Abstract: Objective： To analyze and characterize the phenotype and genetics of Chinese kindred with mid?-frequency hearing loss. Methods： Pedigree was drawn after investigation. Eight family members were checked up, and detailed audiological examination were preformed,（including pure tone assay, acoustic immittance measurement）. Results： The proband of the kindred had been diagnosed with senserineural hearing loss. The pattern of the family was autosomal dominant based on the investigated information. Common phenotypie features shared among affected family members included bilateral involvement and postlingual, moderate to severe sensorineural hearing loss affecting mainly mid frequencies． Conclusions： Pedigree analysis suggests an autosomal dominant hereditary pattern in this family with predominantly mid frequency hearing loss. The information may be important for future molecular linkage studies and identification of genes contributing to autosomal dominant hearing loss．

Key words: Hearing loss, sensorineural, Genetic diseases, inborn, Genetic screening, Chromosome aberrations, Phenotype, Pedigree