The study of the progressive familial deafness was rather not easy because of difficult evaluation for family history. The authors recently experienced a case of progressive familial deafness in through 3 generations. Audiograms showed decending forms and total deafness, but grandfather could not be studied because of dead but his family mentioned he had severe hearing loss. These cases were progressive bilateral perceptive hearing loss and hereditary disease as a Mendelian dominant character. The onset of deafness began at childhood. So we present this report and discussed with some literatures.