Otologic Features in Children With Primary Ciliary Dyskinesia

Abstract

To analyze otologic features in patients with primary ciliary dyskinesia (PCD) aged 0 to 18 years and to evaluate the correlation between ultrastructural defects and severity of otologic features. Retrospective study. Pediatric referral center. Fifty-eight patients with PCD were evaluated in the following 4 age intervals: group 1, preschool (≤ 5 years [n = 47]); group 2, school (6-11 years [n = 50]); group 3, teenagers (12-17 years [n = 34]); and group 4, young adults (≥ 18 years; 27 years for the oldest [n = 10]). Follow-up was 2 to 6 years in each age group; 26 patients had total follow-up of more than 12 years. Ultrastructural defects occurred in the outer dynein arm (n = 33), the inner dynein arm (n = 13), and the central complex (n = 11). One patient had typical Kartagener syndrome with typical PCD features but normal ciliary ultrastructure. Frequency of acute otitis media, otitis media with effusion, otorrhea, chronic otitis media, hearing loss, and middle ear surgery and type of antibiotic regimen according to age and type of defect. Recurrent acute otitis media decreased from group 1 (32 of 47 [68%]) to group 4 (0 of 10 [0%]) (P < .001). Otitis media with effusion was more severe in groups 1 through 3 than in group 4 (P = .02). Otorrhea decreased in group 4: 30% vs 80% (3 of 10 vs 36 of 41) in the other groups (P < .001). Half of the patients with tympanostomy tubes eventually had tympanic perforation. Hearing loss was moderate in groups 1 through 3 and mild in group 4. Continuous antibiotic therapy could be slightly reduced only in group 4. Central complex defect was a significant marker of severity for all these criteria. Despite continuous antibiotic therapy, the middle ear condition in PCD remained severe throughout childhood, with improvement only after age 18 years. Armstrong grommet placement did not improve the middle ear condition. Central complex defect is a marker of severity.