Listening Effort and Its Relation to Spatial Localization, Vestibular and Visual Impairment in Usher Syndrome—Our Experience

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Background/Objectives: to analyse the listening effort in Usher Syndrome (USH) cases types 1 and 2 and its relation to age, genetic diagnosis, visual field, visual acuity, degree of HL, vestibular impairment and spatial orientation. Methods: it was a retrospective monocentric study. Twenty children with genetically confirmed USH (USH2 in 15/20 - 75% and USH1 in 5/20 - 25%) - age range 3-17 years (mean 9.6 ± 4.7) -underwent: Van-derbilt fatigue scale questionnaire (VFS), audiological and vestibular assessment comprehensive of Oldenburg Matrix test in Italian and video head impulse test (VHIT), sound localization test and ophthalmologic exami-nation. Results: we confirmed greater HL and worse vestibular function in USH1. They also employed signif-icant more time and head movements to localize sounds compared to USH2 and had worst visual field on eye examination. VFS was not significantly different between the two groups, except for the physical fatigue part referred by parents. Mean VFS was linearly related to age, the hearing threshold of the worse ear, data logging hours of hearing device, time and head movements of the localization test, VHIT asymmetry and balance problems referred by parents and visual field. Conclusions: listening effort in difficult environments such as school rooms in USH patients is not only associated to the hearing function but also to the spatial awareness determined in part by vestibular and visual function. Teachers should be informed and made aware of multi-ple comorbidities in order to facilitate learning.

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