Table 2

Symptoms of Wolfram syndrome: age at onset, prevalence, and symptom-free survival with regard to glucose control in WSD

Optic atrophyDiabetes insipidusDeafnessBladder dysfunctionNeurologic problemsPsychologic problems
Mean (range)Mean (range)Mean (range)Mean (range)Mean (range)Mean (range)
Age in years at symptom onset
 All patients9 (2–18)13 (5–22)10 (1–20)15 (1–22)11 (1–22)14 (8–20)
 Patients with a mean HbA1c ≤7.5%9 (4–18)13.5 (5–18)6 (1–18)16 (1–22)10 (1–22)14.5 (14–15)
 Patients with a mean HbA1c >7.5%9 (2–17)11 (5.5–22)11 (1–18)13.5 (7–18)11 (4–19)14 (8–20)
%%%%%%
Prevalence of symptoms
 All patients925268486234
 Patients with a mean HbA1c ≤7.5%94.731.65847.442.110.5
 Patients with a mean HbA1c >7.5%89.264.375507553.6
Median (95% CI)Median (95% CI)Median (95% CI)Median (95% CI)Median (95% CI)Median (95% CI)
Symptom-free survival in years after WSD diagnosis*
 All patients4.7 (3.2–6.2)13 (9.7–16.3)9.8 (7.4–12.2)14.0 (12.1–15.9)9.9 (6.4–13.4)14 (10.2–17.8)
 Patients with a mean HbA1c ≤7.5%2.2 (0.8–3.6)16.7 (8.21–25.2)9.8 (0.8–18.8)11.9 (9.4–14.4)20.7 (—)
 Patients with a mean HbA1c >7.5%5.2 (4.6–5.8)13 (6.6–20.4)8.9 (6.3–11.5)14 (11.8–16.2)8 (4.7–11.8)12.3 (10.7–13.8)
PPPPPP
Significance of differences
 Each individual symptom0.3490.1150.8650.8410.0890.033
 All symptoms0.0310.0310.0310.0310.0310.031
  • *Cox regression.

  • †The symptom-free survival of all neurodegenerative symptoms pooled in Wolfram syndrome is significantly different (P = 0.031) in patients with a mean HbA1c higher than 7.5%, compared with patients with a mean HbA1c lower than or equal to 7.5%.