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October 1981

Friedreich's Ataxia-Reply

Author Affiliations

Tel-Aviv, Israel

Arch Otolaryngol. 1981;107(10):651. doi:10.1001/archotol.1981.00790460063021

In Reply.—Our study of a family with Friedreich's ataxia was completed in the early months of 1980, and at that time we were not aware of an additional work on this subject.

Dr Satya-Murti and colleagues stressed the fact that they did not obtain any recognizable waves in their patients,2 whereas we observed a consistent loss of N1 responses early in the disease, a loss of N3 later in the course of the disease, and a preservation of the more rostrally evoked auditory brainstem potentials.

The audiometric curve observed by Satya-Murti and colleagues did not show a specific pattern. In patient 4, for example, a high-frequency hearing loss was noted similar to that recorded in our patients. However, patient 1 had a dome-shaped audiometric curve analogous to that described by Spoendlin.2

The idea raised by Satya-Murti and colleagues that the pathologic condition of the spiral

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