Mutations of BSND Result in Nonsyndromic and Syndromic Hearing Loss in Family PKDF815
(A) Affected individuals V:15-17, V:19, and VI:2-5 have distal and proximal breakpoints at D1S2661 and D1S475, respectively, defining the smallest linkage interval to ∼4 cM. Individuals VI:15-17 are segregating prelingual profound deafness along with elevated renin and nephrocalcinosis (Table 1).
(B) Electropherograms of amplimers from genomic DNA templates illustrate homozygosity for the c.35T>C mutant allele among affected family members segregating isolated hearing loss. Affected family members segregating deafness along with nephrocalcinosis and elevated renin are compound heterozygous for c.35T>C and c.10G>T.