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Since a case of hydrocephalus in humans considered to be caused by ciliary dysfunction was first reported by Greenstone et al. in 1984, numerous papers onthe correlation between ciliary function and hydrocephalus have been published. We reviewed the published literature on primary ciliary dyskinesia inhumans causing hydrocephalus, focusing on articles specifically examining the relation between ciliary function and hydrocephalus and its treatment. In addition, theauthors' experience is briefly discussed. Full texts of 16 articles report