WOLFRAM (DIDMOAD) SYNDROME AND ITS UROLOGICAL MANIFESTATION

We report 2 patients with DIDMOAD syndrome (diabetes insipidus, diabet es mellitus, optic atrophy and neural deafness), with emphasis on the urological aspects and their management. Both patients underwent thoro ugh radiological endoscopic and urodynamic evaluation, in addition to detailed evaluation of other systems involved. Each had the characteri stic hyper-reflexive neurogenic bladder with sphincteric dyssynergia, which resulted in severe urinary tract dilation. One patient was diagn osed at this institution and managed conservatively with clean intermi ttent catheterisation and anticholinergic medication; the second patie nt was referred to us after several attempts at surgical correction. T he presentation, details of the urological evaluation with special emp hasis on the urodynamic findings, and the outcome of different means o f management are discussed.