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Audiological evaluation in spinocerebellar ataxia
Codas
|January 14, 2014
Summary
Spinocerebellar ataxia (SCA) patients frequently exhibit hearing loss and abnormal auditory brainstem responses. These audiological and electrophysiological findings highlight the widespread neurological impact of SCA.
Area of Science:
- Neurology
- Audiology
- Neurophysiology
Background:
- Spinocerebellar ataxia (SCA) is a group of inherited neurodegenerative disorders.
- These disorders primarily affect the cerebellum, leading to progressive problems with gait, speech, and coordination.
- Auditory and electrophysiological dysfunctions are not well-characterized in SCA populations.
Purpose of the Study:
- To investigate and report the audiological and electrophysiological results in patients diagnosed with spinocerebellar ataxia (SCA).
Main Methods:
- A retrospective and cross-sectional study was conducted involving 43 patients with SCA.
- Procedures included patient history, otolaryngologic examination, pure tone audiometry, acoustic immittance measures, and brainstem auditory evoked potential (BAEP) testing.
Main Results:
- Patients commonly presented with gait abnormalities (83.7%), speech disorders (48.8%), dizziness (41.8%), and dysphagia (39.5%).
- Hearing loss was reported in 27.9% of cases, with audiometric abnormalities found in 32.5% of patients.
- Brainstem auditory evoked potential (BAEP) abnormalities were observed in 46.5% of patients, and acoustic immittance measures revealed disorders in 44.1%.
Conclusions:
- Audiological evaluation showed a prevalent down-sloping hearing loss pattern and absent acoustic reflexes at 3 and 4 kHz.
- Electrophysiological assessment indicated abnormalities in 50% of patients, characterized by increased wave latencies and interpeak intervals in BAEP.
