Postural Instability in Subjects With Usher Syndrome

This study investigated postural performances and vestibular impairment in Usher patients. The three groups studied were: 11 patients with Usher type I (with visual and vestibular impairment), 14 patients with Usher type II (with only visual impairment), and 14 healthy control subjects. Postural stability was measured with a Framiral Multitest Equilibre platform with three visual conditions: eyes open (EO), eyes closed (EC), and vision disturbed by optokinetic stimulation (OPT), and two different postural conditions: stable or unstable platform. The surface and mean velocity of the center of pressure displacement (CoP) were measured and a postural instability index (PII) was calculated. Usher type I and II patients were more unstable than control subjects, but only for the unstable platform. Patients with Usher type I (with severe vestibular impairment) were also significantly more unstable than patients with Usher type II (with normal vestibular function) on the unstable platform. The severity of the vestibular impairment was correlated with the surface of the CoP displacement. We suggest that poor postural control of Usher patients is due to the abnormalities in their visual and, when defective, vestibular inputs. Measurements of postural stability on an unstable platform can distinguish type I from type II Usher patients. We emphasize the importance of multisensory evaluation in these patients to guide development of personalized visuo-vestibular rehabilitation techniques to improve their postural stability and improve their quality of life.