Why do patients with PSP fall? Evidence for abnormal otolith responses

Background: Patients with progressive supranuclear palsy (PSP) fall frequently, beginning early in the course of their disease. Abnormal vestibulospinal reflexes are suspected, but the angular vestibulo-ocular reflex, which is mediated by the labyrinthine semicircular canals, survives late into the course of the disease. Objective: To test the hypothesis that otolithic-mediated reflexes are abnormal in PSP. Methods: We tested otolith-ocular reflexes (the translational vestibulo-ocular reflex [tVOR]) during combined rotation-translation in nine patients with PSP and nine age-matched control subjects; subjects viewed far and near targets. We also tested click-induced otolith-spinal reflexes (vestibular-evoked myogenic potentials [VEMPs]) in 10 patients with PSP and 30 age-matched controls. Results: All patients with PSP had small tVOR responses during near viewing that were, on average, only 12% of those of control subjects (p = 0.001). Patients with PSP also showed a reduction of the amplitude of VEMPs compared to control subjects (median [range]: 54.3 [16.8 to 214] vs 149 [11.6 to 466], p = 0.001). Conclusions: Taken together, these results indicate that abnormal otolith-mediated reflexes may be at least partly responsible for frequent falls in progressive supranuclear palsy, and deserve further study.