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Abstract

Highlights

• The estimation of verticality (assessed with Subjective visual vertical (SVV)) is more variable in patients with Usher (type I and II) compared to healthy participants.

• Visual and vestibular information are essential for the visual vertical (VV) perception.

• A reweighting of sensory information from the central nervous system seems to be able to compensate for the absence of vestibular function in patients with Usher type I.

BACKGROUND:

Verticality, or more precisely the ability to perceive spatial orientation with regard to gravity, is based on the integration of visual, vestibular and somesthetic information.

OBJECTIVE:

The purpose of the present study was to compare the subjective visual vertical (SVV) in patients with Usher (type I and type II) with visual or vestibular impairment, and in healthy participants, in order to explore the importance of the visual and vestibular functions on the vertical’s perception.

METHODS:

We evaluated the SVV using a wall housing which projects on the opposite wall a red-light line of about 2 meters, obtained by laser cannon. The evaluation was carried out under two tilt conditions: clockwise and counter-clockwise randomly performed five times in each direction. The response to the SVV task was quantified by the mean of the absolute values of the SVV.

RESULTS:

Responses to the SVV were significantly less accurate in patients with Usher with respect to healthy participants while it was similar for the two groups of patients with Usher.

CONCLUSIONS:

We hypothesize that visual inputs play a very important role in the perception of verticality and that the symmetrical bilateral vestibular deficit in Usher type I does not have a strong impact in perception of verticality.

Keywords

Subjective visual vertical Usher syndrome visuo-vestibular inputs somes- thesia perception of space verticality

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Subjective visual vertical in patients with Usher syndrome