DFNB28, a novel locus for prelingual nonsyndromic autosomal recessive hearing loss maps to 22q13 in a large consanguineous Palestinian kindred. T.D. Walsh1, H. Shahin2, 3, J. Morrow1, M-C. King1, E. Lynch1, K. Avraham2, M. Kanaan3. 1) Medical Genetics, University of Washington, Seattle, WA; 2) Department of Human Genetics and Molecular Medicine, Sackler School of Medicine, Tel Aviv University, Israel; 3) Department of Life Sciences, Bethlehem University, Palestinian Authority.
Nonsyndromic hearing loss (NSHL) is genetically heterogeneous. Most autosomal recessive NSHL is prelingual and severe to profound. Thus far, more than 30 autosomal recessive NSHL loci have been mapped and six genes have been cloned. We mapped recessive, prelingual, NSHL in a four-generation consanguineous Palestinian family. The maximum LOD score is 5.23 at the 6.5Mb interval on chromosome 22q13 bound by markers D22S1045 and D22S282. The DFNB28 region is contained completely within the much larger interval linked to DFNA17. DFNB28 is distal to the region deleted in velo-cardio-facial syndrome. Several candidate genes on chromosome 22q13 have been excluded as the cause of deafness in the family by sequence analysis. The DFNB28 region includes more than 70 genes, so ascertainment of distantly related affected individuals would be helpful to narrow the region.