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Yazdani Qadikollaee P, Zamanfar D, Rostami Maskopaii S. A case report of Wolfram syndrome. Clin Exc 2017; 6 (1) :84-94
URL: http://ce.mazums.ac.ir/article-1-330-en.html

Wolfram syndrome is a rare neurodegenerative autosomal recessive disorder that usually appears with diabetes mellitus, diabetes insipidus, bilateral optic nerve atrophy and neurosensory deafness. But it has been reported incomplete forms of this syndrome and cases where has a variety of other symptoms, also.
The current report presents a 12-year-old male patient that was under treatment with the diagnosis of diabetes from 1.5 years ago. Patient was complained from decreased vision, brief hearing loss and polydipsia and polyuria that was observed macula and head of optic nerve atrophy on both sides in the examination of the retina. In addition, he had mild neurosensory hearing loss at high frequencies in the audiometry. Also, this patient had bilateral hydronephrosis due to posterior urethral valve (PUV), which despite surgery, the plaintiff was the process of urination and disposal of urine with urine catheter, daily. One of the sick sisters which was Suffering from diabetes died at age 15 years old, but there is no diabetes or vision or hearing problems in other family members.
In patients under 16 years old with non-autoimmune-insulin-dependent diabetes, the smallest neurological disorders should be considered to perform neurological examinations to early diagnose & control of complications of this syndrome.