Mechanisms underlying auditory processing deficits in Fragile X syndrome.

Title

Mechanisms underlying auditory processing deficits in Fragile X syndrome.

Creator

McCullagh Elizabeth A; Rotschafer Sarah E; Auerbach Benjamin D; Klug Achim; Kaczmarek Leonard K; Cramer Karina S; Kulesza Randy J Jr; Razak Khaleel A; Lovelace Jonathan W; Lu Yong; Koch Ursula; Wang Yuan

Publisher

FASEB journal : official publication of the Federation of American Societies for Experimental Biology

Date

2020
2020-03

Description

Autism spectrum disorders (ASD) are strongly associated with auditory hypersensitivity or hyperacusis (difficulty tolerating sounds). Fragile X syndrome (FXS), the most common monogenetic cause of ASD, has emerged as a powerful gateway for exploring underlying mechanisms of hyperacusis and auditory dysfunction in ASD. This review discusses examples of disruption of the auditory pathways in FXS at molecular, synaptic, and circuit levels in animal models as well as in FXS individuals. These examples highlight the involvement of multiple mechanisms, from aberrant synaptic development and ion channel deregulation of auditory brainstem circuits, to impaired neuronal plasticity and network hyperexcitability in the auditory cortex. Though a relatively new area of research, recent discoveries have increased interest in auditory dysfunction and mechanisms underlying hyperacusis in this disorder. This rapidly growing body of data has yielded novel research directions addressing critical questions regarding the timing and possible outcomes of human therapies for auditory dysfunction in ASD.

Subject

auditory system; autism spectrum disorders; synaptic transmission; Fragile X syndrome; circuit development; hyperacusis

Rights

Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).

Format

Journal Article

Search for Full-text

Users with a NEOMED Library login can search for full-text journal articles at the following url: https://libraryguides.neomed.edu/home

Pages

3501-3518

Issue

3

Volume

34

NEOMED College

NEOMED College of Medicine

NEOMED Department

Department of Anatomy & Neurobiology

Update Year & Number

March 2020 Update

Citation

McCullagh Elizabeth A; Rotschafer Sarah E; Auerbach Benjamin D; Klug Achim; Kaczmarek Leonard K; Cramer Karina S; Kulesza Randy J Jr; Razak Khaleel A; Lovelace Jonathan W; Lu Yong; Koch Ursula; Wang Yuan, “Mechanisms underlying auditory processing deficits in Fragile X syndrome.,” NEOMED Bibliography Database, accessed September 25, 2021, https://neomed.omeka.net/items/show/10985.

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