[Juvenile diabetes mellitus with optic atrophy and labyrinthine deafness. An autosomal-recessive inherited syndrome].

A now 12 year old boy developed diabetes mellitus when he was 7 years old. From 9 years on he developed progressive optic atrophy and deafness. Type I diabetes mellitus, together with progressive optic atrophy and several other symptoms like deafness, diabetes insipidus and ectasy of urinary tract are known for more than 40 years as autosomal-recessively inherited conditions. Therefore, in case of diabetes mellitus combined with optic atrophy it is necessary to search for further symptoms, developing during the course of the disease. Genetic counselling to the families is mandatory.