MYO7A gene encodes an unconventional myosin, which is essential for well functioned hair cells of inner ear.Mutations in MYO7A have been associated with nonsyndromic hearing loss.Here we report clinical and genetic characterization of a Chinese pedigree carrying compound heterozygous mutations.MYO7A is considered to be responsible for the WLT151 pedigree by exome capture sequencing and pathogenicity prediction.The proband has less severe hearing loss in low frequency without defects in vestibular or retinal, though her parents have normal hearing.