Objective：To investigate the clinical characters of a large family with non-syndromic hereditary hearing loss，and to find the mutational genes. Methods：Clinical and audiological examinations were performed to rule out syndromic hearing impairment，and the inheritance mode of the family was evaluated. The known deafness-associated genes were sequenced by targeted next-generation sequencing. Results：The family has 97 members in 6 generations，of whom 18 persons were affected. The mode of inheritance should be autosomal dominant according to the pedigree. Audiograms showed the Ⅲ4，Ⅳ20，Ⅲ2 and his offspring of this family were late-onset，progressive non-syndromic sensorineural hearing loss. The age of onset was about 28-year-old. V10 and V35 exhibit hearing impairment in high frequencies. Ⅲ4 and her children（V36，V37，V38，V39）showed non-progressive hearing impairment at 3 and 4 year-old. We did not find any known deafness-associated gene mutations by target sequence capture sequencing technology except mitochondrial A1555G mutation in Ⅲ4 and her children（V36，V37，V38，V39）. Conclusion：Pedigree analysis showed an autosomal dominant hereditary pattern in this family. Hearing loss was congenital，bilateral symmetric，and sensorineural. The known deafness genes seem not contribute to the pathogenesis of the hearing loss in this family，suggesting new gene（s） involvement.