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Published on: January 19, 2022
Management of middle ear disease in pediatric primary ciliary dyskinesia
Ethan G Muhonen1, Austin Zhu2, Sera Sempson3
1Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine, Aurora, CO, USA; Department of Pediatric Otolaryngology, Children's Hospital Colorado, Aurora, CO, USA.
Insights
Children with primary ciliary dyskinesia (PCD) often experience middle ear issues. This study suggests a management plan to limit tympanostomy tube use and improve outcomes for otitis media with effusion and hearing loss.
Area of Science:
- Otolaryngology
- Pediatric Otology
- Genetics
Background:
- Primary ciliary dyskinesia (PCD) frequently causes middle ear disease, including otitis media with effusion (OME) and conductive hearing loss (CHL) from early childhood.
- Tympanostomy tube (TT) placement in PCD patients carries risks of chronic otorrhea and tympanic membrane perforation.
- The optimal management strategy for OME and CHL in PCD remains a subject of ongoing debate.
Purpose of the Study:
- To propose and evaluate a management schema for middle ear conditions in pediatric patients diagnosed with PCD.
- To compare clinical and audiological outcomes between patients with and without tympanostomy tube (TT) placement.
Main Methods:
- Retrospective review of a pediatric PCD cohort at Children's Hospital Colorado (CHCO).
- Analysis of electronic health records, focusing on middle ear status and audiological data.
- Comparison of outcomes across four age groups, stratified by TT status (no-TT vs. TT).
Main Results:
- No significant differences in normal tympanic membrane (TM) prevalence or OME between no-TT and TT groups across all age categories.
- The no-TT subgroup showed a higher percentage of normal TM in the 10 to <15 years age group (p=0.006).
- Fewer ears with hearing loss were observed in the no-TT subgroup for the ≥15 years age group (p=0.008).
- The TT subgroup experienced tympanic membrane perforations in 9 out of 13 subjects/ears, with a total of 130 TTs and other procedures performed.
Conclusions:
- Children with PCD exhibit a high incidence of otologic complications.
- A management schema is proposed to judiciously select candidates for TT placement.
- The proposed schema aims to actively manage CHL and prevent adverse sequelae of OME for improved clinical outcomes.
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.
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