Health-Related Quality of Life in Adults With Classical Infratentorial Superficial Siderosis: A Cross-sectional Study.

Background And Objectives: Infratentorial superficial siderosis (iSS) is a rare but disabling neurologic condition characterized by progressive hearing loss and balance and mobility problems. The functional decline in these neurologic domains with iSS progression is likely to adversely affect health-related quality of life (HRQoL). We studied the HRQoL of adults with iSS using 2 common generic HRQoL measures (Health Utilities Index Mark III [HUI3] and EuroQoL EQ5D [5 Level]) to determine the most affected domains and evaluate the association between HRQoL scores and disease duration.