Familial incidence of juvenile diabets mellitus, progressive optic atrophy, and neurogenic deafness.

Wolfram (1938) published a report from the Mayo Clinic concerning the incidence of diabetes mellitus and optic atrophy in four siblings. He commented that "an aetiological relationship between diabetes mellitus and simple progressive optic atrophy is by no means well established". During the ensuing years other families have been described, members ofwhich have shown a similar association between juvenile diabetes mellitus and progressive optic atrophy (Tyrer, I943; Stansbury, I948; Tunbridge and Paley, 1956; Walsh, 1957; Shaw and Duncan, 1958; Barjon, Labauge, Cazaban, and Fabre, I963; Rose, Fraser, Friedman, and Kohner, I966; Rorsman and Soderstrom, I967), suggesting that the relationship between the two is due to more than chance. Indeed, Rose and others (I966) and Rorsman and Soderstrom (I967) expressed the view that an autosomal recessive mode of inheritance was likely. In his original report, Wolfram (I 938) made no mention of deafness, but H. P. Wagner, in a personal communication to Tunbridge and Paley (1956), stated that "three of Wolfram's patients had subnormal hearing". Tyrer (I943) and Stansbury (1948) did not state whether their patients were deaf, but Tunbridge and Paley (1956), Shaw and Duncan (1958), Barjon and others (I963), and Rose and others (I966) reported bilateral nerve deafness in twelve out of fourteen cases. Until now only 29 cases in fourteen families have been recorded. The present report concerns another family, two members of which exhibit this uncommon syndrome.