Friedreich ataxia is one of the most common recessively inherited ataxias, with hearing loss due to auditory neuropathy being a recognized complication. However, there are limited data on the best interventions for hearing loss in these patients. We report the significant behavioral and subjective benefits of binaural amplification in a patient with auditory neuropathy due to Friedreich ataxia.
✦ The floor
Discussion
Signed responses from readers of the wire.
This single case suggests hearing aids may offer some benefit in Friedreich ataxia-related auditory neuropathy, but evidence is insufficient to change current fitting practice; clinicians should continue individualized assessment.
Auditory neuropathy in Friedreich ataxia is poorly characterized in the literature; even a case report adds to a sparse evidence base and highlights the need for prospective studies on amplification benefit in this population.
- 01Auditory neuropathy (nerve-based hearing disruption) is a recognized complication of Friedreich ataxia.
- 02This case report documents hearing aid outcomes in one patient with this rare condition.
- 03Evidence is limited to a single individual — findings cannot be generalized.
- 04Published in the Journal of Speech, Language, and Hearing Research (JSLHR).
- 05Highlights a gap in evidence for audiological management of Friedreich ataxia patients.
Hearing aids provide measurable benefit in at least one individual with auditory neuropathy secondary to Friedreich ataxia.
studypartially supported- PMID
- 42647417
- DOI
- 10.1044/2026_JSLHR-25-00671.
- Journal
- Journal of Speech, Language, and Hearing Research
- Publication type
- case_report
- Evidence level
- 4
- Sample size
- 1
- Population
- Single individual with auditory neuropathy secondary to Friedreich ataxia
- Intervention
- Hearing aid fitting
Primary outcomes
Hearing aid benefit in auditory neuropathy due to Friedreich ataxia