A NON-SYNDROMIC FORM OF NEUROSENSORY, RECESSIVE DEAFNESS MAPS TO THE PERICENTROMERIC REGION OF CHROMOSOME-13Q

Non-syndromic, recessively inherited deafness is the most predominant form of severe inherited childhood deafness. Until now, no gene respon sible for this type of deafness has been localized, due to extreme gen etic heterogeneity and limited clinical differentiation. Linkage analy ses using highly polymorphic microsatellite markers were performed on two consanguineous families from Tunisia affected by this form of deaf ness. The deafness was profound, fully penetrant and prelingual. A max imum two-point lod score of 9.88 (theta = 0.001) was found with a mark er detecting a 13q locus (D13S175). Linkage was also observed to the p ericentromeric 13q12 loci D13S115 and D13S143. These data map this neu rosensory deafness gene to the same region of chromosome 13q as the ge ne for severe, childhood autosomal recessive muscular dystrophy.