We present here two DIDMOAD syndrome cases (Diabetes Mellitus, Diabetes Ins
ipidus, Optic Atrophy, Deafness) in a Turkish family In the examination of
the propositus who had consanguineous parents, diabetes mellitus, diabetes
insipidus, optic atrophy, and deafness were observed in addition to myopia,
juvenile glaucoma, posterior polar cataract, and dilatation of the urinary
tract. Diabetes mellitus, diabetes inspidus, optic atrophy, deafness, myop
ia, and ventricular septal defect were observed in his elder brother. Juven
ile onset diabetes mellitus, congenital glaucoma, deafness, and heart disea
se were the other remarkable findings observed in relatives to this family.
Juvenile glaucoma, posterior polar cataract observed in our propositus and
myopia in both our DIDMOAD syndrome cases are the first ophthalmic manifes
tations described in the DIDMOAD syndrome.