×
Entrez Id: 51475
Gene Symbol: CABP2
CABP2
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Ca2+ -binding protein 2 inhibits Ca2+ -channel inactivation in mouse inner hair cells.
28183797 2017
×
Entrez Id: 10518
Gene Symbol: CIB2
CIB2
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
CIB2, defective in isolated deafness, is key for auditory hair cell mechanotransduction and survival.
29084757 2017
ATP6V1B1
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Hearing loss without overt metabolic acidosis in ATP6V1B1 deficient MRL mice, a new genetic model for non-syndromic deafness with enlarged vestibular aqueducts.
28934385 2017
×
Entrez Id: 9294
Gene Symbol: S1PR2
S1PR2
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
S1PR2 variants associated with auditory function in humans and endocochlear potential decline in mouse.
27383011 2016
×
Entrez Id: 9294
Gene Symbol: S1PR2
S1PR2
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Autosomal-Recessive Hearing Impairment Due to Rare Missense Variants within S1PR2.
26805784 2016
×
Entrez Id: 286262
Gene Symbol: TPRN
TPRN
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Progressive hearing loss and degeneration of hair cell stereocilia in taperin gene knockout mice.
27693694 2016
×
Entrez Id: 286676
Gene Symbol: ILDR1
ILDR1
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Deficiency of angulin-2/ILDR1, a tricellular tight junction-associated membrane protein, causes deafness with cochlear hair cell degeneration in mice.
25822906 2015
×
Entrez Id: 286676
Gene Symbol: ILDR1
ILDR1
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
ILDR1 null mice, a model of human deafness DFNB42, show structural aberrations of tricellular tight junctions and degeneration of auditory hair cells.
25217574 2015
MARVELD2
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Deletion of Tricellulin Causes Progressive Hearing Loss Associated with Degeneration of Cochlear Hair Cells.
26677943 2015
×
Entrez Id: 340990
Gene Symbol: OTOG
OTOG
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
A new Otogelin ENU mouse model for autosomal-recessive nonsyndromic moderate hearing impairment.
26636018 2015
×
Entrez Id: 286676
Gene Symbol: ILDR1
ILDR1
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
ILDR1 deficiency causes degeneration of cochlear outer hair cells and disrupts the structure of the organ of Corti: a mouse model for human DFNB42.
25819842 2015
×
Entrez Id: 51168
Gene Symbol: MYO15A
MYO15A
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
The 133-kDa N-terminal domain enables myosin 15 to maintain mechanotransducing stereocilia and is essential for hearing.
26302205 2015
×
Entrez Id: 65217
Gene Symbol: PCDH15
PCDH15
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
The circling mutant Pcdh15roda is a new mouse model for hearing loss.
24044941 2014
×
Entrez Id: 7007
Gene Symbol: TECTA
TECTA
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Three deaf mice: mouse models for TECTA-based human hereditary deafness reveal domain-specific structural phenotypes in the tectorial membrane.
24363064 2014
×
Entrez Id: 253827
Gene Symbol: MSRB3
MSRB3
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Methionine sulfoxide reductase B3 deficiency causes hearing loss due to stereocilia degeneration and apoptotic cell death in cochlear hair cells.
24191262 2014
MARVELD2
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Tricellulin deficiency affects tight junction architecture and cochlear hair cells.
23979167 2013
CEACAM16
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Loss of mammal-specific tectorial membrane component carcinoembryonic antigen cell adhesion molecule 16 (CEACAM16) leads to hearing impairment at low and high frequencies.
22544735 2012
×
Entrez Id: 117531
Gene Symbol: TMC1
TMC1
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Identification of three novel hearing loss mouse strains with mutations in the Tmc1 gene.
22330676 2012
×
Entrez Id: 146183
Gene Symbol: OTOA
OTOA
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
A mouse model for human deafness DFNB22 reveals that hearing impairment is due to a loss of inner hair cell stimulation.
23129639 2012
×
Entrez Id: 64072
Gene Symbol: CDH23
CDH23
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
A new mouse mutant of the Cdh23 gene with early-onset hearing loss facilitates evaluation of otoprotection drugs.
20644563 2012
×
Entrez Id: 64072
Gene Symbol: CDH23
CDH23
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
An ENU-induced mutation of Cdh23 causes congenital hearing loss, but no vestibular dysfunction, in mice.
21689626 2011
×
Entrez Id: 53904
Gene Symbol: MYO3A
MYO3A
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
A mouse model for human hearing loss DFNB30 due to loss of function of myosin IIIA.
21165622 2011
×
Entrez Id: 10804
Gene Symbol: GJB6
GJB6
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
The human deafness-associated connexin 30 T5M mutation causes mild hearing loss and reduces biochemical coupling among cochlear non-sensory cells in knock-in mice.
20858605 2010
×
Entrez Id: 25861
Gene Symbol: WHRN
WHRN
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Cochlear outer hair cells undergo an apical circumference remodeling constrained by the hair bundle shape.
20332152 2010
×
Entrez Id: 64072
Gene Symbol: CDH23
CDH23
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
A mouse model for nonsyndromic deafness (DFNB12) links hearing loss to defects in tip links of mechanosensory hair cells.
19270079 2009