Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4 -insufficient mouse model of large vestibular aqueduct syndrome

Highlights • SLC26A4 mutations can cause fluctuating and progressive hearing loss in humans. • Slc26a4 -insufficient mice have irreversible hearing loss after 6 months of age. • Stria vascularis degeneration underlies hearing loss in Slc26a4 -insufficient ears. • Slc26a4 -insufficient mice may be us...

Full description

Saved in:
Bibliographic Details
Published in:Neuroscience 2015-12, Vol.310, p.188-197
Main Authors: Ito, T, Nishio, A, Wangemann, P, Griffith, A.J
Format: Article
Language:English
Subjects:
Acoustic Stimulation
Animals
Anion Transport Proteins - deficiency
Anion Transport Proteins - genetics
Auditory Threshold - physiology
Cell Death - genetics
deafness
Disease Models, Animal
doxycycline
Evoked Potentials, Auditory, Brain Stem - genetics
Evoked Potentials, Auditory, Brain Stem - physiology
fluctuation
Gene Expression Regulation - genetics
Genotype
Hair Cells, Auditory - pathology
Hearing Loss - etiology
Hearing Loss - genetics
Hearing Loss - physiopathology
hypomorphic
KCNQ1 Potassium Channel - genetics
KCNQ1 Potassium Channel - metabolism
Mice
Mice, Transgenic
Mutation - genetics
Neurology
Potassium Channels, Inwardly Rectifying - genetics
Potassium Channels, Inwardly Rectifying - metabolism
SLC26A4
stria vascularis
Stria Vascularis - pathology
Sulfate Transporters
Vestibular Aqueduct - pathology
Vestibular Diseases - complications
Vestibular Diseases - genetics
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Highlights • SLC26A4 mutations can cause fluctuating and progressive hearing loss in humans. • Slc26a4 -insufficient mice have irreversible hearing loss after 6 months of age. • Stria vascularis degeneration underlies hearing loss in Slc26a4 -insufficient ears. • Slc26a4 -insufficient mice may be used to study primary strial presbycusis.
ISSN:0306-4522
1873-7544
DOI:10.1016/j.neuroscience.2015.09.016