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.
| Original language | English |
|---|---|
| Pages (from-to) | 109-112 |
| Number of pages | 4 |
| Journal | Journal of the Neurological Sciences |
| Volume | 324 |
| Issue number | 1-2 |
| DOIs | |
| Publication status | Published - Jan 15 2013 |
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Keywords
- Asidan
- Ataxia
- Audiogram
- Brainstem auditory evoked potential
- GGCCTG repeat
- Motor neuron disease
- NOP56
- SCA36
ASJC Scopus subject areas
- Clinical Neurology
- Neurology
Cite this
Acoustic impairment is a distinguishable clinical feature of Asidan/SCA36. / Ikeda, Yoshio; Ohta, Yasuyuki; Kurata, Tomoko; Shiro, Yoshihiko; Takao, Yoshiki; Abe, Koji.
In: Journal of the Neurological Sciences, Vol. 324, No. 1-2, 15.01.2013, p. 109-112.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Acoustic impairment is a distinguishable clinical feature of Asidan/SCA36
AU - Ikeda, Yoshio
AU - Ohta, Yasuyuki
AU - Kurata, Tomoko
AU - Shiro, Yoshihiko
AU - Takao, Yoshiki
AU - Abe, Koji
PY - 2013/1/15
Y1 - 2013/1/15
N2 - 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.
AB - 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.
KW - Asidan
KW - Ataxia
KW - Audiogram
KW - Brainstem auditory evoked potential
KW - GGCCTG repeat
KW - Motor neuron disease
KW - NOP56
KW - SCA36
UR - http://www.scopus.com/inward/record.url?scp=84870928563&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84870928563&partnerID=8YFLogxK
U2 - 10.1016/j.jns.2012.10.013
DO - 10.1016/j.jns.2012.10.013
M3 - Article
C2 - 23140984
AN - SCOPUS:84870928563
VL - 324
SP - 109
EP - 112
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
SN - 0022-510X
IS - 1-2
ER -