Primary ciliary dyskinesia is a hereditary defect in the ultrastructur
e of cilia, leading to poor ciliary motility. The sinonasal and the br
onchial manifestations of the disease are well documented; whereas its
otological aspects have received less attention. In this report, we d
escribe the clinical profile of 16 patients with primary ciliary dyski
nesia laying particular emphasis on the otological manifestations. All
children (11 patients) had bilateral otitis media with effusion. Of t
he five adults, three had tympanosclerosis; one had bilateral choleste
atoma; and one patient had bilateral keratosis obturans in combination
with tympanosclerosis. Hearing improvement and a dry ear was achieved
in all the children treated by tympanostomy tube insertion. The study
suggests that otitis media is a prominent feature of this disorder. M
ost subjects suffer from protracted bilateral otitis media with effusi
on throughout childhood.