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High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
Published on: January 19, 2022
Otologic features in children with primary ciliary dyskinesia
Virginie Prulière-Escabasse1, Andre Coste, Pierre Chauvin
1Department of Otolaryngology-Head and Neck Surgery, Hôpital Intercommunal de Créteil, 94000 Créteil CEDEX, France. virginie.escabasse@chicreteil.fr
Insights
Middle ear disease in primary ciliary dyskinesia (PCD) remains severe in children despite antibiotics, improving after age 18. Central complex defects indicate greater severity in PCD patients.
Area of Science:
- Otolaryngology
- Genetics
- Pediatrics
Background:
- Primary ciliary dyskinesia (PCD) is a genetic disorder affecting cilia function.
- Otologic manifestations are common in PCD but require detailed analysis across age groups.
- Understanding the correlation between ciliary ultrastructural defects and otologic severity is crucial for management.
Purpose of the Study:
- To analyze otologic features in pediatric patients with primary ciliary dyskinesia (PCD).
- To evaluate the correlation between specific ciliary ultrastructural defects and the severity of otologic conditions in PCD patients aged 0-18 years.
Main Methods:
- Retrospective study at a pediatric referral center.
- Evaluation of 58 PCD patients across four age intervals: preschool, school-aged, teenagers, and young adults.
- Analysis of otologic outcomes including acute otitis media, otitis media with effusion, otorrhea, hearing loss, and middle ear surgery, correlated with ultrastructural defects (outer dynein arm, inner dynein arm, central complex).
Main Results:
- Recurrent acute otitis media and otorrhea significantly decreased with age, particularly after 18 years.
- Otitis media with effusion was more severe in younger age groups (preschool to teenagers).
- Central complex defects were identified as a significant marker for increased severity of otologic features across all evaluated criteria.
Conclusions:
- Otologic conditions in PCD patients remain severe throughout childhood, with notable improvement observed only after 18 years of age.
- Continuous antibiotic therapy showed limited benefit in improving the middle ear condition during childhood.
- Central complex ciliary defects serve as a key indicator of disease severity in pediatric PCD patients.
Objectives:
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.
Design:
Retrospective study.
Setting:
Pediatric referral center.
Patients:
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.
Main Outcome Measures:
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.
Results:
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.
Conclusions:
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.
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