Verpy et al. (2001) identified mutations in a protein called stereocilin, encoded by a gene symbolized STRC (606440). Using a candidate deafness gene approach, they reported a transcript that is expressed almost exclusively in the inner ear. Genomic ... Verpy et al. (2001) identified mutations in a protein called stereocilin, encoded by a gene symbolized STRC (606440). Using a candidate deafness gene approach, they reported a transcript that is expressed almost exclusively in the inner ear. Genomic analysis showed that STRC is located on chromosome 15q15. It contains 29 exons encompassing approximately 19 kb. STRC is tandemly duplicated, with the coding sequence of the second copy interrupted by a stop codon in exon 20. In 2 families affected by autosomal recessive nonsyndromal sensorineural deafness linked to the DFNB16 locus on 15q, Verpy et al. (2001) found 2 frameshift mutations and a large deletion in the copy of the gene containing 29 coding exons. In one of the consanguineous deaf families linked to 15q22 reported by Campbell et al. (1997), Verpy et al. (2001) failed to find a mutation in the STRC gene. The deafness in these families was of a prelingual profound type and may be due to mutation in a second deafness gene in the 15q21.1 region.