Primary Ciliary Dyskinesia Patients: To Tube or Not to Tube?

Introduction: To analyze otologic manifestations in patients with primary ciliary dyskinesia (PCD) and evaluate the effects of Pressure Equalization Tube (PET) insertion compared to those managed conservatively without PETs.

Methods: A retrospective longitudinal review was conducted on pediatric and adult patients with confirmed PCD diagnosis from two Montreal university-affiliated hospitals. Data on patient characteristics, otologic evaluations, and treatment outcomes were recorded.

Results: Of the 88 PCD patients presenting with otologic symptoms, 67 (76.1%) underwent PET insertion. Among children (48/67; age range: 7 months–17.9 years), 67.5% underwent their first PET before age 5 (mean: 2.3yrs), and 56.3% required repeated PETs (2–11 procedures; mean age: 9.1yrs.). In adults (19/67; age range 19.1–71yrs.), 89.5% had prior childhood PETs, averaging 4.6 insertions (range 2–30). Main adverse events included tube clogging (36%), displacement or premature extrusion (22.4%), and granulation, scarring, or myringosclerosis/tympanosclerosis (34.3%). Persistent otorrhea was reported in 55.2%, with chronic otitis media (COM) including tympanic perforation observed in 19.4%, and 7.5% requiring hearing aids. Conductive hearing loss post-insertion persisted in only 14/67 (20.9%) of patients, primarily adults (42.1%). Among the 21 patients (23.9%) managed conservatively (age range: 0.75–71yrs.), recurrent OM occurred in 52.4%, with 9.5% developing COM with tympanic perforation. Hearing loss was reported in 9/21 (42.9%) of conservatively managed patients, predominantly in adults (46.7%).

Conclusions: PET insertion notably increased normal hearing in our PCD patients, though it carried risks. However, hearing loss remained prevalent in adults, regardless of PET status, underscoring the dilemma and challenges otolaryngologists face in managing otologic symptoms in this population