A New Gene For Autosomal Dominant Nonsyndromic Sensorineural Hearing Loss (DFNA32) Maps to 11p15. X.C.1, H.M.2, T.B.3, R.A.1. 1) Department of Cell and Molecular Biology, House Ear Institute, 2100 W. Los Angles; 2) Human Genetics Division, Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH; 3) Laboratory of Molecular Genetics, NIDCD, NIH, Rockville, MD.
Hearing loss is the most common sensory defect in humans. About one in every 1000 children is affected by severe hearing loss at birth or during early childhood. More than 50% of these cases are due to genetic cause with extensive genetic heterogeneity A genetic linkage study conducted on a large multigenerational US family with nonsyndromic autosomal dominant progressive hearing loss has resulted in the localization of a new deafness locus, DFNA32. The deafness gene segregating in this family is mapped to the telemere region of chromosome 11p15. A maximum lod score of 4.1 was obtained with Marker D11S1984. The DFNB18 and two syndromic deafness genes, Usher syndrome type 1C and Jervell and Lange-Nielson syndrome have been mapped to the proximity of this interval. The DFNA32 interval is approximately 3-4 cM distal to DFNB18 and Usher 1C, but does not overlap with them.