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Auditory dysfunction in Friedreich ataxia

Result of spiral ganglion degeneration

Department of Neurology, Division of Otolaryngology, Albany Medical College, Albany, NY.

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.

Address correspondence and reprint requests to Dr. Satya-Murti, Department of Neurology, Neil Hellman Medical Research Building, Albany Medical College, Albany, NY 12208.

Accepted for publication January 8, 1980.

This work was supported by the Robert W. Graves Memorial Fund and a grant from the Muscular Dystrophy Association.

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