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Case Reports
. 2010 Jan;5(1):53-6.

A case of wolfram syndrome

Gholamali Naderian  1 , Fereshteh Ashtari, Kia Nouri-Mahdavi, Valleh Sajjadi
Affiliations
Case Reports

A case of wolfram syndrome

Gholamali Naderian et al. J Ophthalmic Vis Res. 2010 Jan.

Abstract

Purpose: To report a case of Wolfram syndrome characterized by early onset diabetes mellitus and progressive optic atrophy.

Case report: A 20-year-old male patient with diabetes mellitus type I presented with best corrected visual acuity of 1/10 in both eyes with correction of -0.25+1.50@55 and -0.25+1.50@131 in his right and left eyes, respectively. Bilateral optic atrophy was evident on fundus examination. The patient also had diabetes insipidus, neurosensory deafness, neurogenic bladder, polyuria and extra-residual voiding indicating atony of the urinary tract, combined with delayed sexual maturity.

Conclusion: One should consider Wolfram syndrome in patients with juvenile onset diabetes mellitus and hearing loss. Ophthalmological examination may disclose optic atrophy; urologic examinations are vital in such patients.

Keywords: Deafness; Diabetes Insipidus; Diabetes Mellitus; Optic Atrophy.

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Figures

Figure 1
Figure 1
Optic atrophy in the right eye.
Figure 2
Figure 2
Optic atrophy in the left eye.
Figure 3
Figure 3
Bladder sonography is consistent with a neuorogenic bladder.
See this image and copyright information in PMC

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