Sensory substitution in bilateral vestibular a-reflexic patients

Patients with bilateral vestibular loss have balance problems in darkness, but maintain spatial orientation rather effectively in the light. It has been suggested that these patients compensate for vestibular cues by relying on extravestibular signals, including visual and somatosensory cues, and integrating them with internal beliefs. How this integration comes about is unknown, but recent literature suggests the healthy brain remaps the various signals into a task-dependent reference frame, thereby weighting them according to their reliability. In this paper, we examined this account in six patients with bilateral vestibular a-reflexia, and compared them to six age-matched healthy controls. Subjects had to report the orientation of their body relative to a reference orientation or the orientation of a flashed luminous line relative to the gravitational vertical, by means of a two-alternative-forced-choice response. We tested both groups psychometrically in upright position (0°) and 90° sideways roll tilt. Perception of body tilt was unbiased in both patients and controls. Response variability, which was larger for 90° tilt, did not differ between groups, indicating that body somatosensory cues have tilt-dependent uncertainty. Perception of the visual vertical was unbiased when upright, but showed systematic undercompensation at 90° tilt. Variability, which was larger for 90° tilt than upright, did not differ between patients and controls. Our results suggest that extravestibular signals substitute for vestibular input in patients' perception of spatial orientation. This is in line with the current status of rehabilitation programs in acute vestibular patients, targeting at recognizing body somatosensory signals as a reliable replacement for vestibular loss.