Elsevier

Neuroscience

Volume 171, Issue 2, 1 December 2010, Pages 552-555
Neuroscience

Neurodegeneration, Neuroprotection, and Disease-Oriented Neuroscience
Research Paper
Successful treatment of auditory perceptual disorder in individuals with Friedreich ataxia

https://doi.org/10.1016/j.neuroscience.2010.09.013Get rights and content

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 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.

Section snippets

Experimental procedures

Ten subjects (four females) homozygous for GAA expansion of intron 1 of the FXN gene were recruited through the FRDA clinic at the Monash Medical Centre. As each subject was given the FM device at the end of the trial and as we had a limited number of systems, preference was given to school-aged children and adult subjects known to experience communication difficulties in background noise. Age at assessment ranged from 8 to 42 years and age at disease onset ranged from 5 to 20 years. See Table 1

Results

Detection of sinusoidal amplitude modulation was poorer in FRDA subjects than matched controls. Mean detection threshold for the FRDA group was −3.3±3.6 dB (68.4% of the maximum amplitude) and for the control group was −17.3±2.1 dB (13.6% of maximum) (Fig. 2). Paired T-testing showed a significant difference between matched subjects (T=13.1, P<0.001). AM detection thresholds for each of the FRDA subjects are shown in Table 1.

The masking effect of simultaneous noise was also greater in FRDA

Discussion

These data demonstrate the presence of auditory neuropathy and severe disruption of central auditory processing in individuals with FRDA. Each subject suffered an impaired ability to detect rapid signal changes suggesting a temporally distorted representation of auditory events (Zeng et al., 2005). Furthermore, they all showed a reduced ability to extract a tonal signal from within a background masking noise. Similar results have been reported previously for AN due to other forms of

Conclusion

Basic auditory processing was affected with each FRDA individual showing poorer temporal processing and figure/ground discrimination than their matched control. Functional hearing, particularly the perception of speech in 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

Acknowledgments

We would like to express our appreciation to PHONAK Pty Ltd who donated the FM-devices used in the study. Also, thanks to the Friedreich Ataxia Research Alliance (FARA) for their support to the project. The corresponding author was also supported by the Wagstaff Research Fellowship in Otolaryngology. Finally, special thanks to the participants who gave their time so willingly.

References (12)

  • V. Campuzano et al.

    Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion

    Science

    (1996)
  • M. Cossee et al.

    Friedreich's ataxia: point mutations and clinical presentations of compound heterozygotes

    Ann Neurol

    (1999)
  • D. Hawkins

    Comparisons of speech recognition in noise by mildly-to moderately-hearing-impaired children using hearing aids and FM systems

    J Speech Hear Disord

    (1984)
  • K.N. Johnstone et al.

    Multiple benefits of personal FM system use by children with auditory processing disorder (APD)

    Int J Audiol

    (2009)
  • G. Peterson et al.

    Revised CNC lists for auditory tests

    J Speech Hear Disord

    (1962)
  • G. Rance

    Auditory neuropathy/dys-synchrony and it's perceptual consequences

    Trends Amplif

    (2005)
There are more references available in the full text version of this article.

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