Genetic map positions of mouse genes and mutations affecting hearing and balance

Genes with mouse mutations that provide models of human deafness disorders are followed by NS (non-syndromic) or S (syndromic).

Homologs of genes that underlie human non-syndromic deafness, but without mouse models, are followed by *. (see Mouse Models)

Chr 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, X

Chromosome cM Position Genes and mutations
1 10.4 Eya1, eyes absent 1 homolog (Drosophila) [spontaneous and targeted mutations] S
1 15.0 Col9a1, procollagen, type IX, alpha 1 [targeted mutation]
1 16.5 Dst, dystonin [dystonia musculorum, dt]
1 25.0 Wo, wocko
1 43.0 Epha4, Eph receptor A4 [targeted mutation]
1 44.0 Pax3, paired box gene 3 [splotch, Sp] S
1 51.0 Col4a3, procollagen, type IV, alpha 3 [targeted mutation] S
1 58.5 Otos, otospiralin [targeted mutation]
1 59.9 thd, tilted head
1 60.0 Gbx2, gastrulation brain homeobox 2 [targeted mutation]
1 87.0 Slc19a2, solute carrier family 19, member 2 [targeted mutation]
1 88.5 Lmx1a, LIM homeobox transcription factor 1 alpha [dreher, dr]
1 92.4 Mpz, myelin protein zero [targeted mutation]
1 93.5 Kcnj10, potassium inwardly-rectifying channel, subfamily J, member 10 [targeted mutation]
1 93.7 Vangl2, loop tail associated protein [loop tail, Lp]
1 101.5 Tgfb2, transforming growth factor, beta 2 [targeted mutation]
1 106.3 Ush2a, Usher syndrome type 2A [targeted mutation]
2  7.0 Itga8, integrin alpha 8 [targeted mutation]
2 7.0 Gata3, GATA-binding protein 3 [targeted mutation] S
2 10.0 Myo3a, myosin IIIA [DFNB30] *
2 13.5 Pax8, paired box gene 8 [targeted mutation]
2 14.5 Cacnb2, calcium channel, voltage-dependent beta 2 subunit [targeted mutation]
2 15.0 Notch1, Notch gene homolog [targeted mutation]
2 17.0 Barhl1, BarH-like 1 [targeted mutation]
2 34.0 Fign, fidgetin [fidget, fi]
2 40.0 Lrp2, low density lipoprotein receptor-related protein 2 (megalin) [targeted mutation]
2 43.5 Dlx1, distal-less homeobox 1 [targeted mutations]
2 44.0 Atf2, activating transcription factor 2 [targeted mutation]
2 45.0 Dfnb59, deafness autosomal recessive 59 homolog [targeted mutation] NS
2 46.0 Neurod1, neurogenic differentiation 1 [targeted mutation]
2 51 Slc12a6, solute carrier family 12, member 6; K-Cl co-transporter KCC3  [targeted mutation]
2 62.0 Bdnf, brain derived neurotrophic factor [targeted mutation]
2 62.0 Tlc, tailchaser
2 67.5 Mtap1a [modifier of tubby hearing 1, moth1]
2 68.0 Strc, stereocilin [targeted mutation] NS
2 68.5 Duox2, dual oxidase 2 {thyroid dyshormonogenesis, thyd]
2 69.0 Slc30a4, solute carrier family 30 (zinc transporter), member 4 [lethal milk, lm]
2 69.5 Pldn, pallidin [pallid, pa]
2 74.0 Slc4a11, solute carrier famiy 4, sodium bicarbonate transporter-like, member 11 [targeted mutation]
2 76.0 Mkks, McKusick-Kaufman syndrome protein [targeted mutation] S
2 77.0 Jag1, jagged 1 [targeted mutation; slalom]
2 78.0 Cm, coloboma deletion [contains Jag1]
2 91.0 Mafb, v-maf musculoaponeurotic fibrosarcoma oncogene family, protein B (avian) [Kreisler, kr]
2 104.0 Edn3, endothelin 3 [lethal spotting, ls] S
3 12.0 Evi1, ecotropic viral integration site 1 [Junbo,Jbo]
3  12.6 Cldn11, claudin 11 [targeted mutation]
3  15.0 Sox2, SRY-box containing gene 2  [Lcc, light coat and circling; Ysb, yellow submarine]
3 30.0 Clrn1, clarin 1 [targeted mutation] S
3 41.8 Chrnb2, cholinergic receptor, nicotinic, beta polypeptide 2 [targeted mutation]
3 48.8 Tbx15, T-box 15 [droopy ear, de]
3 53.1 Col11a1, procollagen, type XI, alpha 1 [chondrodysplasia, cho] S
3 68.0 Cisd2, CDGSH iron sulfur domain 2 [targeted mutation] S
3 68.9 Nfkb1, nuclear factor of light chain gene enhancer in B cells 1 (p50 subunit) [targeted mutation]
3 74.8 Mcoln3, mucolipin 3 [varitint-waddler,Va ]
4 0.0 Mos, Moloney sarcoma oncogene [transgene]
4 2.0 Chd7, chromodomain helicase DNA binding protein 7 [wheels, Whl, and others] S
4 11.0 Cga, glycoprotein hormone, alpha subunit [targeted mutation]
4 22.0 Nr4a3, nuclear receptor subfamily 4, group A, member 3 [targeted mutations]
4 22.0 Npr2, natriuretic peptide receptor 2 [achondroplasia, cn]
4 31.4 Whrn, whirlin [whirler, wi] NS
4 45.1 asp2, audiogenic seizure prone 2
4 52.0 Bsnd, Bartter syndrome, infantile, with sensorineural deafness {conditional null] S, NS
4 57.0 Kcnq4, potassium voltage-gated channel, subfamily Q, member 4 [targeted null, knock-in] NS
4 57.5 Gjb3, gap junction membrane channel protein beta 3 [DFNA2] *
4 66 ecl, epistatic circling C57L/J
4 66 Ephb2, Eph receptor B2 [targeted mutation]
4 71 Fbxo2, F-box protein-2 [targeted mutation]
4 80.1 Espn, espin [jerker, je] NS
4 81.5 Hes5, hairy and enhancer of split 5, (Drosophila) [targeted mutation]
5 4.0 Hgf, hetatocyte growth factor [conditional KO, transgene overexpression] NS
5 8.0 Slc26a5, solute carrier family 26 member 5 (prestin) [targeted mutation] NS
5 18.0 Otof, otoferlin [targeted mutation] NS
5 18.0 Ucn, urocortin [targeted mutation]
5 18.0 Mpv17, Mpv17 transgene, kidney disease mutant
5 20.0 Fgfr3, fibroblast growth factor receptor 3 [targeted mutation] S
5 21.0 Wfs1, Wolfram syndrome 1 homolog [DFNA6, DFNA14] *
5 24.0 Otop1, otopetrin [tilted, tlt; mergulhador, mlh]
5 40.0 pi, pirouette; tde, Tasmanian devil
5  35-45 ahl2, age-related hearing loss 2, QTL
5 41.0 Chrna9, cholinergic receptor, nicotinic, alpha polypeptide 9 [targeted mutation]
5 42.0 Kit, kit oncogene [dominant spotting,W] S
5 45.0 Scarb2, scavenger receptor class B, member 2 [targeted mutation] 
5 56.0 Gfi1, growth factor independent 1 [targeted mutation] 
5 58.0 Cplx1, complexiin 1 [targeted mutation]
5 61.5 Trpv4, transient receptor potential cation channel, subfamily V, member 4 [targeted mutation]
5 63.0 bv, Bronx waltzer
5 72.0 Gusb, glucuronidase, beta [mucopolysaccharidosis VII, mps]
5 78.0 Gjc3, gap junction protein, gamma 3 [targeted mutation]
5 86.0 Lfng, lunatic fringe gene homolog (Drosophila) [targeted mutation]
6 2.0 Dlx5, distal-less homeobox 5 [targeted mutation]
6 7.0 Mirn96, microRNA 96 [diminuendo, Dmdo] NS
6 22.5 Dfna5h, deafness, autosomal dominant 5 homolog [DFNA5] *
6 26.28 Hoxa1, Hoxa2, homeo box A1, homeo box A2 [targeted mutations]
6 29.7 Atoh1, atonal homolog 1 (Math1) [targeted mutation]
6 31.0 St3gal5, ST3 beta-galactoside alpha-2,3-sialyltransferase 5 [targeted mutation]
6 35.5 Alms1, Alstrom syndrome 1 homolog [gene-trap KO]
6 36.0 Tgfa, transforming growth factor alpha [waved 1, wa1]
6 40.0 Mitf, microphthalmia-associated transcription factor [microphthalmia, mi] S
6 48.0 Atp2b2, ATPase, Ca++ transporting, plasma membrane 2 [deaf waddler, dfw]
6 60.0 Ntf3, neurotrophin 3 [targeted mutation]
6 62.0 Cdkn1b, cyclin-dependent kinase inhibitor 1B [targeted mutation]
7  4.2 nv, Nijmegen waltzer
7 7.5 Spnb4, beta-spectrin 4 [quivering, qv] S
7 14.0 Hpn, hepsin [targeted mutation]
7 23.0 Myh14, myosin heavy chain 14, non-muscle [DFNA4]* 
7 23.5 Ush1c, harmonin [deaf circler, dfcr] NS, S
7 23.6 Otog, otogelin [targeted mutation; twister, twt]
7 24.5 Nav2, neuron navigator 2 [gene trap KO]
7 35.0 asp3, audiogenic seizure prone 3
7 39.0 Agc1, aggrecan 1 [cartilage matrix deficiency, cmd]
7 39.0 Ntrk3, neurotrophic tyrosine kinase, receptor, type 3 [targeted mutation]
7 44.5 Fzd4, frizzled homolog 4 (Drosophila) [targeted mutation]
7 48.1 Myo7a, myosin VIIa [shaker 1, sh1] NS S
7 50.0 Tomt, transmembrane O-methyltransferase [ENU-induced add mutation] NS
7 51.45 Tub, tubby
7 55.0 Crym, crystallin, mu [DFNA] *
7 57.0 Otoa, otoancorin [DFNB22] *
7 63.0 Fgfr2, fibroblast growth factor receptor 2 [targeted mutation]
7 64 hb, head bobber
7 65.0 Hmx3, Hmx2, H6 homeo box 3, H6 homeo box 2 [(targeted mutations]
7 69.3 Kcnq1, potassium voltage-gated channel, subfamily Q, member 1 [targeted mutation] S
7 72.4 Fgf3, fibroblast growth factor 3 [targeted mutation]
8 2.0 Efnb2, ephrin-B2 [targeted mutation]
8 10.0 Fgfr1, fibroblast growth factor receptor 1 [Foxg1 promotor conditional targeted mutation] 
8 26.0 Casp3, caspase 3, apoptosis related cysteine protease [targeted mutation]
8 33.0 Large, like-glycosyltransferase [myd, myodystrophy]
8 38.0 Q, quinky
8 41.0 Sall1, sal-like 1 [targeted gain-of-function mutation] S
9 6.0 Edg5, endothelial differentiation, sphingolipid G-prptein-coupled receptor, 5 [targeted mutation]
9    8.0 Cdkn2d, cyclin-dependent kinase inhibitor 2D [targeted mutation]
9  19.0 vs, variable spotting
9 25.5 Tecta, tectorin alpha [targeted mutation] NS
9 29.0 Rdx, radixin [targeted mutation] NS
9 33.0 Bbs4, Bardet-Biedl syndrome 4 [targeted mutation] S
9 44.0 Myo6, myosin VI [Snell's waltzer, sv] NS
9 48.5 Tbx18, T-box 18 [targeted mutation + transgene]
9 56.0 Ephb1, Eph receptor B1 [targeted mutation]
9 57.0 Gpx1, glutathione peroxidase 1 [targeted mutation]
9 60.0 Bsn, bassoon [targeted mutation]
9 61.0 Tmie, transmembrane inner ear [spinner, sr] NS
10  18.0 Eya4, eyes absent 4 homolog [DFNA10] NS
10 20.0 Lama2, laminin alpha 2 [dystrophia muscularis, dy]
10 28.0 Sobp, sine oculis-binding protein, synonym Jxc1 [jc, Jackson circler]
10 29.0 Gja1, gap junction membrane channel protein alpha 1 *
10 30.0 Cdh23, cadherin 23 [waltzer, v] NS S
10 30.0 ahl, age related hearing loss = Cdh23
10 30.0 mdfw, modifier of deaf waddler = Cdh23
10 35.0 Psap, prosaposin [targeted mutation]
10 40.0 Pcdh15, protocadherin 15 [Ames waltzer, av] NS S
10 43.0 Ap3d1, adaptor-related protein complex AP-3, delta 1 subunit [mocha, mh]
10 48.0 Igf1, insulin-like growth factor 1 [targeted mutation]
10 48.5 Apaf1, apoptotic peptidase activating factor 1 [targeted mutation]
10 49.0 Slc17a8, solute carrier family 17, member 8 (Vglut3) [targeted mutation]
10 57.0 Kitl, kit ligand [steel, Sl]
10 60.0 Ptprq, protein tyrosine phosphatase, receptor type, Q [targeted mutation]
10 68.0 Lrig3, leucine-rich repeats and immunoglobulin-l9ike domainis 3 [targeted mutation]

10 

70.0

Myo1a, myosin IA [DFNA48] *

10 65-75 ahl4, age-related hearing loss 4, QTL
11  13.0 Otx1, orthodenticle homolog 1 [targeted mutation; Jackson waltzer, jv]

11

17.0

Foxi1, forkhead box I1 [targeted mutation]

11 25.0 Prop1, paired like homeodomain factor 1 [Ames dwarf, df]
11 33.9 Myo15, myosin XV [shaker 2, sh2] NS
11 34.45 Pmp22, peripheral myelin protein, 22 kDa [trembler, Tr] S
11 37.0 Ntn1, netrin 1 [targeted mutation]
11 46.0 co cocked
11 50.5 Nog, noggin [targeted mutation, heterozygote] S
11 55.6 Ngfr, nerve growth factor receptor [targeted mutation]
11 56.0 Col1a1, procollagen, type I, alpha 1 [Mov13 transgene disruption; targeted mutation] S
11 69.5 Sox9, SRY-box containing gene 9 [conditional KO]
11 73.5 Ts, tail-short
11 77.0 Ush1g, Usher syndrome 1G homolog [spontaneous mutation js, Jackson shaker] S
11 79.0 Actg, actin, gamma, cytoplasmic [targeted mutation]  NS
11 70-80 ahl8, age-related hearing loss 8, QTL
12  syntenic asp1, audiogenic seizure prone 1
12 ~15 Slc26a4, solute carrier family 26, member 4 (Pendred syndrome homolog) [targeted mutation] NS S
12 21.0 Foxg1, forkhead box G1 [targeted mutation]
12 23.0 Coch, coagulation factor C homolog [targeted mutation] NS
12 31.0 Six1, sine oculis-related homeobox 1 homolog [targeted mutation]
12 33.0 Esr2, estrogen receptor 2 (beta) [targeted mutation]
12 37.0 Tshr, thyroid stimulating hormone, receptor [hypothyroid, hyt] S
12 41.0 Esrrb, estrogen related receptor, beta [targeted conditional mutation] NS
12 44.0 Dio2, deiodinase, iodothyronine, type II [targeted mutation]
12 54.0 Dio3, deiodinase, iodothyronine, type III [targeted mutation]
12 55.0 Ckb, creatine kinase, brain [targeted mutation]
12 57.9 Jag2, jagged 2 [targeted mutation]
13  21.0 Muted, muted
13 25.0 Tcfap2a, transcription factor AP-2, alpha [Doard, ENU-induced mutation]
13 35.0 Neurog1, neurogenic differentiation 3 [targeted mutation]
13 36.0 Ntrk2, neurotrophic tyrosine kinase, receptor, type 2 [targeted mutation]
13 38.0 Ptch1, patched homolog 1 [mesenchymal dysplasia, mes]
13 39.0 Slc12a7, solute carrier family 12, member 7; K-Cl co-transporter KCC4 [targeted mutation]
13 40.0 Gpr98, G protein-coupled receptor 98 [Frings, BUB/BnJ strains, targeted mutation] S
13 51.0 Marveld2, MARVEL (membrane-associating) domain containing 2 [DFNB49] *
14  0.5 Slc4a7, solute carrier family 4, sodium bicarbonate transporter, member 7 [targeted mutation]
14  1.5 Thrb, thyroid hormone receptor beta [targeted mutation] S
14 6.0 Kcnma1, potassium large conductance calcium-activated channel, subfamily M, alpha member 1 [targeted mutation]
14 8.0 Cacna1d, calcium channel, voltage-dependent, L type, alpha 1D subunit [targeted mutation]
14 13.5 Grid1, glutamate receptor ionotropic,delta 1 [targeted mutation]
14 15.0 Bmp4, bone morphogenetic protein 4 [targeted mutation]
14 21.0 Gjb2, gap junction membrane channel protein beta 2 [targeted, conditional mutation] NS
14 21.0 Gjb6, gap junction membrane channel protein beta 6 [targeted mutation] NS
14 21.5 Ift88, intraflagellar transport 88 homolog [targeted conditional mutation]
14 15-30 ecs, epistatic circling SWR/J
14 27.0 Pnoc, prepronociceptin [targeted mutation]
14 51.0 Ednrb, endothelin receptor type B [piebald, s] S
14 51.0 Pou4f1, POU domain, class 4, transcription factor 1 [targeted mutation]
14 51.0 Spry2, sprouty homolog [targeted mutation]
15 6.7 Slc1a3, solute carrier family 1, member 3 [targeted mutation]
15 18.0 Grhl2, grainyhead-like 2 [DFNA28] * 
15 22.0 Tnfrsf11b, tumor necrosis factor receptor superfamily, member 11b (osteoprotegerin) [targeted mutation]
15 37.5 Oc90, otoconin 90 [targeted mutation]
15 43.3 Myh9, myosin heavy chain IX [DFNA17] *
15 43.8 Scrib, scribbled homolog [circletail, Crc]
15 45.2 Cacng2, calcium channel, voltage-dependent, gamma subunit 2 [stargazer, stg]
15 46.5 Triobp, TRIO and F-actin binding protein [DFNB28] *
15 47.0 Sox10, SRY-box containing gene 10 [dominant megacolon, Dom] S
15 49.0 Celsr1, cadherin EGF LAG seven-pass G-type receptor 1 [spin cycle, Scy; crash, Crsh]
15 53.0 Arsa, arylsulfatase A [targeted mutation]
15 54.5

Col2a1, procollagen, type II, alpha 1 [disproportionate micromelia, Dmm; spondyloepiphyseal dysplasia congenita, sedc; targeted mutations] S

16   9.4  Snai2, snail homolog 2 [targeted mutation] S
16  11.4 Tbx1, T-box 1 [targeted mutation] S
16 15.0 Ephb3, Eph receptor B3 [targeted mutation]
16 20.0 Ccdc50, coiled-coil domain containing 50 [DFNA44] *
16 27 Hes1, hairy and enhancer of split 1 (Drosophila) [targeted mutation]
16 28.2 ckr, chakragati
16 44.0 Pou1f1, POU domain, class 1, transcription factor 1 [dwarf, dw]
16 61.0 Sod1, superoxide dismutase 1, soluble [targeted mutation]
16 64.4 Kcne1, potassium voltage-gated channel, Isk-related subfamily, member 1 [targeted mutation; punk rocker, pkr] S
16 67.0 Cldn14, claudin 14 [targeted mutation] NS
17  4.1 Nox3, NADPH oxidase 3 [head tilt, het]
17 5.9 Qk, quaking
17 9.5 Cldn9, claudin 9 [nmf329, ENU-induced mutation]
17 10.0 Noxo1, NADPH oxidase organizer 1 [head slant, hslt]
17 11.8 Axin1, axin 1 [fused, Fu]
17 16.0 Tmhs, tetraspan membrane protein, hair cell stereocilia [hurry-scurry, hscy] NS
17 17.0 Tmprss3, transmembrane protease, serine 3 [DFNB8, DFNB10] *
17 18.5 Col11a2, procollagen, type XI, alpha 2 [targeted mutation] NS S
17 21.5 Ddr1, discoidin domain receptor family, member 1 [targeted mtation]
17 23.0 Clic5, chloride intracellular channel protein 5 [jitterbug, jbg]
17 24.5 Ptk7, PTK7 protein tyrosine kinase 7 [targeted mutation]
17 20-45 ahl3, age-related hearing loss 3, QTL
17 47.0 Fbxo11, F-box protein 11 [jeff Jf]
18  3.0 Tw, twirler
18 6.0 Aqp4, aquaporin 4 [targeted mutation]
18 16.0 Diap1, diaphanous homolog 1 [DFNA1] *
18 24.0 Pou4f3, POU domain, class 4, transcription factor 3 [targeted mutation; dreidel, ddl] NS
18 27.0 Slc12a2, solute carrier family 12, member 2 (Na-K-Cl co-transporter) [targeted and spontaneous mutations]
18 27.0 sy, shaker-with-syndactylism deletion [contains Slc12a2]
18 39.0 Atp8b1, ATPase, class I, type 8B, member 1 [targeted missense mutation]
18 50.0 Loxhd1, lipoxygenase homology domains 1 [samba, ENU-induced mutation] NS
18 55.0 Mbp, myelin basic protein [shiverer, shi]
19  2.0 Tbx10, T-box 10 [dancer, Dc]
19 4.0 Bbs1, Bardet-Biedl syndrome 1 [targeted mutation] S
19 15.0 Tmc1, transmembrane cochlear-expressed gene 1 [deafness, dn; Beethoven, Bth] NS
19 23.0 Fas, Fas (TNF receptor superfamily member) [lymphoproliferation,Fas-lpr]
19 43.0 Pax2, paired box gene 2 [targeted mutation] S
19 44.0 Krd, kidney and retinal defects deletion [contains Pax2]
19 53.5 Emx2, empty spiracles homolog 2 [pardon, Pdo] 
X  5.3 Ndph, Norrie disease homolog [targeted mutation] S
X 32 Dmd, dystrophin [X-linked muscular dystrophy, mdx]
X 49.0 Pou3f4, POU domain, class 3, transcription factor 4 [targeteded mutation; sex-linked fidgit, slf] NS
X 65.0

Phex, phosphate regulating neutral endopeptidases on the X Chromosome [hypophosphotemia, Hyp; included in gyro (Gy) deletion]

X 65.0 Sms, spermine synthase [included in gyro (Gy) deletion]

Chr 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, X

Content maintained by Ken Johnson