Intermediate filaments link glutamate–aspartate transporter deficiency to cochlear synaptopathy

Hidden hearing loss (HHL) describes a pathology where cochlear synapses degenerate despite preserved hair cells and normal audiometric thresholds. While glutamate excitotoxicity is a known driver of cochlear pathology, the underlying molecular mechanisms compromising the ribbon synapse remain incompletely understood. Ma et al. identify a novel link between the glutamate-aspartate transporter (GLAST) and the structural integrity of the postsynaptic cytoskeleton. GLAST deficiency leads to the degradation of intermediate filaments, destabilizing surface AMPA receptors, highlighting the critical role of cytoskeletal architecture in maintaining auditory function and preventing accelerated age-related hearing loss.

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Publication Details

Journal
FEBS Journal
Published
2026-04-01
DOI
https://doi.org/10.1111/febs.70512
Primary Topic
Hearing, Cochlea, Tinnitus, Genetics
Type
article
Field-Weighted Citation Impact
0.00

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article

Intermediate filaments link glutamate–aspartate transporter deficiency to cochlear synaptopathy

Lukas D. Landegger, Paul Emmerich Krumpoeck
FEBS Journal
Hearing, Cochlea, Tinnitus, Genetics
article

Intermediate filaments link glutamate–aspartate transporter deficiency to cochlear synaptopathy

Lukas D. Landegger, Paul Emmerich Krumpoeck
article en

Abstract

Hidden hearing loss (HHL) describes a pathology where cochlear synapses degenerate despite preserved hair cells and normal audiometric thresholds. While glutamate excitotoxicity is a known driver of cochlear pathology, the underlying molecular mechanisms compromising the ribbon synapse remain incompletely understood. Ma et al. identify a novel link between the glutamate-aspartate transporter (GLAST) and the structural integrity of the postsynaptic cytoskeleton. GLAST deficiency leads to the degradation of intermediate filaments, destabilizing surface AMPA receptors, highlighting the critical role of cytoskeletal architecture in maintaining auditory function and preventing accelerated age-related hearing loss.

FEBS Journal
Vienna General Hospital (AT), Christian Doppler Laboratory for Thermoelectricity (AT), Stanford University (US)
Christian Doppler Forschungsgesellschaft
Openalex Percentile: Top 23%
Hearing, Cochlea, Tinnitus, Genetics
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