scholarly journals Mechanism and Prevention of Spiral Ganglion Neuron Degeneration in the Cochlea

2022 ◽  
Vol 15 ◽  
Author(s):  
Li Zhang ◽  
Sen Chen ◽  
Yu Sun
Keyword(s):  
Gene Therapy ◽  
Hearing Loss ◽  
Stem Cell ◽  
Cell Therapy ◽  
Sensory Neurons ◽  
Stem Cell Therapy ◽  
Spiral Ganglion ◽  
Spiral Ganglion Neuron ◽  
Regeneration Ability ◽  
Ganglion Neurons

Sensorineural hearing loss (SNHL) is one of the most prevalent sensory deficits in humans, and approximately 360 million people worldwide are affected. The current treatment option for severe to profound hearing loss is cochlear implantation (CI), but its treatment efficacy is related to the survival of spiral ganglion neurons (SGNs). SGNs are the primary sensory neurons, transmitting complex acoustic information from hair cells to second-order sensory neurons in the cochlear nucleus. In mammals, SGNs have very limited regeneration ability, and SGN loss causes irreversible hearing loss. In most cases of SNHL, SGN damage is the dominant pathogenesis, and it could be caused by noise exposure, ototoxic drugs, hereditary defects, presbycusis, etc. Tremendous efforts have been made to identify novel treatments to prevent or reverse the damage to SGNs, including gene therapy and stem cell therapy. This review summarizes the major causes and the corresponding mechanisms of SGN loss and the current protection strategies, especially gene therapy and stem cell therapy, to promote the development of new therapeutic methods.

scholarly journals Emerging Therapies and Approaches to Treat and Prevent Hearing Loss

2020 ◽  
Vol 5 (5) ◽  
pp. 1147-1165
Author(s):  
Alaa Koleilat ◽  
Colin L. W. Driscoll ◽  
Lisa A. Schimmenti ◽  
Gayla L. Poling
Keyword(s):  
Gene Therapy ◽  
Hearing Loss ◽  
Stem Cell ◽  
Cell Therapy ◽  
Stem Cell Therapy ◽  
Psychosocial Development ◽  
New Therapies ◽  
Recent Developments ◽  
Negative Impacts ◽  
New Treatments

Purpose More than 460 million people are impacted by disabling hearing loss worldwide. Hearing loss has negative impacts across the life span and varies by individual. Specifically, in children, hearing loss has been shown to affect learning outcomes, behavioral and cognitive growth, and psychosocial development. In adults, hearing loss has been shown to affect quality of life, social isolation, depression, and anxiety. The purpose of this review is to advance the understanding of the latest research in the development of new treatments of hearing loss, including gene therapy, stem cell therapy, and pharmacotherapies. We address the use of animals to model human hearing loss and the importance of animals in previous discoveries and developing new therapies. We highlight the importance of early identification for better speech and developmental outcomes specifically in pediatric individuals. Lastly, we review new possibilities in the prevention of hearing loss and the path to translation into practice. Conclusions Research and development of new therapies to treat hearing loss is an ever growing field with many notable successes. However, clinical translation still poses many challenges. Many discoveries related to gene therapy, stem cell therapy, and pharmacotherapy are in the early phases of clinical trials and hold promise. This review empowers audiologists to be aware of these most recent developments and be conscious of the evolving future landscape of treatment and management of hearing loss.

scholarly journals Stem-cell therapy for hearing loss: are we there yet?

2019 ◽  
Vol 85 (4) ◽  
pp. 520-529 ◽  
Author(s):  
Luiz Gustavo Dufner-Almeida ◽  
Dayane Bernardino da Cruz ◽  
Regina Célia Mingroni Netto ◽  
Ana Carla Batissoco ◽  
Jeanne Oiticica ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Stem Cell ◽  
Cell Therapy ◽  
Stem Cell Therapy

scholarly journals Gene therapy as a possible option to treat hereditary hearing loss

2020 ◽  
Vol 32 (2) ◽  
pp. 149-159
Author(s):  
Michael Morgan ◽  
Juliane W. Schott ◽  
Axel Rossi ◽  
Christian Landgraf ◽  
Athanasia Warnecke ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Hearing Loss ◽  
Middle Ear ◽  
Hearing Aids ◽  
Spiral Ganglion ◽  
Primary Auditory Cortex ◽  
Auditory Pathway ◽  
Therapeutic Strategies ◽  
Sound Waves ◽  
Ganglion Neurons

Abstract The process of hearing involves a series of events. The energy of sound is captured by the outer ear and further transferred through the external auditory canal to the middle ear. In the middle ear, sound waves are converted into movements of the tympanic membrane and the ossicles, thereby amplifying the pressure so that it is sufficient to cause movement of the cochlear fluid. The traveling wave within the cochlea leads to depolarization of the inner ear hair cells that, in turn, release the neurotransmitter glutamate. Thereby, the spiral ganglion neurons are activated to transfer the signals via the auditory pathway to the primary auditory cortex. This complex combination of mechanosensory and physiological mechanisms involves many distinct types of cells, the function of which are impacted by numerous proteins, including those involved in ion channel activity, signal transduction and transcription. In the last 30 years, pathogenic variants in over 150 genes were found to be linked to hearing loss. Hearing loss affects over 460 million people world-wide, and current treatment approaches, such as hearing aids and cochlear implants, serve to improve hearing capacity but do not address the underlying genetic cause of hearing loss. Therefore, therapeutic strategies designed to correct the genetic defects causative for hearing loss offer the possibility to treat these patients. In this review, we will discuss genetic causes of hearing loss, novel gene therapeutic strategies to correct hearing loss due to gene defects and some of the preclinical studies in hearing loss animal models as well as the clinical translation of gene therapy approaches to treat hearing loss patients.

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