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
N1 - Funding Information:
We would like to thank the patients and doctors who participated in this work. This study was supported in part by grants-in-aid for scientific research (C) 24591263 (to Y.I.), and Challenging Exploratory Research 24659651 (to K.A.) from the Ministry of Education, Culture, Sports, Science and Technology, Japan , and by grants-in-aid from the Research Committees (Nakano I, Sobue G, Mizusawa H, and Sasaki H), the Ministry of Health, Labour and Welfare, Japan (to K.A.).
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
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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 -