title: Otologic features in children with primary ciliary dyskinesia.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  Pediatric referral center.
  
  
  METHODS
  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.
  
  
  METHODS
  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.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Hôpital Intercommunal de Créteil, 94000 Créteil CEDEX, France. virginie.escabasse@chicreteil.fr
authors.forename: Virginie
authors.initials: V
authors.lastname: Prulière-Escabasse
authors.forename: Andre
authors.initials: A
authors.lastname: Coste
authors.forename: Pierre
authors.initials: P
authors.lastname: Chauvin
authors.forename: Brigitte
authors.initials: B
authors.lastname: Fauroux
authors.forename: Aline
authors.initials: A
authors.lastname: Tamalet
authors.forename: Erea-Noel
authors.initials: EN
authors.lastname: Garabedian
authors.forename: Estelle
authors.initials: E
authors.lastname: Escudier
authors.forename: Gilles
authors.initials: G
authors.lastname: Roger
mesh_terms: D000293:Adolescent; D000900:Anti-Bacterial Agents / Q000008:administration & dosage; D001299:Audiometry; D001987:Bronchiectasis / Q000209:etiology; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D005260:Female; D034381:Hearing Loss / Q000209:etiology*; D006801:Humans; D007223:Infant; D007619:Kartagener Syndrome / Q000150:complications*; D008297:Male; D010033:Otitis Media / Q000209:etiology*; D012189:Retrospective Studies
chemical_list: D000900:Anti-Bacterial Agents