×
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
Random mutagenesis of proximal mouse chromosome 5 uncovers predominantly embryonic lethal mutations.
16024820 2005
×
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: 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
The mouse Snell's waltzer deafness gene encodes an unconventional myosin required for structural integrity of inner ear hair cells.
7493015 1995
×
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
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
Myosin VI is required for normal retinal function.
15978262 2005
×
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: 220074
Gene Symbol: LRTOMT
LRTOMT
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
A catechol-O-methyltransferase that is essential for auditory function in mice and humans.
18794526 2008
×
Entrez Id: 1302
Gene Symbol: COL11A2
COL11A2
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Targeted disruption of Col11a2 produces a mild cartilage phenotype in transgenic mice: comparison with the human disorder otospondylomegaepiphyseal dysplasia (OSMED).
11668593 2001
×
Entrez Id: 246213
Gene Symbol: SLC17A8
SLC17A8
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Impairment of SLC17A8 encoding vesicular glutamate transporter-3, VGLUT3, underlies nonsyndromic deafness DFNA25 and inner hair cell dysfunction in null mice.
18674745 2008
×
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: 494513
Gene Symbol: PJVK
PJVK
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy.
16804542 2006
×
Entrez Id: 59341
Gene Symbol: TRPV4
TRPV4
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Hearing impairment in TRPV4 knockout mice.
15925108 2005
×
Entrez Id: 1302
Gene Symbol: COL11A2
COL11A2
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Mutations in COL11A2 cause non-syndromic hearing loss (DFNA13).
10581026 1999
×
Entrez Id: 117531
Gene Symbol: TMC1
TMC1
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Degeneration followed by partial regeneration of the organ of Corti in deafness (dn/dn) mice.
1728569 1992
×
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
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
Early degeneration of sensory and ganglion cells in the inner ear of mice with uncomplicated genetic deafness (dn): preliminary observations.
6662828 1983
×
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