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Hearing loss and tympanostomy tube outcomes in primary ciliary dyskinesia
Liliana Arida-Moody1, Roberto N Solis2, Michael G O'Connor2
1Vanderbilt University School of Medicine, Nashville, TN, USA.
Insights
Children with primary ciliary dyskinesia (PCD) often experience hearing loss, requiring speech and educational support. Hearing aids may offer better long-term hearing management than tympanostomy tubes due to complications.
Area of Science:
- Otolaryngology
- Genetics
- Pediatrics
Background:
- Primary ciliary dyskinesia (PCD) is a genetic disorder affecting mucociliary clearance.
- Otologic sequelae and hearing loss are common in children with PCD.
- Early diagnosis and management of hearing impairment are crucial for development.
Purpose of the Study:
- To characterize the otologic complications and the impact of hearing loss in patients with primary ciliary dyskinesia (PCD).
- To evaluate the effectiveness of current interventions for hearing loss in PCD patients.
Main Methods:
- Retrospective chart review of 40 patients with PCD diagnosed between 2006-2025.
- Data included audiology results, tympanostomy tube placements, speech concerns, hearing amplification, and individualized education plans (IEPs).
- Diagnosis confirmed by genetic testing, ciliary biopsy, or clinical criteria.
Main Results:
- 65% of PCD patients had confirmed hearing loss.
- 77.5% underwent tympanostomy tube placement, with 74.2% experiencing otorrhea.
- Hearing loss in PCD patients was associated with speech concerns (57.7%), need for IEPs (19.2%), and hearing amplification (19.2%).
Conclusions:
- PCD patients are diagnosed younger due to improved early detection.
- Children with PCD-related hearing loss often need speech and educational support.
- Hearing amplification may be a more consistent alternative to tympanostomy tubes given the high complication rate and fluctuating hearing loss in PCD.
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.
