Abstract
Two cases of hearing disorders with progressive muscular atrophic diseases were reported. The case 1 is an 11-year-old boy with facio-scapulohumeral type of muscular dystrophy, whose audiogram showed the sensorineural type of bilateral deafness with gradual form of high tone loss. The case 2 is a 29-year-old female with congenital multiple arthrogryposis complaining of progressive hearing impairment of both ears. The audiogram showed sensori-neural deafness. Her hearing acuity became worse as the muscular atrophy proceeded. The hearing impairment of both cases might be due to the pathological change in the inner ear because they had recruitment phenomena and bone conduction disorders.
