Successful treatment of auditory perceptual disorder in individuals with Friedreich ataxia

Gary Rance, L. A. Corben, E. Du Bourg, A. King, M. B. Delatycki

Research output: Contribution to journalArticleResearchpeer-review

42 Citations (Scopus)


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 discrimination than their matched control. Speech perception in the presence of background noise was also impaired, with FRDA listeners typically able to access only around 50% of the information available to their normal peers. The use of personal FM-listening devices did however, dramatically improve their ability to hear and communicate in everyday listening situations.

Original languageEnglish
Pages (from-to)552-555
Number of pages4
Issue number2
Publication statusPublished - 1 Dec 2010
Externally publishedYes


  • Auditory neuropathy
  • Auditory processing
  • Friedreich ataxia
  • Speech perception

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