Ernstson S, Afzelius B A, Mossberg B
Acta Otolaryngol. 1984 Jan-Feb;97(1-2):83-92. doi: 10.3109/00016488409130967.
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.
不动纤毛综合征由纤毛的遗传性、先天性超微结构缺陷引起,使纤毛不能运动或运动不良,从而消除了黏液纤毛清除功能。本文报告了5例病例,并对耳部表现进行了较为详细的描述。对于患有持续性分泌性中耳炎并反复发作急性中耳炎,以及持续性咳嗽并反复发生支气管炎的儿童,应怀疑患有该综合征。肺叶不张是常见的表现。半数病例还伴有内脏转位。不动纤毛综合征引起了耳科医生的特别关注,因为它似乎有助于阐明分泌性中耳炎的发病机制。