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.
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: Zachary T McEachin; Tania F Gendron; Nisha Raj; María García-Murias; Anwesha Banerjee; Ryan H Purcell; Patricia J Ward; Tiffany W Todd; Megan E Merritt-Garza; Karen Jansen-West; Chadwick M Hales; Tania García-Sobrino; Beatriz Quintáns; Christopher J Holler; Georgia Taylor; Beatriz San Millán; Susana Teijeira; Toru Yamashita; Ryuichi Ohkubo; Nicholas M Boulis; Chongchong Xu; Zhexing Wen; Nathalie Streichenberger; Brent L Fogel; Thomas Kukar; Koji Abe; Dennis W Dickson; Manuel Arias; Jonathan D Glass; Jie Jiang; Malú G Tansey; María-Jesús Sobrido; Leonard Petrucelli; Wilfried Rossoll; Gary J Bassell Journal: Neuron Date: 2020-05-05 Impact factor: 17.173