Table 1:
Clinical and imaging characteristics of WSS
| Family | Sex | Age (Yr) | Neurologic |
Radiologic |
||||
|---|---|---|---|---|---|---|---|---|
| Dystonia | SNHL | Seizure | WM Changes | ID | Pituitary | |||
| 1 | F | 18 | Focal | − | − | Mild | − | Small |
| M | 19 | − | − | + | Absent | + | Small | |
| 2 | F | 29 | Generalized | − | − | Severe | + | Normal |
| F | 38 | Focal | + | − | Moderate | + | Small | |
| F | 35 | Focal | + | − | Mild | + | Small | |
| 3 | F | 37 | Focal | − | − | Severe | + | Small |
| M | 45 | Generalized | + | − | Severe | + | Small | |
| 4 | M | 22 | Focal | − | − | Mild | + | Normal |
| M | 24 | Generalized | − | − | Mild | + | Normal | |
| 5 | M | 23 | − | − | − | Mild | − | Small |
| F | 21 | − | − | − | Mild | + | Small | |
| M | 16 | − | − | − | Mild | − | Small | |
| M | 26 | Generalized | + | − | Mild | + | Normal | |
| M | 21 | − | + | + | Absent | + | Normal | |
| 6 | M | 20 | Generalized | − | − | Mild | − | Small |
| F | 21 | − | − | − | Absent | + | Small | |
| 7 | F | 32 | Multisegmental | − | − | Mild | + | Small |
| F | 22 | Focal | − | − | Absent | − | Small | |
| F | 35 | Focal | − | + | Severe | + | Small | |
| M | 21 | Multisegmental | + | − | Absent | + | Small | |
| F | 27 | Focal | + | − | Absent | + | Small | |
| 8 | M | 16 | − | − | − | Absent | − | Small |
| 9 | F | 41 | Generalized | − | − | Moderate | + | Small |
| 10 | M | 37 | Multisegmental | + | − | Moderate | + | Small |
| 11 | F | 17 | Multisegmental | − | − | Absent | + | Small |
| 12 | F | 29 | − | − | − | Mild | − | Normal |
Note:—SNHL indicates sensorineural hearing loss; ID, iron deposition; +, present; −, absent.