Author(s)
Sera Sempson, BA
Ethan Muhonen, MD
Austin Zhu, BA
Scott Sagel, MD
Kenny Chan, MD
Affiliation(s)
Children's Hospital of Colorado Department of Otolaryngology; University of Colorado School of Medicine
Abstract:
Introduction: Primary ciliary dyskinesia (PCD) is a ciliopathic, autosomal recessive genetic disorder that causes defective mucociliary clearance throughout the respiratory tract. We previously explored the impact of otitis media (OM) in 54 patients from 2007-2018 (2018 ESPO poster) after which our group began emphasizing avoidance of PE tubes. In this study our specific aims were to:
• analyze the frequency of OM and its medical and surgical complications.
• quantify audiologic outcomes and rehabilitation.
Methods: A 16-year retrospective chart review was performed using a patient registry at the PCD Center and the electronic health record system of an academic tertiary children’s hospital.
Results: Seventy-four PCD subjects were identified, 54 from the 2007-2018 period and 20 from the 2018-2023 period. Of the 62 subjects with otitis media with effusion, 33 had audiologic data. Of the 45 ears with hearing loss, 37 had mild conductive hearing loss. While the proportion of patients using hearing aids was unknown due to our vast service region, 13 patients utilized bone conduction devices at our institution. 48 subjects underwent at least 1 set of PE tubes. Decreased PE tube placement was noted during the 2018-2013 period, from 66.7% to 20.0% (p=.0005). The otorrhea rate was similar at 72.2% from 2007-2018 and 75% from 2018-2023.
Two patients received implantable bone-anchored hearing devices. Four patients required 6 ear-related admissions for intravenous antibiotics. Other otologic surgeries included: removal of intratympanic cyst (n=1), myringoplasty (n=3), tympanoplasty (n=5), and mastoidectomy (n=2).
Discussion: This work represents one of largest cohorts of pediatric patients with PCD in the literature and is the largest to specifically address middle ear disease. Despite the heavy burden of ear disease in PCD patients, serious medical and surgical interventions were limited. Given the preponderance of otorrhea but rarity of other complications, PE tubes should be used judiciously.