Prune Belly Syndrome With Pulmonic Stenosis, Mental Retardation, And Deafness

Lockhart et al. (1979) described a family in which 2 brothers and a sister had this combination. A fourth sib may have been affected; the mother reported that the male, who died 25 hours after birth, had 'a big bladder, big ureters, bad kidneys.' The hearing loss was sensorineural in nature. Because the involvement in the female was less marked than in the males, the possibility of X-linked inheritance was suggested. No information concerning abnormalities in the mother was provided.