Ahmadmehrabi S, Brant J, Epstein DJ, Ruckenstein MJ, Rader DJ (2021) Genetics of Postlingual Sensorineural hearing loss. Laryngoscope 131(2):401–409

Article  PubMed  Google Scholar 

Bergsbaken T, Fink SL, Cookson BT (2009) Pyroptosis: host cell death and inflammation. Nat Rev Microbiol 7(2):99–109

Article  CAS  PubMed  PubMed Central  Google Scholar 

Bischoff AM, Luijendijk MW, Huygen PL, van Duijnhoven G, De Leenheer EM, Oudesluijs GG, Van Laer L, Cremers FP, Cremers CW, Kremer H (2004) A novel mutation identified in the DFNA5 gene in a Dutch family: a clinical and genetic evaluation. Audiol Neurootol 9(1):34–46

Article  CAS  PubMed  Google Scholar 

Chadha S, Kamenov K, Cieza A (2021) The world report on hearing, 2021. Bull World Health Organ 99(4):242–242A

Article  PubMed  PubMed Central  Google Scholar 

Cheng J, Han DY, Dai P, Sun HJ, Tao R, Sun Q, Yan D, Qin W, Wang HY, Ouyang XM, Yang SZ, Cao JY, Feng GY, Du LL, Zhang YZ, Zhai SQ, Yang WY, Liu XZ, He L, Yuan HJ (2007) A novel DFNA5 mutation, IVS8 + 4 A > G, in the splice donor site of intron 8 causes late-onset non-syndromic hearing loss in a Chinese family. Clin Genet 72(5):471–477

Article  CAS  PubMed  Google Scholar 

Croes L, Op de Beeck K, Van Camp G (2015) Role of DFNA5 in hearing loss and cancer - a comment on Rakusic et al. Onco Targets Ther 8:2613–2615

Article  CAS  PubMed  PubMed Central  Google Scholar 

Cunningham LL, Tucci DL (2017) Hearing loss in adults. N Engl J Med 377(25):2465–2473

Article  PubMed  PubMed Central  Google Scholar 

Delmaghani S, del Castillo FJ, Michel V, Leibovici M, Aghaie A, Ron U, Van Laer L, Ben-Tal N, Van Camp G, Weil D, Langa F, Lathrop M, Avan P, Petit C (2006) Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy. Nat Genet 38(7):770–778

Article  CAS  PubMed  Google Scholar 

Ding J, Wang K, Liu W, She Y, Sun Q, Shi J, Sun H, Wang DC, Shao F (2016) Pore-forming activity and structural autoinhibition of the gasdermin family. Nature 535(7610):111–116

Article  CAS  PubMed  Google Scholar 

Gao X, Tao Y, Lamas V, Huang M, Yeh WH, Pan B, Hu YJ, Hu JH, Thompson DB, Shu Y, Li Y, Wang H, Yang S, Xu Q, Polley DB, Liberman MC, Kong WJ, Holt JR, Chen ZY, Liu DR (2018) Treatment of autosomal dominant hearing loss by in vivo delivery of genome editing agents. Nature 553(7687):217–221

Article  CAS  PubMed  Google Scholar 

Hu JJ, Liu X, Xia S, Zhang Z, Zhang Y, Zhao J, Ruan J, Luo X, Lou X, Bai Y, Wang J, Hollingsworth LR, Magupalli VG, Zhao L, Luo HR, Kim J, Lieberman J, Wu H (2020a) FDA-approved disulfiram inhibits pyroptosis by blocking gasdermin D pore formation. Nat Immunol 21(7):736–745

Article  CAS  PubMed  PubMed Central  Google Scholar 

Hu L, Chen M, Chen X, Zhao C, Fang Z, Wang H, Dai H (2020b) Chemotherapy-induced pyroptosis is mediated by BAK/BAX-caspase-3-GSDME pathway and inhibited by 2-bromopalmitate. Cell Death Dis 11(4):281

Article  CAS  PubMed  PubMed Central  Google Scholar 

Humphries F, Shmuel-Galia L, Ketelut-Carneiro N, Li S, Wang B, Nemmara VV, Wilson R, Jiang Z, Khalighinejad F, Muneeruddin K, Shaffer SA, Dutta R, Ionete C, Pesiridis S, Yang S, Thompson PR, Fitzgerald KA (2020) Succination inactivates gasdermin D and blocks pyroptosis. Science 369(6511):1633–1637

Article  CAS  PubMed  PubMed Central  Google Scholar 

Kalinec GM, Webster P, Lim DJ, Kalinec F (2003) A cochlear cell line as an in vitro system for drug ototoxicity screening. Audiol Neurootol 8(4):177–189

Article  CAS  PubMed  Google Scholar 

Karmakar M, Minns M, Greenberg EN, Diaz-Aponte J, Pestonjamasp K, Johnson JL, Rathkey JK, Abbott DW, Wang K, Shao F, Catz SD, Dubyak GR, Pearlman E (2020) N-GSDMD trafficking to neutrophil organelles facilitates IL-1beta release independently of plasma membrane pores and pyroptosis. Nat Commun 11(1):2212

Article  CAS  PubMed  PubMed Central  Google Scholar 

Kayagaki N, Stowe IB, Lee BL, O’Rourke K, Anderson K, Warming S, Cuellar T, Haley B, Roose-Girma M, Phung QT, Liu PS, Lill JR, Li H, Wu J, Kummerfeld S, Zhang J, Lee WP, Snipas SJ, Salvesen GS, Morris LX, Fitzgerald L, Zhang Y, Bertram EM, Goodnow CC, Dixit VM (2015) Caspase-11 cleaves gasdermin D for non-canonical inflammasome signalling. Nature 526(7575):666–671

Article  CAS  PubMed  Google Scholar 

Kim MS, Chang X, Yamashita K, Nagpal JK, Baek JH, Wu G, Trink B, Ratovitski EA, Mori M, Sidransky D (2008) Aberrant promoter methylation and tumor suppressive activity of the DFNA5 gene in colorectal carcinoma. Oncogene 27(25):3624–3634

Article  CAS  PubMed  Google Scholar 

Kovacs SB, Miao EA (2017) Gasdermins: effectors of Pyroptosis. Trends Cell Biol 27(9):673–684

Article  CAS  PubMed  PubMed Central  Google Scholar 

Li CM, Zhang X, Hoffman HJ, Cotch MF, Themann CL, Wilson MR (2014) Hearing impairment associated with depression in US adults, National Health and Nutrition Examination Survey 2005–2010. JAMA Otolaryngol Head Neck Surg 140(4):293–302

Article  PubMed  PubMed Central  Google Scholar 

Li-Yang MN, Shen XF, Wei QJ, Yao J, Lu YJ, Cao X, Xing GQ (2015) IVS8 + 1 DelG, a Novel Splice Site Mutation causing DFNA5 deafness in a Chinese family. Chin Med J (Engl) 128(18):2510–2515

Article  PubMed  Google Scholar 

Lin FR, Yaffe K, Xia J, Xue QL, Harris TB, Purchase-Helzner E, Satterfield S, Ayonayon HN, Ferrucci L, Simonsick EM, Health ABCSG (2013) Hearing loss and cognitive decline in older adults. JAMA Intern Med 173(4):293–299

Article  PubMed  Google Scholar 

Liu W, Xu L, Wang X, Zhang D, Sun G, Wang M, Wang M, Han Y, Chai R, Wang H (2021) PRDX1 activates autophagy via the PTEN-AKT signaling pathway to protect against cisplatin-induced spiral ganglion neuron damage. Autophagy 17(12):4159–4181

Article  CAS  PubMed  PubMed Central  Google Scholar 

Lu H, Zhang S, Wu J, Chen M, Cai MC, Fu Y, Li W, Wang J, Zhao X, Yu Z, Ma P, Zhuang G (2018) Molecular targeted therapies elicit concurrent apoptotic and GSDME-Dependent pyroptotic tumor cell death. Clin Cancer Res 24(23):6066–6077

Article  CAS  PubMed  Google Scholar 

Lu L, Zhang Y, Tan X, Merkher Y, Leonov S, Zhu L, Deng Y, Zhang H, Zhu D, Tan Y, Fu Y, Liu T, Chen Y (2022) Emerging mechanisms of pyroptosis and its therapeutic strategy in cancer. Cell Death Discov 8(1):338

Article  PubMed  PubMed Central  Google Scholar 

Maeda Y, Fukushima K, Kasai N, Maeta M, Nishizaki K (2001) Quantification of TECTA and DFNA5 expression in the developing mouse cochlea. NeuroReport 12(15):3223–3226

Article  CAS  PubMed  Google Scholar 

Mansard L, Vache C, Bianchi J, Baudoin C, Perthus I, Isidor B, Blanchet C, Baux D, Koenig M, Kalatzis V, Roux AF (2022) Identification of the first single GSDME exon 8 Structural Variants Associated with autosomal Dominant hearing loss. Diagnostics (Basel) 12(1)

Nadol JB Jr., Handzel O, Amr S (2015) Histopathology of the human inner ear in a patient with Sensorineural hearing loss caused by a variant in DFNA5. Otol Neurotol 36(10):1616–1621

Article  PubMed  Google Scholar 

Nayagam BA, Muniak MA, Ryugo DK (2011) The spiral ganglion: connecting the peripheral and central auditory systems. Hear Res 278(1–2):2–20

Article  PubMed  PubMed Central  Google Scholar 

Neel DV, Basu H, Gunner G, Bergstresser MD, Giadone RM, Chung H, Miao R, Chou V, Brody E, Jiang X, Lee E, Watts ME, Marques C, Held A, Wainger B, Lagier-Tourenne C, Zhang YJ, Petrucelli L, Young-Pearse TL, Chen-Plotkin AS, Rubin LL, Lieberman J, Chiu IM (2023) Gasdermin-E mediates mitochondrial damage in axons and neurodegeneration. Neuron 111(8):1222–1240 e9

Article  CAS  PubMed  PubMed Central