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Behavioural and subjective benefits of hearing aids in an adult with ANSD
What is it about?
Hearing loss is commonly managed with hearing aids; however, people with hearing loss due to auditory neuropathy spectrum disorder (ANSD) have mixed outcomes and are often not recommended for this patient group. In a patient with ANSD due to Friedreich ataxia, a multisystem neurodegenerative disease, we show that hearing aids (with a remote microphone) can provide meaningful benefit by improving the audibility of soft sounds and reducing listening effort and fatigue.
Why is it important?
This case illustrates the importance of expanding audiological care to include rare neurogenetic conditions, such as Friedreich ataxia. It demonstrates how amplification, even in the context of ANSD, can lead to meaningful improvements in quality of life and overall well-being. Clinicians should consider hearing aid trials in populations with complex needs, supported by both behavioural (speech discrimination testing) and subjective (hearing-related questionnaires) outcome measures to capture the full spectrum of benefit.