Successful treatment of auditory perceptual disorder in individuals with Friedreich ataxia

Abstract

Friedreich ataxia (FRDA) is a neurodegenerative disease affecting motor and sensory systems. This study aimed to investigate the presence and perceptual consequences of auditory neuropathy (AN) in affected individuals and examine the use of personal-FM systems to ameliorate the resulting communication difficulties. Ten individuals with FRDA underwent a battery of auditory function tests and their results were compared with a cohort of matched controls. Friedreich ataxia subjects were then fit with personal FM-listening devices and evaluated over a 6 week period. Basic auditory processing was affected with each FRDA individual showing poorer temporal processing and figure/ground discriminatio..