To evaluate otologic features in children with primary ciliary dyskinesia (PCD).
pediatric referral center
58 PCD patients, distributed in four age-groups (I: 0–5 years; II: 6–11 years; III: 12–17 years and IV: above 18 years with 47, 50, 34 and 10 cases, respectively). Follow-up: 2 to 6 years in each age-group. Ultrastructural defects of outer or inner dynein arms, and central complex (CC): 33, 13 and 11 cases, respectively.
MAIN OUTCOME MEASURES
Frequency of: acute otitis media (AOM), otitis media with effusion (OME), otorrhea, chronic otitis media, hearing loss, middle ear surgery and type of antibiotic regimen according to age and type of defect.
Recurrent AOM decreased from group I (68%) to group IV (0%), p<0.00001. OME was more severe in groups I to III than in group IV, p=0.02. Otorrhea decreased in group IV: 30% versus 80% in other groups, p<0.001. One half of patients with tubes eventually had tympanic perforation. Hearing loss was moderate in groups I to III and mild in group IV. Continuous antibiotics could be slightly reduced only in group IV. CC defect was a significant marker of severity for all of these criteria.
Despite continuous antibiotics, the middle ear condition in PCD remained severe throughout childhood with improvement only after the age of 18 years. Grommet placement failed to improve the middle ear condition. CC defect is a marker of severity.