Otologic manifestations of the immotile-cilia syndrome.
The immotile-cilia syndrome is caused by a hereditary, inborn, ultrastructural defect of the cilia, rendering them immotile or poorly motile and thereby abolishing mucociliary clearance. Five cases are presented and the otologic manifestations are described in some detail. The syndrome should be suspected in children who have a persistent secretory otitis media with recurring bouts of acute otitis media, and a perpetual cough with repeated episodes of bronchitis. A lobar atelectasis is a frequent finding. Half the cases also have situs inversus. The immotile-cilia syndrome is of special interest to the otologist, as it seems to throw some light on the pathogenesis of secretory otitis media.
D000293:Adolescent; D000328:Adult; D001991:Bronchitis / Q000175:diagnosis*; D002923:Cilia / Q000502:physiology* / Q000648:ultrastructure; D005260:Female; D006801:Humans; D008297:Male; D009093:Mucus / Q000502:physiology; D010033:Otitis Media / Q000175:diagnosis*; D010034:Otitis Media with Effusion / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D001261:Pulmonary Atelectasis / Q000175:diagnosis; D012008:Recurrence; D012857:Situs Inversus / Q000175:diagnosis*; D013081:Sperm Motility; D013577:Syndrome