Urinary Excretion of Amino Acids and Hexosamine in Otosclerotic Patients

Abstract
THE SYNDROME consisting of otosclerosis, blue sclerae, and fragility of bones (osteogenesis imperfecta) was described by Adair-Dighton1 in 1912, and abnormal amino-aciduria has been detected in five patients with osteogenesis imperfecta by Chowers et al.2 Arslan and Ricci3 and others have postulated that otosclerosis is a regional mesenchymopathy related to other connective tissue disorders. Among these diseases Kulonen and Kulonen4 found increased urinary elimination of some amino acids in rheumatoid arthritis. Prockop and Sjoerdsma5 observed increased urinary excretion of bound hydroxyproline in some cases of arachnodactylia (Marfan's syndrome), in which ill-defined defects in the connective tissue have been noted. We have therefore found it worthwhile to measure the amino-acid excretion in patients suffering from otosclerosis. Material and Methods Five patients with proven otosclerosis and three control subjects (nonotosclerotic patients) were studied. Case 1.—This patient was a 44-year-old man with a history of progressive loss

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