DR. SWETHA SUBHARAJ

DR.SANDHYA DHARWADKAR

Abstract

OBJECTIVE: To report a rare case of Wolfram syndrome(WS) also known as DIDMOAD syndrome characterized by diabetes insipidus(DI), diabetes mellitus(DM),progressive optic atrophy(OA),and deafness(D)

CASE REPORT: A 14-year old male patient, diagnosed with type I DM since 4 years, presented to paediatric OPD with complains of urinary retention and high blood sugars.He also complained of bilateral painless progressive vision loss since 8months.Fundus examination showed bilateral primary optic atrophy with moderate NPDR changes. Radiological and blood investigations were done which confirmed DIDMOAD syndrome.

CONCLUSION:DIDMOAD syndrome needs to be evaluated in a multidisciplinary manner.In patients with concomitant diabetes mellitus and diabetes insipidus,strong suspicion and careful ophthalmological and auditory evaluation can reveal this condition.Thus, awareness and timely diagnosis is important for improving patient prognosis and enabling timely genetic counseling for family members.