BLUE SCLERAS, BRITTLE BONES AND DEAFNESS

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Abstract
For centuries physicians have witnessed the tragic instance of a child born with multiple fractures of the bones. Lobstein,1in 1833, designated this condition as Ostepsathyrosis, a descriptive term derived from Greek, meaning friable bones. In 1849, Vrolik reported a similar condition, which he named osteogenesis imperfecta, implying defective formation of bone as the cause. Spurway,2in 1896, reported the first instance in which the condition of fragile bones (fragilitas ossium) was associated with blue scleras. Bronson,3in 1917, and van der Hoeve and de Kleyn,4in 1918, added to the syndrome the third feature, deafness. Key,5in 1926, referred to the syndrome as "hereditary hypoplasia of the mesenchyme" and called attention to the hypotonicity of the ligaments with hypermobile joints. In 1928 Bell6collected 75 pedigrees including 489 cases of this abnormality. The condition is not so rare as is generally thought. Recent literature7has shown renewed

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