scholarly journals Inner Ear Gene Therapies Take Off: Current Promises and Future Challenges

2020 ◽  
Vol 9 (7) ◽  
pp. 2309 ◽  
Author(s):  
Sedigheh Delmaghani ◽  
Aziz El-Amraoui

Hearing impairment is the most frequent sensory deficit in humans of all age groups, from children (1/500) to the elderly (more than 50% of the over-75 s). Over 50% of congenital deafness are hereditary in nature. The other major causes of deafness, which also may have genetic predisposition, are aging, acoustic trauma, ototoxic drugs such as aminoglycosides, and noise exposure. Over the last two decades, the study of inherited deafness forms and related animal models has been instrumental in deciphering the molecular, cellular, and physiological mechanisms of disease. However, there is still no curative treatment for sensorineural deafness. Hearing loss is currently palliated by rehabilitation methods: conventional hearing aids, and for more severe forms, cochlear implants. Efforts are continuing to improve these devices to help users to understand speech in noisy environments and to appreciate music. However, neither approach can mediate a full recovery of hearing sensitivity and/or restoration of the native inner ear sensory epithelia. New therapeutic approaches based on gene transfer and gene editing tools are being developed in animal models. In this review, we focus on the successful restoration of auditory and vestibular functions in certain inner ear conditions, paving the way for future clinical applications.

2021 ◽  
Author(s):  
Daniel R. Romano ◽  
Eri Hashino ◽  
Rick F. Nelson

AbstractSensorineural hearing loss (SNHL) is a major cause of functional disability in both the developed and developing world. While hearing aids and cochlear implants provide significant benefit to many with SNHL, neither targets the cellular and molecular dysfunction that ultimately underlies SNHL. The successful development of more targeted approaches, such as growth factor, stem cell, and gene therapies, will require a yet deeper understanding of the underlying molecular mechanisms of human hearing and deafness. Unfortunately, the human inner ear cannot be biopsied without causing significant, irreversible damage to the hearing or balance organ. Thus, much of our current understanding of the cellular and molecular biology of human deafness, and of the human auditory system more broadly, has been inferred from observational and experimental studies in animal models, each of which has its own advantages and limitations. In 2013, researchers described a protocol for the generation of inner ear organoids from pluripotent stem cells (PSCs), which could serve as scalable, high-fidelity alternatives to animal models. Here, we discuss the advantages and limitations of conventional models of the human auditory system, describe the generation and characteristics of PSC-derived inner ear organoids, and discuss several strategies and recent attempts to model hereditary deafness in vitro. Finally, we suggest and discuss several focus areas for the further, intensive characterization of inner ear organoids and discuss the translational applications of these novel models of the human inner ear.


2021 ◽  
Vol 22 (12) ◽  
pp. 6368
Author(s):  
Maurizio Cortada ◽  
Soledad Levano ◽  
Daniel Bodmer

Hearing loss affects many people worldwide and occurs often as a result of age, ototoxic drugs and/or excessive noise exposure. With a growing number of elderly people, the number of people suffering from hearing loss will also increase in the future. Despite the high number of affected people, for most patients there is no curative therapy for hearing loss and hearing aids or cochlea implants remain the only option. Important treatment approaches for hearing loss include the development of regenerative therapies or the inhibition of cell death/promotion of cell survival pathways. The mammalian target of rapamycin (mTOR) pathway is a central regulator of cell growth, is involved in cell survival, and has been shown to be implicated in many age-related diseases. In the inner ear, mTOR signaling has also started to gain attention recently. In this review, we will emphasize recent discoveries of mTOR signaling in the inner ear and discuss implications for possible treatments for hearing restoration.


2021 ◽  
Vol 8 (05) ◽  
pp. 01-03
Author(s):  
Filomena da Ponte

Aging, consensually assumed as a process that affects and alters significantly the cognitive and behavioral functions of the human being , encompassing multiple effects on a number of clinically significant variables , the most important being those that directly relate to the movement, ability to balance the effectiveness of mental functioning and sensory functions such as taste, smell , sight and hearing . Hearing loss in the elderly, scientifically called presbiacustia emerges quietly and is increased progressively with advancing age. This study had as its aim to contribute to the achievement of the best quality of life for elderly individuals with hearing loss (presbycusis). Thus, the objectives targeted were: trying to understand the motivations of the elderly to the use of hearing aids; determine the degree of satisfaction of users of these prostheses; evaluate how Preabycusis affects the quality of life of the elderly. The method used was qualitative oriented once interviewed the elderly users of the home. The sample was composed of 110 participants, with presbycusis, 69 female and 41 male (institutionalized seniors).


2012 ◽  
Vol 13 (3) ◽  
pp. 148-172 ◽  
Author(s):  
Barbara Hutchison ◽  
Eleanor K. Covan ◽  
Janet C. Bogus

We used a novel approach allowing participants in this study to self-monitor their hearing sensitivity to familiar sounds in the environment. The objective of this approach was to establish whether participants in the 80–89 and 90–99 age groups would recognize the degree of their hearing impairment. It was our hope that participants would value audiology treatment and that it would improve their quality of life over a short period. The pilot study took place in a clinical environment where participants answered survey questions before and after audiology treatment, which pertained to their hearing impairment, morale, social support, and life satisfaction. Caregivers were included in the research to rate and record participants’ problem behaviors (e.g., shouting, anger, agitation, repetitive speech dialog, and depression) before, during, and after treatment. This research validates other scientists’ findings that annoying problem behaviors may actually be the result of hearing loss because of a person’s inability to recognize speech. The pilot study shows that participants with mild dementia adapted well to speech and sounds in the natural environment without agitation as well as to management of hearing aids within a 30-day period. The participants with moderate dementia were slower to adapt. All participants with dementia required the cooperation of their caregivers to maintain hygiene of ear canals and hearing aids and insertion. This study suggests that the sooner people receive treatment for hearing loss, the quicker they are to recognize speech and to master hearing aid technology.


2020 ◽  
pp. 014556132093056
Author(s):  
David Ulrich Seidel ◽  
Simon Bode ◽  
Karel Kostev ◽  
Jonas Jae-Hyun Park

Objective: The aim of this study was to determine the incidence of inner ear involvement in various forms of acute otitis media (AOM) in ear, nose, and throat (ENT) practices in Germany. Methods: Patients who had been diagnosed with various forms of AOM in the years 2010 to 2017 were enrolled in the study from a nationwide, representative practice database (Disease Analyzer, IQVIA). In these patients, the incidence of simultaneous or subsequent inner ear disorders (IED) was determined within 7 days and within 12 months from the date of an AOM diagnosis. Results: A total of 286 186 patients with AOM were enrolled. The most frequent diagnoses were “nonsuppurative otitis media, unspecified” (47.6%) and “otitis media, unspecified” (39.0%). The diagnoses of hemorrhagic bullous myringitis (BM) or influenza-induced AOM were very rarely found in the database. The highest incidence of IED after 7 days and 12 months was found in “nonsuppurative otitis media, unspecified” (7.7% and 15.9%, respectively), followed by “otitis media, unspecified” (5.6% and 13.5%, respectively). The incidences of the most frequent IED “hearing loss, unspecified” and “sensorineural hearing loss, unspecified” increased proportionally with increasing patient age, while the rare diagnoses of “labyrinthitis” and “ototoxic hearing loss” were evenly distributed among the age groups. Conclusion: In ENT practices in Germany, both various forms of AOM, as well as simultaneous or subsequent IED, are mostly being coded in an unspecific way, while specific forms such as hemorrhagic BM, influenza-induced AOM, and labyrinthitis are coded very rarely. Older patients have a higher risk of IED in AOM. A visit due to AOM seems to be a regular occasion for the initial diagnosis of hearing impairment in the elderly individuals. The highest risk of IED was found in nonsuppurative AOM.


2021 ◽  
Author(s):  
Alejandro Brice

Hearing loss is very common in the United States and the most widespread disability in the U.S. Hearing loss is the third most chronic health condition in the U.S. Noise induced hearing loss (NIHL) results from damaging external noise. This injury leads to temporarily or permanently affecting sensitive inner ear structures (e.g., cochlea, organ of Corti, and hair cells). NIHL can result from a single high-level noise exposure or repeated exposures to excessively loud noises [i.e., typically 85 dBA or greater, (A weighted decibel)]. Damage to the inner ear can also result from aging (i.e., presbycusis). This case study documents the hearing loss of an otherwise healthy 21-year-old, male individual and his progressive moderate-to-severe sensorineural hearing loss over a period of 41 years. His history will be reported along with his perspective as a speech-language pathologist and speech scientist. The individual with hearing loss has adapted to wearing hearing aids over the last five years. Issues that have occurred affecting comprehension along with compensatory strategies that assisted listening and comprehension will be discussed.


2020 ◽  
Vol 29 (4) ◽  
pp. 738-761
Author(s):  
Tess K. Koerner ◽  
Melissa A. Papesh ◽  
Frederick J. Gallun

Purpose A questionnaire survey was conducted to collect information from clinical audiologists about rehabilitation options for adult patients who report significant auditory difficulties despite having normal or near-normal hearing sensitivity. This work aimed to provide more information about what audiologists are currently doing in the clinic to manage auditory difficulties in this patient population and their views on the efficacy of recommended rehabilitation methods. Method A questionnaire survey containing multiple-choice and open-ended questions was developed and disseminated online. Invitations to participate were delivered via e-mail listservs and through business cards provided at annual audiology conferences. All responses were anonymous at the time of data collection. Results Responses were collected from 209 participants. The majority of participants reported seeing at least one normal-hearing patient per month who reported significant communication difficulties. However, few respondents indicated that their location had specific protocols for the treatment of these patients. Counseling was reported as the most frequent rehabilitation method, but results revealed that audiologists across various work settings are also successfully starting to fit patients with mild-gain hearing aids. Responses indicated that patient compliance with computer-based auditory training methods was regarded as low, with patients generally preferring device-based rehabilitation options. Conclusions Results from this questionnaire survey strongly suggest that audiologists frequently see normal-hearing patients who report auditory difficulties, but that few clinicians are equipped with established protocols for diagnosis and management. While many feel that mild-gain hearing aids provide considerable benefit for these patients, very little research has been conducted to date to support the use of hearing aids or other rehabilitation options for this unique patient population. This study reveals the critical need for additional research to establish evidence-based practice guidelines that will empower clinicians to provide a high level of clinical care and effective rehabilitation strategies to these patients.


Crisis ◽  
2016 ◽  
Vol 37 (2) ◽  
pp. 148-154 ◽  
Author(s):  
Karoly Bozsonyi ◽  
Peter Osvath ◽  
Sandor Fekete ◽  
Lajos Bálint

Abstract. Background: Several studies found a significant relationship between important sport events and suicidal behavior. Aims: We set out to investigate whether there is a significant relationship between the raw suicide rate and the most important international sports events (Olympic Games, FIFA World Cup, UEFA European Championship) in such an achievement-oriented society as the Hungarian one, where these sport events receive great attention. Method: We examined suicide cases occurring over 15,706 days between January 1, 1970, and December 31, 2012 (43 years), separately for each gender. Because of the age-specific characteristics of suicide, the effects of these sport events were analyzed for the middle-aged (30–59 years old) and the elderly (over 60 years old) generations as well as for gender-specific population groups. The role of international sport events was examined with the help of time-series intervention analysis after cyclical and seasonal components were removed. Intervention analysis was based on the ARIMA model. Results: Our results showed that only the Olympic Games had a significant effect in the middle-aged population. Neither in the older male nor in any of the female age groups was a relationship between suicide and Olympic Games detected. Conclusion: The Olympic Games seem to decrease the rate of suicide among middle-aged men, slightly but significantly.


2019 ◽  
pp. 5-34
Author(s):  
Anna L. Lukyanova ◽  
Rostislav I. Kapeliushnikov

The paper analyzes changes in job opportunities of older workers in Russia in the period 2005—2017. The study uses the data from the Russian Labor Force Survey conducted by Rosstat. Changes in the occupational and industrial composition of elderly workers follow the trends pursued by other age groups: employment shifts from low- to high-skilled occupations, from physical to intellectual labor, and from material production to the service sector. We find a stronger polarization among older workers as their occupational structure is biased in favor of, on the one hand, the most and, on the other hand, the least qualified types of jobs. Employment of the elderly has fallen sharply in agriculture and manufacturing with a significant increase in trade, education, and health. Although the employment structure of older workers is generally more “traditionalist”, recent decades have witnessed its transformation in “progressive” directions, similarly to other age groups. These findings suggest that the legislated increase in the state retirement age is not likely to give rise to sizeable unemployment among the elderly. Most of them will be able to work in the occupations and industries previously dominated by young and prime-age workers.


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