Regeneration of sensory hair cells after acoustic trauma

Science ◽  
1988 ◽  
Vol 240 (4860) ◽  
pp. 1772-1774 ◽  
Author(s):  
JT Corwin ◽  
DA Cotanche
Keyword(s):  
Hearing Loss ◽  
Hair Cells ◽  
Acoustic Trauma ◽  
Sensory Cells ◽  
Lesion Site ◽  
Supporting Cells ◽  
Profound Hearing Loss ◽  
Cochlear Hair Cells ◽  
Sensory Hair ◽  
Sensory Hair Cells

Any loss of cochlear hair cells has been presumed to result in a permanent hearing deficit because the production of these cells normally ceases before birth. However, after acoustic trauma, injured sensory cells in the mature cochlea of the chicken are replaced. New cells appear to be produced by mitosis of supporting cells that survive at the lesion site and do not divide in the absence of trauma. This trauma-induced division of normally postmitotic cells may lead to recovery from profound hearing loss.

Hearing loss caused by progressive degeneration of cochlear hair cells in mice deficient for the Barhl1 homeobox gene

Development ◽  
2002 ◽  
Vol 129 (14) ◽  
pp. 3523-3532 ◽  
Author(s):  
Shengguo Li ◽  
Sandy M. Price ◽  
Hugh Cahill ◽  
David K. Ryugo ◽  
Michael M. Shen ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Inner Ear ◽  
Hair Cells ◽  
Organ Of Corti ◽  
Cochlear Hair Cells ◽  
Sensory Hair ◽  
Progressive Degeneration ◽  
Age Related ◽  
Sensory Hair Cells

The cochlea of the mammalian inner ear contains three rows of outer hair cells and a single row of inner hair cells. These hair cell receptors reside in the organ of Corti and function to transduce mechanical stimuli into electrical signals that mediate hearing. To date, the molecular mechanisms underlying the maintenance of these delicate sensory hair cells are unknown. We report that targeted disruption of Barhl1, a mouse homolog of the Drosophila BarH homeobox genes, results in severe to profound hearing loss, providing a unique model for the study of age-related human deafness disorders. Barhl1 is expressed in all sensory hair cells during inner ear development, 2 days after the onset of hair cell generation. Loss of Barhl1 function in mice results in age-related progressive degeneration of both outer and inner hair cells in the organ of Corti, following two reciprocal longitudinal gradients. Our data together indicate an essential role for Barhl1 in the long-term maintenance of cochlear hair cells, but not in the determination or differentiation of these cells.

Fluorocitrate Neural Dystrophy of the Guinea Pig Cochlea

1975 ◽  
Vol 33 ◽  
pp. 368-369
Author(s):  
G.J. Spector ◽  
C.D. Carr ◽  
I. Kaufman Arenberg ◽  
R.H. Maisel
Keyword(s):  
Hearing Loss ◽  
Hair Cells ◽  
Sodium Phosphate ◽  
Sensory Cells ◽  
Barium Salt ◽  
Perfusion Medium ◽  
Cochlear Hair Cells ◽  
Neural Degeneration ◽  
Sodium Phosphate Buffer ◽  
Cochlear Neurons

All studies on primary neural degeneration in the cochlea have evaluated the end stages of degeneration or the indiscriminate destruction of both sensory cells and cochlear neurons. We have developed a model which selectively simulates the dystrophic changes denoting cochlear neural degeneration while sparing the cochlear hair cells. Such a model can be used to define more precisely the mechanism of presbycusis or the hearing loss in aging man.Twenty-two pigmented guinea pigs (200-250 gm) were perfused by the perilymphatic route as live preparations using fluorocitrate in various concentrations (15-250 ug/cc) and at different incubation times (5-150 minutes). The barium salt of DL fluorocitrate, (C6H4O7F)2Ba3, was reacted with 1.0N sulfuric acid to precipitate the barium as a sulfate. The perfusion medium was prepared, just prior to use, as follows: sodium phosphate buffer 0.2M, pH 7.4 = 9cc; fluorocitrate = 15-200 mg/cc; and sucrose = 0.2M.

scholarly journals Hearing Loss in Neurological Disorders

2021 ◽  
Vol 9 ◽  
Author(s):  
Siyu Li ◽  
Cheng Cheng ◽  
Ling Lu ◽  
Xiaofeng Ma ◽  
Xiaoli Zhang ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cells ◽  
Neurological Disorders ◽  
Molecular Mechanisms ◽  
Relevant Literature ◽  
Autism Spectrum ◽  
Supporting Cells ◽  
Cochlear Hair Cells ◽  
Wide Range ◽  
Histological Characteristics

Sensorineural hearing loss (SNHL) affects approximately 466 million people worldwide, which is projected to reach 900 million by 2050. Its histological characteristics are lesions in cochlear hair cells, supporting cells, and auditory nerve endings. Neurological disorders cover a wide range of diseases affecting the nervous system, including Alzheimer’s disease (AD), Parkinson’s disease (PD), Huntington’s disease (HD), autism spectrum disorder (ASD), etc. Many studies have revealed that neurological disorders manifest with hearing loss, in addition to typical nervous symptoms. The prevalence, manifestations, and neuropathological mechanisms underlying vary among different diseases. In this review, we discuss the relevant literature, from clinical trials to research mice models, to provide an overview of auditory dysfunctions in the most common neurological disorders, particularly those associated with hearing loss, and to explain their underlying pathological and molecular mechanisms.

scholarly journals Cross-Species Experiments Reveal Widespread Cochlear Neural Damage in Normal Hearing

2021 ◽  
Author(s):  
Hari M. Bharadwaj ◽  
Alexandra R. Hustedt-Mai ◽  
Hannah M. Ginsberg ◽  
Kelsey M. Dougherty ◽  
Vijaya Prakash Krishnan Muthaiah ◽  
...  
Keyword(s):  
At Risk ◽  
Hearing Loss ◽  
Animal Models ◽  
Hair Cells ◽  
Normal Hearing ◽  
Afferent Nerve ◽  
Sensory Hair ◽  
Neural Damage ◽  
Sensory Hair Cells ◽  
Afferent Nerve Endings

AbstractAnimal models suggest that cochlear afferent nerve endings may be more vulnerable than sensory hair cells to damage from acoustic overexposure and aging, but that such damage cannot be detected in standard clinical audiometry. Co-ordinated experiments in at-risk humans and a chinchilla model using two distinct physiological assays suggest that cochlear neural damage exists even in populations without clinically recognized hearing loss.

scholarly journals Deletion of Clusterin Protects Cochlear Hair Cells against Hair Cell Aging and Ototoxicity

2021 ◽  
Vol 2021 ◽  
pp. 1-14
Author(s):  
Xiaochang Zhao ◽  
Heidi J. Henderson ◽  
Tianying Wang ◽  
Bo Liu ◽  
Yi Li
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Sensorineural Hearing Loss ◽  
Hair Cells ◽  
Sensorineural Hearing ◽  
Cell Aging ◽  
Supporting Cells ◽  
Drug Induced ◽  
Cochlear Hair Cells ◽  
Age Related

Hearing loss is a debilitating disease that affects 10% of adults worldwide. Most sensorineural hearing loss is caused by the loss of mechanosensitive hair cells in the cochlea, often due to aging, noise, and ototoxic drugs. The identification of genes that can be targeted to slow aging and reduce the vulnerability of hair cells to insults is critical for the prevention of sensorineural hearing loss. Our previous cell-specific transcriptome analysis of adult cochlear hair cells and supporting cells showed that Clu, encoding a secreted chaperone that is involved in several basic biological events, such as cell death, tumor progression, and neurodegenerative disorders, is expressed in hair cells and supporting cells. We generated Clu-null mice (C57BL/6) to investigate its role in the organ of Corti, the sensory epithelium responsible for hearing in the mammalian cochlea. We showed that the deletion of Clu did not affect the development of hair cells and supporting cells; hair cells and supporting cells appeared normal at 1 month of age. Auditory function tests showed that Clu-null mice had hearing thresholds comparable to those of wild-type littermates before 3 months of age. Interestingly, Clu-null mice displayed less hair cell and hearing loss compared to their wildtype littermates after 3 months. Furthermore, the deletion of Clu is protected against aminoglycoside-induced hair cell loss in both in vivo and in vitro models. Our findings suggested that the inhibition of Clu expression could represent a potential therapeutic strategy for the alleviation of age-related and ototoxic drug-induced hearing loss.

scholarly journals Chicken Auditory Supporting Cells Express Interferon Response Genes during Regeneration towards Nascent Sensory Hair Cells In Vivo

2021 ◽  
Author(s):  
Amanda S Janesick ◽  
Mirko Scheibinger ◽  
Nesrine Benkafadar ◽  
Sakin Kirti ◽  
Stefan Heller
Keyword(s):  
Hair Cell ◽  
Hair Cells ◽  
Cell Loss ◽  
Basilar Papilla ◽  
Interferon Response ◽  
Hair Cell Loss ◽  
Supporting Cells ◽  
Sensory Hair ◽  
Sensory Hair Cells

The avian hearing organ is the basilar papilla that, in sharp contrast to the mammalian cochlea, can regenerate sensory hair cells and thereby recover from complete deafness within weeks. The mechanisms that trigger, sustain, and terminate the regenerative response in vivo are largely unknown. Here, we profile the changes in gene expression in the chicken basilar papilla after aminoglycoside antibiotic-induced hair cell loss using RNA-sequencing. The most prominent changes in gene expression were linked to the upregulation of interferon response genes which occurred in supporting cells, confirmed by single-cell RNA-sequencing and in situ hybridization. We determined that the JAK/STAT signaling pathway is essential for the interferon gene response in supporting cells, set in motion by hair cell loss. Four days after ototoxic damage, we identified newly regenerated, nascent auditory hair cells that express genes linked to termination of the interferon response. These cells are incipient modified neurons that represent a population of hair cells en route towards obtaining their location-specific and fully functional cell identity. The robust, transient expression of immune-related genes in supporting cells suggests a potential functional involvement of JAK/STAT signaling and interferon in sensory hair cell regeneration.

Effect of Anesthesia on Susceptibility to Acoustic Trauma

1976 ◽  
Vol 85 (2) ◽  
pp. 276-280 ◽  
Author(s):  
Moshe Rubinstein ◽  
Nechama Pluznik
Keyword(s):  
Hair Cells ◽  
General Condition ◽  
Guinea Pigs ◽  
Pure Tone ◽  
Noise Exposure ◽  
Acoustic Trauma ◽  
Outer Hair Cells ◽  
Sensory Cells ◽  
Cochlear Hair Cells ◽  
Susceptibility To Noise

In an effort to ascertain whether differences in susceptibility to noise depend on general condition, awake and anesthetized guinea pigs were given a 4 kHz pure tone overstimulation under identical conditions. Cochlear hair cells were histologically examined four weeks after the noise exposure. The damage was localized in the upper part of the first turn and the lower part of the second turn. One fourth as much damage occurred in the anesthetized group. The distribution of damage in the four rows of sensory cells was different in the two experimental groups. In both groups of animals the damage was localized mainly to the outer hair cells, the first row sustaining the major damage.

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