Almost all Usher syndrome patients in this study had an advanced form of RP. In contrast, auditory function differed considerably between type I and type II. An impairment of the olfactory system could not be detected, and there was no correlation between parameters representing visual function, hearing ability, and olfactory sense.

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With the multifocal ERG disturbances of macular function due to oedema and secondary structural changes were detected in the presented cases and the extension of the central lesions was estimated.

In retinal disorders defects in multifocal ERG presented the similar pattern as scotomata in perimetry. The patient with visual field defects due to disturbances in the chiasma exhibited a normal ERG-topography. In patients with visual field defects multifocal ERG supported differentiation of the location of the lesion.