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Auditory dysfunction in Friedreich ataxia
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1980
Year
Audiometric TestsNeuropsychologyNeurophysiologyNeuroanatomyAuditory Neuropathy Spectrum DisorderAuditory DysfunctionNeurological DisorderBehavioral Audiometric TestsSpiral Ganglion NeuronsAuditory PhysiologyNeuroscienceNeurologyCentral Nervous SystemHuman HearingSpeech PerceptionMedicineNeuropathologyHealth Sciences
Friedreich ataxia patients did not report symptomatic hearing difficulties. Audiometric and brainstem auditory evoked potential tests were conducted on four Friedreich ataxia patients. Audiometric testing revealed eighth‑nerve dysfunction and abnormal brainstem evoked potentials due to spiral ganglion neuron degeneration, yet patients maintained functional hearing.
We performed behavioral audiometric tests and brainstem auditory evoked potentials in four patients with Friedreich ataxia. None of the patients had symptomatic hearing difficulties. Results of the audiometric tests pointed to a disorder of the eighth nerve. In none of the patients could we elicit normal-appearing waves of the brainstem auditory evoked potentials. These abnormalities could be attributed to degeneration of spiral ganglion neurons. Our patients had useful and functional hearing despite very abnormal brainstem auditory evoked potentials.