Table 1 Clinical characteristics and age at onset of symptoms (years) for Wolfram syndrome patients (n = 40)
Patient number | Sex | Age at enrollment | Siblings | Diabetes mellitus | Diabetes insipidus | Hearing loss | Optic nerve atrophy | Number of analyzed sessions | Years between first and last session |
|---|---|---|---|---|---|---|---|---|---|
WFS_01 | M | 12.9 |  | 3.5 | 9.0 | 9.0 | 5.0 | 1 | – |
WFS_02 | F | 10.9 | Â | 6.2 | 9.7 | Normal | 9.5 | 6 | 4.95 |
WFS_03 | M | 17.9 | Â | 4.6 | 6.0 | 6.0 | 6.0 | 6 | 4.95 |
WFS_04 | F | 23.7 | Â | 2.2 | 12.3 | 5.0 | 12.3 | 6 | 4.94 |
WFS_05 | F | 13.7 |  | 3.9 | No dx | 1.7 | 12.6 | Cochlear implant | – |
WFS_07 | M | 7.3 | Â | 2.7 | 7.3 | 11.2 | 7.0 | 5 | 3.95 |
WFS_09 | M | 14.4 | WFS_10, 11 | 10.8 | 14.7 | Normal | 10.9 | 3 | 2.93 |
WFS_10 | F | 11.6 | WFS_9, 11 | 7.0 | 11.0 | Normal | 8.0 | 5 | 3.95 |
WFS_11 | M | 8.3 | WFS_9, 10 | 7.8 | 10.0 | Normal | 6.0 | 3 | 3.93 |
WFS_12 | M | 22.9 | Â | 6.3 | 17.0 | 7.0 | 17.0 | 6 | 4.95 |
WFS_13 | F | 5.4 | Â | 4.7 | 7.0 | 7.4 | 5.2 | 6 | 4.94 |
WFS_14 | F | 12.6 | Â | 6.2 | 11.2 | 9.1 | 7.9 | 4 | 3.93 |
WFS_15 | F | 10.9 | Â | 2.7 | 12.0 | 9.0 | 7.0 | 4 | 2.98 |
WFS_16 | F | 25.8 | Â | 13.0 | 14.5 | 25.8 | 13.0 | 5 | 3.93 |
WFS_17 | F | 17.1 | WFS_29 | 4.9 | Not Diagnosed | 16.3 | 15.3 | 5 | 3.93 |
WFS_18 | M | 12.0 | Â | 5.1 | 10.3 | 11.9 | 10.1 | 4 | 2.97 |
WFS_19 | F | 11.9 |  | Not Diagnosed | Not Diagnosed | 3.0 | 5.7 | Cochlear implant | – |
WFS_22 | M | 15.7 | Â | 13.9 | Not Diagnosed | Normal | 13.0 | 4 | 2.97 |
WFS_23 | F | 17.7 | WFS_24, 25 | 5.0 | Not Diagnosed | 10.0 | 17.0 | 3 | 2.00 |
WFS_24 | F | 16.3 | WFS_23, 25 | 4.0 | 5.5 | 8.5 | 14.0 | 3 | 2.00 |
WFS_25 | F | 7.9 | WFS_23, 24 | 4.0 | Not Diagnosed | 7.9 | 7.0 | 2 | 2.00 |
WFS_27 | M | 10.1 | Â | 3.8 | 8.9 | 10.1 | 8.0 | 3 | 2.00 |
WFS_28 | M | 6.9 | Â | 3.8 | Not Diagnosed | Normal | 3.8 | 3 | 1.99 |
WFS_29 | F | 5.1 | WFS_17 | Not Diagnosed | Not Diagnosed | 3.0 | Not Diagnosed | 2 | 1.00 |
WFS_30 | F | 22.7 | Â | 6.9 | 22.4 | Normal | 19.6 | 2 | 1.00 |
WFS_31 | F | 10.8 | Â | 4.5 | Not Diagnosed | 8.8 | 7.0 | 2 | 1.00 |
WFS_32 | F | 7.6 |  | Not Diagnosed | Not Diagnosed | 0.4 | 6.4 | Cochlear implant | – |
WFS_33 | M | 6.3 | Â | 4.8 | 6.2 | 6.3 | 6.0 | 2 | 0.66 |
WFS_34 | F | 14.0 | WFS_35 | 3.5 | 13.0 | 3.0 | Not Diagnosed | 1 | – |
WFS_35 | F | 17.1 | WFS_34 | 3.5 | 6.2 | 6.0 | 13.0 | 2 | 0.60 |
WFS_36 | F | 11.1 |  | 7.0 | 10.1 | 6.1 | 10.0 | 1 | – |
WFS_37 | F | 21.8 |  | 6.0 | 17.0 | 16.0 | 18.0 | 1 | – |
WFS_38 | M | 12.9 |  | Not Diagnosed | Not Diagnosed | 2.8 | 10.9 | Cochlear implant | – |
WFS_39 | M | 13.0 |  | 3.0 | Not Diagnosed | 13.0 | 9.0 | 1 | – |
WFS_40 | F | 17.1 |  | 13.0 | 13.5 | Normal | 8.0 | 1 | – |
WFS_42 | M | 8.3 |  | 5.0 | No dx | 6.4 | 7.4 | 1 | – |
WFS_43 | M | 21.1 |  | 5.0 | 16.0 | Normal | 14.0 | 1 | – |
WFS_44 | M | 10.9 |  | 5.0 | Not Diagnosed | 7.0 | 9.0 | 1 | – |
WFS_45 | M | 6.0 |  | 4.0 | Not Diagnosed | Normal | Not Diagnosed | 1 | – |
WFS_46 | M | 21.2 |  | 5.0 | Not Diagnosed | 12.0 | 11.0 | 1 | – |
Summary statistics: # or mean (SD) | 18 Males | 13.5 (5.6) | 4 Sibships | 5.6 (2.9) | 11.3 (4.2) | 8.3 (5.1) | 10.0 (4.0) | 4 with cochlear implants | Â |