Adult onset hydrocephalus and situs inversus: new autosomal dominant syndrome?

Familial adult onset hydrocephalus is unusual and its genetic basis is unknown. We report a two-generation family with three members having hydrocephalus, two of them with complete situs inversus. Laterality syndromes were considered in the differential diagnosis and are unlikely. We suggest that this family represents a previously unrecognised form of autosomal dominant late onset hydrocephalus with situs inversus.