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

OBJECTIVE

Otitis media and conductive hearing loss are common in patients with primary ciliary dyskinesia (PCD). We primarily aimed to assess the impact of otologic features of individuals with PCD on health-related quality of life (QOL), and our secondary aims are characterization of the coexisting pulmonary and sinonasal findings.

METHODS

Demographic data, spirometry values, a health-related quality of life questionnaire for PCD patients (QOL-PCD), physical examination data of sinonasal and otologic systems, the sinonasal outcome test-22, and audiologic tests were recorded in this cross-sectional study. The pure tone audiometry, distortion product otoacoustic emission (DPOAE), tympanogram, and the Turkish matrix test (TUR-matrix) evaluating speech understanding in noise were performed.

RESULTS

Fifty-six patients with PCD were included in the study. The scores of the social, upper, and lower respiratory domains of the QOL-PCD were significantly lower in adults than in children and adolescents (p-value < 0.001, 0.02, and 0.01, respectively). The hearing and the TUR-matrix were pathologic in 26 (46.4%) and 36 (64.3%) cases, respectively. Patients with pathological hearing had significantly worse results in regards to the FEV1 z-score (-1.7 vs. -0.8, p = 0.01), the TUR-matrix test (20 vs. 16 cases, p = 0.001), the DPOAE (25 vs. 11 cases, p < 0.001), and the tympanogram (26 vs. 12 cases, p < 0.001) than patients with normal hearing.

CONCLUSION

Sinopulmonary involvement and hearing manifestations are very common in patients with PCD, and speech understanding in noise is often impaired. These manifestations impact QOL in various areas.