Congenital hydrocephalus due to aqueductal stenosis of probable autosomal recessive inheritance

Hereditary stenosis of the aqueduct of Sylvius is known to be transmitted a s an X-linkcd recessive trait, thus affecting only the male offspring. We d escribe a family of consanguineous marriage where 2 sons and 2 daughters we re afflicted with congenital hydrocephalus associated with stenosis; of aqu educt of Sylvius. The pattern of the disease in this family is consistent w ith autosomal recessive inheritance. The authors of this communication are not aware of any literature reporting a similar mode of inheritance regardi ng this condition, Perhaps this discordant genetic mode of inheritance coul d have been caused by the consanguinity between the parents.