Hearing Aid Benefit in Auditory Neuropathy Due to Friedreich Ataxia: A Case Report.
I Zay Melville, Alice H Smith, Gavin B Coad et al.
✦ AI-curated · Sources linked
Case in brief
A 44-year-old woman with genetically confirmed Friedreich ataxia underwent assessment for hearing loss. Audiometry and electrophysiological testing supported auditory neuropathy spectrum disorder, with otoacoustic emissions or a cochlear microphonic alongside an abnormal auditory brainstem response. After bilateral hearing-aid fitting, aided testing showed better detection and discrimination of low-intensity sounds in quiet, but limited benefit in noise. She also reported less fatigue and hearing benefits beyond speech discrimination, as assessed with patient-centered questionnaires.
What made this case unusual
The reported benefit extended beyond speech performance: the patient described reduced fatigue and additional subjective hearing benefits, despite limited improvement in noise.
Diagnostic / clinical pearl
In auditory neuropathy associated with Friedreich ataxia, hearing-aid benefit may be evident for low-intensity speech in quiet but limited in noise; assess both behavioral outcomes and the patient’s broader experience.