u00e9 University, 75018 Paris, France"},{"name":"INSERM 12, F-CRIN, Clinical Research Initiative in Severe Asthma: A Lever for Innovation & Science (CRISALIS), 31059 Toulouse, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estelle","family":"Escudier","sequence":"additional","affiliation":[{"name":"Molecular Genetics Functional Unit, Armand-Trousseau Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Sorbonne University, 75012 Paris, France"},{"name":"INSERM, UMR-S933 Genetic Diseases of Pediatric Expression, Sorbonne University, 75005 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernard","family":"Maitre","sequence":"additional","affiliation":[{"name":"Mondor Institute of Biomedical Research INSERM-UPEC UMR 955, CNRS ERL7000, 94010 Cr\u00e9teil, France"},{"name":"Pneumology Department, Henri Mondor Hospital, Intercommuncal Hospital of Cr\u00e9teil, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris Est University, 94010 Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Fran\u00e7ois","family":"Papon","sequence":"additional","affiliation":[{"name":"ENT Department, Bic\u00eatre Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris-Saclay University, 94270 Le Kremlin-Bic\u00eatre, France"},{"name":"Mondor Institute of Biomedical Research INSERM-UPEC UMR 955, CNRS ERL7000, 94010 Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00e9r\u00f4me","family":"Nevoux","sequence":"additional","affiliation":[{"name":"ENT Department, Bic\u00eatre Hospital, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris-Saclay University, 94270 Le Kremlin-Bic\u00eatre, France"},{"name":"INSERM, U1120, Pasteur Institute, 75724 Paris, 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