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Table 1:

Clinical and imaging characteristics of WSS

FamilySexAge (Yr)NeurologicRadiologic
DystoniaSNHLSeizureWM ChangesIDPituitary
1F18FocalMildSmall
M19+Absent+Small
2F29GeneralizedSevere+Normal
F38Focal+Moderate+Small
F35Focal+Mild+Small
3F37FocalSevere+Small
M45Generalized+Severe+Small
4M22FocalMild+Normal
M24GeneralizedMild+Normal
5M23MildSmall
F21Mild+Small
M16MildSmall
M26Generalized+Mild+Normal
M21++Absent+Normal
6M20GeneralizedMildSmall
F21Absent+Small
7F32MultisegmentalMild+Small
F22FocalAbsentSmall
F35Focal+Severe+Small
M21Multisegmental+Absent+Small
F27Focal+Absent+Small
8M16AbsentSmall
9F41GeneralizedModerate+Small
10M37Multisegmental+Moderate+Small
11F17MultisegmentalAbsent+Small
12F29MildNormal
  • Note:—SNHL indicates sensorineural hearing loss; ID, iron deposition; +, present; −, absent.