scholarly journals Animal Model of Sensorineural Hearing Loss Associated with Lassa Virus Infection

2015 ◽  
Vol 90 (6) ◽  
pp. 2920-2927 ◽  
Author(s):  
Nadezhda E. Yun ◽  
Shannon Ronca ◽  
Atsushi Tamura ◽  
Takaaki Koma ◽  
Alexey V. Seregin ◽  
...  
Keyword(s):  
Animal Model ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Spiral Ganglion ◽  
Major Complication ◽  
Sensorineural Hearing ◽  
Lassa Virus ◽  
Loss Mechanism ◽  
Cochlear Hair Cells ◽  
Human Pathology

ABSTRACTApproximately one-third of Lassa virus (LASV)-infected patients develop sensorineural hearing loss (SNHL) in the late stages of acute disease or in early convalescence. With 500,000 annual cases of Lassa fever (LF), LASV is a major cause of hearing loss in regions of West Africa where LF is endemic. To date, no animal models exist that depict the human pathology of LF with associated hearing loss. Here, we aimed to develop an animal model to study LASV-induced hearing loss using human isolates from a 2012 Sierra Leone outbreak. We have recently established a murine model for LF that closely mimics many features of human disease. In this model, LASV isolated from a lethal human case was highly virulent, while the virus isolated from a nonlethal case elicited mostly mild disease with moderate mortality. More importantly, both viruses were able to induce SNHL in surviving animals. However, utilization of the nonlethal, human LASV isolate allowed us to consistently produce large numbers of survivors with hearing loss. Surviving mice developed permanent hearing loss associated with mild damage to the cochlear hair cells and, strikingly, significant degeneration of the spiral ganglion cells of the auditory nerve. Therefore, the pathological changes in the inner ear of the mice with SNHL supported the phenotypic loss of hearing and provided further insights into the mechanistic cause of LF-associated hearing loss.IMPORTANCESensorineural hearing loss is a major complication for LF survivors. The development of a small-animal model of LASV infection that replicates hearing loss and the clinical and pathological features of LF will significantly increase knowledge of pathogenesis and vaccine studies. In addition, such a model will permit detailed characterization of the hearing loss mechanism and allow for the development of appropriate diagnostic approaches and medical care for LF patients with hearing impairment.

The BALB/c mouse as an animal model for progressive sensorineural hearing loss

1998 ◽  
Vol 115 (1-2) ◽  
pp. 162-174 ◽  
Author(s):  
James F Willott ◽  
Jeremy G Turner ◽  
Stephanie Carlson ◽  
Dalian Ding ◽  
Lori Seegers Bross ◽  
...  
Keyword(s):  
Animal Model ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing

scholarly journals Autophagy Regulates the Survival of Hair Cells and Spiral Ganglion Neurons in Cases of Noise, Ototoxic Drug, and Age-Induced Sensorineural Hearing Loss

2021 ◽  
Vol 15 ◽  
Author(s):  
Lingna Guo ◽  
Wei Cao ◽  
Yuguang Niu ◽  
Shuangba He ◽  
Renjie Chai ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hair Cells ◽  
Noise Exposure ◽  
Spiral Ganglion ◽  
Sensorineural Hearing ◽  
Spiral Ganglion Neurons ◽  
Core Components ◽  
Spontaneous Regeneration ◽  
Ganglion Neurons

Inner ear hair cells (HCs) and spiral ganglion neurons (SGNs) are the core components of the auditory system. However, they are vulnerable to genetic defects, noise exposure, ototoxic drugs and aging, and loss or damage of HCs and SGNs results in permanent hearing loss due to their limited capacity for spontaneous regeneration in mammals. Many efforts have been made to combat hearing loss including cochlear implants, HC regeneration, gene therapy, and antioxidant drugs. Here we review the role of autophagy in sensorineural hearing loss and the potential targets related to autophagy for the treatment of hearing loss.

scholarly journals Cochlear Implantation: An Overview

2018 ◽  
Vol 80 (02) ◽  
pp. 169-177 ◽  
Author(s):  
Nicholas Deep ◽  
Eric Dowling ◽  
Daniel Jethanamest ◽  
Matthew Carlson
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Ganglion Cells ◽  
Spiral Ganglion ◽  
Auditory Brainstem ◽  
Electrical Signal ◽  
Sensorineural Hearing ◽  
Acoustic Energy ◽  
Lateral Skull Base ◽  
Single Sided Deafness

AbstractA cochlear implant (CI) is a surgically implanted device for the treatment of severe to profound sensorineural hearing loss in children and adults. It works by transducing acoustic energy into an electrical signal, which is used to stimulate surviving spiral ganglion cells of the auditory nerve. The past 2 decades have witnessed an exponential rise in the number of CI surgeries performed. Continual developments in programming strategies, device design, and minimally traumatic surgical technique have demonstrated the safety and efficacy of CI surgery. As a result, candidacy guidelines have expanded to include both pre and postlingually deaf children as young as 1 year of age, and those with greater degrees of residual hearing. A growing proportion of patients are undergoing CI for off-label or nontraditional indications including single-sided deafness, retrocochlear hearing loss, asymmetrical sensorineural hearing loss (SNHL) in adults and children with at least 1 ear that is better than performance cut-off for age, and children less than 12 months of age. Herein, we review CI design, clinical evaluation, indications, operative technique, and outcomes. We also discuss the expanding indications for CI surgery as it relates to lateral skull base pathology, comparing CI to auditory brainstem implants, and address the concerns with obtaining magnetic resonance imaging (MRI) in CI recipients.

The effect of fractionated radiotherapy in sensorineural hearing loss: An animal model

2014 ◽  
Vol 124 (10) ◽  
pp. E418-E424 ◽  
Author(s):  
Mario A. Mujica‐Mota ◽  
Farid F. Ibrahim ◽  
Aren Bezdjian ◽  
Slobodan Devic ◽  
Sam J. Daniel
Keyword(s):  
Animal Model ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Fractionated Radiotherapy

scholarly journals Congenital Human Cytomegalovirus Infection Inducing Sensorineural Hearing Loss

2021 ◽  
Vol 12 ◽  
Author(s):  
Wenwen Xia ◽  
Hui Yan ◽  
Yiyuan Zhang ◽  
Congcong Wang ◽  
Wei Gao ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Human Cytomegalovirus ◽  
Cytomegalovirus Infection ◽  
Hcmv Infection ◽  
Spiral Ganglion ◽  
Sensorineural Deafness ◽  
Sensorineural Hearing ◽  
Inflammatory Factors ◽  
Congenital Hcmv Infection

Human cytomegalovirus (HCMV) is the primary cause of congenital infections. Despite its clinical significance, congenital HCMV infection is frequently overlooked clinically since most affected infants are asymptomatic. Sensorineural hearing loss (SNHL) is one of the most widely known disorders caused by congenital HCMV infection. The potential mechanism, however, remains unknown to date. The mechanism by which congenital HCMV infection induces sensorineural deafness has been partly characterized, leading to advancements in diagnosis, therapy, and prevention strategies. HCMV-induced hearing loss primarily involves immune responses, the release of inflammatory factors by natural killer (NK) cells, apoptosis of cochlear spiral ganglion, and potential changes due to vascular dysfunction. The diagnosis of HCMV induced SNHL includes serological examination to mothers, imaging, and amniotic fluid examination. Ganciclovir, mainly used for antiviral therapy and behavioral prevention, can, to some degree, prevent congenital HCMV infection. The role of HCMV infection in hearing loss needs further investigation since the mechanism of hearing loss caused by cytomegalovirus infection is not well understood. Although some advancement has been made in diagnosing and treating SNHL, more improvement is needed. A comprehensive understanding of cytomegalovirus’s pathogenesis is of key importance for preventing, diagnosing, and treating SNHL.

GRβ Regulates Glucocorticoid Resistance in Sudden Sensorineural Hearing Loss

2020 ◽  
Vol 21 ◽  
Author(s):  
Xubo Chen ◽  
Qi Zhang ◽  
Chunping Yang ◽  
Yuehui Liu ◽  
Lihua Li
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cell Line ◽  
Mtt Assay ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
High Expression ◽  
Sudden Deafness ◽  
Cochlear Hair Cells ◽  
First Choice

Background: In recent years, the incidence of sudden deafness has gradually increased, with a very limited understanding of the etiology and the pathogenesis. Glucocorticoids are the first choice for the treatment, but some hormoneresistant patients are not sensitive to glucocorticoid therapy. The pathogenesis is not yet known. In this study, we aim to construct HEI-OC1 cell line stably overexpressing glucocorticoid receptor beta (GRβ), and identify its exact role in the cases of glucocorticoid-resistant sudden deafness. Method: We used the endotoxin lipopolysaccharide-stimulated cochlear hair cells (HEI-OC1) to investigate the relationship of inflammation factor IL-2, TNF alpha, and SRp30c with the high expression GRβ. We build a stable GRβ high expression HEI-OC1 cell line and clarified its effects on the therapeutic effect from Dexamethasone. MTT assay, colony formation assay, CCK-8 assay, Western blot, and RT-qPCR were utilized for the characterizations. Results: Dexamethasone reduced LPS-induced inflammatory response in HEI-OC1 cells (p<0.05), detected by MTT assay. Dexamethasone could protect HEI-OC1 cells, but its protective effect was weakened due to the transfection of SRp30c overexpression plasmid (p<0.05). The transfection of SRp30c over-expression plasmid in HEI-OC1 cells could elevate the expressions of GRβ (p<0.05). Conclusion: We clarified the mechanisms of high expression of GRβ in glucocorticoid-resistant sudden sensorineural hearing loss, and proved that the inhibition of SRp30c may act as a new treatment way of glucocorticoid-resistant sudden sensorineural hearing loss.

Sign in / Sign up

Export Citation Format

Share Document