OTOLOGICAL MANIFESTATIONS OF PRIMARY CILIARY DYSKINESIA

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.