Table 1.

Comparison of our patient to WS and MIDD.

	Case patient	Wolfram syndrome	Maternally inherited diabetes mellitus and deafness
Onset of diabetes mellitus	Diagnosed at the age of 39 years with gestational diabetes mellitus	Present in 98% [11]. Average age of diagnosis 6 years [1, 5]	Average age of diagnosis 37 years [12]
Anti-GAD and anti-islet cell antibodies	Absent	Absent	Absent
Diabetic ketoacidosis at presentation	Absent	3% [2]	No data reported
Retinal disease	Absent	35% diabetic retinopathy after 15 years [13]	Macular pattern dystrophy [14]
Renal disorders	Absent	8% [13]	Focal segmental glomerulosclerosis with hyalinised glomeruli, myocyte necrosis in afferent arterioles, and small arteries [15]
Optic atrophy	Diagnosed at the age of 10 years	Present in 82% [11]. Average age of diagnosis 10-11 years [5, 11]	No macular retinal dystrophy more common [14]
Sensorineural hearing loss	Diagnosed at the age of 25 years	Present in 48% [11]. Average age of diagnosis 16 years [1, 5]	Present in 75% Diagnosed between 2 and 61 years, frequently precedes diagnosis of DM, mean age of onset of hearing loss 33.2 years [15]
Diabetes insipidus	Absent	Present in classical WS 38% [11]. Average age of diagnosis 14-15 years [1, 5]. Absent in WS (type 2) [16]	Not routinely screened for
Neurological manifestations	Present, symptomatic by the age of 25 years	Present in 53% [5]. Average age of diagnosis 15 years [5] cerebellar ataxia, peripheral neuropathy, and dementia	Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) [17]
Urological manifestations	Absent	Present in 19% [11]. Average age of diagnosis 12–20 years [1, 5]	Not routinely screened for
Gastrointestinal manifestations	Absent	Gastrointestinal dysmotility in 24% [1]. Severe gastrointestinal ulcer and bleeding in WS (type 2) [18]	Gastrointestinal dysmotility
Psychiatric manifestations	Absent	39% [5]	Depression, dementia, and psychosis [19]
Median age of death	n/a	39 years [1]	No reported data
Cause of death	n/a	Neurological complications [1, 2]	Lactic acidosis, renal failure [19]