Hearing loss and tympanostomy tube outcomes in primary ciliary dyskinesia.

OBJECTIVE

Characterize otologic sequelae and impact of hearing loss in primary ciliary dyskinesia (PCD) STUDY

DESIGN

Retrospective chart review.

SETTING

Tertiary care academic children's hospital.

METHODS

Medical records of patients with PCD seen 2006-2025 were reviewed. Diagnosis was confirmed by positive genetic testing, ciliary biopsy, or clinical criteria. Data collection included audiology, tympanostomy tube placements, speech concerns, hearing amplification, and individualized education plans (IEPs).

RESULTS

Of 40 patients with PCD, 26 had confirmed hearing loss (65%). Thirty-one patients (77.5%) underwent at least one tympanostomy tube placement, with a median of 3 procedures (range 1-7). 23 of these patients (74.2%) experienced otorrhea after tube placement, and 13 patients (41.9%) had otorrhea for >12 months. Of the patients with hearing loss, 15 (57.7%) had documented speech concerns, 5 patients (19.2%) required an IEP in school, and 5 (19.2%) required hearing amplification. Air conduction pure tone averages were significantly higher after tubes had extruded or been removed (20.3 ± 10.2) compared to when they were in place (17.1 ± 8, p = 0.001).

CONCLUSION

PCD patients born more recently have received their diagnoses at a younger age than their older counterparts, likely due to improvements in early detection. Children with PCD-related hearing loss frequently required speech intervention and educational support. Given the fluctuant nature of hearing loss in PCD and the high complication burden associated with tympanostomy tubes, hearing amplification as an alternative treatment may provide more consistent functional hearing support throughout childhood.