메뉴 건너뛰기




Volumn 12, Issue 3, 2016, Pages 135-149

Auditory neuropathy-neural and synaptic mechanisms

Author keywords

[No Author keywords available]

Indexed keywords

CALCIUM CHANNEL; DYNAMIN; EXCITATORY AMINO ACID TRANSPORTER; MEMBRANE PROTEIN; OTOFERLIN PROTEIN; PERIPHERAL MYELIN PROTEIN 22; PROTEIN DFNB59; PROTEIN DIAPH3; PROTEIN OPA1; REGULATOR PROTEIN; SYNAPTOTAGMIN I; THIAMINE; UNCLASSIFIED DRUG; VESICULAR GLUTAMATE TRANSPORTER 3;

EID: 84959368381     PISSN: 17594758     EISSN: 17594766     Source Type: Journal    
DOI: 10.1038/nrneurol.2016.10     Document Type: Review
Times cited : (247)

References (143)
  • 1
    • 33847662000 scopus 로고    scopus 로고
    • World Health Organization. Advanced level. [online
    • World Health Organization. Primary ear and hearing care training resource. Advanced level. [online], http://www.who.int/pbd/deafness/activities/hearing-care/advanced.pdf (2006
    • (2006) Primary Ear and Hearing Care Training Resource
  • 3
    • 19344362785 scopus 로고    scopus 로고
    • Perceptual consequences of disrupted auditory nerve activity
    • Zeng F. G., Kong Y. Y., Michalewski H. J., & Starr A. Perceptual consequences of disrupted auditory nerve activity. J. Neurophysiol. 93, 3050-3063 (2005
    • (2005) J. Neurophysiol , vol.93 , pp. 3050-3063
    • Zeng, F.G.1    Kong, Y.Y.2    Michalewski, H.J.3    Starr, A.4
  • 4
    • 33845387728 scopus 로고    scopus 로고
    • Diagnostik und therapie der auditorischen synaptopathie/neuropathie
    • in German
    • Moser T., et al. Diagnostik und therapie der auditorischen synaptopathie/neuropathie. HNO 54, 833-841 (in German) (2006
    • (2006) HNO , vol.54 , pp. 833-841
    • Moser, T.1
  • 7
    • 84881516385 scopus 로고    scopus 로고
    • Prevalence of auditory neuropathy spectrum disorder in an auditory health care service
    • Penido R. C., & Isaac M. L. Prevalence of auditory neuropathy spectrum disorder in an auditory health care service. Braz. J. Otorhinolaryngol. 79, 429-433 (2013
    • (2013) Braz. J. Otorhinolaryngol , vol.79 , pp. 429-433
    • Penido, R.C.1    Isaac, M.L.2
  • 8
    • 0033003831 scopus 로고    scopus 로고
    • Clinical findings for a group of infants and young children with auditory neuropathy
    • Rance G., et al. Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear. 20, 238-252 (1999
    • (1999) Ear Hear , vol.20 , pp. 238-252
    • Rance, G.1
  • 9
    • 33744986774 scopus 로고    scopus 로고
    • Prevalence of auditory neuropathy/synaptopathy in a population of children with profound hearing loss
    • Foerst A., et al. Prevalence of auditory neuropathy/synaptopathy in a population of children with profound hearing loss. Int. J. Pediatr. Otorhinolaryngol. 70, 1415-1422 (2006
    • (2006) Int. J. Pediatr. Otorhinolaryngol , vol.70 , pp. 1415-1422
    • Foerst, A.1
  • 10
    • 44849114793 scopus 로고    scopus 로고
    • A multicenter study on the prevalence and spectrum of mutations in the otoferlin gene (otof) in subjects with nonsyndromic hearing impairment and auditory neuropathy
    • Rodríguez-Ballesteros M., et al. A multicenter study on the prevalence and spectrum of mutations in the otoferlin gene (OTOF) in subjects with nonsyndromic hearing impairment and auditory neuropathy. Hum. Mutat. 29, 823-831 (2008
    • (2008) Hum. Mutat , vol.29 , pp. 823-831
    • Rodríguez-Ballesteros, M.1
  • 12
    • 78650515181 scopus 로고    scopus 로고
    • The diverse roles of ribbon synapses in sensory neurotransmission
    • Matthews G., & Fuchs P. The diverse roles of ribbon synapses in sensory neurotransmission. Nat. Rev. Neurosci. 11, 812-822 (2010
    • (2010) Nat. Rev. Neurosci , vol.11 , pp. 812-822
    • Matthews, G.1    Fuchs, P.2
  • 13
    • 0041104621 scopus 로고    scopus 로고
    • Loss of function mutations in a calcium-channel α1-subunit gene in Xp11.23 cause incomplete X linked congenital stationary night blindness
    • Bech-Hansen N. T., et al. Loss of function mutations in a calcium-channel α1-subunit gene in Xp11.23 cause incomplete X linked congenital stationary night blindness. Nat. Genet. 19, 264-267 (1998
    • (1998) Nat. Genet , vol.19 , pp. 264-267
    • Bech-Hansen, N.T.1
  • 14
    • 17344366487 scopus 로고    scopus 로고
    • An L type calcium-channel gene mutated in incomplete X linked congenital stationary night blindness
    • Strom T. M., et al. An L type calcium-channel gene mutated in incomplete X linked congenital stationary night blindness. Nat. Genet. 19, 260-263 (1998
    • (1998) Nat. Genet , vol.19 , pp. 260-263
    • Strom, T.M.1
  • 15
    • 33749005104 scopus 로고    scopus 로고
    • Mutations in CABP4, the gene encoding the Ca2+-binding protein 4, cause autosomal recessive night blindness
    • Zeitz C., et al. Mutations in CABP4, the gene encoding the Ca2+-binding protein 4, cause autosomal recessive night blindness. Am. J. Hum. Genet. 79, 657-667 (2006
    • (2006) Am. J. Hum. Genet , vol.79 , pp. 657-667
    • Zeitz, C.1
  • 16
    • 17244366230 scopus 로고    scopus 로고
    • Hair cell synaptic ribbons are essential for synchronous auditory signalling
    • Khimich D., et al. Hair cell synaptic ribbons are essential for synchronous auditory signalling. Nature 434, 889-894 (2005
    • (2005) Nature , vol.434 , pp. 889-894
    • Khimich, D.1
  • 17
    • 77953204546 scopus 로고    scopus 로고
    • Onset coding is degraded in auditory nerve fibers from mutant mice lacking synaptic ribbons
    • Buran B. N., et al. Onset coding is degraded in auditory nerve fibers from mutant mice lacking synaptic ribbons. J. Neurosci. 30, 7587-7597 (2010
    • (2010) J. Neurosci , vol.30 , pp. 7587-7597
    • Buran, B.N.1
  • 18
    • 84946500434 scopus 로고    scopus 로고
    • Disruption of adaptor protein 2 (AP 2) in cochlear hair cells impairs vesicle reloading of synaptic release sites and hearing
    • Jung S., et al. Disruption of adaptor protein 2 (AP 2) in cochlear hair cells impairs vesicle reloading of synaptic release sites and hearing. EMBO J. 34, 2686-2702 (2015
    • (2015) EMBO J. , vol.34 , pp. 2686-2702
    • Jung, S.1
  • 19
    • 77954143206 scopus 로고    scopus 로고
    • Hearing requires otoferlin-dependent efficient replenishment of synaptic vesicles in hair cells
    • Pangrsic T., et al. Hearing requires otoferlin-dependent efficient replenishment of synaptic vesicles in hair cells. Nat. Neurosci. 13, 869-876 (2010
    • (2010) Nat. Neurosci , vol.13 , pp. 869-876
    • Pangrsic, T.1
  • 20
    • 0034863510 scopus 로고    scopus 로고
    • Mutant β-spectrin 4 causes auditory and motor neuropathies in quivering mice
    • Parkinson N. J., et al. Mutant β-spectrin 4 causes auditory and motor neuropathies in quivering mice. Nat. Genet. 29, 61-65 (2001
    • (2001) Nat. Genet , vol.29 , pp. 61-65
    • Parkinson, N.J.1
  • 21
    • 4644286228 scopus 로고    scopus 로고
    • ΒIV sigma;1 spectrin stabilizes the nodes of ranvier and axon initial segments
    • Lacas-Gervais S., et al. βIVsigma;1 spectrin stabilizes the nodes of Ranvier and axon initial segments. J. Cell Biol. 166, 983-990 (2004
    • (2004) J. Cell Biol , vol.166 , pp. 983-990
    • Lacas-Gervais, S.1
  • 22
    • 84933674259 scopus 로고    scopus 로고
    • Relating structure and function of inner hair cell ribbon synapses
    • Wichmann C., & Moser T. Relating structure and function of inner hair cell ribbon synapses. Cell Tissue Res. 361, 95-114 (2015
    • (2015) Cell Tissue Res , vol.361 , pp. 95-114
    • Wichmann, C.1    Moser, T.2
  • 23
    • 22144466901 scopus 로고    scopus 로고
    • Time and intensity coding at the hair cell's ribbon synapse
    • Fuchs P. A. Time and intensity coding at the hair cell's ribbon synapse. J. Physiol. 566, 7-12 (2005
    • (2005) J. Physiol , vol.566 , pp. 7-12
    • Fuchs, P.A.1
  • 24
    • 33748886717 scopus 로고    scopus 로고
    • Mechanisms underlying the temporal precision of sound coding at the inner hair cell ribbon synapse
    • Moser T., Neef A., & Khimich D. Mechanisms underlying the temporal precision of sound coding at the inner hair cell ribbon synapse. J. Physiol. 576, 55-62 (2006
    • (2006) J. Physiol , vol.576 , pp. 55-62
    • Moser, T.1    Neef, A.2    Khimich, D.3
  • 25
    • 84867906699 scopus 로고    scopus 로고
    • Otoferlin: A multi C2 domain protein essential for hearing
    • Pangršič, T., Reisinger E., & Moser T. Otoferlin: a multi C2 domain protein essential for hearing. Trends Neurosci. 35, 671-680 (2012
    • (2012) Trends Neurosci , vol.35 , pp. 671-680
    • Pangršič, T.1    Reisinger, E.2    Moser, T.3
  • 26
    • 79953219741 scopus 로고    scopus 로고
    • Exocytosis at the hair cell ribbon synapse apparently operates without neuronal snare proteins
    • Nouvian R., et al. Exocytosis at the hair cell ribbon synapse apparently operates without neuronal SNARE proteins. Nat. Neurosci. 14, 411-413 (2011
    • (2011) Nat. Neurosci , vol.14 , pp. 411-413
    • Nouvian, R.1
  • 27
    • 67649342625 scopus 로고    scopus 로고
    • Complexin i is required for high-fidelity transmission at the endbulb of held auditory synapse
    • Strenzke N., et al. Complexin I is required for high-fidelity transmission at the endbulb of held auditory synapse. J. Neurosci. 29, 7991-8004 (2009
    • (2009) J. Neurosci , vol.29 , pp. 7991-8004
    • Strenzke, N.1
  • 28
    • 77956850256 scopus 로고    scopus 로고
    • Molecular anatomy of the hair cell's ribbon synapse
    • Uthaiah R. C., & Hudspeth A. J. Molecular anatomy of the hair cell's ribbon synapse. J. Neurosci. 30, 12387-12399 (2010
    • (2010) J. Neurosci , vol.30 , pp. 12387-12399
    • Uthaiah, R.C.1    Hudspeth, A.J.2
  • 29
    • 84924704521 scopus 로고    scopus 로고
    • Unconventional molecular regulation of synaptic vesicle replenishment in cochlear inner hair cells
    • Vogl C., et al. Unconventional molecular regulation of synaptic vesicle replenishment in cochlear inner hair cells. J. Cell. Sci. 128, 638-644 (2015
    • (2015) J. Cell. Sci , vol.128 , pp. 638-644
    • Vogl, C.1
  • 30
    • 33749994043 scopus 로고    scopus 로고
    • Otoferlin, defective in a human deafness form, is essential for exocytosis at the auditory ribbon synapse
    • Roux I., et al. Otoferlin, defective in a human deafness form, is essential for exocytosis at the auditory ribbon synapse. Cell 127, 277-289 (2006
    • (2006) Cell , vol.127 , pp. 277-289
    • Roux, I.1
  • 31
    • 38149120796 scopus 로고    scopus 로고
    • Sensorineural deafness and seizures in mice lacking vesicular glutamate transporter 3
    • Seal R. P., et al. Sensorineural deafness and seizures in mice lacking vesicular glutamate transporter 3. Neuron 57, 263-275 (2008
    • (2008) Neuron , vol.57 , pp. 263-275
    • Seal, R.P.1
  • 32
    • 48349111572 scopus 로고    scopus 로고
    • Impairment of slc17a8 encoding vesicular glutamate transporter 3, vglut3, underlies nonsyndromic deafness dfna25 and inner hair cell dysfunction in null mice
    • Ruel J., et al. Impairment of SLC17A8 encoding vesicular glutamate transporter 3, VGLUT3, underlies nonsyndromic deafness DFNA25 and inner hair cell dysfunction in null mice. Am. J. Hum. Genet. 83, 278-292 (2008
    • (2008) Am. J. Hum. Genet , vol.83 , pp. 278-292
    • Ruel, J.1
  • 33
    • 0034616911 scopus 로고    scopus 로고
    • Congenital deafness and sinoatrial node dysfunction in mice lacking class D L type Ca2+ channels
    • Platzer J., et al. Congenital deafness and sinoatrial node dysfunction in mice lacking class D L type Ca2+ channels. Cell 102, 89-97 (2000
    • (2000) Cell , vol.102 , pp. 89-97
    • Platzer, J.1
  • 34
    • 0345491881 scopus 로고    scopus 로고
    • Cav1 3 channels are essential for development and presynaptic activity of cochlear inner hair cells
    • Brandt A., Striessnig J., & Moser T. Cav1. 3 channels are essential for development and presynaptic activity of cochlear inner hair cells. J. Neurosci. 23, 10832-10840 (2003
    • (2003) J. Neurosci , vol.23 , pp. 10832-10840
    • Brandt, A.1    Striessnig, J.2    Moser, T.3
  • 37
    • 0036686046 scopus 로고    scopus 로고
    • Dendrotoxin-sensitive K+ currents contribute to accommodation in murine spiral ganglion neurons
    • Mo Z. L., Adamson C. L., & Davis R. L. Dendrotoxin-sensitive K+ currents contribute to accommodation in murine spiral ganglion neurons. J. Physiol. 542, 763 (2002
    • (2002) J. Physiol , vol.542 , pp. 763
    • Mo, Z.L.1    Adamson, C.L.2    Davis, R.L.3
  • 38
    • 84859318083 scopus 로고    scopus 로고
    • Spike encoding of neurotransmitter release timing by spiral ganglion neurons of the cochlea
    • Rutherford M. A., Chapochnikov N. M., & Moser T. Spike encoding of neurotransmitter release timing by spiral ganglion neurons of the cochlea. J. Neurosci. 32, 4773-4789 (2012
    • (2012) J. Neurosci , vol.32 , pp. 4773-4789
    • Rutherford, M.A.1    Chapochnikov, N.M.2    Moser, T.3
  • 39
    • 0036159084 scopus 로고    scopus 로고
    • Transmitter release at the hair cell ribbon synapse
    • Glowatzki E., & Fuchs P. A. Transmitter release at the hair cell ribbon synapse. Nat. Neurosci. 5, 147-154 (2002
    • (2002) Nat. Neurosci , vol.5 , pp. 147-154
    • Glowatzki, E.1    Fuchs, P.A.2
  • 40
    • 84909638235 scopus 로고    scopus 로고
    • Uniquantal release through a dynamic fusion pore is a candidate mechanism of hair cell exocytosis
    • Chapochnikov N. M., et al. Uniquantal release through a dynamic fusion pore is a candidate mechanism of hair cell exocytosis. Neuron 17, 1389-1403 (2014
    • (2014) Neuron , vol.17 , pp. 1389-1403
    • Chapochnikov, N.M.1
  • 41
    • 22544439641 scopus 로고    scopus 로고
    • Where is the spike generator of the cochlear nerve? Voltage-gated sodium channels in the mouse cochlea
    • Hossain W. A., Antic S. D., Yang Y., Rasband M. N., & Morest D. K. Where is the spike generator of the cochlear nerve? Voltage-gated sodium channels in the mouse cochlea. J. Neurosci. 25, 6857-6868 (2005
    • (2005) J. Neurosci , vol.25 , pp. 6857-6868
    • Hossain, W.A.1    Antic, S.D.2    Yang, Y.3    Rasband, M.N.4    Morest, D.K.5
  • 42
    • 0032947634 scopus 로고    scopus 로고
    • A mutation in OTOF, encoding otoferlin, a FER 1 like protein, causes DFNB9, a nonsyndromic form of deafness
    • Yasunaga S., et al. A mutation in OTOF, encoding otoferlin, a FER 1 like protein, causes DFNB9, a nonsyndromic form of deafness. Nat. Genet. 21, 363-369 (1999
    • (1999) Nat. Genet , vol.21 , pp. 363-369
    • Yasunaga, S.1
  • 43
    • 33745907313 scopus 로고    scopus 로고
    • OTOF mutations revealed by genetic analysis of hearing loss families including a potential temperature sensitive auditory neuropathy allele
    • Varga R., et al. OTOF mutations revealed by genetic analysis of hearing loss families including a potential temperature sensitive auditory neuropathy allele. J. Med. Genet. 43, 576-581 (2006
    • (2006) J. Med. Genet , vol.43 , pp. 576-581
    • Varga, R.1
  • 44
    • 77950519874 scopus 로고    scopus 로고
    • Temperature-sensitive auditory neuropathy associated with an otoferlin mutation: Deafening fever! Biochem
    • Marlin S., et al. Temperature-sensitive auditory neuropathy associated with an otoferlin mutation: deafening fever! Biochem. Biophys. Res. Commun. 394, 737-742 (2010
    • (2010) Biophys. Res. Commun , vol.394 , pp. 737-742
    • Marlin, S.1
  • 45
    • 77952643063 scopus 로고    scopus 로고
    • Screening mutations of OTOF gene in Chinese patients with auditory neuropathy, including a familial case of temperature-sensitive auditory neuropathy
    • Wang D. Y., et al. Screening mutations of OTOF gene in Chinese patients with auditory neuropathy, including a familial case of temperature-sensitive auditory neuropathy. BMC Med. Genet. 11, 79 (2010
    • (2010) BMC Med. Genet , vol.11 , pp. 79
    • Wang, D.Y.1
  • 46
    • 68449091256 scopus 로고    scopus 로고
    • Novel OTOF mutations in Brazilian patients with auditory neuropathy
    • Romanos J., et al. Novel OTOF mutations in Brazilian patients with auditory neuropathy. J. Hum. Genet. 54, 382-385 (2009
    • (2009) J. Hum. Genet , vol.54 , pp. 382-385
    • Romanos, J.1
  • 47
    • 84867658612 scopus 로고    scopus 로고
    • A prevalent founder mutation and genotype-phenotype correlations of OTOF in Japanese patients with auditory neuropathy
    • Matsunaga T., et al. A prevalent founder mutation and genotype-phenotype correlations of OTOF in Japanese patients with auditory neuropathy. Clin. Genet. 82, 425-432 (2012
    • (2012) Clin. Genet , vol.82 , pp. 425-432
    • Matsunaga, T.1
  • 48
    • 20344402550 scopus 로고    scopus 로고
    • An emergency response team for membrane repair
    • McNeil P. L., & Kirchhausen T. An emergency response team for membrane repair. Nat. Rev. Mol. Cell. Biol. 6, 499-505 (2005
    • (2005) Nat. Rev. Mol. Cell. Biol , vol.6 , pp. 499-505
    • McNeil, P.L.1    Kirchhausen, T.2
  • 49
    • 17344365600 scopus 로고    scopus 로고
    • Dysferlin, a novel skeletal muscle gene, is mutated in Miyoshi myopathy and limb girdle muscular dystrophy
    • Liu J., et al. Dysferlin, a novel skeletal muscle gene, is mutated in Miyoshi myopathy and limb girdle muscular dystrophy. Nat. Genet. 20, 31-36 (1998
    • (1998) Nat. Genet , vol.20 , pp. 31-36
    • Liu, J.1
  • 50
    • 0037738510 scopus 로고    scopus 로고
    • Defective membrane repair in dysferlin-deficient muscular dystrophy
    • Bansal D., et al. Defective membrane repair in dysferlin-deficient muscular dystrophy. Nature 423, 168-172 (2003
    • (2003) Nature , vol.423 , pp. 168-172
    • Bansal, D.1
  • 51
    • 34547792309 scopus 로고    scopus 로고
    • In silico functional and structural characterisation of ferlin proteins by mapping disease-causing mutations and evolutionary information onto three-dimensional models of their C2 domains
    • Jim?nez J. L., & Bashir R. In silico functional and structural characterisation of ferlin proteins by mapping disease-causing mutations and evolutionary information onto three-dimensional models of their C2 domains. J. Neurol. Sci. 260, 114-123 (2007
    • (2007) J. Neurol. Sci , vol.260 , pp. 114-123
    • Jimnez, J.L.1    Bashir, R.2
  • 52
    • 77957731333 scopus 로고    scopus 로고
    • Otoferlin is a calcium sensor that directly regulates SNARE-mediated membrane fusion
    • Johnson C. P., & Chapman E. R. Otoferlin is a calcium sensor that directly regulates SNARE-mediated membrane fusion. J. Cell Biol. 191, 187-197 (2010
    • (2010) J. Cell Biol , vol.191 , pp. 187-197
    • Johnson, C.P.1    Chapman, E.R.2
  • 53
    • 84905695329 scopus 로고    scopus 로고
    • Characterization of the lipid binding properties of Otoferlin reveals specific interactions between PI 4 5)P2 and the C2C and C2F domains
    • Padmanarayana M., et al. Characterization of the lipid binding properties of Otoferlin reveals specific interactions between PI(4,5)P2 and the C2C and C2F domains. Biochemistry 53, 5023-5033 (2014
    • (2014) Biochemistry , vol.53 , pp. 5023-5033
    • Padmanarayana, M.1
  • 54
    • 59449093847 scopus 로고    scopus 로고
    • Direct interaction of otoferlin with syntaxin 1A SNAP 25, and the L type voltage-gated calcium channel Cav1.3
    • Ramakrishnan N. A., Drescher M. J., & Drescher D. G. Direct interaction of otoferlin with syntaxin 1A, SNAP 25, and the L type voltage-gated calcium channel Cav1.3. J. Biol. Chem. 284, 1364-1372 (2009
    • (2009) J. Biol. Chem , vol.284 , pp. 1364-1372
    • Ramakrishnan, N.A.1    Drescher, M.J.2    Drescher, D.G.3
  • 55
    • 79551689695 scopus 로고    scopus 로고
    • The crystal structure of the C2A domain of otoferlin reveals an unconventional top loop region
    • Helfmann S., et al. The crystal structure of the C2A domain of otoferlin reveals an unconventional top loop region. J. Mol. Biol. 406, 479-490 (2011
    • (2011) J. Mol. Biol , vol.406 , pp. 479-490
    • Helfmann, S.1
  • 56
    • 84891837776 scopus 로고    scopus 로고
    • Alternate splicing of dysferlin C2A confers Ca2+-dependent and Ca2+-independent binding for membrane repair
    • Fuson K., et al. Alternate splicing of dysferlin C2A confers Ca2+-dependent and Ca2+-independent binding for membrane repair. Structure 22, 104-115 (2014
    • (2014) Structure , vol.22 , pp. 104-115
    • Fuson, K.1
  • 57
    • 84947797883 scopus 로고    scopus 로고
    • Audibility, speech perception and processing of temporal cues in ribbon synaptic disorders due to OTOF mutations
    • Pt B
    • Santarelli R., del Castillo I., Cama E., Scimemi P., & Starr A. Audibility, speech perception and processing of temporal cues in ribbon synaptic disorders due to OTOF mutations. Hear. Res. 330 (Pt B), 200-212 (2015
    • (2015) Hear. Res , vol.330 , pp. 200-212
    • Santarelli, R.1    Del Castillo, I.2    Cama, E.3    Scimemi, P.4    Starr, A.5
  • 58
    • 79955777937 scopus 로고    scopus 로고
    • Probing the functional equivalence of otoferlin and synaptotagmin 1 in exocytosis
    • Reisinger E., et al. Probing the functional equivalence of otoferlin and synaptotagmin 1 in exocytosis. J. Neurosci. 31, 4886-4895 (2011
    • (2011) J. Neurosci , vol.31 , pp. 4886-4895
    • Reisinger, E.1
  • 59
    • 69449092161 scopus 로고    scopus 로고
    • Otoferlin is critical for a highly sensitive and linear calcium-dependent exocytosis at vestibular hair cell ribbon synapses
    • Dulon D., Safieddine S., Jones S. M., & Petit C. Otoferlin is critical for a highly sensitive and linear calcium-dependent exocytosis at vestibular hair cell ribbon synapses. J. Neurosci. 29, 10474-10487 (2009
    • (2009) J. Neurosci , vol.29 , pp. 10474-10487
    • Dulon, D.1    Safieddine, S.2    Jones, S.M.3    Petit, C.4
  • 60
    • 0037238597 scopus 로고    scopus 로고
    • Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene
    • Varga R., et al. Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene. J. Med. Genet. 40, 45-50 (2003
    • (2003) J. Med. Genet , vol.40 , pp. 45-50
    • Varga, R.1
  • 61
    • 33847407503 scopus 로고    scopus 로고
    • A forward genetics screen in mice identifies recessive deafness traits and reveals that pejvakin is essential for outer hair cell function
    • Schwander M., et al. A forward genetics screen in mice identifies recessive deafness traits and reveals that pejvakin is essential for outer hair cell function. J. Neurosci. 27, 2163-2175 (2007
    • (2007) J. Neurosci , vol.27 , pp. 2163-2175
    • Schwander, M.1
  • 62
    • 84877295705 scopus 로고    scopus 로고
    • Loudness adaptation accompanying ribbon synapse and auditory nerve disorders
    • Wynne D. P., et al. Loudness adaptation accompanying ribbon synapse and auditory nerve disorders. Brain 136, 1626-1638 (2013
    • (2013) Brain , vol.136 , pp. 1626-1638
    • Wynne, D.P.1
  • 63
    • 84878302482 scopus 로고    scopus 로고
    • Otoferlin couples to clathrin-mediated endocytosis in mature cochlear inner hair cells
    • Duncker S. V., et al. Otoferlin couples to clathrin-mediated endocytosis in mature cochlear inner hair cells. J. Neurosci. 33, 9508-9519 (2013
    • (2013) J. Neurosci , vol.33 , pp. 9508-9519
    • Duncker, S.V.1
  • 64
    • 0035168047 scopus 로고    scopus 로고
    • DFNA25 a novel locus for dominant nonsyndromic hereditary hearing impairment maps to 12q21 24
    • Greene C. C., et al. DFNA25, a novel locus for dominant nonsyndromic hereditary hearing impairment, maps to 12q21 24. Am. J. Hum. Genet. 68, 254-260 (2001
    • (2001) Am. J. Hum. Genet , vol.68 , pp. 254-260
    • Greene, C.C.1
  • 65
    • 39849091711 scopus 로고    scopus 로고
    • Vesicular glutamate transporter 3 is required for synaptic transmission in zebrafish hair cells
    • Obholzer N., et al. Vesicular glutamate transporter 3 is required for synaptic transmission in zebrafish hair cells. J. Neurosci. 28, 2110-2118 (2008
    • (2008) J. Neurosci , vol.28 , pp. 2110-2118
    • Obholzer, N.1
  • 66
    • 0042279177 scopus 로고    scopus 로고
    • Molecular characterization of a 12q22 q24 deletion associated with congenital deafness: Confirmation and refinement of the DFNA25 locus
    • Petek E., et al. Molecular characterization of a 12q22 q24 deletion associated with congenital deafness: confirmation and refinement of the DFNA25 locus. Am. J. Med. Genet. A 117A, 122-126 (2003
    • (2003) Am. J. Med. Genet. A , vol.117 A , pp. 122-126
    • Petek, E.1
  • 67
    • 78650676678 scopus 로고    scopus 로고
    • Loss of Cav1.3 (CACNA1D) function in a human channelopathy with bradycardia and congenital deafness
    • Baig S. M., et al. Loss of Cav1.3 (CACNA1D) function in a human channelopathy with bradycardia and congenital deafness. Nat. Neurosci. 14, 77-84 (2011
    • (2011) Nat. Neurosci , vol.14 , pp. 77-84
    • Baig, S.M.1
  • 68
    • 34548657565 scopus 로고    scopus 로고
    • Calcium ageing, and neuronal vulnerability in Parkinson's disease
    • Surmeier D. J. Calcium, ageing, and neuronal vulnerability in Parkinson's disease. Lancet Neurol. 6, 933-938 (2007
    • (2007) Lancet Neurol , vol.6 , pp. 933-938
    • Surmeier, D.J.1
  • 69
    • 33749368862 scopus 로고    scopus 로고
    • The L Type voltage-gated calcium channel Cav1.3 mediates consolidation, but not extinction, of contextually conditioned fear in mice
    • McKinney B. C., & Murphy G. G. The L Type voltage-gated calcium channel Cav1.3 mediates consolidation, but not extinction, of contextually conditioned fear in mice. Learn. Mem. 13, 584-589 (2006
    • (2006) Learn. Mem , vol.13 , pp. 584-589
    • McKinney, B.C.1    Murphy, G.G.2
  • 70
    • 69449105032 scopus 로고    scopus 로고
    • The Ca2+ channel subunit β2 regulates Ca2+ channel abundance and function in inner hair cells and is required for hearing
    • Neef J., et al. The Ca2+ channel subunit β2 regulates Ca2+ channel abundance and function in inner hair cells and is required for hearing. J. Neurosci. 29, 10730 (2009
    • (2009) J. Neurosci , vol.29 , pp. 10730
    • Neef, J.1
  • 71
    • 33751110923 scopus 로고    scopus 로고
    • Mutation in the auxiliary calcium-channel subunit CACNA2D4 causes autosomal recessive cone dystrophy
    • Wycisk K. A., et al. Mutation in the auxiliary calcium-channel subunit CACNA2D4 causes autosomal recessive cone dystrophy. Am. J. Hum. Genet. 79, 973-977 (2006
    • (2006) Am. J. Hum. Genet , vol.79 , pp. 973-977
    • Wycisk, K.A.1
  • 72
    • 84867267589 scopus 로고    scopus 로고
    • A mutation in CABP2, expressed in cochlear hair cells, causes autosomal-recessive hearing impairment
    • Schrauwen I., et al. A mutation in CABP2, expressed in cochlear hair cells, causes autosomal-recessive hearing impairment. Am. J. Hum. Genet. 91, 636-645 (2012
    • (2012) Am. J. Hum. Genet , vol.91 , pp. 636-645
    • Schrauwen, I.1
  • 73
    • 16544392171 scopus 로고    scopus 로고
    • Essential role of Ca2+-binding protein 4, a Cav1.4 channel regulator, in photoreceptor synaptic function
    • Haeseleer F., et al. Essential role of Ca2+-binding protein 4, a Cav1.4 channel regulator, in photoreceptor synaptic function. Nat. Neurosci. 7, 1079-1087 (2004
    • (2004) Nat. Neurosci , vol.7 , pp. 1079-1087
    • Haeseleer, F.1
  • 74
    • 84927964290 scopus 로고    scopus 로고
    • OPA1 related auditory neuropathy: Site of lesion and outcome of cochlear implantation
    • Santarelli R., et al. OPA1 related auditory neuropathy: site of lesion and outcome of cochlear implantation. Brain 138, 563-576 (2015
    • (2015) Brain , vol.138 , pp. 563-576
    • Santarelli, R.1
  • 75
    • 77950244975 scopus 로고    scopus 로고
    • Multi-system neurological disease is common in patients with OPA1 mutations
    • Yu Wai-Man P., et al. Multi-system neurological disease is common in patients with OPA1 mutations. Brain 133, 771-786 (2010
    • (2010) Brain , vol.133 , pp. 771-786
    • Yu Wai-Man, P.1
  • 77
    • 0033772264 scopus 로고    scopus 로고
    • OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28
    • Alexander C., et al. OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28. Nat. Genet. 26, 211-215 (2000
    • (2000) Nat. Genet , vol.26 , pp. 211-215
    • Alexander, C.1
  • 78
    • 20244381365 scopus 로고    scopus 로고
    • Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy
    • Delettre C., et al. Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy. Nat. Genet. 26, 207-210 (2000
    • (2000) Nat. Genet , vol.26 , pp. 207-210
    • Delettre, C.1
  • 79
    • 84923685347 scopus 로고    scopus 로고
    • Mitochondria: From cell death executioners to regulators of cell differentiation
    • Kasahara A., & Scorrano L. Mitochondria: from cell death executioners to regulators of cell differentiation. Trends Cell Biol. 24, 761-770 (2014
    • (2014) Trends Cell Biol , vol.24 , pp. 761-770
    • Kasahara, A.1    Scorrano, L.2
  • 80
    • 67649658061 scopus 로고    scopus 로고
    • Molecular screening of 980 cases of suspected hereditary optic neuropathy with a report on 77 novel OPA1 mutations
    • Ferr? M., et al. Molecular screening of 980 cases of suspected hereditary optic neuropathy with a report on 77 novel OPA1 mutations. Hum. Mutat. 30, E692-E705 (2009
    • (2009) Hum. Mutat , vol.30 , pp. E692-E705
    • Ferr, M.1
  • 81
    • 34249693479 scopus 로고    scopus 로고
    • A splice site mutation in the murine Opa1 gene features pathology of autosomal dominant optic atrophy
    • Alavi M. V., et al. A splice site mutation in the murine Opa1 gene features pathology of autosomal dominant optic atrophy. Brain 130, 1029-1042 (2006
    • (2006) Brain , vol.130 , pp. 1029-1042
    • Alavi, M.V.1
  • 83
    • 0038157208 scopus 로고    scopus 로고
    • Pathology and physiology of auditory neuropathy with a novel mutation in the MPZ gene (Tyr145→Ser
    • Starr A. Pathology and physiology of auditory neuropathy with a novel mutation in the MPZ gene (Tyr145→Ser). Brain 126, 1604-1619 (2003
    • (2003) Brain , vol.126 , pp. 1604-1619
    • Starr, A.1
  • 84
    • 38449104895 scopus 로고    scopus 로고
    • Late-onset charcot-marie-tooth type 2 disease with hearing impairment associated with a novel pro105thr mutation in the mpz gene
    • Kabzińska D., et al. Late-onset Charcot-Marie-Tooth type 2 disease with hearing impairment associated with a novel Pro105Thr mutation in the MPZ gene. Am. J. Med. Genet. A 143A, 2196-2199 (2007
    • (2007) Am. J. Med. Genet. A , vol.143 A , pp. 2196-2199
    • Kabzińska, D.1
  • 85
    • 18644383046 scopus 로고    scopus 로고
    • Sensorineural hearing impairment in patients with Pmp22 duplication, deletion, and frameshift mutations
    • Verhagen W. I. M., et al. Sensorineural hearing impairment in patients with Pmp22 duplication, deletion, and frameshift mutations. Otol. Neurotol. 26, 405-414 (2005
    • (2005) Otol. Neurotol , vol.26 , pp. 405-414
    • Verhagen, W.I.M.1
  • 86
    • 0036532248 scopus 로고    scopus 로고
    • Anticipation in a unique family with charcot-marie-tooth syndrome and deafness: Delineation of the clinical features and review of the literature
    • Kovach M. J., et al. Anticipation in a unique family with Charcot-Marie-Tooth syndrome and deafness: delineation of the clinical features and review of the literature. Am. J. Med. Genet. 108, 295-303 (2002
    • (2002) Am. J. Med. Genet , vol.108 , pp. 295-303
    • Kovach, M.J.1
  • 87
    • 84947760432 scopus 로고    scopus 로고
    • Pathophysiological mechanisms and functional hearing consequences of auditory neuropathy
    • Rance G., & Starr A. Pathophysiological mechanisms and functional hearing consequences of auditory neuropathy. Brain 138, 3141-3158 (2015
    • (2015) Brain , vol.138 , pp. 3141-3158
    • Rance, G.1    Starr, A.2
  • 88
    • 33745577619 scopus 로고    scopus 로고
    • Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy
    • Delmaghani S., et al. Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy. Nat. Genet. 38, 770-778 (2006
    • (2006) Nat. Genet , vol.38 , pp. 770-778
    • Delmaghani, S.1
  • 89
    • 84865080106 scopus 로고    scopus 로고
    • High frequency of autosomal-recessive DFNB59 hearing loss in an isolated Arab population in Israel
    • Borck G., et al. High frequency of autosomal-recessive DFNB59 hearing loss in an isolated Arab population in Israel. Clin. Genet. 82, 271-276 (2012
    • (2012) Clin. Genet , vol.82 , pp. 271-276
    • Borck, G.1
  • 90
    • 34248389211 scopus 로고    scopus 로고
    • Truncating mutation of the DFNB59 gene causes cochlear hearing impairment and central vestibular dysfunction
    • Ebermann I., et al. Truncating mutation of the DFNB59 gene causes cochlear hearing impairment and central vestibular dysfunction. Hum. Mutat. 28, 571-577 (2007
    • (2007) Hum. Mutat , vol.28 , pp. 571-577
    • Ebermann, I.1
  • 91
    • 34548131104 scopus 로고    scopus 로고
    • Novel mutations in the pejvakin gene are associated with autosomal recessive non-syndromic hearing loss in Iranian families
    • Hashemzadeh Chaleshtori M., et al. Novel mutations in the pejvakin gene are associated with autosomal recessive non-syndromic hearing loss in Iranian families. Clin. Genet. 72, 261-263 (2007
    • (2007) Clin. Genet , vol.72 , pp. 261-263
    • Hashemzadeh Chaleshtori, M.1
  • 92
    • 34447260468 scopus 로고    scopus 로고
    • Involvement of DFNB59 mutations in autosomal recessive nonsyndromic hearing impairment
    • Collin R. W. J., et al. Involvement of DFNB59 mutations in autosomal recessive nonsyndromic hearing impairment. Hum. Mutat. 28, 718-723 (2007
    • (2007) Hum. Mutat , vol.28 , pp. 718-723
    • Collin, R.W.J.1
  • 93
    • 84946234112 scopus 로고    scopus 로고
    • Hypervulnerability to sound exposure through impaired adaptive proliferation of peroxisomes
    • Delmaghani S., et al. Hypervulnerability to sound exposure through impaired adaptive proliferation of peroxisomes. Cell 163, 894-906 (2015
    • (2015) Cell , vol.163 , pp. 894-906
    • Delmaghani, S.1
  • 94
    • 8744288856 scopus 로고    scopus 로고
    • A gene responsible for autosomal dominant auditory neuropathy (AUNA1) maps to 13q14-21
    • Kim T. B., et al. A gene responsible for autosomal dominant auditory neuropathy (AUNA1) maps to 13q14-21. J. Med. Genet. 41, 872 (2004
    • (2004) J. Med. Genet , vol.41 , pp. 872
    • Kim, T.B.1
  • 95
    • 13844264405 scopus 로고    scopus 로고
    • A dominantly inherited progressive deafness affecting distal auditory nerve and hair cells
    • Starr A., et al. A dominantly inherited progressive deafness affecting distal auditory nerve and hair cells. J. Assoc. Res. Otolaryngol. 5, 411-426 (2004
    • (2004) J. Assoc. Res. Otolaryngol , vol.5 , pp. 411-426
    • Starr, A.1
  • 96
    • 77955787251 scopus 로고    scopus 로고
    • Increased activity of diaphanous homolog 3 (diaph3)/diaphanous causes hearing defects in humans with auditory neuropathy and in drosophila
    • Schoen C. J., et al. Increased activity of Diaphanous homolog 3 (DIAPH3)/diaphanous causes hearing defects in humans with auditory neuropathy and in Drosophila. Proc. Natl Acad. Sci. USA 107, 13396-13401 (2010
    • (2010) Proc. Natl Acad. Sci. USA , vol.107 , pp. 13396-13401
    • Schoen, C.J.1
  • 97
    • 84874127342 scopus 로고    scopus 로고
    • Diaphanous homolog 3 (Diap3) overexpression causes progressive hearing loss and inner hair cell defects in a transgenic mouse model of human deafness
    • Schoen C. J., Burmeister M., & Lesperance M. M. Diaphanous homolog 3 (Diap3) overexpression causes progressive hearing loss and inner hair cell defects in a transgenic mouse model of human deafness. PLoS ONE 8, e56520 (2013
    • (2013) Plos One , vol.8 , pp. e56520
    • Schoen, C.J.1    Burmeister, M.2    Lesperance, M.M.3
  • 98
    • 49449099721 scopus 로고    scopus 로고
    • Speech perception ability in individuals with Friedreich ataxia
    • Rance G., et al. Speech perception ability in individuals with Friedreich ataxia. Brain 131, 2002-2012 (2008
    • (2008) Brain , vol.131 , pp. 2002-2012
    • Rance, G.1
  • 99
    • 50649094475 scopus 로고    scopus 로고
    • The frequency of auditory neuropathy detected by universal newborn hearing screening program
    • Kirkim G., Serbetcioglu B., Erdag T. K., & Ceryan K. The frequency of auditory neuropathy detected by universal newborn hearing screening program. Int. J. Pediatr. Otorhinolaryngol. 72, 1461-1469 (2008
    • (2008) Int. J. Pediatr. Otorhinolaryngol , vol.72 , pp. 1461-1469
    • Kirkim, G.1    Serbetcioglu, B.2    Erdag, T.K.3    Ceryan, K.4
  • 100
    • 0018390844 scopus 로고
    • Brain stem evoked response audiometry in newborn hearing screening
    • Schulman-Galambos C., & Galambos R. Brain stem evoked response audiometry in newborn hearing screening. Arch. Otolaryngol. 105, 86-90 (1979
    • (1979) Arch. Otolaryngol , vol.105 , pp. 86-90
    • Schulman-Galambos, C.1    Galambos, R.2
  • 101
    • 0141959121 scopus 로고    scopus 로고
    • Association between peak serum bilirubin and neurodevelopmental outcomes in extremely low birth weight infants
    • Oh W., et al. Association between peak serum bilirubin and neurodevelopmental outcomes in extremely low birth weight infants. Pediatrics 112, 773-779 (2003
    • (2003) Pediatrics , vol.112 , pp. 773-779
    • Oh, W.1
  • 102
    • 84922922441 scopus 로고    scopus 로고
    • Audiologic impairment associated with bilirubin-induced neurologic damage
    • Olds C., & Oghalai J. S. Audiologic impairment associated with bilirubin-induced neurologic damage. Semin. Fetal Neonatal Med. 20, 42-46 (2015
    • (2015) Semin. Fetal Neonatal Med , vol.20 , pp. 42-46
    • Olds, C.1    Oghalai, J.S.2
  • 103
    • 8644220512 scopus 로고    scopus 로고
    • Auditory brainstem response detects early bilirubin neurotoxicity at low indirect bilirubin values
    • Smith C. M., Barnes G. P., Jacobson C. A., & Oelberg D. G. Auditory brainstem response detects early bilirubin neurotoxicity at low indirect bilirubin values. J. Perinatol. 24, 730-732 (2004
    • (2004) J. Perinatol , vol.24 , pp. 730-732
    • Smith, C.M.1    Barnes, G.P.2    Jacobson, C.A.3    Oelberg, D.G.4
  • 104
    • 0034905038 scopus 로고    scopus 로고
    • Selective inner hair cell loss in premature infants and cochlea pathological patterns from neonatal intensive care unit autopsies
    • Amatuzzi M. G., et al. Selective inner hair cell loss in premature infants and cochlea pathological patterns from neonatal intensive care unit autopsies. Arch. Otolaryngol. Head Neck Surg. 127, 629-636 (2001
    • (2001) Arch Otolaryngol. Head Neck Surg , vol.127 , pp. 629-636
    • Amatuzzi, M.G.1
  • 105
    • 0020625389 scopus 로고
    • The Gunn rat: An experimental model for central deafness
    • Uziel A., Marot M., & Pujol R. The Gunn rat: an experimental model for central deafness. Acta Otolaryngol. 95, 651-656 (1983
    • (1983) Acta Otolaryngol , vol.95 , pp. 651-656
    • Uziel, A.1    Marot, M.2    Pujol, R.3
  • 106
    • 78649702914 scopus 로고    scopus 로고
    • Acute hyperbilirubinaemia induces presynaptic neurodegeneration at a central glutamatergic synapse
    • Haustein M. D., et al. Acute hyperbilirubinaemia induces presynaptic neurodegeneration at a central glutamatergic synapse. J. Physiol. 588, 4683-4693 (2010
    • (2010) J. Physiol , vol.588 , pp. 4683-4693
    • Haustein, M.D.1
  • 107
    • 0036768228 scopus 로고    scopus 로고
    • Changes in calcium-binding protein expression in the auditory brainstem nuclei of the jaundiced Gunn rat
    • Spencer R. F., Shaia W. T., Gleason A. T., Sismanis A., & Shapiro S. M. Changes in calcium-binding protein expression in the auditory brainstem nuclei of the jaundiced Gunn rat. Hear. Res. 171, 129-141 (2002
    • (2002) Hear. Res , vol.171 , pp. 129-141
    • Spencer, R.F.1    Shaia, W.T.2    Gleason, A.T.3    Sismanis, A.4    Shapiro, S.M.5
  • 108
  • 109
    • 0036850440 scopus 로고    scopus 로고
    • Targeted disruption of Slc19a2, the gene encoding the high-affinity thiamin transporter Thtr 1, causes diabetes mellitus, sensorineural deafness and megaloblastosis in mice
    • Oishi K., et al. Targeted disruption of Slc19a2, the gene encoding the high-affinity thiamin transporter Thtr 1, causes diabetes mellitus, sensorineural deafness and megaloblastosis in mice. Hum. Mol. Genet. 11, 2951-2960 (2002
    • (2002) Hum. Mol. Genet , vol.11 , pp. 2951-2960
    • Oishi, K.1
  • 110
    • 33747048167 scopus 로고    scopus 로고
    • Deletion of SLC19A2, the high affinity thiamine transporter, causes selective inner hair cell loss and an auditory neuropathy phenotype
    • Liberman M. C., Tartaglini E., Fleming J. C., & Neufeld E. J. Deletion of SLC19A2, the high affinity thiamine transporter, causes selective inner hair cell loss and an auditory neuropathy phenotype. J. Assoc. Res. Otolaryngol. 7, 211-217 (2006
    • (2006) J. Assoc. Res. Otolaryngol , vol.7 , pp. 211-217
    • Liberman, M.C.1    Tartaglini, E.2    Fleming, J.C.3    Neufeld, E.J.4
  • 111
    • 84940188745 scopus 로고    scopus 로고
    • Age-related hearing impairment and the triad of acquired hearing loss
    • Yang C. H., Schrepfer T., & Schacht J. Age-related hearing impairment and the triad of acquired hearing loss. Front. Cell. Neurosci. 9, 276 (2015
    • (2015) Front. Cell. Neurosci , vol.9 , pp. 276
    • Yang, C.H.1    Schrepfer, T.2    Schacht, J.3
  • 112
    • 0028901569 scopus 로고
    • Afferent synaptic changes in auditory hair cells during noise-induced temporary threshold shift
    • Henry W. R., & Mulroy M. J. Afferent synaptic changes in auditory hair cells during noise-induced temporary threshold shift. Hear. Res. 84, 81-90 (1995
    • (1995) Hear. Res , vol.84 , pp. 81-90
    • Henry, W.R.1    Mulroy, M.J.2
  • 113
    • 0026058665 scopus 로고
    • Α-amino 3 hydroxy-5 methyl-4 isoxazole propionic acid electrophysiological and neurotoxic effects in the Guinea-pig cochlea
    • Puel J. L., Pujol R., Ladrech S., & Eybalin M. α-amino 3 hydroxy-5 methyl-4 isoxazole propionic acid electrophysiological and neurotoxic effects in the guinea-pig cochlea. Neuroscience 45, 63-72 (1991
    • (1991) Neuroscience , vol.45 , pp. 63-72
    • Puel, J.L.1    Pujol, R.2    Ladrech, S.3    Eybalin, M.4
  • 114
    • 0032557933 scopus 로고    scopus 로고
    • Excitotoxicity and repair of cochlear synapses after noise-trauma induced hearing loss
    • Puel J. L., Ruel J., Gervais d'Aldin C., & Pujol R. Excitotoxicity and repair of cochlear synapses after noise-trauma induced hearing loss. Neuroreport 9, 2109-2114 (1998
    • (1998) Neuroreport , vol.9 , pp. 2109-2114
    • Puel, J.L.1    Ruel, J.2    Gervais D'aldin, C.3    Pujol, R.4
  • 115
    • 33749257090 scopus 로고    scopus 로고
    • Synaptic alterations at inner hair cells precede spiral ganglion cell loss in aging C57BL/6J mice
    • Stamataki S., Francis H. W., Lehar M., May B. J., & Ryugo D. K. Synaptic alterations at inner hair cells precede spiral ganglion cell loss in aging C57BL/6J mice. Hear. Res. 221, 104-118 (2006
    • (2006) Hear. Res , vol.221 , pp. 104-118
    • Stamataki, S.1    Francis, H.W.2    Lehar, M.3    May, B.J.4    Ryugo, D.K.5
  • 116
    • 70449643246 scopus 로고    scopus 로고
    • Adding insult to injury: Cochlear nerve degeneration after 'temporary' noise-induced hearing loss
    • Kujawa S. G., & Liberman M. C. Adding insult to injury: cochlear nerve degeneration after 'temporary' noise-induced hearing loss. J. Neurosci. 29, 14077 (2009
    • (2009) J. Neurosci , vol.29 , pp. 14077
    • Kujawa, S.G.1    Liberman, M.C.2
  • 117
    • 84882720760 scopus 로고    scopus 로고
    • Age-related cochlear synaptopathy: An early-onset contributor to auditory functional decline
    • Sergeyenko Y., Lall K., Liberman M. C., & Kujawa S. G. Age-related cochlear synaptopathy: an early-onset contributor to auditory functional decline. J. Neurosci. 33, 13686-13694 (2013
    • (2013) J. Neurosci , vol.33 , pp. 13686-13694
    • Sergeyenko, Y.1    Lall, K.2    Liberman, M.C.3    Kujawa, S.G.4
  • 118
    • 84940173805 scopus 로고    scopus 로고
    • Synaptopathy in the noise-exposed and aging cochlea: Primary neural degeneration in acquired sensorineural hearing loss
    • Pt B
    • Kujawa S. G., & Liberman M. C. Synaptopathy in the noise-exposed and aging cochlea: primary neural degeneration in acquired sensorineural hearing loss. Hear. Res. 330(Pt B), 191-199 (2015
    • (2015) Hear. Res , vol.330 , pp. 191-199
    • Kujawa, S.G.1    Liberman, M.C.2
  • 119
    • 0034554777 scopus 로고    scopus 로고
    • Exacerbation of noise-induced hearing loss in mice lacking the glutamate transporter GLAST
    • Hakuba N., Koga K., Gyo K., Usami S. I., & Tanaka K. Exacerbation of noise-induced hearing loss in mice lacking the glutamate transporter GLAST. J. Neurosci. 20, 8750-8753 (2000
    • (2000) J. Neurosci , vol.20 , pp. 8750-8753
    • Hakuba, N.1    Koga, K.2    Gyo, K.3    Usami, S.I.4    Tanaka, K.5
  • 120
    • 63649100112 scopus 로고    scopus 로고
    • Tuning of synapse number, structure and function in the cochlea
    • Meyer A. C., et al. Tuning of synapse number, structure and function in the cochlea. Nat. Neurosci. 12, 444-453 (2009
    • (2009) Nat. Neurosci , vol.12 , pp. 444-453
    • Meyer, A.C.1
  • 121
    • 84880985531 scopus 로고    scopus 로고
    • Noise-induced cochlear neuropathy is selective for fibers with low spontaneous rates
    • Furman A. C., Kujawa S. G., & Liberman M. C. Noise-induced cochlear neuropathy is selective for fibers with low spontaneous rates. J. Neurophysiol. 110, 577-586 (2013
    • (2013) J. Neurophysiol , vol.110 , pp. 577-586
    • Furman, A.C.1    Kujawa, S.G.2    Liberman, M.C.3
  • 122
    • 84906909919 scopus 로고    scopus 로고
    • Contribution of auditory nerve fibers to compound action potential of the auditory nerve
    • Bourien J., et al. Contribution of auditory nerve fibers to compound action potential of the auditory nerve. J. Neurophysiol. 112, 1025-1039 (2014
    • (2014) J. Neurophysiol , vol.112 , pp. 1025-1039
    • Bourien, J.1
  • 123
    • 84930654678 scopus 로고    scopus 로고
    • Neurotrophin 3 regulates ribbon synapse density in the cochlea and induces synapse regeneration after acoustic trauma
    • Wan G., Gsigma;mez-Casati M. E., Gigliello A. R., Liberman M. C., & Corfas G. Neurotrophin 3 regulates ribbon synapse density in the cochlea and induces synapse regeneration after acoustic trauma. eLIFE 3, e03564 (2014
    • (2014) Elife , vol.3 , pp. e03564
    • Wan, G.1    Gsigma2    mez-Casati, M.E.3    Gigliello, A.R.4    Liberman, M.C.5    Corfas, G.6
  • 124
    • 80053003469 scopus 로고    scopus 로고
    • Tinnitus with a normal audiogram: Physiological evidence for hidden hearing loss and computational model
    • Schaette R., & McAlpine D. Tinnitus with a normal audiogram: physiological evidence for hidden hearing loss and computational model. J. Neurosci. 31, 13452-13457 (2011
    • (2011) J. Neurosci , vol.31 , pp. 13452-13457
    • Schaette, R.1    McAlpine, D.2
  • 125
    • 0025785479 scopus 로고
    • Absence of both auditory evoked potentials and auditory percepts dependent on timing cues
    • Starr A., et al. Absence of both auditory evoked potentials and auditory percepts dependent on timing cues. Brain 114, 1157-1180 (1991
    • (1991) Brain , vol.114 , pp. 1157-1180
    • Starr, A.1
  • 126
    • 0035066528 scopus 로고    scopus 로고
    • Cochlear receptor (microphonic and summating potentials, otoacoustic emissions) and auditory pathway (auditory brain stem potentials) activity in auditory neuropathy
    • Starr A., et al. Cochlear receptor (microphonic and summating potentials, otoacoustic emissions) and auditory pathway (auditory brain stem potentials) activity in auditory neuropathy. Ear Hear. 22, 91 (2001
    • (2001) Ear Hear , vol.22 , pp. 91
    • Starr, A.1
  • 127
    • 72249092101 scopus 로고    scopus 로고
    • Abnormal cochlear potentials from deaf patients with mutations in the otoferlin gene
    • Santarelli R., et al. Abnormal cochlear potentials from deaf patients with mutations in the otoferlin gene. J. Assoc. Res. Otolaryngol. 10, 545-556 (2009
    • (2009) J. Assoc. Res. Otolaryngol , vol.10 , pp. 545-556
    • Santarelli, R.1
  • 128
    • 35448995721 scopus 로고    scopus 로고
    • Detection and differentiation of sensorineural hearing loss in mice using auditory steady-state responses and transient auditory brainstem responses
    • Pauli-Magnus D., et al. Detection and differentiation of sensorineural hearing loss in mice using auditory steady-state responses and transient auditory brainstem responses. Neuroscience 149, 673-684 (2007
    • (2007) Neuroscience , vol.149 , pp. 673-684
    • Pauli-Magnus, D.1
  • 129
    • 84937710511 scopus 로고    scopus 로고
    • Massively parallel sequencing for genetic diagnosis of hearing loss: The new standard of care
    • Shearer A. E., & Smith R. J. H. Massively parallel sequencing for genetic diagnosis of hearing loss: the new standard of care. Otolaryngol. Head Neck Surg. 153, 175-182 (2015
    • (2015) Otolaryngol. Head Neck Surg , vol.153 , pp. 175-182
    • Shearer, A.E.1    Smith, R.J.H.2
  • 130
    • 84892813394 scopus 로고    scopus 로고
    • Analysis of speech perception outcomes among patients receiving cochlear implants with auditory neuropathy spectrum disorder
    • Dean C., Felder G., & Kim A. H. Analysis of speech perception outcomes among patients receiving cochlear implants with auditory neuropathy spectrum disorder. Otol. Neurotol. 34, 1610-1614 (2013
    • (2013) Otol. Neurotol , vol.34 , pp. 1610-1614
    • Dean, C.1    Felder, G.2    Kim, A.H.3
  • 131
    • 84879223439 scopus 로고    scopus 로고
    • Does cochlear implantation improve speech recognition in children with auditory neuropathy spectrum disorder? A systematic review
    • Humphriss R., et al. Does cochlear implantation improve speech recognition in children with auditory neuropathy spectrum disorder? A systematic review. Int. J. Audiol. 52, 442-454 (2013
    • (2013) Int. J. Audiol , vol.52 , pp. 442-454
    • Humphriss, R.1
  • 132
    • 83055185558 scopus 로고    scopus 로고
    • Audiologic management of auditory neuropathy spectrum disorder in children: A systematic review of the literature
    • Roush P., Frymark T., Venediktov R., & Wang B. Audiologic management of auditory neuropathy spectrum disorder in children: a systematic review of the literature. Am. J. Audiol. 20, 159-170 (2011
    • (2011) Am. J. Audiol , vol.20 , pp. 159-170
    • Roush, P.1    Frymark, T.2    Venediktov, R.3    Wang, B.4
  • 133
    • 84855677898 scopus 로고    scopus 로고
    • Auditory neuropathies: Understanding their pathogenesis to illuminate intervention strategies
    • Giraudet F., & Avan P. Auditory neuropathies: understanding their pathogenesis to illuminate intervention strategies. Curr. Opin. Neurol. 25, 50-56 (2012
    • (2012) Curr. Opin. Neurol , vol.25 , pp. 50-56
    • Giraudet, F.1    Avan, P.2
  • 134
    • 70350511665 scopus 로고    scopus 로고
    • Speech and language outcomes in children with auditory neuropathy/dys-synchrony managed with either cochlear implants or hearing AIDS
    • Rance G., & Barker E. J. Speech and language outcomes in children with auditory neuropathy/dys-synchrony managed with either cochlear implants or hearing aids. Int. J. Audiol. 48, 313-320 (2009
    • (2009) Int. J. Audiol , vol.48 , pp. 313-320
    • Rance, G.1    Barker, E.J.2
  • 135
    • 84891062327 scopus 로고    scopus 로고
    • Impact of the presence of auditory neuropathy spectrum disorder (ANSD) on outcomes of children at three years of age
    • Ching T. Y. C., et al. Impact of the presence of auditory neuropathy spectrum disorder (ANSD) on outcomes of children at three years of age. Int. J. Audiol. 52, S55-S64 (2013
    • (2013) Int. J. Audiol , vol.52 , pp. S55-S64
    • Ching, T.Y.C.1
  • 136
    • 0025000379 scopus 로고
    • Estimation of surviving spiral ganglion cells in the deaf rat using the electrically evoked auditory brainstem response
    • Hall R. D. Estimation of surviving spiral ganglion cells in the deaf rat using the electrically evoked auditory brainstem response. Hear. Res. 49, 155-168 (1990
    • (1990) Hear. Res , vol.49 , pp. 155-168
    • Hall, R.D.1
  • 137
    • 0025215515 scopus 로고
    • Estimation of surviving spiral ganglion cells in the deaf rat using the electrically evoked auditory brainstem response
    • Hall R. D. Estimation of surviving spiral ganglion cells in the deaf rat using the electrically evoked auditory brainstem response. Hear. Res. 45, 123-136 (1990
    • (1990) Hear. Res , vol.45 , pp. 123-136
    • Hall, R.D.1
  • 138
    • 0028892676 scopus 로고
    • Electrically evoked auditory brainstem response in peripherally myelin-deficient mice
    • Zhou R., Abbas P. J., & Assouline J. G. Electrically evoked auditory brainstem response in peripherally myelin-deficient mice. Hear Res. 88, 98-106 (1995
    • (1995) Hear Res , vol.88 , pp. 98-106
    • Zhou, R.1    Abbas, P.J.2    Assouline, J.G.3
  • 139
    • 33344472435 scopus 로고    scopus 로고
    • Results of cochlear implantation in two children with mutations in the OTOF gene
    • Rouillon I., et al. Results of cochlear implantation in two children with mutations in the OTOF gene. Int. J. Pediatr. Otorhinolaryngol. 70, 689-696 (2006
    • (2006) Int. J. Pediatr. Otorhinolaryngol , vol.70 , pp. 689-696
    • Rouillon, I.1
  • 140
    • 84896786196 scopus 로고    scopus 로고
    • Optogenetic stimulation of the auditory pathway
    • Hernandez V. H., et al. Optogenetic stimulation of the auditory pathway. J. Clin. Invest. 124, 1114-1129 (2014
    • (2014) J. Clin. Invest , vol.124 , pp. 1114-1129
    • Hernandez, V.H.1
  • 141
    • 84925414360 scopus 로고    scopus 로고
    • Considering optogenetic stimulation for cochlear implants
    • Jeschke M., & Moser T. Considering optogenetic stimulation for cochlear implants. Hear. Res. 322, 224-234 (2015
    • (2015) Hear. Res , vol.322 , pp. 224-234
    • Jeschke, M.1    Moser, T.2
  • 142
    • 84864297772 scopus 로고    scopus 로고
    • Restoration of hearing in the VGLUT3 knockout mouse using virally mediated gene therapy
    • Akil O., et al. Restoration of hearing in the VGLUT3 knockout mouse using virally mediated gene therapy. Neuron 75, 283-293 (2012
    • (2012) Neuron , vol.75 , pp. 283-293
    • Akil, O.1
  • 143
    • 84938215789 scopus 로고    scopus 로고
    • US National Library of Medicine
    • US National Library of Medicine. ClinicalTrials.gov [online] https://clinicaltrials.gov/ct2/show/NCT02132130 (2015
    • (2015) ClinicalTrials.gov [Online


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.