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Evaluation of Pulmonary and Otological Manifestations in Primary Ciliary Dyskinesia and Their Effect on Quality of Life

Pediatric Pulmonology·August 26Open Access
Respiratory SystemLimited evidenceBronchiectasisChronic RhinosinusitisConductive Hearing LossOtitis Media With EffusionPrimary Ciliary DyskinesiaCross-Sectional StudyMixed

Summary

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What was studied

This cross-sectional study (Jan 2023–Jan 2024) evaluated 56 PCD patients aged ≥6 years (mean age 15.2 years) for otologic, pulmonary, and sinonasal manifestations and their impact on health-related QOL (QOL-PCD), using pure-tone audiometry, DPOAE, tympanogram, speech-in-noise testing (TUR-Matrix), spirometry, and SNOT-22.

Key findings

Pathologic hearing was found in 46.4% (26/56) and abnormal speech-in-noise (TUR-Matrix) in 64.3% (36/56). Patients with pathologic hearing had significantly worse FEV1 z-scores (−1.7 vs. −0.8, p=0.01), more abnormal DPOAE (25 vs. 11, p<0.001), and worse tympanograms (26 vs. 12, p<0.001). Adults had significantly lower QOL scores in social, upper, and lower respiratory domains than children and adolescents (p<0.001, 0.02, and 0.01, respectively).

Study limitations

- Single-center design with a small sample (n=56) limits generalizability. - No multiple testing correction applied despite numerous comparisons. - Cross-sectional design prevents causal inference between hearing loss and pulmonary decline.

Clinical implications

Nearly half of PCD patients have measurable hearing loss, and nearly two-thirds struggle with speech understanding in noise — both often go unreported. Routine multidisciplinary assessment including objective audiologic testing (not just symptom inquiry) should be standard in PCD care, especially in patients with lower FEV1.

Caveats

  • No multiple testing correction was applied; several exploratory comparisons should be interpreted cautiously.
  • Speech-in-noise impairment rate (64.3%) is novel — this is the first study to apply this test in PCD — so reference ranges and clinical thresholds in this population are not yet established.
  • The study is single-center with n=56; 17 of 73 eligible patients were excluded due to refusal or missed appointments, introducing potential selection bias.

Related Questions

Explore related topics

How common is hearing loss in primary ciliary dyskinesia and how should it be managed?Does speech-in-noise testing add value to audiologic follow-up in pediatric PCD patients?How does sinonasal disease severity relate to quality of life outcomes in primary ciliary dyskinesia?

Publication Details

Year
2026
Journal
Pediatric Pulmonology
Sample Size
n=56
Source
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