LiSN-S Testing and Auditory Dysfunction in Friedreich's Ataxia

Key Takeaways

• Participants with Friedreich ataxia generally scored worse than controls on LiSN-S measures, although the reported score distributions still overlapped.
• LiSN-S measures tracked most clearly with FARS, and regression models showed GAA1 was significantly associated with all variables while disease duration showed weaker relationships.
• The authors concluded that LiSN-S reflects audiologic dysfunction in Friedreich ataxia, but cross-sectional data and incomplete group separation limited stronger conclusions.

Among 101 people with Friedreich ataxia and 38 healthy controls, the LiSN-S study showed poorer performance across all LiSN-S sub-scores in the FRDA group. Scores still overlapped between groups, but the overall pattern consistently favored controls. Across analyses, LiSN-S results aligned more closely with genetic severity than with disease duration in this cohort. These findings point to measurable auditory dysfunction on LiSN-S within this cross-sectional sample.

The study used a cross-sectional analysis from the FACOMS natural history cohort, and participants with Friedreich ataxia had biallelic FXN mutations. Investigators evaluated LiSN-S as a measure of speech-in-noise and spatial hearing by comparing affected participants with healthy controls. Auditory symptomatology was reported by 51% of participants with Friedreich ataxia, although symptom capture in FACOMS was incomplete.

Controls outperformed the Friedreich ataxia group on every LiSN-S sub-score, including speech reception thresholds and the derived advantage measures. Score distributions nevertheless overlapped, with effect sizes in the modest range of about 0.35 to 0.55. The largest abnormalities appeared in the spatially separated target speech and noise conditions, DV90 and SV90. Participants with clinical auditory complaints also had numerically worse LiSN-S results, but the difference was only borderline significant, making the findings measurable rather than individually definitive.

LiSN-S correlations were moderate with FARS, ranging from roughly 0.5 to 0.7 across the test measures. Associations were lower for GAA1 repeat length and age at onset, at about 0.32 to 0.54, and weaker overall for disease duration, with significant correlations generally around 0.25 to 0.29. In linear regression, GAA1 was associated with all LiSN-S variables, while age was associated with several variables, including spatial advantage and total advantage, but not DV0 or talker advantage. Stratified analyses also suggested greater abnormality above the cohort mean GAA1 length, whereas age alone showed less clear separation. Overall, these association patterns favored genetic severity more clearly than time-related measures within this cross-sectional cohort.

The authors concluded that LiSN-S captures audiologic dysfunction in Friedreich ataxia and appears to depend more on genetic severity than on time-related measures. They also noted that the cross-sectional design does not establish progression over time for individual patients. Because scores overlapped between groups, LiSN-S did not cleanly distinguish every affected participant from a control individual. Auditory dysfunction was also not fully captured by symptom report alone, and longitudinal data may further clarify the test's role.