[en] [en] IMPORTANCE: Otologic disease is common among people with primary ciliary dyskinesia (PCD), yet little is known about its spectrum and severity.
OBJECTIVE: To characterize otologic disease among participants with PCD using data from the Ear-Nose-Throat Prospective International Cohort.
DESIGN, SETTING, AND PARTICIPANTS: This cross-sectional analysis of baseline cohort data from February 2020 through July 2022 included participants from 12 specialized centers in 10 countries. Children and adults with PCD diagnoses; routine ear, nose, and throat examinations; and completed symptom questionnaires at the same visit or within 2 weeks were prospectively included.
EXPOSURES: Potential risk factors associated with increased risk of ear disease.
MAIN OUTCOMES AND MEASURES: The prevalence and characteristics of patient-reported otologic symptoms and findings from otologic examinations, including potential factors associated with increased risk of ear inflammation and hearing impairment.
RESULTS: A total of 397 individuals were eligible to participate in this study (median [range] age, 15.2 [0.2-72.4] years; 186 (47%) female). Of the included participants, 204 (51%) reported ear pain, 110 (28%) reported ear discharge, and 183 (46%) reported hearing problems. Adults reported ear pain and hearing problems more frequently when compared with children. Otitis media with effusion-usually bilateral-was the most common otoscopic finding among 121 of 384 (32%) participants. Retracted tympanic membrane and tympanic sclerosis were more commonly seen among adults. Tympanometry was performed for 216 participants and showed pathologic type B results for 114 (53%). Audiometry was performed for 273 participants and showed hearing impairment in at least 1 ear, most commonly mild. Season of visit was the strongest risk factor for problems associated with ear inflammation (autumn vs spring: odds ratio, 2.40; 95% CI, 1.51-3.81) and age 30 years and older for hearing impairment (41-50 years vs ≤10 years: odds ratio, 3.33; 95% CI, 1.12-9.91).
CONCLUSION AND RELEVANCE: In this cross-sectional study, many people with PCD experienced ear problems, yet frequency varied, highlighting disease expression differences and possible clinical phenotypes. Understanding differences in otologic disease expression and progression during lifetime may inform clinical decisions about follow-up and medical care. Multidisciplinary PCD management should be recommended, including regular otologic assessments for all ages, even without specific complaints.
Disciplines :
Pediatrics
Author, co-author :
Goutaki, Myrofora; Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland ; Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Bern, Switzerland
Lam, Yin Ting; Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland
Alexandru, Mihaela; Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Saclay, Hôpital Bicêtre, Service d'ORL, Le Kremlin-Bicêtre, France ; Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
Anagiotos, Andreas; Department of Otorhinolaryngology, Nicosia General Hospital, Nicosia, Cyprus
Armengot, Miguel; Department of Otorhinolaryngology and Primary Ciliary Dyskinesia Unit, La Fe University and Polytechnic Hospital, Valencia, Spain ; University of Valencia Medical School, Valencia, Spain
Boon, Mieke; Department of Paediatrics, University Hospital, Leuven, Belgium
Burgess, Andrea; Primary Ciliary Dyskinesia Centre, Southampton Children's Hospital, Southampton NHS Foundation Trust, Southampton, England, United Kingdom
Caversaccio, Nathalie; Department of Otorhinolaryngology-Head and Neck Surgery, University Hospital of Bern, University of Bern, Bern, Switzerland
Crowley, Suzanne; Paediatric Department of Allergy and Lung Diseases, Oslo University Hospital, Oslo, Norway
Dheyauldeen, Sinan Ahmed D; Department of Otorhinolaryngology-Head and Neck Surgery, Oslo University Hospital, Oslo, Norway ; Faculty of Medicine, University of Oslo, Oslo, Norway
Emiralioglu, Nagehan; Department of Pediatric Pulmonology, Hacettepe University School of Medicine, Ankara, Turkey
Erdem, Ela; Department of Pediatric Pulmonology, Marmara University School of Medicine, Istanbul, Turkey
van Gogh, Christine; Department of Otorhinolaryngology-Head and Neck Surgery, Amsterdam UMC, Amsterdam, the Netherlands
Gunaydin, Onder; Department of Otorhinolaryngology, Hacettepe University School of Medicine, Ankara, Turkey
Haarman, Eric G; Department of Pediatric Pulmonology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit, Amsterdam, the Netherlands
Harris, Amanda; Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton, Southampton, England, United Kingdom
Hayn, Isolde; Department of Otorhinolaryngology-Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Berlin, Germany
Ismail-Koch, Hasnaa; Primary Ciliary Dyskinesia Centre, Southampton Children's Hospital, Southampton NHS Foundation Trust, Southampton, England, United Kingdom
Karadag, Bulent; Department of Pediatric Pulmonology, Marmara University School of Medicine, Istanbul, Turkey
Kempeneers, Céline ; Centre Hospitalier Universitaire de Liège - CHU > > Service de pédiatrie
Kim, Sookyung; Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Saclay, Hôpital Bicêtre, Service d'ORL, Le Kremlin-Bicêtre, France
Lorent, Natalie; Department of Respiratory Diseases, University Hospital, Leuven, Belgium
Ozcelik, Ugur; Department of Pediatric Pulmonology, Hacettepe University School of Medicine, Ankara, Turkey
Pioch, Charlotte; Department of Pediatric Pulmonology, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany
Poirrier, Anne-Lise ; Centre Hospitalier Universitaire de Liège - CHU > > Service d'ORL, d'audiophonologie et de chir. cervico-faciale
Reula, Ana; Biomedical Sciences Department, CEU-Cardenal Herrera University, Castellón, Spain ; Molecular, Cellular and Genomic Biomedicine Group, Instituto de Investigación Sanitaria La Fe, Valencia, Spain
Roehmel, Jobst; Department of Pediatric Pulmonology, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany
Yiallouros, Panayiotis; University of Cyprus Medical School, Nicosia, Cyprus ; Pediatric Pulmonology Unit, Archbishop Makarios III Hospital, Nicosia, Cyprus
Yumusakhuylu, Ali Cemal; Department of Otolaryngology, Marmara University School of Medicine, Istanbul, Turkey
Papon, Jean-François; Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Saclay, Hôpital Bicêtre, Service d'ORL, Le Kremlin-Bicêtre, France ; Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
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