title: Acoustic impairment is a distinguishable clinical feature of Asidan/SCA36.
abstract: OBJECTIVE
  To investigate acoustic function of Asidan/spinocerebellar ataxia type 36 (SCA36) in which sensorineural hearing loss may be found as one of extracerebellar symptom that can be a distinguishable feature from other degenerative ataxias.
  
  
  METHODS
  Acoustic function in the groups of normal control (n=31), Asidan/SCA36 (n=13), cortical cerebellar atrophy (CCA, n=28), multiple system atrophy of cerebellar predominance (MSA-C, n=48), SCA31 (n=4), and other forms of SCAs (n=14) was evaluated by pure tone average (PTA) calculated by the results of audiogram and brainstem auditory evoked potentials (BAEPs).
  
  
  RESULTS
  PTA was significantly decreased in Asidan/SCA36 in comparison to normal control and other ataxic groups, but not significant within other ataxic groups and normal control. In comparison to other groups, Asidan/SCA36 showed a constant depression at 7 different frequencies in audiogram, especially at 4000 and 8000 Hz. BAEPs in 2 Asidan/SCA36 cases suggested possible involvement in the inner ear or the peripheral part of the auditory system. PTA in Asidan/SCA36 cases significantly correlated with their severity of ataxia.
  
  
  CONCLUSIONS
  In addition to signs for motor neuron involvement, acoustic impairment in Asidan/SCA36 is another characteristic clinical feature that is distinguishable from other forms of SCAs.
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikatacho, Okayama 700-8558, Japan.
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Ikeda
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Ohta
authors.forename: Tomoko
authors.initials: T
authors.lastname: Kurata
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Shiro
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Takao
authors.forename: Koji
authors.initials: K
authors.lastname: Abe
mesh_terms: D020121:5' Untranslated Regions; D000368:Aged; D001299:Audiometry; D001301:Audiometry, Pure-Tone; D003937:Diagnosis, Differential; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D006311:Hearing Disorders / Q000209:etiology*; D006319:Hearing Loss, Sensorineural / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D016133:Polymerase Chain Reaction; D020754:Spinocerebellar Ataxias / Q000145:classification / Q000150:complications* / Q000175:diagnosis
chemical_list: D020121:5' Untranslated Regions