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
SHANON E. Friedreich's Ataxia-Reply. Arch Otolaryngol. 1981;107(10):651. doi:10.1001/archotol.1981.00790460063021
Artificial Intelligence Resource Center
Customize your JAMA Network experience by selecting one or more topics from the list below.