Metaphyseal dysplasia demonstrated by clinical, audiological, and roentgenological studies, occurred in seven members of a large kindred. Serial audiograms, roentgenograms, and close clinical reexamination over a ten-year period were obtained and described. In contradistinction to previous reports, the disease in this family is progressive, inherited by an autosomal dominant mode, and manifests itself in all members by hearing loss first presenting to the otolaryngologist.