WOLFRAM (DIDMOAD) SYNDROME AND ITS UROLOGICAL MANIFESTATION

We report 2 patients with DIDMOAD syndrome (diabetes insipidus, diabetes mellitus, optic atrophy and neural deafness), with emphasis on the urological aspects and their management. Both patients underwent thorough radiological endoscopic and urodynamic evaluation, in addition to detailed evaluation of other systems involved. Each had the characteristic hyper-reflexive neurogenic bladder with sphincteric dyssynergia, which resulted in severe urinary tract dilation. One patient was diagnosed at this institution and managed conservatively with clean intermittent catheterisation and anticholinergic medication; the second patient was referred to us after several attempts at surgical correction. The presentation, details of the urological evaluation with special emphasis on the urodynamic findings, and the outcome of different means of management are discussed.