Objective: to describe the audiological and electrophysiological results  in patients with sporadic ataxia (SA).

Background: Patients with adult onset non-familial progressive ataxia are classified in sporadic ataxia group. There are several disease categories that may manifest with sporadic ataxia: toxic causes, immune-mediated ataxias, vitamin deficiency, infectious diseases, degenerative disorders and even genetic conditions.

Methods: Eleven patients were assessed using the following procedures: anamnesis, otolaryngologic exam, pure tone audiometry, acoustic immittance measures and brainstem auditory evoked potential (BAEP).

Results: a) patients showed gait abnormality (81.8%), dysarthria (63.8%) and dysphagia (54.5%). Hearing loss was referred in 36.3% of the cases; b) audiometric exams were abnormal in four patients (36.4%); c) BAEP were abnormal in four patients (36.4%), 18.2% occurred bilaterally and left ear, respectively; and d)  acoustic immittance measurements were abnormal in five patients (45.4%) with predominance of bilaterally.

Conclusions:

The most evident abnormalities in the audiological evaluation were the predominance of the down-sloping audiometric configuration beginning at 4 kHz and the bilateral absence of acoustic reflex at the frequencies of 3-4 kHz. In the electrophysiological evaluation the patients showed abnormalities with prevalence of an increase of the latency of waves I, II and V, and of the interval in the interpeaks I-III, I-V and III-V.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/audiological-evaluation-in-sporadic-ataxia/