Middle ear ventilation in children with primary ciliary dyskinesia

Objective: Otitis media with effusion (OME) in children with primary ciliary dyskinesia (PCD) is recurrent and persistent and presents a complex clinical problem for otolaryngologists. We sought to review the otological presentation of children with PCD treated medically and surgically and to compare audiological outcomes and complications of surgical and medical management in children with PCD and correlate these findings with known PCD ultrastructural ciliary phenotype.

Methods: A retrospective review was performed of all patients with PCD and OME presenting from 1991 to 2009. Patients were grouped into "medical management" and "ventilation tube (VT) insertion" groups to observe changes in hearing and post-operative complications.

Results: Of 31 patients with PCD and OME, 20 received VTs and 11 were treated by medical management strategies. A median of one set of VTs (range 1-5) were inserted per patient. Hearing thresholds improved to normal (<25 dB HL) in 80% ears with VTs (mean improvement from 27.1 dB to 22.1 dB (p=0.034)), whereas patients managed medically had stable thresholds (22.7-23.6 dB (p=0.397) over the study period. At least one episode of post-operative otorrhea occurred in eight (42.1%) VT insertion patients during the study period, and four had multiple episodes. We could not demonstrate any differences in post-surgical outcomes between patient groups based on ultrastructural ciliary phenotype. Otorrhea was well controlled with medical therapy with only one requiring tube removal. Bilateral cholesteatoma was found in two patients.

Conclusions: Our data support the use of VT insertion as an option for patients with PCD and OME with mild to moderate hearing loss. Patients should be counseled on the possibility of multiple insertions and the likelihood of post-operative otorrhea, although this was not very troublesome in our group. Ultrastructural ciliary phenotype did not appear to alter the likelihood of post-operative otorrhea in our series. Cholesteatoma should be considered as a potential cause of otorrhea in PCD.