POU4F3

Summary

Gene Symbol: POU4F3
Description: POU class 4 homeobox 3
Alias: BRN3C, DFNA15, DFNA42, DFNA52, POU domain, class 4, transcription factor 3, brain-3C, brain-specific homeobox/POU domain protein 3C, brn-3C, deafness, autosomal dominant 42
Species: human
Products:     POU4F3

Top Publications

  1. Clough R, Sud R, Davis Silberman N, Hertzano R, Avraham K, Holley M, et al. Brn-3c (POU4F3) regulates BDNF and NT-3 promoter activity. Biochem Biophys Res Commun. 2004;324:372-81 pubmed
    ..Additionally, BDNF expression is reduced in the inner ear of a Brn-3c mutant mouse during embryogenesis. Our data suggest that Brn-3c may play a role in regulating neurotrophin gene expression in the inner ear. ..
  2. Pauw R, van Drunen F, Collin R, Huygen P, Kremer H, Cremers C. Audiometric characteristics of a Dutch family linked to DFNA15 with a novel mutation (p.L289F) in POU4F3. Arch Otolaryngol Head Neck Surg. 2008;134:294-300 pubmed publisher
    To report on the audiometric characteristics of a large Dutch family linked to DFNA15 with a novel mutation (p.L289F) in POU4F3 (OMIM 602460). Clinical investigation. Tertiary referral center...
  3. Vahava O, Morell R, Lynch E, Weiss S, Kagan M, Ahituv N, et al. Mutation in transcription factor POU4F3 associated with inherited progressive hearing loss in humans. Science. 1998;279:1950-4 pubmed
    ..Linkage analysis placed this deafness locus, DFNA15, on chromosome 5q31. The human homolog of mouse Pou4f3, a member of the POU-domain family of transcription factors whose targeted inactivation causes profound deafness ..
  4. Erkman L, McEvilly R, Luo L, Ryan A, Hooshmand F, O Connell S, et al. Role of transcription factors Brn-3.1 and Brn-3.2 in auditory and visual system development. Nature. 1996;381:603-6 pubmed
    ..Mutation of Brn-3.1 results in complete deafness, owing to a failure of hair cells to appear in the inner ear, with subsequent loss of cochlear and vestibular ganglia. ..
  5. Weiss S, Gottfried I, Mayrose I, Khare S, Xiang M, Dawson S, et al. The DFNA15 deafness mutation affects POU4F3 protein stability, localization, and transcriptional activity. Mol Cell Biol. 2003;23:7957-64 pubmed
    A mutation in the POU4F3 gene (BRN-3.1, BRN3C) is responsible for DFNA15 (MIM 602459), autosomal-dominant nonsyndromic hearing loss...
  6. Collin R, Chellappa R, Pauw R, Vriend G, Oostrik J, van Drunen W, et al. Missense mutations in POU4F3 cause autosomal dominant hearing impairment DFNA15 and affect subcellular localization and DNA binding. Hum Mutat. 2008;29:545-54 pubmed publisher
    ..suffering from autosomal dominant nonsyndromic hearing impairment (ADNSHI), linkage was found to the locus for DFNA15, with a two-point logarithm of the odds (LOD) score of 5.1...
  7. Kim H, Won H, Park K, Hong S, Ki C, Cho S, et al. SNP linkage analysis and whole exome sequencing identify a novel POU4F3 mutation in autosomal dominant late-onset nonsyndromic hearing loss (DFNA15). PLoS ONE. 2013;8:e79063 pubmed publisher
    ..977G>A) in the POU homeodomain of the POU4F3 gene as the candidate disease-causing mutation in the family...
  8. Lee H, Park H, Lee K, Park R, Kim U. A novel frameshift mutation of POU4F3 gene associated with autosomal dominant non-syndromic hearing loss. Biochem Biophys Res Commun. 2010;396:626-30 pubmed publisher
    Autosomal dominant mutations in the transcription factor POU4F3 gene are associated with non-syndromic hearing loss in humans; however, there have been few reports of mutations in this gene worldwide...
  9. Xiang M, Zhou L, Macke J, Yoshioka T, Hendry S, Eddy R, et al. The Brn-3 family of POU-domain factors: primary structure, binding specificity, and expression in subsets of retinal ganglion cells and somatosensory neurons. J Neurosci. 1995;15:4762-85 pubmed
    ..ganglion cells (alpha and beta cells); anti-Brn-3b immunoreactivity was present in all ganglion cells; and anti-Brn3c immunoreactivity was confined to the small ganglion cells...

More Information

Publications60

  1. Sweet E, Vemaraju S, Riley B. Sox2 and Fgf interact with Atoh1 to promote sensory competence throughout the zebrafish inner ear. Dev Biol. 2011;358:113-21 pubmed publisher
    ..resulted in ectopic expression of early markers of sensory development within 2h, and mature hair cells marked by brn3c:GFP began to accumulate 9h after heat shock...
  2. Kasica Jarosz N, Podlasz P, Kaleczyc J. Pituitary adenylate cyclase-activating polypeptide (PACAP-38) plays an inhibitory role against inflammation induced by chemical damage to zebrafish hair cells. PLoS ONE. 2018;13:e0198180 pubmed publisher
    ..zebrafish larvae of three diverse genetic lines [transgenic lines Tg(MPX:GFP) with GFP-labelled neutrophils and Tg(pou4f3:GAP-GFP) with GFP-labelled hair cells and the wild-type Tuebingen] were used to investigate an inhibitory role of ..
  3. Baker C, Modrell M. Insights into Electroreceptor Development and Evolution from Molecular Comparisons with Hair Cells. Integr Comp Biol. 2018;58:329-340 pubmed publisher
    ..electrosensory organs express transcription factors essential for hair cell development, including Atoh1 and Pou4f3. Previous hypotheses for electroreceptor evolution suggest either that electroreceptors and hair cells evolved ..
  4. Zhong C, Fu Y, Pan W, Yu J, Wang J. Atoh1 and other related key regulators in the development of auditory sensory epithelium in the mammalian inner ear: function and interplay. Dev Biol. 2019;446:133-141 pubmed publisher
    ..Next, we review the roles of Gfi1, Pou4f3, and Barhl1 in hair cell maturation and maintenance, and suggest that manipulation of these genes and their ..
  5. Mahmoudian Sani M, Jami M, Mahdavinezhad A, Amini R, Farnoosh G, Saidijam M. The Effect of the MicroRNA-183 Family on Hair Cell-Specific Markers of Human Bone Marrow-Derived Mesenchymal Stem Cells. Audiol Neurootol. 2018;23:208-215 pubmed publisher
    ..Then, the relative expression levels of SOX2, POU4F3, MYO7A, and calretinin were assayed using real-time polymerase chain reaction according to the ΔΔCt method...
  6. Kaur T, Hirose K, Rubel E, Warchol M. Macrophage recruitment and epithelial repair following hair cell injury in the mouse utricle. Front Cell Neurosci. 2015;9:150 pubmed publisher
    ..mice in which the gene for the human diphtheria toxin receptor (huDTR) was inserted under regulation of the Pou4f3 promoter. Hair cells in such mice can be selectively lesioned by systemic treatment with diphtheria toxin (DT)...
  7. Tong L, Strong M, Kaur T, Juiz J, Oesterle E, HUME C, et al. Selective deletion of cochlear hair cells causes rapid age-dependent changes in spiral ganglion and cochlear nucleus neurons. J Neurosci. 2015;35:7878-91 pubmed publisher
    ..Hair cells express the human diphtheria toxin (DT) receptor behind the Pou4f3 promoter...
  8. Badea T, Nathans J. Morphologies of mouse retinal ganglion cells expressing transcription factors Brn3a, Brn3b, and Brn3c: analysis of wild type and mutant cells using genetically-directed sparse labeling. Vision Res. 2011;51:269-79 pubmed publisher
    ..The roles of the closely related family members, Brn3a/Pou4f1 and Brn3c/Pou4f3 in RGC development are less clear...
  9. Wei Q, Zhu H, Qian X, Chen Z, Yao J, Lu Y, et al. Targeted genomic capture and massively parallel sequencing to identify novel variants causing Chinese hereditary hearing loss. J Transl Med. 2014;12:311 pubmed publisher
    ..Of the 23 probands, six had mutations in DFNA genes [WFS1 (n = 2), COCH, ACTG1, TMC1, and POU4F3] known to cause autosomal dominant nonsyndromic hearing loss...
  10. Ishii J, Sato H, Yazawa T, Shishido Hara Y, Hiramatsu C, Nakatani Y, et al. Class III/IV POU transcription factors expressed in small cell lung cancer cells are involved in proneural/neuroendocrine differentiation. Pathol Int. 2014;64:415-22 pubmed publisher
    ..Because class III POU genes (POU3F1, POU3F2, POU3F3, and POU3F4) and class IV POU genes (POU4F1, POU4F2, and POU4F3) share similar properties in neural development, we analyzed the association between class III/IV POU genes and a ..
  11. Badea T, Williams J, Smallwood P, Shi M, Motajo O, Nathans J. Combinatorial expression of Brn3 transcription factors in somatosensory neurons: genetic and morphologic analysis. J Neurosci. 2012;32:995-1007 pubmed publisher
    The three members of the Brn3 family of POU-domain transcription factors (Brn3a/Pou4f1, Brn3b/Pou4f2, and Brn3c/Pou4f3) are expressed in overlapping subsets of visual, auditory/vestibular, and somatosensory neurons...
  12. Frenz S, Rak K, Völker J, Jürgens L, Scherzad A, Schendzielorz P, et al. Mosaic pattern of Cre recombinase expression in cochlear outer hair cells of the Brn3.1 Cre mouse. Neuroreport. 2015;26:309-13 pubmed publisher
    ..Mutation leads to nonsyndromic human progressive hearing loss (DFNA15). To investigate the suitability of the Brn3...
  13. Costa A, Powell L, Lowell S, Jarman A. Atoh1 in sensory hair cell development: constraints and cofactors. Semin Cell Dev Biol. 2017;65:60-68 pubmed publisher
    ..We particularly focus on the possible roles of Gfi1 and Pou4f3, drawing from studies in mouse, Drosophila and C. elegans.
  14. Hanovice N, McMains E, Gross J. A GAL4-inducible transgenic tool kit for the in vivo modulation of Rho GTPase activity in zebrafish. Dev Dyn. 2016;245:844-53 pubmed publisher
    ..Construct expression was confined to proper cells when combined with pou4f3:gal4 or ptf1a:gal4...
  15. Costa A, Sánchez Guardado L, Juniat S, Gale J, Daudet N, Henrique D. Generation of sensory hair cells by genetic programming with a combination of transcription factors. Development. 2015;142:1948-59 pubmed publisher
    ..Here, we show that combined expression of the transcription factors Gfi1, Pou4f3 and Atoh1 can induce direct programming towards HC fate, both during in vitro mouse embryonic stem cell ..
  16. Kopecky B, DeCook R, Fritzsch B. N-Myc and L-Myc are essential for hair cell formation but not maintenance. Brain Res. 2012;1484:1-14 pubmed publisher
    ..shown that the deletion of Atoh1 results in embryonic loss of hair cells while the absence of Barhl1, Gfi1, and Pou4f3 leads to the progressive loss of hair cells in newborn mice...
  17. Zak M, van Oort T, Hendriksen F, Garcia M, Vassart G, Grolman W. LGR4 and LGR5 Regulate Hair Cell Differentiation in the Sensory Epithelium of the Developing Mouse Cochlea. Front Cell Neurosci. 2016;10:186 pubmed publisher
    ..cells are likely formed due to an up-regulation of the "pro-hair cell" transcription factors Atoh1, Nhlh1, and Pou4f3. Using a hypomorphic Lgr4 mouse model we showed a mild overproduction of OHCs in the heterozygous and homozygous ..
  18. Kaur T, Zamani D, Tong L, Rubel E, Ohlemiller K, Hirose K, et al. Fractalkine Signaling Regulates Macrophage Recruitment into the Cochlea and Promotes the Survival of Spiral Ganglion Neurons after Selective Hair Cell Lesion. J Neurosci. 2015;35:15050-61 pubmed publisher
    ..mouse model in which the human diphtheria toxin receptor (huDTR) is selectively expressed under the control of Pou4f3, a hair cell-specific transcription factor...
  19. Kopecky B, Jahan I, Fritzsch B. Correct timing of proliferation and differentiation is necessary for normal inner ear development and auditory hair cell viability. Dev Dyn. 2013;242:132-47 pubmed publisher
    ..be due to mis-regulation of genes necessary for neurosensory formation and maintenance, such as Neurod1, Atoh1, Pou4f3, and Barhl1...
  20. Hickox A, Wong A, Pak K, Strojny C, Ramirez M, Yates J, et al. Global Analysis of Protein Expression of Inner Ear Hair Cells. J Neurosci. 2017;37:1320-1339 pubmed publisher
    ..By using Pou4f3/eGFP-transgenic mice in which HCs express GFP driven by Pou4f3, we FACS purified a population of HCs to analyze ..
  21. Li X, Gaillard F, Monckton E, Glubrecht D, Persad A, Moser M, et al. Loss of AP-2delta reduces retinal ganglion cell numbers and axonal projections to the superior colliculus. Mol Brain. 2016;9:62 pubmed publisher
    ..AP-2? knockout results in loss of Brn3c (Pou4f3) expression in AP-2? -positive RGCs...
  22. Masuda M, Li Y, Pak K, Chavez E, Mullen L, Ryan A. The Promoter and Multiple Enhancers of the pou4f3 Gene Regulate Expression in Inner Ear Hair Cells. Mol Neurobiol. 2017;54:5414-5426 pubmed publisher
    ..of enhanced green fluorescent protein (eGFP) reporter constructs and bioinformatics, we evaluated the control of pou4f3 gene expression, since it is expressed only in HCs within the inner ear and continues to be expressed throughout ..
  23. Walters B, Coak E, Dearman J, Bailey G, Yamashita T, Kuo B, et al. In Vivo Interplay between p27Kip1, GATA3, ATOH1, and POU4F3 Converts Non-sensory Cells to Hair Cells in Adult Mice. Cell Rep. 2017;19:307-320 pubmed publisher
    ..Co-activation of GATA3 or POU4F3 and ATOH1 promoted conversion of SCs to HCs in adult mice...
  24. Pan N, Jahan I, Kersigo J, Duncan J, Kopecky B, Fritzsch B. A novel Atoh1 "self-terminating" mouse model reveals the necessity of proper Atoh1 level and duration for hair cell differentiation and viability. PLoS ONE. 2012;7:e30358 pubmed publisher
    ..Gene expression analyses of Atoh1, Barhl1 and Pou4f3, genes required for survival and maturation of hair cells, reveal earlier and higher expression levels in the ..
  25. Weatherstone J, Kopp Scheinpflug C, Pilati N, Wang Y, Forsythe I, Rubel E, et al. Maintenance of neuronal size gradient in MNTB requires sound-evoked activity. J Neurophysiol. 2017;117:756-766 pubmed publisher
    ..was corroborated by selective elimination of auditory hair cell activity with either hair cell elimination in Pou4f3 DTR mice or inner ear tetrodotoxin (TTX) treatment...
  26. Jahan I, Pan N, Kersigo J, Fritzsch B. Neurog1 can partially substitute for Atoh1 function in hair cell differentiation and maintenance during organ of Corti development. Development. 2015;142:2810-21 pubmed publisher
    ..Replacement of Atoh1 with Neurog1 maintains limited expression of Pou4f3 and Barhl1 and rescues HCs quantitatively, but not qualitatively...
  27. Sajgo S, Ali S, Popescu O, Badea T. Dynamic expression of transcription factor Brn3b during mouse cranial nerve development. J Comp Neurol. 2016;524:1033-61 pubmed publisher
    ..Brn3a, Brn3b, and Brn3c, in combination with each other and/or transcription factors of other families, can define subgroups of retinal ..
  28. Chang J, Choi J, Rah Y, Yoo M, Oh K, Im G, et al. Sodium Selenite Acts as an Otoprotectant against Neomycin-Induced Hair Cell Damage in a Zebrafish Model. PLoS ONE. 2016;11:e0151557 pubmed publisher
    ..we investigated the effect of sodium selenite on neomycin ototoxicity in wild-type and transgenic zebrafish (Brn3C: EGFP)...
  29. Monroe J, Manning D, Uribe P, Bhandiwad A, Sisneros J, Smith M, et al. Hearing sensitivity differs between zebrafish lines used in auditory research. Hear Res. 2016;341:220-231 pubmed publisher
    ..We previously observed reduced auditory sensitivity in adult Brn3c:mGFP transgenic zebrafish, which express membrane-bound green fluorescent protein (GFP) in sensory hair cells...
  30. Bu F, Peng Y, Wang S, Pan Q, Hu Z, Gong H, et al. [Mutation screening of 20 candidate genes located in chromo-some 5q31-5q32 for DFNA52 locus]. Yi Chuan. 2009;31:43-9 pubmed
    Previously, we mapped the DFNA52 (OMIM: 607683) locus to an 8.8 cM interval between STR D5S2056 and D5S638 on human chromosome 5q31.1-q32 in a large consanguineous Chinese family with congenital sensorineural hearing loss...
  31. Sayyad Z, Sirohi K, Radha V, Swarup G. 661W is a retinal ganglion precursor-like cell line in which glaucoma-associated optineurin mutants induce cell death selectively. Sci Rep. 2017;7:16855 pubmed publisher
    ..this cell line expressed certain markers specific to retinal ganglion cells such as Rbpms, Brn3b (Pou4f2), Brn3c (Pou4f3), Thy1 and ?-synuclein (Sncg), and some other markers of neuronal cells (beta-III tubulin, NeuN and MAP2)...
  32. de Heer A, Huygen P, Collin R, Kremer H, Cremers C. Mild and variable audiometric and vestibular features in a third DFNA15 family with a novel mutation in POU4F3. Ann Otol Rhinol Laryngol. 2009;118:313-20 pubmed
    Cochleovestibular characteristics were investigated in a Dutch DFNA15 family with a novel POU4F3 mutation, L223P. A 4-generation pedigree was constructed of the Dutch family with the novel L223P POU4F3 mutation...
  33. Parmhans N, Sajgo S, Niu J, Luo W, Badea T. Characterization of retinal ganglion cell, horizontal cell, and amacrine cell types expressing the neurotrophic receptor tyrosine kinase Ret. J Comp Neurol. 2018;526:742-766 pubmed publisher
    ..Ret expression overlaps with Brn3a in 4 RGC types, with Brn3b in 5 RGC types, and with Brn3c in one RGC type, respectively...
  34. Freitas E, Oiticica J, Silva A, Bittar R, Rosenberg C, Mingroni Netto R. Deletion of the entire POU4F3 gene in a familial case of autosomal dominant non-syndromic hearing loss. Eur J Med Genet. 2014;57:125-8 pubmed publisher
    ..A deletion in the 5q32 region encompassing only one gene, POU4F3, which corresponds to DFNA15, was detected in one family...
  35. Kitano T, Miyagawa M, Nishio S, Moteki H, Oda K, Ohyama K, et al. POU4F3 mutation screening in Japanese hearing loss patients: Massively parallel DNA sequencing-based analysis identified novel variants associated with autosomal dominant hearing loss. PLoS ONE. 2017;12:e0177636 pubmed publisher
    A variant in a transcription factor gene, POU4F3, is responsible for autosomal dominant nonsyndromic hereditary hearing loss, DFNA15...
  36. Leonard J, Cook A, Van Gele M, Boyle G, Inglis K, Speleman F, et al. Proneural and proneuroendocrine transcription factor expression in cutaneous mechanoreceptor (Merkel) cells and Merkel cell carcinoma. Int J Cancer. 2002;101:103-10 pubmed
  37. Lin Y, Lin Y, Lu Y, Liu T, Chen C, Hsu C, et al. A novel missense variant in the nuclear localization signal of POU4F3 causes autosomal dominant non-syndromic hearing loss. Sci Rep. 2017;7:7551 pubmed publisher
    ..a massively parallel sequencing panel targeting 159 deafness genes, we identified a novel missense variant of POU4F3 (c.982A>G, p...
  38. Cai X, Li Y, Xia L, Peng Y, He C, Jiang L, et al. Exome sequencing identifies POU4F3 as the causative gene for a large Chinese family with non-syndromic hearing loss. J Hum Genet. 2017;62:317-320 pubmed publisher
    ..have focused on this pedigree since 2002, and we have mapped a deafness locus named DFNA42 (which has been renamed DFNA52, OMIM entry 607683) via a genome-wide scan...
  39. Requena T, Espinosa Sanchez J, Lopez Escamez J. Genetics of dizziness: cerebellar and vestibular disorders. Curr Opin Neurol. 2014;27:98-104 pubmed publisher
    ..We have summarized clinical and molecular genetics findings in neuro-otolology during the last 2 years...
  40. Zhang C, Wang M, Xiao Y, Zhang F, Zhou Y, Li J, et al. A Novel Nonsense Mutation of POU4F3 Gene Causes Autosomal Dominant Hearing Loss. Neural Plast. 2016;2016:1512831 pubmed publisher
    i>POU4F3 gene encodes a transcription factor which plays an essential role in the maturation and maintenance of hair cells in cochlea and vestibular system...
  41. Ikeda R, Pak K, Chavez E, Ryan A. Transcription factors with conserved binding sites near ATOH1 on the POU4F3 gene enhance the induction of cochlear hair cells. Mol Neurobiol. 2015;51:672-84 pubmed
    ..Evaluating DNA 5Œ to the coding sequence of the pou4f3 gene, a target of ATOH1 in HCs, we identified in three regions containing clustered binding sites for ATOH1 and ..
  42. Zhang L, Wahlin K, Li Y, Masuda T, Yang Z, Zack D, et al. RIT2, a neuron-specific small guanosine triphosphatase, is expressed in retinal neuronal cells and its promoter is modulated by the POU4 transcription factors. Mol Vis. 2013;19:1371-86 pubmed
  43. van Drunen F, Pauw R, Collin R, Kremer H, Huygen P, Cremers C. Vestibular impairment in a Dutch DFNA15 family with an L289F mutation in POU4F3. Audiol Neurootol. 2009;14:303-7 pubmed publisher
    ..This is the second DFNA15 family worldwide to have a novel L289F mutation in POU4F3. Vestibular involvement appeared to be present in 2 affected individuals according to their medical history...
  44. He L, Pang X, Chen P, Wu H, Yang T. Mutation in the Hair Cell Specific Gene POU4F3 Is a Common Cause for Autosomal Dominant Nonsyndromic Hearing Loss in Chinese Hans. Neural Plast. 2016;2016:9890827 pubmed publisher
    ..So far the genetic etiological contribution of the gene POU4F3 associated with ADNSHL has been rarely reported. In our previous study, a c...
  45. Ouji Y, Sakagami M, Omori H, Higashiyama S, Kawai N, Kitahara T, et al. Efficient induction of inner ear hair cell-like cells from mouse ES cells using combination of Math1 transfection and conditioned medium from ST2 stromal cells. Stem Cell Res. 2017;23:50-56 pubmed publisher
    ..to generate approximately 30% HC-like cells expressing HC-related markers (myosin6, myosin7a, calretinin, ?9AchR, Brn3c), which showed remarkable formation of stereocilia-like structures...
  46. Qiong P, Hu Z, Feng Y, Pan Q, Xia J, Xia K. Bioinformatics analysis of candidate genes and mutations in a congenital sensorineural hearing loss pedigree: detection of 52 genes for the DFNA52 locus. J Laryngol Otol. 2008;122:1029-36 pubmed publisher
    Previously, we have mapped the DFNA52 (Online Mendelian Inheritance in Man (OMIM) 607683) locus, using an 8.8-cM interval on the human chromosome 5q31...
  47. Frenz C, Lefebvre P. Molecular modelling insights into DFNA15 mediated enhancement of POU4F3 stability. Int J Comput Biol Drug Des. 2008;1:295-301 pubmed
    ..The DFNA15 truncation mutation has been demonstrated to result in a loss of transcriptional activity, but an increase in the ..
  48. Xu X, Yang Q, Jiao J, He L, Yu S, Wang J, et al. Genetic Variation in POU4F3 and GRHL2 Associated with Noise-Induced Hearing Loss in Chinese Population: A Case-Control Study. Int J Environ Res Public Health. 2016;13: pubmed publisher
    ..The purpose of this study was to examine whether genetic variations in POU4F3 and GRHL2 may influence susceptibility to NIHL in the Chinese population...
  49. Tornari C, Towers E, Gale J, Dawson S. Regulation of the orphan nuclear receptor Nr2f2 by the DFNA15 deafness gene Pou4f3. PLoS ONE. 2014;9:e112247 pubmed publisher
    Hair cells are the mechanotransducing cells of the inner ear that are essential for hearing and balance. POU4F3--a POU-domain transcription factor selectively expressed by these cells--has been shown to be essential for hair cell ..
  50. Frejo L, Giegling I, Teggi R, Lopez Escamez J, Rujescu D. Genetics of vestibular disorders: pathophysiological insights. J Neurol. 2016;263 Suppl 1:S45-53 pubmed publisher
    ..Some Mendelian sensorineural hearing loss also exhibits vestibular dysfunction, including DFNA9, DFNA11, DFNA15 and DFNA28...
  51. Nolan L, Jagutpal S, Cadge B, Woo P, Dawson S. Identification and functional analysis of common sequence variants in the DFNA15 gene, Brn-3c. Gene. 2007;400:89-97 pubmed
    A rare mutation in Brn-3c (Brn3.1, POU4F3) underlies adult onset hearing loss (DFNA15) and targeted deletion of this gene in mice leads to complete deafness due to loss of sensory hair cells from the cochlea...