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Brain Sciences
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Recent Advances in Hearing Impairment
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Recent Advances in Hearing Impairment

A special issue of Brain Sciences (ISSN 2076-3425). This special issue belongs to the section "Neuro-otology and Neuro-ophthalmology".

Deadline for manuscript submissions: 14 February 2025 | Viewed by 6925

Special Issue Editor

Prof. Dr. Agnieszka Szczepek

E-Mail Website
Guest Editor
Department of Otorhinolaryngology, Head and Neck Surgery, Charité Universitätsmedizin, Berlin, Germany
Interests: hearing research; inner ear immunology; ototoxicity
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Hearing impairment is a common condition that has received increased attention recently. Scientific studies showing a link between untreated hearing loss and the development of dementia have been particularly instrumental in raising the profile of hearing loss. The classic causes of hearing loss or deafness are genetic predisposition, ototoxic substances and drugs, noise, and aging. Some diseases, such as diabetes, multiple sclerosis, autoimmune diseases, or certain cancers, are also affected by the development of hearing loss. Because this field of research is evolving exponentially, a thematic collection of the latest discoveries could help us keep up to date with these developments.

This Special Issue aims to collate recent clinical and basic research that provides new information on the ototoxic effects of drugs (including biologics), as well as the lesser-known disease entities associated with hearing impairment, such as (but not limited to) temporomandibular joint disorders, kidney diseases, Hashimoto's disease, or mitochondrial diseases. New biomarkers for hearing loss are also covered.

Original papers (clinical and basic research), case reports, and review articles are welcome. Opinion papers will also be considered in special cases.

Prof. Dr. Agnieszka Szczepek
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. Brain Sciences is an international peer-reviewed open access monthly 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 2200 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

  • ototoxicity
  • autoimmune disease
  • cochlear synaptopathy
  • hair cell loss
  • spiral ganglion damage
  • translational research
  • audiology
  • animal models
  • biomarkers for hearing loss

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Published Papers (7 papers)

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Research

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11 pages, 1749 KiB  
Article
Window Coverage and Liquid Biopsy in the First-Line Therapy of Severe Sudden Sensorineural Hearing Loss
by Alexander Kilgue, Rayoung Kim, Lars-Uwe Scholtz, Conrad Riemann, Christoph J. Pfeiffer, Matthias Schürmann and Ingo Todt
Brain Sci. 2024, 14(11), 1154; https://doi.org/10.3390/brainsci14111154 - 19 Nov 2024
Viewed by 312
Abstract
Introduction: Based on clinical practice guidelines, the application of corticosteroids as a first-line therapy is common. Although sudden sensorineural hearing loss (SSHL) etiology is primarily idiopathic, hearing loss can result from a perilymphatic fistula (PLF). Recent findings show evidence of a specific rate [...] Read more.
Introduction: Based on clinical practice guidelines, the application of corticosteroids as a first-line therapy is common. Although sudden sensorineural hearing loss (SSHL) etiology is primarily idiopathic, hearing loss can result from a perilymphatic fistula (PLF). Recent findings show evidence of a specific rate of PLF based on a cochlin-tomoprotein (CTP) detection test. Based on this rate of PLF treatment, the concepts of SSNHL need to be re-evaluated. The present study aimed to evaluate CTP in SSNHL patients and compare pre-surgical and post-surgical pure tone hearing thresholds after round and oval window sealing as first-line treatment. Material and Methods: A total of 30 patients with unilateral SSNHL with a pure tone average (PTA) (4 Freq. of 60 dB or more were enrolled in a prospective study. All patients underwent tympanoscopy for middle ear exploration as a first-line treatment. After intraoperative observation of a possible PLF, all patients obtained middle ear lavage to gain CTP samples for following ELISA-based CTP detection tests. All patients received round window and oval window sealing with fascia. PTA hearing thresholds were analyzed post-surgically 3 weeks after treatment based on 4-frequency bone conduction (BC). Results: The average preoperative pure tone BC threshold was 97.7 dB compared with the 69 dB postoperative BC threshold. Mean BC improved by 20.3 dB after middle ear exploration and window sealing. A total of 56% (17 of 30) of patients recovered at least 10 dB. The middle ear cochlin-tomoprotein detection rate was 70% positive. Conclusions: The combination of early tympanoscopy and inner ear-specific cochlin-tomoprotein as a detection tool for suspected PLF showed evidence of PLF as a key causative in SSNHL. Full article
(This article belongs to the Special Issue Recent Advances in Hearing Impairment)
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23 pages, 2556 KiB  
Article
Investigation of Deficits in Auditory Emotional Content Recognition by Adult Cochlear Implant Users through the Study of Electroencephalographic Gamma and Alpha Asymmetry and Alexithymia Assessment
by Giulia Cartocci, Bianca Maria Serena Inguscio, Andrea Giorgi, Dario Rossi, Walter Di Nardo, Tiziana Di Cesare, Carlo Antonio Leone, Rosa Grassia, Francesco Galletti, Francesco Ciodaro, Cosimo Galletti, Roberto Albera, Andrea Canale and Fabio Babiloni
Brain Sci. 2024, 14(9), 927; https://doi.org/10.3390/brainsci14090927 - 17 Sep 2024
Viewed by 921
Abstract
Background/Objectives: Given the importance of emotion recognition for communication purposes, and the impairment for such skill in CI users despite impressive language performances, the aim of the present study was to investigate the neural correlates of emotion recognition skills, apart from language, in [...] Read more.
Background/Objectives: Given the importance of emotion recognition for communication purposes, and the impairment for such skill in CI users despite impressive language performances, the aim of the present study was to investigate the neural correlates of emotion recognition skills, apart from language, in adult unilateral CI (UCI) users during a music in noise (happy/sad) recognition task. Furthermore, asymmetry was investigated through electroencephalographic (EEG) rhythm, given the traditional concept of hemispheric lateralization for emotional processing, and the intrinsic asymmetry due to the clinical UCI condition. Methods: Twenty adult UCI users and eight normal hearing (NH) controls were recruited. EEG gamma and alpha band power was assessed as there is evidence of a relationship between gamma and emotional response and between alpha asymmetry and tendency to approach or withdraw from stimuli. The TAS-20 questionnaire (alexithymia) was completed by the participants. Results: The results showed no effect of background noise, while supporting that gamma activity related to emotion processing shows alterations in the UCI group compared to the NH group, and that these alterations are also modulated by the etiology of deafness. In particular, relative higher gamma activity in the CI side corresponds to positive processes, correlated with higher emotion recognition abilities, whereas gamma activity in the non-CI side may be related to positive processes inversely correlated with alexithymia and also inversely correlated with age; a correlation between TAS-20 scores and age was found only in the NH group. Conclusions: EEG gamma activity appears to be fundamental to the processing of the emotional aspect of music and also to the psychocognitive emotion-related component in adults with CI. Full article
(This article belongs to the Special Issue Recent Advances in Hearing Impairment)
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12 pages, 1354 KiB  
Article
Neonatal Markers of Prematurity as Predictors of Permanent Childhood Hearing Loss and Neurodevelopmental Impairment in Children Admitted to the Neonatal Intensive Care Unit
by Hayma Moosan, Derek J. Hoare, Dulip Jayasinghe, Karen R. Willis, Katherine Martin and Sally K. Thornton
Brain Sci. 2024, 14(9), 926; https://doi.org/10.3390/brainsci14090926 - 17 Sep 2024
Viewed by 756
Abstract
Need for admission to the neonatal intensive care unit (NICU) confers an increased risk of hearing loss in the newborn and of later neurodevelopmental impairment. In this retrospective longitudinal case-controlled study, we assess how the degree of prematurity, measured via gestational age, birth [...] Read more.
Need for admission to the neonatal intensive care unit (NICU) confers an increased risk of hearing loss in the newborn and of later neurodevelopmental impairment. In this retrospective longitudinal case-controlled study, we assess how the degree of prematurity, measured via gestational age, birth weight, and z-scores, in 138 infants admitted to the NICU are associated with permanent childhood hearing loss (PCHI) and 2-year developmental outcomes. Logistic regression analyses, Kruskal–Wallis analysis of variance, and Chi-squared tests were used. Independent of prematurity, PCHI and NICU admission were predictive of poor developmental outcomes. Twenty-one (47%) children with PCHI had a moderate-to-severe developmental delay, compared to three (7%) matched controls. Days in the NICU but not z-scores predicted PCHI. Z-score was not prognostic of moderate or severe developmental impairment in children with PCHI. The odds ratio of moderate-to-severe neurodevelopmental impairment with PCHI was high, at 12.48 [95% CI = 3.37–46.40]. Children with PCHI were significantly more likely to have cerebral palsy than their matched counterparts (30% vs. 2%). These findings challenge the conventional focus on gestational age and birth weight on neurodevelopmental outcomes for children with PCHI and NICU admission. A more nuanced approach to monitoring and intervention is needed. Full article
(This article belongs to the Special Issue Recent Advances in Hearing Impairment)
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12 pages, 2433 KiB  
Article
MRI-Based Inner Ear Assessment and Cochlin Tomoprotein-Based Evaluation of Perilymphatic Fistula in Patients with Sudden Hearing Loss
by Rayoung Kim, Denis Mihailovic, Conrad Riemann, Alexander Kilgué, Christoph Joachim Pfeiffer, Hans-Björn Gehl, Lars-Uwe Scholtz and Ingo Todt
Brain Sci. 2024, 14(7), 681; https://doi.org/10.3390/brainsci14070681 - 5 Jul 2024
Viewed by 1056
Abstract
Objectives: To study the correlation between positive cochlin tomoprotein testing (CTP), magnetic resonance (MR) imaging, and the auditory and vestibular function amongst patients with sudden hearing loss. Study Design: Prospective case series. Methods: We prospectively examined eight patients who presented with sudden hearing [...] Read more.
Objectives: To study the correlation between positive cochlin tomoprotein testing (CTP), magnetic resonance (MR) imaging, and the auditory and vestibular function amongst patients with sudden hearing loss. Study Design: Prospective case series. Methods: We prospectively examined eight patients who presented with sudden hearing loss (>60 dB) with or without vertigo or tinnitus. We performed an ELISA-based CTP detection test using middle ear lavage samples. In addition to the CTP examination, a magnetic resonance imaging (MRI) examination was performed using different sequences (T1 and a T1 sequence with a contrast medium (CM), a T2 sequence, 4 h delayed intravenous gadolinium-enhanced three-dimensional fluid-attenuated inversion recovery (3D FLAIR)). Results: All patients with sudden hearing loss (>60 dB) presented a non-specific contrast enhancement in the cochlea and vestibulum on the affected side on delayed 3D-FLAIR MRI. Four patients had a positive CTP test, suggesting a perilymphatic fistula (PLF). However, no specific MRI signal for a PLF was observed. Conclusions: Using multimodal diagnostic measures, such as CTP testing and different MRI sequences, no correlation could be found in patients with a PLF. Full article
(This article belongs to the Special Issue Recent Advances in Hearing Impairment)
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15 pages, 262 KiB  
Article
Wideband Tympanometry and Pressurized Otoacoustic Emissions in Children with Surgical Excision of Palatine and/or Pharyngeal Tonsils
by Aline Buratti Sanches, Milaine Dominici Sanfins, Piotr Henryk Skarzynski, Magdalena Beata Skarżyńska, Henrique Costa Penatti, Caroline Donadon, Ingrid Pereira de Souza, Ingridy Vitoria da Silva and Maria Francisca Colella-Santos
Brain Sci. 2024, 14(6), 598; https://doi.org/10.3390/brainsci14060598 - 14 Jun 2024
Viewed by 905
Abstract
Palatine and pharyngeal tonsil hypertrophy may lead to dysfunction of the auditory tube due to a propensity for infection, potentially giving rise to otitis media. This is a quantitative and longitudinal study, developed from 2019 to 2021, at the State University of Campinas [...] Read more.
Palatine and pharyngeal tonsil hypertrophy may lead to dysfunction of the auditory tube due to a propensity for infection, potentially giving rise to otitis media. This is a quantitative and longitudinal study, developed from 2019 to 2021, at the State University of Campinas (UNICAMP). The studied sample comprised 15 participants aged 5 to 12 years (mean 7.9 years), 12 male and 3 female, arranged into two groups: children diagnosed with pharyngeal and/or palatine tonsil hypertrophy who were candidates for surgery (G1), and children who were later evaluated after surgery (G2). As part of the test, an otoscopy and measurements of logoaudiometry, pure-tone threshold audiometry, wideband tympanometry (ambient and peak pressure), and otoacoustic emissions (TEOAEs and DPOAEs, both at ambient and peak pressure) were all performed. There were statistically significant differences between phases in pure-tone audiometry, in terms of 226 Hz tympanometry, wideband tympanometry in peak pressure conditions, in the amplitude measurement TEOAEs in both pressure conditions, in DPOAEs in ambient pressure conditions, and in the signal/noise measurement in both pressures in DPOAEs. Overall, it was found that hearing tests were different for subjects with palatine and pharyngeal tonsil hypertrophy compared to the post-surgical group. Full article
(This article belongs to the Special Issue Recent Advances in Hearing Impairment)

Review

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17 pages, 645 KiB  
Review
Otorhinolaryngological Problems in Mucopolysaccharidoses: A Review of Common Symptoms in a Rare Disease
by Anna Waśniewska-Włodarczyk, Renata Pepaś, Oskar Rosiak and Wiesław Konopka
Brain Sci. 2024, 14(11), 1085; https://doi.org/10.3390/brainsci14111085 - 29 Oct 2024
Viewed by 546
Abstract
Background: The mucopolysaccharidoses (MPSs) are very rare lysosomal diseases. MPSs belong to inherited diseases; however, newborns are usually asymptomatic. A deficiency of one of the enzymes, which is responsible for glycosaminoglycan (GAG) catabolism, results in the accumulation of this material. GAGs lead to [...] Read more.
Background: The mucopolysaccharidoses (MPSs) are very rare lysosomal diseases. MPSs belong to inherited diseases; however, newborns are usually asymptomatic. A deficiency of one of the enzymes, which is responsible for glycosaminoglycan (GAG) catabolism, results in the accumulation of this material. GAGs lead to progressive damage to tissues. More than 90% of patients with MPS suffer from otitis media with effusion or recurrent otitis media, craniofacial dysmorphia, obstructive sleep apnea, different types of hearing loss, and progressive upper and lower airway dysfunction. Patients visit otolaryngologists often before the diagnosis of MPS. Thus, the awareness of symptoms of MPS is crucial for otolaryngologists and pediatricians. The earlier the diagnosis is made, the more effective treatment is. Ineffective or delayed treatment leads to premature death. Two principal treatments for MPS are currently available: hematopoietic stem cell transplantation (HSCT) and enzyme replacement therapy (ERT). In recent years, there has been a growing interest in gene therapy as a potential treatment for patients with MPS. Mortality in patients with MPS typically occurs during childhood and early adolescence as a consequence of upper and lower respiratory diseases. Methods: This systematic review is based on papers available in the following scientific databases: MEDLINE (via PubMed), Web of Science, Scopus, and the Cochrane Library. Results: After screening, 72 articles met our inclusion criteria. Conclusions: It is of paramount importance that otolaryngologists are involved in this field. This narrative review examines and synthesizes the otolaryngologic issues encountered in patients with MPS. Full article
(This article belongs to the Special Issue Recent Advances in Hearing Impairment)
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Other

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12 pages, 1874 KiB  
Hypothesis
Cross-Modal Tinnitus Remediation: A Tentative Theoretical Framework
by Antoine J. Shahin, Mariel G. Gonzales and Andrew Dimitrijevic
Brain Sci. 2024, 14(1), 95; https://doi.org/10.3390/brainsci14010095 - 19 Jan 2024
Viewed by 1682
Abstract
Tinnitus is a prevalent hearing-loss deficit manifested as a phantom (internally generated by the brain) sound that is heard as a high-frequency tone in the majority of afflicted persons. Chronic tinnitus is debilitating, leading to distress, sleep deprivation, anxiety, and even suicidal thoughts. [...] Read more.
Tinnitus is a prevalent hearing-loss deficit manifested as a phantom (internally generated by the brain) sound that is heard as a high-frequency tone in the majority of afflicted persons. Chronic tinnitus is debilitating, leading to distress, sleep deprivation, anxiety, and even suicidal thoughts. It has been theorized that, in the majority of afflicted persons, tinnitus can be attributed to the loss of high-frequency input from the cochlea to the auditory cortex, known as deafferentation. Deafferentation due to hearing loss develops with aging, which progressively causes tonotopic regions coding for the lost high-frequency coding to synchronize, leading to a phantom high-frequency sound sensation. Approaches to tinnitus remediation that demonstrated promise include inhibitory drugs, the use of tinnitus-specific frequency notching to increase lateral inhibition to the deafferented neurons, and multisensory approaches (auditory–motor and audiovisual) that work by coupling multisensory stimulation to the deafferented neural populations. The goal of this review is to put forward a theoretical framework of a multisensory approach to remedy tinnitus. Our theoretical framework posits that due to vision’s modulatory (inhibitory, excitatory) influence on the auditory pathway, a prolonged engagement in audiovisual activity, especially during daily discourse, as opposed to auditory-only activity/discourse, can progressively reorganize deafferented neural populations, resulting in the reduced synchrony of the deafferented neurons and a reduction in tinnitus severity over time. Full article
(This article belongs to the Special Issue Recent Advances in Hearing Impairment)
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