This family is of Hispanic ethnicity that was negative for mutations in GJB2, and we identified a mutation in CIB2. Mutations in CIB2, encoding a calcium and integrin binding protein 2 (OMIM# 605564), were previously reported in Pakistani and Turkish families with ARSNHL (DFNB48) as well as Usher syndrome type 1J [30]. The gene discussed is CIB2; the disease is Usher syndrome.