BILATERAL SENSORINEURAL DEAFNESS AND HYDROCEPHALUS DUE TO FORAMEN OF MONRO OBSTRUCTION IN SIBS - A NEWLY DESCRIBED AUTOSOMAL RECESSIVE DISORDER

We identified a Canadian-Mennonite family in which a brother and siste r have hydrocephalus due to obstruction at the foramen of Monro and pr ofound bilateral sensorineural deafness. This appears to be a unique c ombination of anomalies and, to our knowledge, has not been reported p reviously. Both parents and a brother are phenotypically normal. The p arents are second cousins. Thus, on the basis of consanguinity, affect ed sibs of both sexes, and in the absence of evidence for intrauterine infections or other adverse perinatal events, this syndrome is likely inherited in an autosomal recessive fashion. (C) 1997 Wiley-Liss, Inc .