1 顾翔，陈知己，蔡婷，等.衔接蛋白AP-2在不同发育时期小鼠耳蜗毛细胞中的表达[J].听力学及言语疾病杂志，2015,23(6):607-611.

2 Kujawa SG,Liberman MC.Synaptopathy in the noise-exposed and aging cochlea:primary neural degeneration in acquired sensorineural hearing loss[J]?Hear Res,2015,330(Pt B):191-199.

3 李晓瑞，蒋兴旺，张延平.C57BL/6J小鼠随年龄增长耳蜗内毛细胞带状突触数量变化的观察[J].听力学及言语疾病杂志，2015,23(2):176-180.

4 Yuan Y,Shi F,Yin Y,et al.Ouabain-induced cochlear nerve degeneration:synaptic loss and plasticity in a mouse model of auditory neuropathy [J].J Assoc Res Otolaryngol,2014,15(1):31-43.

5 Piatto VB,Nascimento EC,Alexandrino F,et al.Molecular genetics of non-syndromic deafness[J]?Braz J Otorhinolaryngol,2005,71(2):216-223.

6 Moser T,Starr A.Auditory neuropathy--neural and synaptic mechanisms [J].Nat Rev Neurol,2016,12(3):135-149.

7 张秋静，王秋菊.听神经病谱系障碍的病变部位和定位分型诊断[J].中华耳科学杂志，2015,13(2):202-205.

8 Wan G,Corfas G.No longer falling on deaf ears:mechanisms of degeneration and regeneration of cochlear ribbon synapses[J]?Hear Res,2015,329:1-10.

9 Becker L,Schnee ME,Niwa M,et al.The presynaptic ribbon maintains vesicle populations at the hair cell afferent fiber synapse [J].Elife,2018,7:e30241.DOI:10.7554/eLife.30241.

10 Coate TM,Scott MK,Gurjar M.Current concepts in cochlear ribbon synapse formation [J].Synapse,2019,73(5):e22087.DOI:10.1002/syn.22087.

11 Sheets L,Trapani JG,Mo W,et al.Ribeye is required for presynaptic Ca(V)1.3a channel localization and afferent innervation of sensory hair cells [J].Development,2011,138(7):1309-1319.

12 Lv C,Stewart WJ,Akanyeti O,et al.Synaptic ribbons require ribeye for electron density,proper synaptic localization,and recruitment of calcium channels [J].Cell Rep,2016,15(12):2784-2795.

13 Jean P,Lopez de la Morena D,Michanski S,et al.The synaptic ribbon is critical for sound encoding at high rates and with temporal precision [J].Elife,2018,7:e29275.DOI:10.7554/eLife.29275.

14 Butola T,Wichmann C,Moser T.Piccolo promotes vesicle replenishment at a fast central auditory synapse [J].Front Synaptic Neurosci,2017,9:14.

15 Frank T,Rutherford MA,Strenzke N,et al.Bassoon and the synaptic ribbon organize Ca2+ channels and vesicles to add release sites and promote refilling [J].Neuron,2010,68(4):724-738.

16 Buran BN,Strenzke N,Neef A,et al.Onset coding is degraded in auditory nerve fibers from mutant mice lacking synaptic ribbons [J].The Journal of Neuroscience,2010,30(22):7587-7597.

17 Yang SM,Hou ZH,Yang G,et al.Chondrocyte-specific Smad4 gene conditional knockout results in hearing loss and inner ear malformation in mice [J].Dev Dyn,2009,238(8):1897-1908.

18 Liu K,Ji F,Yang G,et al.SMAD4 defect causes auditory neuropathy via specialized disruption of cochlear ribbon synapses in mice [J].Mol Neurobiol,2016,53(8):5679-5691.

19 Carrott L,Bowl MR,Aguilar C,et al.Absence of neuroplastin-65 affects synaptogenesis in mouse inner hair cells and causes profound hearing loss [J].J Neurosci,2016,36(1):222-234.

20 Harley RJ,Murdy JP,Wang Z,et al.Neuronal cell adhesion molecule (NrCAM) is expressed by sensory cells in the cochlea and is necessary for proper cochlear innervation and sensory domain patterning during development [J].Dev Dyn,2018,247(7):934-950.

21 Obholzer N,Wolfson S,Trapani JG,et al.Vesicular glutamate transporter 3 is required for synaptic transmission in zebrafish hair cells [J].J Neurosci,2008,28(9):2110-2118.

22 Ruel J,Emery S,Nouvian R et al.Impairment of SLC17A8 encoding vesicular glutamate transporter-3,VGLUT3,underlies nonsyndromic deafness DFNA25 and inner hair cell dysfunction in null mice [J].Am J Hum Genet,2008,83(2):278-292.

23 Seal RP,Akil O,Yi E,et al.Sensorineural deafness and seizures in mice lacking vesicular glutamate transporter 3 [J].Neuron,2008,57(2):263-275.

24 Neef J,Jung S,Wong AB,et al.Modes and regulation of endocytic membrane retrieval in mouse auditory hair cells [J].J Neurosci,2014,34(3):705-716.

25 Jung S,Maritzen T,Wichmann C,et al.Disruption of adaptor protein 2mu (AP-2mu) in cochlear hair cells impairs vesicle reloading of synaptic release sites and hearing [J].EMBO J,2015,34(21):2686-2702.

26 Michalski N,Goutman JD,Auclair SM,et al.Otoferlin acts as a Ca(2+) sensor for vesicle fusion and vesicle pool replenishment at auditory hair cell ribbon synapses [J].Elife,2017,6:e31013.DOI:10.7554/eLife.31013.

27 Takago H,Oshima-Takago T,Moser T.Disruption of otoferlin alters the mode of exocytosis at the mouse inner hair cell ribbon synapse [J].Front Mol Neurosci,2018,11:492.

28 Al-Moyed H,Cepeda AP,Jung S,et al.A dual-AAV approach restores fast exocytosis and partially rescues auditory function in deaf otoferlin knock-out mice [J].EMBO Mol Med,2019,11(1):e9396.DOI:10.15252/emmm.201809396.

29 Manchanda A,Chatterjee P,Bonventre JA,et al.Otoferlin depletion results in abnormal synaptic ribbons and altered intracellular calcium levels in zebrafish [J].Sci Rep,2019,9(1):14273.

30 Lin SY,Vollrath MA,Mangosing S,et al.The zebrafishpinball wizardgene encodes WRB,a tail-anchored-protein receptor essential for inner-ear hair cells and retinal photoreceptors [J].J Physiol,2016,594(4):895-914.

31 Vogl C,Panou I,Yamanbaeva G,et al.Tryptophan-rich basic protein (WRB) mediates insertion of the tail-anchored protein otoferlin and is required for hair cell exocytosis and hearing [J].EMBO J,2016,35(23):2536-2552.

32 Yang T,Hu N,Pangr?i? T,Green S,et al.Functions of CaBP1 and CaBP2 in the peripheral auditory system [J].Hear Res,2018,364:48-58.

33 Schrauwen I,Helfmann S,Inagaki A,et al.A mutation in CABP2,expressed in cochlear hair cells,causes autosomal-recessive hearing impairment [J].Am J Hum Genet,2012,91(4):636-645.

34 Picher MM,Gehrt A,Meese S,et al.Ca(2+)-binding protein 2 inhibits Ca2+-channel inactivation in mouse inner hair cells [J].Proc Natl Acad Sci USA,2017,114(9):E1717-E1726.

35 Ortner NJ,Pinggera A,Hofer NT,et al.RBP2 stabilizes slow Cav1.3 Ca2+ channel inactivation properties of cochlear inner hair cells [J].Pflugers Arch,2019,472(1):3-25.

36 Baig SM,Koschak A,Lieb A,et al.Loss of Cav1.3 (CACNA1D) function in a human channelopathy with bradycardia and congenital deafness [J].Nat Neurosci,2011,14(1):77-84.

37 Brandt A,Striessnig J,Moser T.CaV1.3 Channels are essential for development and presynaptic activity of cochlear inner hair cells [J].J Neurosci,2003,23(34):10832-10840.

38 Eckrich S,Hecker D,Sorg K,et al.Cochlea-Specific deletion of Cav1.3 calcium channels arrests inner hair cell differentiation and unravels pitfalls of conditional mouse models [J].Front Cell Neurosci,2019,13:225.

39 Sheets L,Kindt KS,Nicolson T.Presynaptic CaV1.3 channels regulate synaptic ribbon size and are required for synaptic maintenance in sensory hair cells [J].J Neurosci,2012,32(48):17273-17286.

40 Schoen CJ,Emery SB,Thorne MC,et al.Increased activity of diaphanous homolog 3 (DIAPH3)/diaphanous causes hearing defects in humans with auditory neuropathy and in Drosophila [J].Proc Natl Acad Sci USA,2010,107(30):13396-13401.

41 Schoen CJ,Burmeister M,Lesperance MM et al.Diaphanous homolog 3 (Diap3) overexpression causes progressive hearing loss and inner hair cell defects in a transgenic mouse model of human deafness [J].PLoS One,2013,8(2):e56520.DOI:10.1371/journal.pone.0056520.

42 Ogun O,Zallocchi M.Clarin-1 acts as a modulator of mechanotransduction activity and presynaptic ribbon assembly [J].J Cell Biol,2014,207(3):375-391.

43 Dulon D,Papal S,Patni P,et al.Clarin-1 gene transfer rescues auditory synaptopathy in model of Usher syndrome [J].J Clin Invest,2018,128(8):3382-3401.

44 Mehta A,Prabhakar M,Kumar P,et al.Excitotoxicity:bridge to various triggers in neurodegenerative disorders [J].Eur J Pharmacol,2013,698(1-3):6-18.