title: Audiological evaluation in spinocerebellar ataxia.
abstract: OBJECTIVE
  To describe the audiological and electrophysiological results in patients with spinocerebellar ataxia (SCA).
  
  
  METHODS
  Retrospective and cross-sectional studies were performed. Forty-three patients were assessed using the following procedures: anamnesis and otolaryngologic exam, pure tone audiometry, acoustic immittance measures and brainstem auditory evoked potential (BAEP).
  
  
  RESULTS
  Patients showed gait abnormality (83.7%), speech disorder (48.8%), dizziness (41.8%) and dysphagia (39.5%). Hearing loss was referred in 27.9% of the cases; in the audiometric exams, 14 patients (32.5%) presented disorders; in SCA 3, 33.3%; in SCA 2, 12.5%; in SCA 4, 100.0%; in SCA 6, 100.0%; in SCA 7, 100.0%; in SCA 10, 50.0%; and in undetermined SCA, 21.4%. In BAEP, 20 patients (46.5%) were abnormal, being 58.3% in SCA 3, 62.5% in SCA 2, 100.0% in SCA 6, 100.0% in SCA 7, 66.7% in SCA 10 and 14.2% in undetermined SCA. While in acoustic immittance, 19 patients (44.1%) presented disorders, being 50.0% in SCA 3, 50.0% in SCA 2, 100.0% in SCA 4, 100.0% in SCA 6, 100.0% in SCA 7, 33.3% in SCA 10 and 28.5% in undetermined SCA.
  
  
  CONCLUSIONS
  The most evident abnormalities in the audiological evaluation were the predominance of the down-sloping audiometric configuration beginning at 4 kHz bilaterally and the bilateral absence of acoustic reflex at the frequencies of 3 and 4 kHz. In the electrophysiological evaluation, 50% of 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.
authors.forename: Bianca Simone
authors.initials: BS
authors.lastname: Zeigelboim
authors.forename: Hélio Afonso Ghizoni
authors.initials: HA
authors.lastname: Teive
authors.forename: Rosane Sampaio
authors.initials: RS
authors.lastname: Santos
authors.forename: Walter Oleschko
authors.initials: WO
authors.lastname: Arruda
authors.forename: Ari Leon
authors.initials: AL
authors.lastname: Jurkiewicz
authors.forename: Heidi
authors.initials: H
authors.lastname: Mengelberg
authors.forename: Denise
authors.initials: D
authors.lastname: França
authors.forename: Jair Mendes
authors.initials: JM
authors.lastname: Marques
mesh_terms: D000328:Adult; D000368:Aged; D001301:Audiometry, Pure-Tone; D001307:Auditory Perception / Q000502:physiology*; D003430:Cross-Sectional Studies; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011930:Reaction Time; D012189:Retrospective Studies; D020754:Spinocerebellar Ataxias / Q000150:complications / Q000503:physiopathology*; D055815:Young Adult