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Psychoacoustics and neurophysiological auditory processing in patients with Charcot‐Marie‐Tooth disease types 1A and 2A.

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Title: Psychoacoustics and neurophysiological auditory processing in patients with Charcot‐Marie‐Tooth disease types 1A and 2A.
Authors: Choi, J. E. (AUTHOR), Seol, H. Y. (AUTHOR), Seok, J. M. (AUTHOR), Hong, S. H. (AUTHOR), Choi, B.‐O. (AUTHOR), Moon, I. J. (AUTHOR)
Source: European Journal of Neurology. Oct2020, Vol. 27 Issue 10, p2079-2088. 10p.
Subjects: Charcot-Marie-Tooth disease, Psychoacoustics, Hearing disorders, Auditory processing disorder, Presbycusis, Myelin proteins, Audiometry
Abstract: Background and purpose: Hidden hearing loss has been reported in patients with Charcot‐Marie‐Tooth (CMT) disease; however, the auditory‐processing deficits have not been widely explored. We investigated the psychoacoustic and neurophysiological aspects of auditory processing in patients with CMT disease type 1A (CMT1A) and type 2A (CMT2A). Methods: A total of 43 patients with CMT1A and 15 patients with CMT2A were prospectively enrolled. All patients with CMT disease had normal sound‐detection ability by using pure‐tone audiometry. Spectral‐ripple discrimination, temporal modulation detection and auditory frequency‐following response were compared between CMT1A, CMT2A and control groups. Results: Although all participants had normal audiograms, patients with CMT disease had difficulty understanding speech in noise. The psychoacoustic auditory processing was somewhat different depending on the underlying pathophysiology of CMT disease. Patients with CMT1A had degraded auditory temporal and spectral processing. Patients with CMT2A had no reduced spectral resolution, but they showed further reduced temporal resolution than the patients with CMT1A. The amplitudes of the frequency‐following response were reduced in patients with CMT1A and CMT2A, but the neural timing remained relatively intact. Conclusions: When we first assessed the neural representation to speech at the brainstem level, the grand average brainstem responses were reduced in both patients with CMT1A and CMT2A compared with healthy controls. As the psychoacoustic aspects of auditory dysfunctions in CMT1A and CMT2A were somewhat different, it is necessary to consider future auditory rehabilitation methods based on their pathophysiology. [ABSTRACT FROM AUTHOR]
Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Psychology and Behavioral Sciences Collection
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  Data: Psychoacoustics and neurophysiological auditory processing in patients with Charcot‐Marie‐Tooth disease types 1A and 2A.
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  Data: <searchLink fieldCode="AR" term="%22Choi%2C+J%2E+E%2E%22">Choi, J. E.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Seol%2C+H%2E+Y%2E%22">Seol, H. Y.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Seok%2C+J%2E+M%2E%22">Seok, J. M.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Hong%2C+S%2E+H%2E%22">Hong, S. H.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Choi%2C+B%2E‐O%2E%22">Choi, B.‐O.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Moon%2C+I%2E+J%2E%22">Moon, I. J.</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22European+Journal+of+Neurology%22">European Journal of Neurology</searchLink>. Oct2020, Vol. 27 Issue 10, p2079-2088. 10p.
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  Data: <searchLink fieldCode="DE" term="%22Charcot-Marie-Tooth+disease%22">Charcot-Marie-Tooth disease</searchLink><br /><searchLink fieldCode="DE" term="%22Psychoacoustics%22">Psychoacoustics</searchLink><br /><searchLink fieldCode="DE" term="%22Hearing+disorders%22">Hearing disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Auditory+processing+disorder%22">Auditory processing disorder</searchLink><br /><searchLink fieldCode="DE" term="%22Presbycusis%22">Presbycusis</searchLink><br /><searchLink fieldCode="DE" term="%22Myelin+proteins%22">Myelin proteins</searchLink><br /><searchLink fieldCode="DE" term="%22Audiometry%22">Audiometry</searchLink>
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  Data: Background and purpose: Hidden hearing loss has been reported in patients with Charcot‐Marie‐Tooth (CMT) disease; however, the auditory‐processing deficits have not been widely explored. We investigated the psychoacoustic and neurophysiological aspects of auditory processing in patients with CMT disease type 1A (CMT1A) and type 2A (CMT2A). Methods: A total of 43 patients with CMT1A and 15 patients with CMT2A were prospectively enrolled. All patients with CMT disease had normal sound‐detection ability by using pure‐tone audiometry. Spectral‐ripple discrimination, temporal modulation detection and auditory frequency‐following response were compared between CMT1A, CMT2A and control groups. Results: Although all participants had normal audiograms, patients with CMT disease had difficulty understanding speech in noise. The psychoacoustic auditory processing was somewhat different depending on the underlying pathophysiology of CMT disease. Patients with CMT1A had degraded auditory temporal and spectral processing. Patients with CMT2A had no reduced spectral resolution, but they showed further reduced temporal resolution than the patients with CMT1A. The amplitudes of the frequency‐following response were reduced in patients with CMT1A and CMT2A, but the neural timing remained relatively intact. Conclusions: When we first assessed the neural representation to speech at the brainstem level, the grand average brainstem responses were reduced in both patients with CMT1A and CMT2A compared with healthy controls. As the psychoacoustic aspects of auditory dysfunctions in CMT1A and CMT2A were somewhat different, it is necessary to consider future auditory rehabilitation methods based on their pathophysiology. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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              Text: Oct2020
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