The ear in osteogenesis imperfecta

Abstract
A review of the pertinent historical, clinical, pathological and otologic features of osteogenesis imperfecta is presented. A study of the genetic aspects of the Van der Hoeve Syndrome supports the view that the disease results from one gene or gene complex with wide variations in expressivity. A case history and family pedigree as well as the treatment for the deafness are discussed.

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