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Results of tympanoplasty in children with primary ciliary dyskinesia
F Denoyelle1, G Roger, V Ducroz
1Department of Pediatric Otolaryngology, Armand-Trousseau Children's Hospital, Paris, France.
Insights
Tympanoplasty in children with primary ciliary dyskinesia shows high graft success and hearing improvement. However, serous otitis media frequently recurs, requiring ongoing management.
Area of Science:
- Otolaryngology
- Pediatric Surgery
- Genetics
Background:
- Primary ciliary dyskinesia (PCD) is a genetic disorder affecting mucociliary clearance.
- PCD often leads to chronic ear infections, tympanic perforations, and cholesteatoma in children.
- These complications can result in hearing loss and recurrent otorrhea.
Purpose of the Study:
- To evaluate the outcomes of tympanoplasty in pediatric patients with primary ciliary dyskinesia.
- To assess graft success, hearing improvement, and recurrence of otorrhea after surgery.
- To analyze the impact of tympanoplasty on cholesteatoma management in this patient group.
Main Methods:
- Retrospective study of seven children with primary ciliary dyskinesia.
- Inclusion criteria: tympanic perforation or attic cholesteatoma with hearing loss and/or otorrhea.
- Surgical procedures included type 1 tympanoplasty and canal wall-down tympanoplasty with mastoidectomy.
Main Results:
- Nine type 1 tympanoplasties resulted in intact grafts in all cases, with no otorrhea recurrence.
- Significant auditory improvement was observed, averaging a 17-dB gain in speech frequencies.
- One case of attic cholesteatoma treated with canal wall-down tympanoplasty showed no residual disease or otorrhea at 46 months.
- Serous otitis media recurred in 6 out of 9 ears post-tympanoplasty.
Conclusions:
- Tympanoplasty offers a high likelihood of graft success and hearing restoration in children with primary ciliary dyskinesia.
- Despite surgical success, the frequent recurrence of serous otitis media highlights the chronic nature of the condition.
- Surgical intervention is effective for managing tympanic perforations and cholesteatoma in this population.
Objective:
To assess the results of tympanoplasty in children with primary ciliary dyskinesia complicated by tympanic perforation or cholesteatoma with hearing loss and/or recurrent otorrhea.
Design:
Retrospective study. Postoperative follow-up of 26.3 months in the type 1 tympanoplasty group and 46 months in the child with cholesteatoma.
Setting:
Hospitalized care, referral center.
Patients:
Seven children with primary ciliary dyskinesia, complicated in 6 children by 9 tympanic perforations (3 bilateral perforations) and in 1 child by an attical cholesteatoma.
Results:
After 9 type 1 tympanoplasties, the grafts were intact in 9 ears, with no recurrence of otorrhea, but serous otitis media was present in 6 of the 9 ears. Auditory improvement was significant, with an average gain of 17-dB hearing level in speech frequencies. After a canal wall-down tympanoplasty with mastoidectomy for attic cholesteatoma in 1 ear, the cavity that was operated on showed no signs of otorrhea or residual cholesteatoma after a follow-up of 46 months.
Conclusion:
In children with primary ciliary dyskinesia, tympanoplasty has a high probability of graft success and auditory improvement, despite the frequent recurrence of serous otitis media.