Journal
FASEB JOURNAL
Volume 34, Issue 3, Pages 3501-3518Publisher
WILEY
DOI: 10.1096/fj.201902435R
Keywords
auditory system; autism spectrum disorders; circuit development; Fragile X syndrome; hyperacusis; synaptic transmission
Categories
Funding
- HHS \ NIH \ National Institute on Deafness and Other Communication Disorders (NIDCD) [3T32DC012280-05S1, R01DC01919, NS102239, R01DC010796, R01DC017924, R01DC016054, R01DC13074, R21DC17267, F32DC015160-01A1]
- FRAXA Research Foundation (FRAXA)
- Deutsche Forschungsgemeinschaft (DFG) [SFB665]
- HHS \ NIH \ Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) [U54 HD082008]
- DOD \ United States Army \ MEDCOM \ Congressionally Directed Medical Research Programs (CDMRP) [PR140683]
- United States-Israel Binational Science Foundation
- SFARI
- CDMRP [794307, PR140683] Funding Source: Federal RePORTER
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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.
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