Auditory evoked potentials (early, middle, late components) and audiological tests in Friedreich's ataxia.

A functional assessment of the acoustic pathways in a group of 9 patients with Friedreich's ataxia was performed. In none of them was symptomatic auditory impairment noticeable. All patients were submitted to audiological tests and AEP recording. AEPs included early (BAEPs), middle (MLCs) and long (LLCs) latency components. The absence of one or more BAEP waves was observed in 5 out of 9 patients. N85 latency was significantly increased in all but one patient. MLCs, particularly P12, N15, P25 were often normal in amplitude and latency, scarcely showing a positive relationship with abnormalities of preceding and following waves. Pure tone audiometry revealed cochlear or neural impairment only in 2 patients. Speech audiometry showed deficits in all patients. These investigations, taken as a whole, showed a prevalent involvement of the brain-stem acoustic pathways, presumably at different levels, seldom associated with eighth nerve impairment. In some cases LLC (N85) abnormalities could not be interpreted as a consequence of impaired function in peripheral and brain-stem acoustic sites and might suggest a cortical dysfunction. Major BAEP abnormalities were observed in patients with longer disease duration, while N85 changes were unrelated to the duration and the severity of the illness. The former may prove to be a reliable means of monitoring the progression of the disease, the latter could be employed as a complementary procedure in its early diagnosis.