scholarly journals miR-29b overexpression induces cochlear hair cell apoptosis through the regulation of SIRT1/PGC-1α signaling: Implications for age-related hearing loss

2016 ◽  
Vol 38 (5) ◽  
pp. 1387-1394 ◽  
Author(s):  
Tao Xue ◽  
Li Wei ◽  
Ding-Jun Zha ◽  
Jian-Hua Qiu ◽  
Fu-Quan Chen ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Cell Apoptosis ◽  
Cochlear Hair Cell ◽  
Age Related ◽  
Age Related Hearing Loss

RETRACTED: miR-34a/Bcl-2 signaling pathway contributes to age-related hearing loss by modulating hair cell apoptosis

2017 ◽  
Vol 661 ◽  
pp. 51-56 ◽  
Author(s):  
Qiuhong Huang ◽  
Yiqing Zheng ◽  
Yongkang Ou ◽  
Hao Xiong ◽  
Haidi Yang ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Signaling Pathway ◽  
Cell Apoptosis ◽  
Age Related ◽  
Age Related Hearing Loss

Recent advances in cochlear hair cell regeneration—A promising opportunity for the treatment of age-related hearing loss

2017 ◽  
Vol 36 ◽  
pp. 149-155 ◽  
Author(s):  
Miren Revuelta ◽  
Francisco Santaolalla ◽  
Olatz Arteaga ◽  
Antonia Alvarez ◽  
Ana Sánchez-del-Rey ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Cell Regeneration ◽  
Hair Cell Regeneration ◽  
Cochlear Hair Cell ◽  
Recent Advances ◽  
Age Related ◽  
Age Related Hearing Loss

SIRT1 protects cochlear hair cell and delays age-related hearing loss via autophagy

2019 ◽  
Vol 80 ◽  
pp. 127-137 ◽  
Author(s):  
Jiaqi Pang ◽  
Hao Xiong ◽  
Yongkang Ou ◽  
Haidi Yang ◽  
Yaodong Xu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Cochlear Hair Cell ◽  
Age Related ◽  
Age Related Hearing Loss

scholarly journals THOC1 deficiency leads to late-onset nonsyndromic hearing loss through p53-mediated hair cell apoptosis

2019 ◽  
Author(s):  
Luping Zhang ◽  
Yu Gao ◽  
Ru Zhang ◽  
Feifei Sun ◽  
Cheng Cheng ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Hair Cells ◽  
Cell Apoptosis ◽  
Late Onset ◽  
Nonsyndromic Hearing Loss ◽  
Progressive Hearing Loss ◽  
Age Related ◽  
Age Related Hearing Loss ◽  
Shed Light

AbstractApoptosis of cochlear hair cells is a key step towards age-related hearing loss. Although numerous genes have been implicated in the genetic causes of late-onset, progressive hearing loss, few show direct links to the proapoptotic process. By genome-wide linkage analysis and whole exome sequencing, we identified a heterozygous p.L183V variant in THOC1 as the probable cause of the late-onset, progressive, non-syndromic hearing loss in a large dominant family. Thoc1, a member of the conserved multisubunit THO/TREX ribonucleoprotein complex, is highly expressed in mouse and zebrafish hair cells. The Thoc1 mutant zebrafish generated by gRNA-Cas9 system lacks the C-startle response, indicative of the hearing dysfunction. Both Thoc1 mutant and knockdown zebrafish have greatly reduced hair cell numbers, while the latter can be rescued by embryonic microinjection of human wild-type THOC1 mRNA but to significantly lesser degree by the p.L183V mutant mRNA. The Thoc1 deficiency resulted in marked apoptosis in zebrafish hair cells. Consistently, transcriptome sequencing of the mutants showed significantly increased gene expression in the p53-associated signaling pathway. Depletion of p53 or applying the p53 inhibitor Pifithrin-α significantly rescued the hair cell loss in the Thoc1 knockdown zebrafish. Our results suggested that THOC1 deficiency lead to late-onset, progressive hearing loss through p53-mediated hair cell apoptosis. This is to our knowledge the first human disease associated with THOC1 mutations and may shed light on the molecular mechanism underlying the age-related hearing loss.Significance StatementFor the first time, we found that THOC1 deficiency leads to late-onset nonsyndromic hearing loss. Furthermore, we revealed the hypomorphic THOC1 induced p53-mediated hair cell apoptosis. This is to our knowledge the first human disease associated with THOC1 mutations and may shed light on the molecular mechanism underlying the age-related hearing loss.

scholarly journals Noise-induced and age-related hearing loss:  new perspectives and potential therapies

F1000Research ◽  
2017 ◽  
Vol 6 ◽  
pp. 927 ◽  
Author(s):  
M Charles Liberman
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Sensorineural Hearing Loss ◽  
Hair Cells ◽  
Auditory Nerve ◽  
Cell Loss ◽  
Sensorineural Hearing ◽  
Hair Cell Loss ◽  
Age Related ◽  
Age Related Hearing Loss

The classic view of sensorineural hearing loss has been that the primary damage targets are hair cells and that auditory nerve loss is typically secondary to hair cell degeneration. Recent work has challenged that view. In noise-induced hearing loss, exposures causing only reversible threshold shifts (and no hair cell loss) nevertheless cause permanent loss of >50% of the synaptic connections between hair cells and the auditory nerve. Similarly, in age-related hearing loss, degeneration of cochlear synapses precedes both hair cell loss and threshold elevation. This primary neural degeneration has remained a “hidden hearing loss” for two reasons: 1) the neuronal cell bodies survive for years despite loss of synaptic connection with hair cells, and 2) the degeneration is selective for auditory nerve fibers with high thresholds. Although not required for threshold detection when quiet, these high-threshold fibers are critical for hearing in noisy environments. Research suggests that primary neural degeneration is an important contributor to the perceptual handicap in sensorineural hearing loss, and it may be key to the generation of tinnitus and other associated perceptual anomalies. In cases where the hair cells survive, neurotrophin therapies can elicit neurite outgrowth from surviving auditory neurons and re-establishment of their peripheral synapses; thus, treatments may be on the horizon.

A DPOAE assessment of outer hair cell integrity in ears with age-related hearing loss

2016 ◽  
Vol 332 ◽  
pp. 137-150 ◽  
Author(s):  
Margarete A. Ueberfuhr ◽  
Hannah Fehlberg ◽  
Shawn S. Goodman ◽  
Robert H. Withnell
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Outer Hair Cell ◽  
Cell Integrity ◽  
Age Related ◽  
Age Related Hearing Loss
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