Primary ciliary dyskinesia (PCD) is a rare, genetically heterogeneous disorder of motile cilia that leads to impaired mucociliary clearance and recurrent airway infections. Children with PCD often present with ear and sinus disease resembling common pediatric conditions, yet the true burden and management remain incompletely defined. To address this gap, a systematic search for pediatric cohort studies published between 2020 and 2025 reporting otologic and sinonasal features of PCD was performed. Searches of PubMed, Embase, Scopus, and Web of Science identified 12 eligible studies, encompassing 524 children with confirmed PCD. Data extracted focused on demographics, otologic and sinonasal manifestations, vestibular findings, radiographic imaging, and interventions. Across studies, 60.3% had a history of otitis media and 39.1% had hearing loss, predominantly conductive. Tympanostomy tubes were utilized in more than half of patients, with many requiring multiple sets over time due to recurrent effusions or tube occlusion. Sinonasal disease was nearly universal, with 78.5% demonstrating chronic rhinosinusitis and most reporting nasal congestion and rhinorrhea; nasal polyps were uncommonly noted. Vestibular symptoms were also infrequently assessed but present in some patient cohorts. In conclusion, otologic and sinonasal disease are highly co-prevalent in pediatric PCD, highlighting the need for early recognition, regular surveillance, and standardized outcome reporting to guide long-term management of this complex chronic disease.
Nguyen et al. (Wed,) studied this question.