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: 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: 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: 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
A mouse model for nonsyndromic deafness (DFNB12) links hearing loss to defects in tip links of mechanosensory hair cells.
19270079 2009
×
Entrez Id: 64072
Gene Symbol: CDH23
CDH23
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
A forward genetics screen in mice identifies recessive deafness traits and reveals that pejvakin is essential for outer hair cell function.
17329413 2007
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: 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
×
Entrez Id: 23562
Gene Symbol: CLDN14
CLDN14
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Claudin 14 knockout mice, a model for autosomal recessive deafness DFNB29, are deaf due to cochlear hair cell degeneration.
12913076 2003
×
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: 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: 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: 81624
Gene Symbol: DIAPH3
DIAPH3
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
×
Entrez Id: 2706
Gene Symbol: GJB2
GJB2
Sensorineural hearing loss, bilateral
0.340
Biomarker
MGD
×
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: 10804
Gene Symbol: GJB6
GJB6
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafness.
17227867 2007
×
Entrez Id: 10804
Gene Symbol: GJB6
GJB6
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Connexin30 deficiency causes instrastrial fluid-blood barrier disruption within the cochlear stria vascularis.
17400755 2007
×
Entrez Id: 10804
Gene Symbol: GJB6
GJB6
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
ATP release through connexin hemichannels and gap junction transfer of second messengers propagate Ca2+ signals across the inner ear.
19047635 2008
×
Entrez Id: 10804
Gene Symbol: GJB6
GJB6
Sensorineural hearing loss, bilateral
0.200
Biomarker
MGD
Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potential.
12490528 2003
×
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
×
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: 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
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