Hearing Aid Benefit in Auditory Neuropathy Due to Friedreich Ataxia: A Case Report

PURPOSE: 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. METHOD: Auditory function was assessed in a 44-year-old woman with genetically confirmed Friedreich ataxia using pure-tone audiometry, speech audiometry, and immittance testing, along with auditory evoked potentials. Auditory neuropathy spectrum disorder (ANSD) was diagnosed based on the presence of otoacoustic emissions or a cochlear microphonic, alongside an abnormal auditory brainstem response. Hearing aids were fitted bilaterally, and speech discrimination (in quiet and in noise) was assessed in unaided and aided conditions. Validated and patient-centered questionnaires assessed subjective hearing difficulties and fatigue before and after hearing aid fitting. RESULTS: Behavioral and electrophysiological testing confirmed ANSD. Aided testing showed improved speech detection and discrimination for low-intensity sounds in quiet, although benefit in noise was limited. The patient experienced reduced fatigue and reported additional unique hearing benefits that extended beyond speech discrimination. CONCLUSIONS: Hearing aids have the potential to improve hearing, hearing-related quality of life, and fatigue in patients with Friedreich ataxia and auditory neuropathy, with benefits extending beyond speech discrimination. A trial with an amplification device should be considered for all adults with Friedreich ataxia and hearing loss due to ANSD, verified using subjective and behavioral assessments. Use of open-ended, patient-centered validation tools is recommended to capture the full scope of benefit.