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<p>Auditory dysfunction is a clinically significant component of Friedreich ataxia (FRDA), but its features are not readily captured in standard audiology. We examined the Listening in Spatialized Noise–Sentences (LiSN-S) test as a measure of auditory function in a large cohort of subjects with this disease. Methods: We examined 101 individuals with FRDA with the LiSN -S, compared the results with 38 controls, and correlated results with disease features. Results. Control participants scored better than individuals with FRDA on all of the LiSN-S sub-scores. Correlations of LiSN-S scores with the Friedreich Ataxia Rating Scale (FARS) exams were moderate (0.5–0.7), while correlations were lower with genetic markers such as GAA1 and age of onset (0.32–0.54). Correlations were lower still with disease duration (0.25–0.29). Linear regressions to define the independent effects of age and GAA1 demonstrated that GAA1 uniformly predicted LiSN-S advantage scores (p &lt; 0.01) but age predicted spatial advantage and total advantage ( P &lt; 0.01) but not talker advantage. LiSN-S scores were slightly more affected in individuals with clinical auditory complaints. Conclusions: LiSN -S testing captures audiologic dysfunction in FRDA in a manner that appears to dependent on genetic severity and to a lesser degree time.</p>

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Listening in Spatialized Noise–Sentences (LiSN-S) as a Measure of Auditory Function in Friedreich Ataxia

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年4月24日

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  • Shahd Al Haj Ali,
  • Jessica Early,
  • Jennifer M. Farmer,
  • Sarah Gelbard,
  • Louise Corben,
  • Gary Rance,
  • David R. Lynch

摘要

Auditory dysfunction is a clinically significant component of Friedreich ataxia (FRDA), but its features are not readily captured in standard audiology. We examined the Listening in Spatialized Noise–Sentences (LiSN-S) test as a measure of auditory function in a large cohort of subjects with this disease. Methods: We examined 101 individuals with FRDA with the LiSN -S, compared the results with 38 controls, and correlated results with disease features. Results. Control participants scored better than individuals with FRDA on all of the LiSN-S sub-scores. Correlations of LiSN-S scores with the Friedreich Ataxia Rating Scale (FARS) exams were moderate (0.5–0.7), while correlations were lower with genetic markers such as GAA1 and age of onset (0.32–0.54). Correlations were lower still with disease duration (0.25–0.29). Linear regressions to define the independent effects of age and GAA1 demonstrated that GAA1 uniformly predicted LiSN-S advantage scores (p < 0.01) but age predicted spatial advantage and total advantage ( P < 0.01) but not talker advantage. LiSN-S scores were slightly more affected in individuals with clinical auditory complaints. Conclusions: LiSN -S testing captures audiologic dysfunction in FRDA in a manner that appears to dependent on genetic severity and to a lesser degree time.