A gene for a dominant form of non-syndromic sensorineural deafness (DFNA11) maps within the region containing the DFNB2 recessive deafness gene

Hereditary hearing loss is divided into two groups, syndromic and non-syndromic, the latter being more common and highly heterogeneous. Linkageanalyses were performed on a Japanese family showing a dominant form ofnon-syndromic progressive sensorineural hearing loss. This gene (DFNA11)was localized within the region of chromosome 11q which contains the secondgene for a recessive form of non-syndromic sensorineural hearing loss(DFNB2). Since it has been reported that another gene for dominantnon-syndromic hearing loss (DFNA3) has been mapped to the same region asthe first gene for recessive hearing loss (DFNB1), it is possible thatdifferent mutations in the DFNB2 gene may result in either dominant orrecessive hearing loss.