Management of middle ear disease in pediatric primary ciliary dyskinesia.

Introduction: Primary ciliary dyskinesia (PCD) adversely affects the middle ear from early childhood resulting in otitis media with effusion (OME) and conductive hearing loss (CHL). Chronic otorrhea and tympanic membrane (TM) perforation are serious postoperative complications from tympanostomy tube (TT) placement. Management of OME and CHL has been debated for decades. This study reports on a proposed management schema for the middle ear in individuals with PCD.

Methods: A pediatric PCD cohort was retrospectively reviewed at Children's Hospital Colorado (CHCO) using electronic health record systems. Paired middle-ear/audiologic data for all subjects at their most recent CHCO visits were organized in a 4-age-group grid to compare clinical/audiologic outcomes between no-TT and TT subgroups.

Results: 68 PCD subjects with middle-ear and audiologic data were tabulated. No-TT and TT subgroup comparisons showed no difference in clinical (normal TM and OME prevalence) and hearing [CHL percentage and pure-tone average (PTA)] outcomes across 4 age-groups except for higher percentage of normal TM (10 to <15 years category; p = 0.006) and fewer hearing loss ears (≥15 years category; p = 0.008), both favoring the no-TT subgroup. There were 9/13 (subjects/ears) in the TT subgroup with TM perforations, and a total of 130 sets of TT and other medical/surgical procedures were performed in this sub-cohort.

Conclusion: Children with PCD have a high prevalence of otologic complications. A schema is proposed to restrict the indications for TT placement and guide active management of CHL with the goal of preventing adverse sequalae of OME and achieving best clinical outcomes.