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.
Keywords
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Article info
Publication history
Published online: November 09, 2012
Accepted:
October 17,
2012
Received in revised form:
October 15,
2012
Received:
August 6,
2012
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
