×
Entrez Id:
9381
Gene Symbol:
OTOF
OTOF
Sensorineural hearing loss, bilateral
0.500
Biomarker
MGD
Synaptic transmission between end bulbs of Held and bushy cells in the cochlear nucleus of mice with a mutation in Otoferlin.
25253474
2014
×
Entrez Id:
9381
Gene Symbol:
OTOF
OTOF
Sensorineural hearing loss, bilateral
0.500
Biomarker
MGD
A missense mutation in the conserved C2B domain of otoferlin causes deafness in a new mouse model of DFNB9.
17967520
2007
×
Entrez Id:
9381
Gene Symbol:
OTOF
OTOF
Sensorineural hearing loss, bilateral
0.500
Biomarker
MGD
Otoferlin, defective in a human deafness form, is essential for exocytosis at the auditory ribbon synapse.
17055430
2006
×
Entrez Id:
9381
Gene Symbol:
OTOF
OTOF
Sensorineural hearing loss, bilateral
0.500
Biomarker
MGD
Random mutagenesis of proximal mouse chromosome 5 uncovers predominantly embryonic lethal mutations.
16024820
2005
×
Entrez Id:
2706
Gene Symbol:
GJB2
GJB2
Sensorineural hearing loss, bilateral
0.340
Biomarker
MGD
×
Entrez Id:
161497
Gene Symbol:
STRC
STRC
Sensorineural hearing loss, bilateral
0.310
Biomarker
MGD
Stereocilin-deficient mice reveal the origin of cochlear waveform distortions.
18849963
2008
×
Entrez Id:
4647
Gene Symbol:
MYO7A
MYO7A
Sensorineural hearing loss, bilateral
0.300
Biomarker
MGD
Inner ear morphology is perturbed in two novel mouse models of recessive deafness.
23251483
2012
×
Entrez Id:
4646
Gene Symbol:
MYO6
MYO6
Sensorineural hearing loss, bilateral
0.300
Biomarker
MGD
Myosin VI is required for normal retinal function.
15978262
2005
×
Entrez Id:
4646
Gene Symbol:
MYO6
MYO6
Sensorineural hearing loss, bilateral
0.300
Biomarker
MGD
Myo15 function is distinct from Myo6, Myo7a and pirouette genes in development of cochlear stereocilia.
12966030
2003
×
Entrez Id:
4646
Gene Symbol:
MYO6
MYO6
Sensorineural hearing loss, bilateral
0.300
Biomarker
MGD
Role of myosin VI in the differentiation of cochlear hair cells.
10525338
1999
×
Entrez Id:
4646
Gene Symbol:
MYO6
MYO6
Sensorineural hearing loss, bilateral
0.300
Biomarker
MGD
The mouse Snell's waltzer deafness gene encodes an unconventional myosin required for structural integrity of inner ear hair cells.
7493015
1995
×
Entrez Id:
1690
Gene Symbol:
COCH
COCH
Sensorineural hearing loss, bilateral
0.220
Biomarker
MGD
Hearing and vestibular deficits in the Coch(-/-) null mouse model: comparison to the Coch(G88E/G88E) mouse and to DFNA9 hearing and balance disorder.
21073934
2011
×
Entrez Id:
1690
Gene Symbol:
COCH
COCH
Sensorineural hearing loss, bilateral
0.220
Biomarker
MGD
A targeted Coch missense mutation: a knock-in mouse model for DFNA9 late-onset hearing loss and vestibular dysfunction.
18697796
2008
×
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