Optic atrophy and nerve deafness in diabetes mellitus.

The belief, once held, that an aetiological relationship exists between diabetes mellitus and optic atrophy is not now generally accepted. The observations of early authors such as Allbutt (1871) and von Noorden (1917), who regarded optic atrophy as a diabetic complication, may have been partially vitiated by the fact that both visual loss and changes in the optic discs may result from other ocular complications of diabetes and need not be an expression of primary nerve degeneration. Waite and Beetham (1935), in a comprehensive survey of the visual mechanism in diabetes, reported optic atrophy in 27 (0-69%) of the 3,915 fundi that they were able to examine in 2,002 diabetic patients, and in four (044 %) of the 901 visible fundi of 457 non-diabetic persons. These findings have, however, been repeatedly misinterpreted by subsequent authors (Wagener, 1938; Tunbridge and Paley, 1956) who, by overlooking the fact that the percentage figures related to individual fundi and not to patients, quote a higher incidence of optic atrophy in both groups than was actually recorded. It is not possible to derive from these data the percentage of patients in each group having optic atrophy in one or both eyes. On the basis of their findings Waite and Beetham (1935) considered the two conditions to be fortuitously associated: that no aetiological relationship exists between them is the view now generally accepted. The development of both optic atrophy and diabetes in two or more members of a family is thus of special interest. Wolfram (1938) reported a family ofeight children, four of whom, two boys and two girls, developed diabetes mellitus and bilateral optic atrophy by the age of 12. Tyrer (1943) described an 18-year-old boy who suffered from infantilism with goitre, diabetes mellitus, mental defect, and bilateral primary optic atrophy. One sibling suffered from diabetes and epilepsy and had pronounced bilateral primary optic atrophy; another, who was only 5 years old when examined, was also diabetic and epileptic and had pallor of the optic discs. A third family was reported by Tunbridge and Paley (1956) in which two members, brother and sister, were diabetic and had primary optic atrophy; they had, in addition, bilateral nerve deafness. We report here a fourth family in which the association of diabetes mellitus with optic atrophy and nerve deafness is again encountered but in which, unlike the other three, more than one generation is involved.