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Therapies for Hearing Loss
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Therapies for Hearing Loss

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Otolaryngology".

Deadline for manuscript submissions: closed (1 April 2020) | Viewed by 143953

Special Issue Editor

Prof. Dr. Eric Truy

E-Mail Website
Guest Editor
1. Departments of ENT (Hôpital Edouard herriot and Hôpital Femme Mère Enfant), Centre Hospitalier et Universitaire de Lyon, Lyon, France
2. Université de Lyon, Lyon, France
3. INSERM U1028 - CNRS UMR5292, Lyon Neuroscience Research Center, Equipe IMPACT, Lyon, France
Interests: cochlear implants; middle ear surgery; deafness; auditiory implants
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

It is my pleasure to invite you to submit papers for a Special Issue of the Journal of Clinical Medicine focused on “Therapies for Hearing Loss”.

Deafness is a major handicap, impending social, emotional, and professional life of millions of people, in an entire lifespan. Babies can be screened at birth and treated very efficiently to date; these major advances in care of deafness in children improve dramatically their well-being. It has been demonstrated that hearing loss can impact cognitive skills in the elderly and that auditory rehabilitation can reduce this impact.

Improvements in knowledge have been very important in the last few decades in the fields of genetics, bioengineering, and auditory function assessment. These have led to possible new therapies for hearing loss. Inner ear therapies are emerging, genetic therapies are not so far for some diseases, and cochlear and other auditory implants afford efficient restoration of audition for severe to profoundly deaf people.

The main ambition of this Special Issue is to share the state-of-the-art in therapies for hearing loss, to scientists and physicians who are not so familiar with deafness.

Prof. Dr. Eric Truy
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • deafness
  • hearing loss
  • genetics
  • auditory implants
  • cochlear implants

Published Papers (18 papers)

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Research

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15 pages, 1543 KiB  
Article
Speech Perception Changes in the Acoustically Aided, Nonimplanted Ear after Cochlear Implantation: A Multicenter Study
by Mario A. Svirsky, Arlene C. Neuman, Jonathan D. Neukam, Annette Lavender, Margaret K. Miller, Ksenia A. Aaron, Piotr H. Skarzynski, Katarzyna B. Cywka, Henryk Skarzynski, Eric Truy, Fabien Seldran, Ruben Hermann, Paul Govaerts, Geert De Ceulaer, Francois Bergeron, Matthieu Hotton, Michelle Moran, Richard C. Dowell, Maria Valeria Schmidt Goffi-Gomez, Ana Tereza de Matos Magalhães, Rosamaria Santarelli and Pietro Scimemiadd Show full author list remove Hide full author list
J. Clin. Med. 2020, 9(6), 1758; https://doi.org/10.3390/jcm9061758 - 5 Jun 2020
Cited by 2 | Viewed by 2841
Abstract
In recent years there has been an increasing percentage of cochlear implant (CI) users who have usable residual hearing in the contralateral, nonimplanted ear, typically aided by acoustic amplification. This raises the issue of the extent to which the signal presented through the [...] Read more.
In recent years there has been an increasing percentage of cochlear implant (CI) users who have usable residual hearing in the contralateral, nonimplanted ear, typically aided by acoustic amplification. This raises the issue of the extent to which the signal presented through the cochlear implant may influence how listeners process information in the acoustically stimulated ear. This multicenter retrospective study examined pre- to postoperative changes in speech perception in the nonimplanted ear, the implanted ear, and both together. Results in the latter two conditions showed the expected increases, but speech perception in the nonimplanted ear showed a modest yet meaningful decrease that could not be completely explained by changes in unaided thresholds, hearing aid malfunction, or several other demographic variables. Decreases in speech perception in the nonimplanted ear were more likely in individuals who had better levels of speech perception in the implanted ear, and in those who had better speech perception in the implanted than in the nonimplanted ear. This raises the possibility that, in some cases, bimodal listeners may rely on the higher quality signal provided by the implant and may disregard or even neglect the input provided by the nonimplanted ear. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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15 pages, 1752 KiB  
Article
Biocompatibility of Bone Marrow-Derived Mesenchymal Stem Cells in the Rat Inner Ear following Trans-Tympanic Administration
by Adrien A. Eshraghi, Emre Ocak, Angela Zhu, Jeenu Mittal, Camron Davies, David Shahal, Erdogan Bulut, Rahul Sinha, Viraj Shah, Mario M. Perdomo and Rahul Mittal
J. Clin. Med. 2020, 9(6), 1711; https://doi.org/10.3390/jcm9061711 - 2 Jun 2020
Cited by 8 | Viewed by 2763
Abstract
Recent advancements in stem cell therapy have led to an increased interest within the auditory community in exploring the potential of mesenchymal stem cells (MSCs) in the treatment of inner ear disorders. However, the biocompatibility of MSCs with the inner ear, especially when [...] Read more.
Recent advancements in stem cell therapy have led to an increased interest within the auditory community in exploring the potential of mesenchymal stem cells (MSCs) in the treatment of inner ear disorders. However, the biocompatibility of MSCs with the inner ear, especially when delivered non-surgically and in the immunocompetent cochlea, is not completely understood. In this study, we determined the effect of intratympanic administration of rodent bone marrow MSCs (BM-MSCs) on the inner ear in an immunocompetent rat model. The administration of MSCs did not lead to the generation of any oxidative stress in the rat inner ear. There was no significant production of proinflammatory cytokines, tumor necrosis factor (TNF)-α, interleukin (IL)-1β, IL-6 and IL-12, due to BM-MSCs administration into the rat cochlea. BM-MSCs do not activate caspase 3 pathway, which plays a central role in sensory cell damage. Additionally, transferase dUTP nick end labeling (TUNEL) staining determined that there was no significant cell death associated with the administration of BM-MSCs. The results of the present study suggest that trans-tympanic administration of BM-MSCs does not result in oxidative stress or inflammatory response in the immunocompetent rat cochlea. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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18 pages, 1190 KiB  
Article
Listening in Noise Remains a Significant Challenge for Cochlear Implant Users: Evidence from Early Deafened and Those with Progressive Hearing Loss Compared to Peers with Normal Hearing
by Yael Zaltz, Yossi Bugannim, Doreen Zechoval, Liat Kishon-Rabin and Ronen Perez
J. Clin. Med. 2020, 9(5), 1381; https://doi.org/10.3390/jcm9051381 - 8 May 2020
Cited by 24 | Viewed by 3341
Abstract
Cochlear implants (CIs) are the state-of-the-art therapy for individuals with severe to profound hearing loss, providing them with good functional hearing. Nevertheless, speech understanding in background noise remains a significant challenge. The purposes of this study were to: (1) conduct a novel within-study [...] Read more.
Cochlear implants (CIs) are the state-of-the-art therapy for individuals with severe to profound hearing loss, providing them with good functional hearing. Nevertheless, speech understanding in background noise remains a significant challenge. The purposes of this study were to: (1) conduct a novel within-study comparison of speech-in-noise performance across ages in different populations of CI and normal hearing (NH) listeners using an adaptive sentence-in-noise test, and (2) examine the relative contribution of sensory information and cognitive–linguistic factors to performance. Forty CI users (mean age 20 years) were divided into “early-implanted” <4 years (n = 16) and “late-implanted” >6 years (n = 11), all prelingually deafened, and “progressively deafened” (n = 13). The control group comprised 136 NH subjects (80 children, 56 adults). Testing included the Hebrew Matrix test, word recognition in quiet, and linguistic and cognitive tests. Results show poorer performance in noise for CI users across populations and ages compared to NH peers, and age at implantation and word recognition in quiet were found to be contributing factors. For those recognizing 50% or more of the words in quiet (n = 27), non-verbal intelligence and receptive vocabulary explained 63% of the variance in noise. This information helps delineate the relative contribution of top-down and bottom-up skills for speech recognition in noise and can help set expectations in CI counseling. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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12 pages, 1868 KiB  
Article
Evaluating the Efficacy of L-N-acetylcysteine and Dexamethasone in Combination to Provide Otoprotection for Electrode Insertion Trauma
by Adrien A. Eshraghi, David Shahal, Camron Davies, Jeenu Mittal, Viraj Shah, Erdogan Bulut, Carolyn Garnham, Priyanka Sinha, Dibyanshi Mishra, Hannah Marwede and Rahul Mittal
J. Clin. Med. 2020, 9(3), 716; https://doi.org/10.3390/jcm9030716 - 6 Mar 2020
Cited by 6 | Viewed by 3216
Abstract
Background: Electrode insertion trauma (EIT) during cochlear implantation (CI) can cause loss of residual hearing. L-N-acetylcysteine (L-NAC) and dexamethasone (Dex) have been individually shown to provide otoprotection albeit at higher concentrations that may be associated with adverse effects. Objective/Aims: The aim of this [...] Read more.
Background: Electrode insertion trauma (EIT) during cochlear implantation (CI) can cause loss of residual hearing. L-N-acetylcysteine (L-NAC) and dexamethasone (Dex) have been individually shown to provide otoprotection albeit at higher concentrations that may be associated with adverse effects. Objective/Aims: The aim of this study is to determine whether L-NAC and Dex could be combined to decrease their effective dosage. Materials and Methods: The organ of Corti (OC) explants were divided into various groups: 1) control; 2) EIT; 3) EIT treated with different concentrations of Dex; 4) EIT treated with different concentrations of L-NAC; 5) EIT treated with L-NAC and Dex in combination. Hair cell (HC) density, levels of oxidative stress, proinflammatory cytokines and nitric oxide (NO) was determined. Results: There was a significant loss of HCs in explants subjected to EIT compared to the control group. L-NAC and Dex in combination was able to provide significant otoprotection at lower concentrations compared to individual drugs. Conclusions and Significance: A combination containing L-NAC and Dex is effective in protecting sensory cells at lower protective doses than each compound separately. These compounds can be combined allowing a decrease of potential side effects of each compound and providing significant otoprotection for EIT. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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23 pages, 481 KiB  
Article
The Effect of Hearing Aid Use on Cognition in Older Adults: Can We Delay Decline or Even Improve Cognitive Function?
by Julia Sarant, David Harris, Peter Busby, Paul Maruff, Adrian Schembri, Ulrike Lemke and Stefan Launer
J. Clin. Med. 2020, 9(1), 254; https://doi.org/10.3390/jcm9010254 - 17 Jan 2020
Cited by 76 | Viewed by 33685
Abstract
Hearing loss is a modifiable risk factor for dementia in older adults. Whether hearing aid use can delay the onset of cognitive decline is unknown. Participants in this study (aged 62–82 years) were assessed before and 18 months after hearing aid fitting on [...] Read more.
Hearing loss is a modifiable risk factor for dementia in older adults. Whether hearing aid use can delay the onset of cognitive decline is unknown. Participants in this study (aged 62–82 years) were assessed before and 18 months after hearing aid fitting on hearing, cognitive function, speech perception, quality of life, physical activity, loneliness, isolation, mood, and medical health. At baseline, multiple linear regression showed hearing loss and age predicted significantly poorer executive function performance, while tertiary education predicted significantly higher executive function and visual learning performance. At 18 months after hearing aid fitting, speech perception in quiet, self-reported listening disability and quality of life had significantly improved. Group mean scores across the cognitive test battery showed no significant decline, and executive function significantly improved. Reliable Change Index scores also showed either clinically significant improvement or stability in executive function for 97.3% of participants, and for females for working memory, visual attention and visual learning. Relative stability and clinically and statistically significant improvement in cognition were seen in this participant group after 18 months of hearing aid use, suggesting that treatment of hearing loss with hearing aids may delay cognitive decline. Given the small sample size, further follow up is required. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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11 pages, 1298 KiB  
Article
Cochlear Implantation Outcome in Children with DFNB1 locus Pathogenic Variants
by Dominika Oziębło, Anita Obrycka, Artur Lorens, Henryk Skarżyński and Monika Ołdak
J. Clin. Med. 2020, 9(1), 228; https://doi.org/10.3390/jcm9010228 - 15 Jan 2020
Cited by 11 | Viewed by 2505
Abstract
Almost 60% of children with profound prelingual hearing loss (HL) have a genetic determinant of deafness, most frequently two DFNB1 locus (GJB2/GJB6 genes) recessive pathogenic variants. Only few studies combine HL etiology with cochlear implantation (CI) outcome. Patients with profound prelingual HL [...] Read more.
Almost 60% of children with profound prelingual hearing loss (HL) have a genetic determinant of deafness, most frequently two DFNB1 locus (GJB2/GJB6 genes) recessive pathogenic variants. Only few studies combine HL etiology with cochlear implantation (CI) outcome. Patients with profound prelingual HL who received a cochlear implant before 24 months of age and had completed DFNB1 genetic testing were enrolled in the study (n = 196). LittlEARS questionnaire scores were used to assess auditory development. Our data show that children with DFNB1-related HL (n = 149) had good outcome from the CI (6.85, 22.24, and 28 scores at 0, 5, and 9 months post-CI, respectively). A better auditory development was achieved in patients who receive cochlear implants before 12 months of age. Children without residual hearing presented a higher rate of auditory development than children with responses in hearing aids over a wide frequency range prior to CI, but both groups reached a similar level of auditory development after 9 months post-CI. Our data shed light upon the benefits of CI in the homogenous group of patients with HL due to DFNB1 locus pathogenic variants and clearly demonstrate that very early CI is the most effective treatment method in this group of patients. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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8 pages, 1365 KiB  
Article
Beware of a Fragile Footplate: Lessons from Ossiculoplasty in Patients with Ossicular Anomalies Related to Second Pharyngeal Arch Defects
by Sun A Han, Goun Choe, Yoonjoong Kim, Ja-Won Koo, Byung Yoon Choi and Jae-Jin Song
J. Clin. Med. 2019, 8(12), 2130; https://doi.org/10.3390/jcm8122130 - 3 Dec 2019
Cited by 8 | Viewed by 7130
Abstract
Background and objectives: We review the intraoperative findings and postoperative outcomes of ossiculoplasty in subjects with second pharyngeal arch (SPA)-derived ossicular anomalies. We summarize potential intraoperative complications and recommend precautions that may reduce the risk of fracture. Materials and Methods: Twenty-four patients with [...] Read more.
Background and objectives: We review the intraoperative findings and postoperative outcomes of ossiculoplasty in subjects with second pharyngeal arch (SPA)-derived ossicular anomalies. We summarize potential intraoperative complications and recommend precautions that may reduce the risk of fracture. Materials and Methods: Twenty-four patients with SPA-derived ossicular anomalies were included, and pre- and postoperative audiometric results were compared. Results: The mean air conduction threshold (56.0 ± 12.4 dB HL) was significantly improved 1 month (27.6 ± 10.1 dB HL) and 6 months (23.8 ± 13.2 dB HL) after surgery (p < 0.001). The preoperative air–bone gap (ABG) (40.4 ± 7.4 dB HL) was significantly decreased at 1 month (15.1 ± 5.9 dB HL) and 6 months (11.3 ± 8.9 dB HL) postoperation. ABG closure was successful (<20 dB HL) in 21 (87.5%) patients 6 months after surgery. Intraoperative footplate fractures occurred in 3 of 24 patients. The fractures were managed successfully, and the ABG closure was successful in all cases. Conclusions: The stapes footplate is likely to be relatively thin in subjects with SPA-derived ossicular anomalies because the footplate is partially or totally derived from the SPA. Thus, a fragile footplate should be expected, and care is needed when handling the footplate. However, when complications are overcome, the audiological outcomes are excellent in most cases. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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17 pages, 2030 KiB  
Article
Solid Lipid Nanoparticles Loaded with Glucocorticoids Protect Auditory Cells from Cisplatin-Induced Ototoxicity
by Blanca Cervantes, Lide Arana, Silvia Murillo-Cuesta, Marina Bruno, Itziar Alkorta and Isabel Varela-Nieto
J. Clin. Med. 2019, 8(9), 1464; https://doi.org/10.3390/jcm8091464 - 14 Sep 2019
Cited by 35 | Viewed by 4460
Abstract
Cisplatin is a chemotherapeutic agent that causes the irreversible death of auditory sensory cells, leading to hearing loss. Local administration of cytoprotective drugs is a potentially better option co-therapy for cisplatin, but there are strong limitations due to the difficulty of accessing the [...] Read more.
Cisplatin is a chemotherapeutic agent that causes the irreversible death of auditory sensory cells, leading to hearing loss. Local administration of cytoprotective drugs is a potentially better option co-therapy for cisplatin, but there are strong limitations due to the difficulty of accessing the inner ear. The use of nanocarriers for the efficient delivery of drugs to auditory cells is a novel approach for this problem. Solid lipid nanoparticles (SLNs) are biodegradable and biocompatible nanocarriers with low solubility in aqueous media. We show here that stearic acid-based SLNs have the adequate particle size, polydispersity index and ζ-potential, to be considered optimal nanocarriers for drug delivery. Stearic acid-based SLNs were loaded with the fluorescent probe rhodamine to show that they are efficiently incorporated by auditory HEI-OC1 (House Ear Institute-Organ of Corti 1) cells. SLNs were not ototoxic over a wide dose range. Glucocorticoids are used to decrease cisplatin-induced ototoxicity. Therefore, to test SLNs’ drug delivery efficiency, dexamethasone and hydrocortisone were tested either alone or loaded into SLNs and tested in a cisplatin-induced ototoxicity in vitro assay. Our results indicate that the encapsulation in SLNs increases the protective effect of low doses of hydrocortisone and lengthens the survival of HEI-OC1 cells treated with cisplatin. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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12 pages, 890 KiB  
Article
“Third Window” and “Single Window” Effects Impede Surgical Success: Analysis of Retrofenestral Otosclerosis Involving the Internal Auditory Canal or Round Window
by Yun Jung Bae, Ye Ji Shim, Byung Se Choi, Jae-Hyoung Kim, Ja-Won Koo and Jae-Jin Song
J. Clin. Med. 2019, 8(8), 1182; https://doi.org/10.3390/jcm8081182 - 7 Aug 2019
Cited by 9 | Viewed by 4240
Abstract
Background and Objectives: We aimed to identify prognostic computed tomography (CT) findings in retrofenestral otosclerosis, with particular attention paid to the role of otosclerotic lesion area in predicting post-stapedotomy outcome. Materials and Methods: We included 17 subjects (23 ears) with retrofenestral otosclerosis who [...] Read more.
Background and Objectives: We aimed to identify prognostic computed tomography (CT) findings in retrofenestral otosclerosis, with particular attention paid to the role of otosclerotic lesion area in predicting post-stapedotomy outcome. Materials and Methods: We included 17 subjects (23 ears) with retrofenestral otosclerosis who underwent stapedotomy. On preoperative CT, the presence of cavitating lesion and involvement of various subsites (cochlea, round window [RW], vestibule, and semicircular canal) were assessed. Pre- and post-stapedotomy audiometric results were compared according to the CT findings. The surgical outcomes were analyzed using logistic regression with Firth correction. Results: Cavitating lesions were present in 15 of 23 ears (65.2%). Involvement of the RW was the strongest predictor of unsuccessful surgical outcome, followed by involvement of the internal auditory canal (IAC) and the cochlea. Conclusions: RW and IAC involvement in retrofenestral otosclerosis were shown to predict unsuccessful outcomes. While a “third window” effect caused by extension of a cavitating lesion into the IAC may dissipate sound energy and thus serve as a barrier to desirable postoperative audiological outcome, a “single window” effect due to an extension of retrofenestral otosclerosis into the RW may preclude a good surgical outcome, even after successful stapedotomy, due to less compressible cochlear fluid and thus decreased linear movement of the piston. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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Review

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27 pages, 3658 KiB  
Review
Inner Ear Gene Therapies Take Off: Current Promises and Future Challenges
by Sedigheh Delmaghani and Aziz El-Amraoui
J. Clin. Med. 2020, 9(7), 2309; https://doi.org/10.3390/jcm9072309 - 21 Jul 2020
Cited by 68 | Viewed by 10988
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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29 pages, 6016 KiB  
Review
Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports
by Romolo Daniele De Siati, Flora Rosenzweig, Guillaume Gersdorff, Anaïs Gregoire, Philippe Rombaux and Naïma Deggouj
J. Clin. Med. 2020, 9(4), 1074; https://doi.org/10.3390/jcm9041074 - 10 Apr 2020
Cited by 46 | Viewed by 14212
Abstract
Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoacoustic emissions (OAEs). These electrophysiological characteristics have led [...] Read more.
Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoacoustic emissions (OAEs). These electrophysiological characteristics have led to the hypothesis that ANSD may be caused by various dysfunctions at the cochlear inner hair cell (IHC) and spiral ganglion neuron (SGN) levels, while the activity of outer hair cells (OHCs) is preserved, resulting in discrepancies between pure-tone and speech comprehension thresholds. The exact prevalence of ANSD remains unknown; clinical findings show a large variability among subjects with hearing impairment ranging from mild to profound hearing loss. A wide range of prenatal and postnatal etiologies have been proposed. The study of genetics and of the implicated sites of lesion correlated with clinical findings have also led to a better understanding of the molecular mechanisms underlying the various forms of ANSD, and may guide clinicians in better screening, assessment and treatment of ANSD patients. Besides OAEs and ABRs, audiological assessment includes stapedial reflex measurements, supraliminal psychoacoustic tests, electrocochleography (ECochG), auditory steady-state responses (ASSRs) and cortical auditory evoked potentials (CAEPs). Hearing aids are indicated in the treatment of ANSD with mild to moderate hearing loss, whereas cochlear implantation is the first choice of treatment in case of profound hearing loss, especially in case of IHC presynaptic disorders, or in case of poor auditory outcomes with conventional hearing aids. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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13 pages, 7265 KiB  
Review
Hearing with One Ear: Consequences and Treatments for Profound Unilateral Hearing Loss
by Hillary A. Snapp and Sebastian A. Ausili
J. Clin. Med. 2020, 9(4), 1010; https://doi.org/10.3390/jcm9041010 - 3 Apr 2020
Cited by 37 | Viewed by 8853
Abstract
There is an increasing global recognition of the negative impact of hearing loss, and its association to many chronic health conditions. The deficits and disabilities associated with profound unilateral hearing loss, however, continue to be under-recognized and lack public awareness. Profound unilateral hearing [...] Read more.
There is an increasing global recognition of the negative impact of hearing loss, and its association to many chronic health conditions. The deficits and disabilities associated with profound unilateral hearing loss, however, continue to be under-recognized and lack public awareness. Profound unilateral hearing loss significantly impairs spatial hearing abilities, which is reliant on the complex interaction of monaural and binaural hearing cues. Unilaterally deafened listeners lose access to critical binaural hearing cues. Consequently, this leads to a reduced ability to understand speech in competing noise and to localize sounds. The functional deficits of profound unilateral hearing loss have a substantial impact on socialization, learning and work productivity. In recognition of this, rehabilitative solutions such as the rerouting of signal and hearing implants are on the rise. This review focuses on the latest insights into the deficits of profound unilateral hearing impairment, and current treatment approaches. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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20 pages, 1369 KiB  
Review
Research Insights on Neural Effects of Auditory Deprivation and Restoration in Unilateral Hearing Loss: A Systematic Review
by Jolijn Vanderauwera, Elisabeth Hellemans and Nicolas Verhaert
J. Clin. Med. 2020, 9(3), 812; https://doi.org/10.3390/jcm9030812 - 17 Mar 2020
Cited by 23 | Viewed by 5203
Abstract
Neuroplasticity following bilateral deafness and auditory restoration has been repeatedly investigated. In clinical practice, however, a significant number of patients present a severe-to-profound unilateral hearing loss (UHL). To date, less is known about the neuroplasticity following monaural hearing deprivation and auditory input restoration. [...] Read more.
Neuroplasticity following bilateral deafness and auditory restoration has been repeatedly investigated. In clinical practice, however, a significant number of patients present a severe-to-profound unilateral hearing loss (UHL). To date, less is known about the neuroplasticity following monaural hearing deprivation and auditory input restoration. This article provides an overview of the current research insights on the impact of UHL on the brain and the effect of auditory input restoration with a cochlear implant (CI). An exhaustive systematic review of the literature was performed selecting 38 studies that apply different neural analyses techniques. The main results show that the hearing ear becomes functionally dominant after monaural deprivation, reshaping the lateralization of the neural network for auditory processing, a process that can be considered to influence auditory restoration. Furthermore, animal models predict that the onset time of UHL impacts auditory restoration. Hence, the results seem to advocate for early restoration of UHL, although further research is required to disambiguate the effects of duration and onset of UHL on auditory restoration and on structural neuroplasticity following UHL deprivation and restoration. Ongoing developments on CI devices compatible with Magnetic Resonance Imaging (MRI) examinations will provide a unique opportunity to investigate structural and functional neuroplasticity following CI restoration more directly. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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12 pages, 247 KiB  
Review
Does Treating Hearing Loss in Older Adults Improve Cognitive Outcomes? A Review
by Hélène Amieva and Camille Ouvrard
J. Clin. Med. 2020, 9(3), 805; https://doi.org/10.3390/jcm9030805 - 16 Mar 2020
Cited by 46 | Viewed by 6830
Abstract
Hearing loss is the third most prevalent health condition in older age. In recent years, research has consistently reported an association between hearing loss and mental health outcomes, including poorer cognitive performances. Whether treating hearing loss in elders improves cognition has been directly [...] Read more.
Hearing loss is the third most prevalent health condition in older age. In recent years, research has consistently reported an association between hearing loss and mental health outcomes, including poorer cognitive performances. Whether treating hearing loss in elders improves cognition has been directly or indirectly addressed by several studies. This review aims at providing a synthesis of those results. Regarding the literature on hearing aids’ use and cognition, although the lack of interventional studies has to be underlined, observational data suggest that hearing aids positively impact long-term cognition, even though more research is necessary to ascertain this statement and provide information on the length or frequency of use required in order to observe benefits. Regarding cochlear implants in elders experiencing more severe auditory deprivation, the literature is scarcer. The available studies have many limitations and do not allow the drawing of clear conclusions. Taken together, the results are encouraging. Nevertheless, because hearing loss is suspected to account for 9% of dementia cases, and also because hearing loss is one of the few potentially modifiable factors from a dementia prevention perspective, the need to stimulate research to have clearer knowledge of the benefits of treating hearing loss on cognitive outcomes is urgent. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
21 pages, 4157 KiB  
Review
rAAV-Mediated Cochlear Gene Therapy: Prospects and Challenges for Clinical Application
by Fabian Blanc, Michel Mondain, Alexis-Pierre Bemelmans, Corentin Affortit, Jean-Luc Puel and Jing Wang
J. Clin. Med. 2020, 9(2), 589; https://doi.org/10.3390/jcm9020589 - 21 Feb 2020
Cited by 12 | Viewed by 5921
Abstract
Over the last decade, pioneering molecular gene therapy for inner-ear disorders have achieved experimental hearing improvements after a single local or systemic injection of adeno-associated, virus-derived vectors (rAAV for recombinant AAV) encoding an extra copy of a normal gene, or ribozymes used to [...] Read more.
Over the last decade, pioneering molecular gene therapy for inner-ear disorders have achieved experimental hearing improvements after a single local or systemic injection of adeno-associated, virus-derived vectors (rAAV for recombinant AAV) encoding an extra copy of a normal gene, or ribozymes used to modify a genome. These results hold promise for treating congenital or later-onset hearing loss resulting from monogenic disorders with gene therapy approaches in patients. In this review, we summarize the current state of rAAV-mediated inner-ear gene therapies including the choice of vectors and delivery routes, and discuss the prospects and obstacles for the future development of efficient clinical rAAV-mediated cochlear gene medicine therapy. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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22 pages, 2356 KiB  
Review
Presbycusis: An Update on Cochlear Mechanisms and Therapies
by Jing Wang and Jean-Luc Puel
J. Clin. Med. 2020, 9(1), 218; https://doi.org/10.3390/jcm9010218 - 14 Jan 2020
Cited by 106 | Viewed by 16659
Abstract
Age-related hearing impairment (ARHI), also referred to as presbycusis, is the most common sensory impairment seen in the elderly. As our cochlea, the peripheral organ of hearing, ages, we tend to experience a decline in hearing and are at greater risk of cochlear [...] Read more.
Age-related hearing impairment (ARHI), also referred to as presbycusis, is the most common sensory impairment seen in the elderly. As our cochlea, the peripheral organ of hearing, ages, we tend to experience a decline in hearing and are at greater risk of cochlear sensory-neural cell degeneration and exacerbated age-related hearing impairments, e.g., gradual hearing loss, deterioration in speech comprehension (especially in noisy environments), difficulty in the localization sound sources, and ringing sensations in the ears. However, the aging process does not affect people uniformly; nor, in fact, does the aging process appear to be uniform even within an individual. Here, we outline recent research into chronological cochlear age in healthy people, and exacerbated hearing impairments during aging due to both extrinsic factors including noise and ototoxic medication, and intrinsic factors such as genetic predisposition, epigenetic factors, and aging. We review our current understanding of molecular pathways mediating ARHL and discuss recent discoveries in experimental hearing restoration and future prospects. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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15 pages, 1142 KiB  
Opinion
Musical Training for Auditory Rehabilitation in Hearing Loss
by Jacques Pesnot Lerousseau, Céline Hidalgo and Daniele Schön
J. Clin. Med. 2020, 9(4), 1058; https://doi.org/10.3390/jcm9041058 - 8 Apr 2020
Cited by 8 | Viewed by 4563
Abstract
Despite the overall success of cochlear implantation, language outcomes remain suboptimal and subject to large inter-individual variability. Early auditory rehabilitation techniques have mostly focused on low-level sensory abilities. However, a new body of literature suggests that cognitive operations are critical for auditory perception [...] Read more.
Despite the overall success of cochlear implantation, language outcomes remain suboptimal and subject to large inter-individual variability. Early auditory rehabilitation techniques have mostly focused on low-level sensory abilities. However, a new body of literature suggests that cognitive operations are critical for auditory perception remediation. We argue in this paper that musical training is a particularly appealing candidate for such therapies, as it involves highly relevant cognitive abilities, such as temporal predictions, hierarchical processing, and auditory-motor interactions. We review recent studies demonstrating that music can enhance both language perception and production at multiple levels, from syllable processing to turn-taking in natural conversation. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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15 pages, 1407 KiB  
Perspective
Contemporary Speech and Oral Language Care for Deaf and Hard-of-Hearing Children Using Hearing Devices
by François Bergeron, Aurore Berland, Dominique Demers and Suzie Gobeil
J. Clin. Med. 2020, 9(2), 378; https://doi.org/10.3390/jcm9020378 - 30 Jan 2020
Cited by 7 | Viewed by 5162
Abstract
Contemporary speech and language interventions are not limited to disabilities but embrace the pragmatics of communication behaviors from the perspective of functional social participation. Accordingly, current speech and language therapies for deaf and hard-of-hearing children include a broad spectrum of approaches and techniques. [...] Read more.
Contemporary speech and language interventions are not limited to disabilities but embrace the pragmatics of communication behaviors from the perspective of functional social participation. Accordingly, current speech and language therapies for deaf and hard-of-hearing children include a broad spectrum of approaches and techniques. This paper explores contemporary approaches and techniques for speech and oral language interventions for deaf and hard-of-hearing children using hearing devices, evidence of efficacy and how they are implemented in diverse clinical practices. Full article
(This article belongs to the Special Issue Therapies for Hearing Loss)
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