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.
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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.
Perspectives
This study is a great demonstration of how behavioural results should not be the only measure of hearing aid benefit. Our patient experienced reduced fatigue and reported additional hearing benefits that extended beyond speech clarity. We highlight the importance of subjective validation in people with complex needs, including open-ended, patient-driven comments.
I. Zay Melville
University of Auckland
Read the Original
This page is a summary of: Hearing Aid Benefit in Auditory Neuropathy Due to Friedreich Ataxia: A Case Report, Journal of Speech Language and Hearing Research, August 2026, American Speech-Language-Hearing Association (ASHA),
DOI: 10.1044/2026_jslhr-25-00671.
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