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Search Results (432)

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Keywords = functional hearing loss

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13 pages, 872 KB  
Article
Diode Laser Stapedotomy: Audiological Results and Clinical Safety
by Daniela Parrino, Guglielmo Romano, Graziano Pavan, Paolo Castelnuovo and Maurizio Bignami
Audiol. Res. 2026, 16(1), 22; https://doi.org/10.3390/audiolres16010022 - 2 Feb 2026
Viewed by 50
Abstract
Background and objectives: Stapedotomy is the surgical treatment for otosclerosis, with excellent results in terms of hearing recovery. Various laser systems have proven to be an interesting alternative to the conventional technique, allowing for a more precise footplate fenestration with apparently less trauma [...] Read more.
Background and objectives: Stapedotomy is the surgical treatment for otosclerosis, with excellent results in terms of hearing recovery. Various laser systems have proven to be an interesting alternative to the conventional technique, allowing for a more precise footplate fenestration with apparently less trauma to the inner ear. The diode laser, more recently introduced, seems to offer more controlled tissue interaction, potentially reducing thermal damage to surrounding structures. However, the literature remains limited. The aim of the present study was to evaluate the functional outcomes and clinical safety of diode laser stapedotomy by comparing the observed results with those previously reported. Materials and methods: A retrospective analysis of 105 patients who underwent diode laser stapedotomy was conducted. The audiological data and the complications were analyzed and compared with a cohort of patients who underwent stapedotomy performed using the conventional technique. Results: In patients who underwent diode laser stapedotomy, the postoperative air–bone gap (ABG) improved significantly at all frequencies. Hearing outcomes were excellent (ABG ≤ 10 dB) in 60.9% of cases, good (ABG ≤ 20 dB) in 89.5%, and poor (ABG > 20 dB) in 10.5% of patients. Intraoperative complications occurred in seven patients (6.7%), including two cases (1.9%) of footplate damage. Postoperatively, 13 cases of vertigo (12.4%), three cases of tinnitus (2.8%), and one case of sensorineural hearing loss (0.9%) were reported. Conclusions: Diode laser stapedotomy is an effective and safe procedure, providing excellent audiological outcomes without increasing the risk of surgical complications. The possibility of thermal damage to the inner ear must be considered, and appropriate laser parameters should be used to minimize these risks. Full article
(This article belongs to the Section Hearing)
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13 pages, 1092 KB  
Review
Coenzyme Q10 in Hearing Disorders: Replacement Therapy in Mitochondrial Deafness and Neuroprotective Use in Acquired Hearing Loss
by Agnieszka J. Szczepek and Heidi Olze
J. Otorhinolaryngol. Hear. Balance Med. 2026, 7(1), 8; https://doi.org/10.3390/ohbm7010008 - 2 Feb 2026
Viewed by 39
Abstract
Background/Objectives: Coenzyme Q10 (CoQ10) is crucial for mitochondrial bioenergetics and redox balance and has been studied in hearing disorders. Its clinical use ranges from genetic mitochondrial deafness to acquired hearing loss associated with oxidative stress. This review aimed to map human clinical evidence [...] Read more.
Background/Objectives: Coenzyme Q10 (CoQ10) is crucial for mitochondrial bioenergetics and redox balance and has been studied in hearing disorders. Its clinical use ranges from genetic mitochondrial deafness to acquired hearing loss associated with oxidative stress. This review aimed to map human clinical evidence on CoQ10 in hearing issues and differentiate its therapeutic roles based on underlying causes. Methods: This review was conducted following the PRISMA Extension for Scoping Reviews (PRISMA-ScR). A systematic search of PubMed, Europe PubMed Central, the Directory of Open Access Journals (DOAJ), and ClinicalTrials.gov was performed. Human clinical studies evaluating CoQ10 or water-soluble CoQ10 formulations with hearing-related outcomes were included and synthesized descriptively. Results: Fourteen studies met the inclusion criteria, including randomized controlled trials, non-randomized clinical studies, case series, and case reports. Two distinct therapeutic roles of CoQ10 emerged: in primary mitochondrial hearing disorders caused by defects in mitochondrial DNA or CoQ10 biosynthesis pathways, CoQ10 acted as a replacement therapy and was consistently linked to stabilization or prevention of progressive sensorineural hearing loss. Conversely, in acquired or age-related conditions—including presbycusis, noise-induced hearing loss, ototoxicity, tinnitus, and sudden sensorineural hearing loss—CoQ10 was used as an antioxidant or neuroprotective supplement, with outcomes showing functional preservation, symptom reduction, or decreased cochlear injury. Internal validity varied across studies: most evidence for replacement therapy was derived from observational designs, and antioxidant applications were mainly supported by small or preliminary clinical trials. Conclusions: The available evidence suggests two distinct clinical roles of CoQ10 in hearing disorders: (i) replacement therapy in genetically defined mitochondrial deafness and (ii) adjunctive antioxidant/neuroprotective use in acquired conditions. Given heterogeneity and limited study quality, further well-designed trials are needed before broad clinical recommendations can be made. Full article
(This article belongs to the Section Otology and Neurotology)
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10 pages, 344 KB  
Article
Towards a Personalized Vestibular Assessment in Older Patients with Cochlear Implant
by Tiziana Di Cesare, Pasqualina Maria Picciotti, Walter Di Nardo, Daniela Rodolico and Jacopo Galli
J. Pers. Med. 2026, 16(2), 81; https://doi.org/10.3390/jpm16020081 - 1 Feb 2026
Viewed by 108
Abstract
Background: Age-related vestibular decline frequently accompanies presbycusis, and older adults undergoing cochlear implantation (CI) may be particularly vulnerable to postoperative dizziness due to a reduced compensatory capacity and a higher burden of comorbidities. Although CI is an effective treatment for severe-to-profound sensorineural [...] Read more.
Background: Age-related vestibular decline frequently accompanies presbycusis, and older adults undergoing cochlear implantation (CI) may be particularly vulnerable to postoperative dizziness due to a reduced compensatory capacity and a higher burden of comorbidities. Although CI is an effective treatment for severe-to-profound sensorineural hearing loss in the elderly, its impact on vestibular function remains a critical concern. This study aimed to compare pre and postoperative vestibular performance in older patients (≥65 years) versus younger adults undergoing CI in order to identify the risk factors for postoperative vestibular deterioration and critical issues that characterize this category and carry out personalized preoperative counseling. Methods: In this monocentric observational study, adults undergoing CI were divided into two groups: older patients (OPS, ≥65 years) and younger patients (YPS, <65 years). Vestibular function was assessed preoperatively and one month postoperatively through a Dizziness Handicap Inventory (DHI), history of recurrent falls, clinical examination, video head impulse test (VHIT), bithermal caloric testing, and computerized dynamic posturography (Sensory Organization Test, SOT). Risk factors for postoperative vestibular worsening were analyzed using ANOVA test and chi-square statistics, with significance set at p < 0.05. Results: A total of 63 patients were included, with 18 surgeries involving OPS and 45 involving YPS. Preoperatively, OPS showed significantly higher rates of vestibular abnormalities on caloric testing (55.5% vs. 17.7% bilateral hyporeflexia, p < 0.05) and a higher prevalence of recurrent falls (33.3% vs. 4.4%, p < 0.05). Early postoperative dizziness (DHI1) increased significantly in both groups, but age ≥ 65 was a risk factor for ≥10% worsening (OR 2.2, p < 0.05). At one month, YPS returned to baseline DHI values, whereas OPS showed persistent dizziness with significantly higher DHI2 scores (29.2 vs. 12.9, p < 0.05). Vestibular worsening was identified in 33.3% of VHIT assessments and 44.4% of caloric tests in OPS, with caloric testing proving more sensitive than VHIT. Implantation on the better-functioning vestibular side and the presence of ≥3 comorbidities increased the likelihood of persistent postoperative dizziness. Conclusions: Older age is a significant risk factor for persistent dizziness and vestibular impairment one month after CI. Given the reduced compensatory capacity typical of older adults, vestibular assessment should play a central role in preoperative decision-making, particularly for side selection. Bithermal caloric stimulation is recommended as the most sensitive tool for detecting clinically relevant vestibular changes. Preoperative counseling for older CI candidates should include a detailed discussion of vestibular risks and the possible need for postoperative rehabilitation. Full article
(This article belongs to the Special Issue Personalized Medicine for Otolaryngology (ENT))
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16 pages, 1652 KB  
Article
Impact of Amplification and Noise on Subjective Cognitive Effort and Fatigue in Older Adults with Hearing Loss
by Devan M. Lander and Christina M. Roup
Brain Sci. 2026, 16(2), 182; https://doi.org/10.3390/brainsci16020182 - 31 Jan 2026
Viewed by 110
Abstract
Background/Objectives: Older adults with hearing loss frequently report increased listening effort and fatigue, particularly in complex auditory environments. These subjective experiences may reflect increased cognitive resource allocation during both auditory and visual tasks, yet the impact of hearing aids on task-related effort [...] Read more.
Background/Objectives: Older adults with hearing loss frequently report increased listening effort and fatigue, particularly in complex auditory environments. These subjective experiences may reflect increased cognitive resource allocation during both auditory and visual tasks, yet the impact of hearing aids on task-related effort and fatigue remains unclear. This study examined subjective effort and fatigue in experienced older adult hearing aid users while completing cognitively demanding auditory and visual tasks in quiet and background noise, with and without hearing aids. Methods: Thirty-one adults aged 60–87 years completed a cognitive battery assessing inhibition, attention, executive function, and auditory and visual working memory across four listening conditions: aided-quiet, unaided-quiet, aided-noise, and unaided-noise. Subjective effort was measured using the NASA Task Load Index, and task-related fatigue was assessed using a situational fatigue scale. Linear mixed-effects models controlled for age and pure-tone average hearing thresholds. Results: Participants reported significantly lower effort and fatigue in quiet compared to background noise, regardless of hearing aid use. The aided-quiet condition was rated as the least effortful and fatiguing, whereas the unaided-noise condition was rated as the most demanding. Subjective effort and fatigue were moderately to strongly correlated across conditions, particularly in noise. Auditory working memory performance was significantly associated with subjective fatigue across listening conditions, while visual working memory was not associated with effort or fatigue. Hearing aid use did not produce significant reductions in effort or fatigue across conditions. Conclusions: Background noise substantially increases perceived task-related effort and fatigue during cognitively demanding auditory and visual tasks in older adults with hearing loss. While hearing aids did not significantly reduce effort or fatigue across conditions, optimal listening environments were associated with the lowest subjective reports. Auditory working memory emerged as a key factor related to fatigue, highlighting the interplay between hearing, cognition, and subjective listening experiences in older adulthood. Full article
16 pages, 686 KB  
Review
Audiovestibular Dysfunction Related to Long COVID-19 Syndrome: A Systematic Review of Characteristics, Pathophysiology, Diagnosis, and Management
by Jiann-Jy Chen, Chih-Wei Hsu, Hung-Yu Wang, Brendon Stubbs, Tien-Yu Chen, Chih-Sung Liang, Yen-Wen Chen, Bing-Syuan Zeng and Ping-Tao Tseng
Int. J. Mol. Sci. 2026, 27(3), 1417; https://doi.org/10.3390/ijms27031417 - 30 Jan 2026
Viewed by 249
Abstract
Long COVID-19 syndrome (or so-called post-COVID-19) is indicated by miscellaneous symptoms, usually starting 3 months from the COVID-19 infection and lasting for at least 2 months, which cannot be explained by an alternative diagnosis. There has been more and more reports addressing the [...] Read more.
Long COVID-19 syndrome (or so-called post-COVID-19) is indicated by miscellaneous symptoms, usually starting 3 months from the COVID-19 infection and lasting for at least 2 months, which cannot be explained by an alternative diagnosis. There has been more and more reports addressing the audiovestibular dysfunction related to long COVID-19 syndrome. Emerging evidence suggests that the linkage between audiovestibular dysfunction and long COVID-19 syndrome might rely on (a) direct inner ear system damage related to viral invasion and consequent inflammation, (b) micro thromboembolic events, which might result from the COVID-19-induced autoimmune reaction against endothelial cells, and consequent transient-ischemia and hypoxia of the auditory pathways, (c) the disturbed nerve conduction in vestibulocochlear nerves due to viral invasion, and finally (d) altered auditory cortex function, either imbalanced central gain or neurotransmitter disturbance. However, most of the aforementioned mechanism remained hypothetic and still needed further studies to approve or refute. This systematic review synthesizes current evidence on the characteristics, pathophysiology, diagnostic approaches, and management of audiovestibular dysfunction related to long COVID-19 syndrome. Literature searches across PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect (up to 15 December 2025) were conducted in accordance with PRISMA guidelines. Through this systematic review, we provided a schematic diagram of the physiopathology of long COVID-19 syndrome-related audiovestibular dysfunction. Further, we summarized the currently available diagnostic tools to explore the audiovestibular function in such patients. The currently available treatment, either pharmacotherapy or nonpharmacotherapy, mainly tackles idiopathic audiovestibular dysfunction but not specifically long COVID-19 syndrome-related audiovestibular dysfunction. Timely recognition and intervention may prevent progression to permanent hearing loss or vestibular disability, improving quality of life. Trial registration: PROSPERO CRD420251265741. Full article
20 pages, 2617 KB  
Article
Evaluation of Spiral Ganglion Lesions by Electrophysiological Measures
by Max Meuser, Susanne Schwitzer, Parisa Sadat, Horst Hessel, Rainer Seidl, Philipp Mittmann and Dietmar Basta
Brain Sci. 2026, 16(2), 140; https://doi.org/10.3390/brainsci16020140 - 28 Jan 2026
Viewed by 93
Abstract
Background: Through the direct electrical stimulation of spiral ganglion neurons (SGNs) of the hearing nerve, cochlear implants overcome functionally impaired or missing hair cells in patients with profound to severe hearing loss. In routine clinical fitting, regions with severe local SGN degeneration (modiolar [...] Read more.
Background: Through the direct electrical stimulation of spiral ganglion neurons (SGNs) of the hearing nerve, cochlear implants overcome functionally impaired or missing hair cells in patients with profound to severe hearing loss. In routine clinical fitting, regions with severe local SGN degeneration (modiolar “dead regions”) cannot be identified. As a result, the electrical fields of neighboring electrodes are broadened, which can lead to increased channel interaction and, consequently, poorer speech understanding and hearing. The objective of this study was to ascertain whether neural health status can be evaluated by using cochlear implants’ inbuilt measures. Methods: Electrode impedance (MP1-, MP2-, MP1/MP2-, common ground mode), transimpedance matrix (TIM) and electrically evoked compound action potential (eCAP) measurements were performed before and after laser-induced induction of lesions on the modiolus of the guinea pig. Laser treatment-related shifts in impedance, TIM, and eCAP characteristics (threshold, amplitude, and a modified version of the failure index, referred to as the efficiency index (EI)) were correlated with the histologically assessed damage in three predefined areas of the basal modiolus within the electrode region. Results: Modiolar damage resulted in a significant reduction in the electrode impedance in MP2- and MP1/2-mode, the eCAP amplitude, and the EI. In contrast, TIM values and eCAP thresholds were significantly elevated. MP1, MP1/MP2 electrode impedance, TIM, and the eCAP thresholds were not correlated with the extent of modiolar damage. The shifts in eCAP amplitudes and the EI were significantly correlated with the damage at all regions of the basal modiolus. Conclusions: The eCAP amplitude and the EI are both capable of objectively evaluating the neural health status of the cochlea. Thus, a modiolar dead region could be expected from a local drop in eCAP amplitude values or the modified EI within the electrode array. Full article
(This article belongs to the Section Neurotechnology and Neuroimaging)
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16 pages, 1105 KB  
Review
NLRP3 Inflammasome Role and NLRP3 Inhibitors in Sensorineural Hearing Loss
by Mireia Toledano-Pinedo, José Marco-Contelles and Alexey V. Dobrydnev
Biomolecules 2026, 16(2), 186; https://doi.org/10.3390/biom16020186 - 26 Jan 2026
Viewed by 170
Abstract
Herein, we will review the function of the NLRP3 inflammasome in hearing loss (HL), summarize the most significant experimental results described in recent years, describe the biological mechanisms involved in the role of NLRP3 inflammasome in HL, and highlight some of the most [...] Read more.
Herein, we will review the function of the NLRP3 inflammasome in hearing loss (HL), summarize the most significant experimental results described in recent years, describe the biological mechanisms involved in the role of NLRP3 inflammasome in HL, and highlight some of the most promising small NLRP3 inhibitors for its clinical therapy, showing excellent pharmacological effects and good pharmacodynamic/pharmacokinetic profiles. Full article
(This article belongs to the Section Biological Factors)
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20 pages, 466 KB  
Review
The Impacts of Age-Related Peripheral Hearing Loss, Central Auditory Processing, and Cognition on Quality of Life in Older Adults: A Scoping Review
by Samantha E. Vasquez, Anna J. Bierma and Brian M. Kreisman
J. Ageing Longev. 2026, 6(1), 12; https://doi.org/10.3390/jal6010012 - 16 Jan 2026
Viewed by 247
Abstract
This scoping review aims to synthesize peer-reviewed literature exploring quality of life (QoL) for individuals with age-related hearing loss (ARHL), age-related central auditory processing (ARCAP) deficits, and age-related cognitive decline. A growing body of research has identified ARHL as a risk factor for [...] Read more.
This scoping review aims to synthesize peer-reviewed literature exploring quality of life (QoL) for individuals with age-related hearing loss (ARHL), age-related central auditory processing (ARCAP) deficits, and age-related cognitive decline. A growing body of research has identified ARHL as a risk factor for the development of dementia, highlighting the connection between the sensory and cognitive systems. As the aging population continues to grow, examining comorbid age-related hearing and cognitive decline is especially relevant. These conditions may have potential negative consequences on the daily functioning, social participation, mental health, and overall wellbeing of older adults. A systematic search of peer-reviewed literature was conducted across multiple databases, adhering to the PRISMA guidelines for scoping reviews. Studies that focused on the impact of ARHL, ARCAP deficits, and/or related cognitive deficits on QoL were included in the present review. Key data extracted included QoL measures categorized into the ICF framework, the effects of hearing loss intervention on QoL, and the impact of ARHL on QoL for aging individuals. This review summarizes the reported effects that ARHL, ARCAP, and/or cognitive decline have on older adults, and discusses the clinical and practical implications for managing clients with these conditions. In addition to preventative measures and deficit management, maintenance of life participation, social engagement, and overall wellbeing should be considered when caring for aging adults with hearing and/or cognitive impairment. Full article
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13 pages, 252 KB  
Article
Vestibular Function in Long-Term Hearing Aid Users: A Preliminary Investigation
by M. Ramiz Malik, Kaushlendra Kumar, Mohan Kumar Kalaiah, Niraj Kumar Singh and Mayur Bhat
Audiol. Res. 2026, 16(1), 10; https://doi.org/10.3390/audiolres16010010 - 15 Jan 2026
Viewed by 199
Abstract
Background/Objectives: This study compared vestibular evoked myogenic potentials (VEMP) and video head impulse test (vHIT) findings between long-term hearing aid users and non-users with moderately severe to profound sensorineural hearing loss (SNHL) to investigate whether prolonged use of high-power hearing aids has any [...] Read more.
Background/Objectives: This study compared vestibular evoked myogenic potentials (VEMP) and video head impulse test (vHIT) findings between long-term hearing aid users and non-users with moderately severe to profound sensorineural hearing loss (SNHL) to investigate whether prolonged use of high-power hearing aids has any effect on the functioning of peripheral vestibular organs. Methods: A cross-sectional study was conducted in the audiology clinic of a tertiary care hospital. Using convenience sampling, 67 adults aged 20–64 years who visited for hearing evaluation or hearing aid services were recruited and allocated into hearing aid user and non-user groups. VEMP latency and amplitude and vHIT vestibulo-ocular reflex (VOR) gain values were compared between groups. Multiple linear regression was performed to examine the association between the duration of hearing aid use and vestibular function. Results: No significant group differences were observed for any VEMP or vHIT parameter, except for the latency of P1 of the cVEMP in the left ear. Regression analyses indicated that the duration of hearing aid use was not significantly associated with any vestibular test measure. Conclusions: Long-term use of high-power hearing aids does not appear to be associated with measurable alterations in vestibular function. Nonetheless, longitudinal studies with improved control of confounding variables are recommended to validate these preliminary findings. Full article
21 pages, 3413 KB  
Article
The Whole Transcriptome Sequencing Profile of Serum-Derived Exosomes and Potential Pathophysiology of Age-Related Hearing Loss
by Guijun Yang, Zhongqin Xie, Yu Huang, Jing Ke, Ziyi Tang, Zhiji Chen, Shaojing Kuang, Feixian Li, Huan Luo, Qin Lai, Bo Wang, Juhong Zhang and Wei Yuan
Diagnostics 2026, 16(2), 248; https://doi.org/10.3390/diagnostics16020248 - 12 Jan 2026
Viewed by 343
Abstract
Objectives: To systematically analyze the expression profiles of long non-coding RNAs (lncRNAs) in serum-derived exosomes from patients with age-related hearing loss (ARHL), and to further identify key regulatory lncRNAs involved in the pathogenesis and progression of ARHL. Methods: Peripheral blood samples were collected [...] Read more.
Objectives: To systematically analyze the expression profiles of long non-coding RNAs (lncRNAs) in serum-derived exosomes from patients with age-related hearing loss (ARHL), and to further identify key regulatory lncRNAs involved in the pathogenesis and progression of ARHL. Methods: Peripheral blood samples were collected from patients with ARHL and age-matched normal-hearing controls. Serum was separated and exosomes were extracted. The exosomes were identified by nanoparticle tracking analysis (NTA), transmission electron microscopy (TEM), and Western blot. Subsequently, total RNA was extracted from the purified exosomes for lncRNA transcriptome sequencing. Based on the sequencing results, we identified differentially expressed lncRNAs and mRNAs and conducted multi-dimensional functional analysis, including Gene Ontology (GO), Kyoto Encyclopedia of Genes and Genomes (KEGG), Reactome pathway database (Reactome), and Disease Ontology (DO). Finally, four key mRNAs (THAP2, ZNF225, MED12, and RNF141) and four differentially expressed lncRNAs (DE-lncRNAs), namely MSTRG.150961.7, ENSG00000273015, MSTRG.336598.1, and ENSG00000273493, were experimentally verified by quantitative real-time polymerase chain reaction (RT-qPCR) technology. Results: Exosomes were successfully isolated from serum and confirmed by particle size, morphological examination, and the expression of exosome-labeled proteins. A total of 2874 DE-lncRNAs were identified, among which 988 were downregulated and 1886 were upregulated. Similarly, 2132 DE-mRNAs were detected, among which 882 were downregulated and 1250 were upregulated. GO analysis revealed significant enrichment in biological processes such as “phospholipid binding”, “phosphatidylinositol binding”, “phosphatase binding”, “phosphatidylinositol bisphosphate binding”, “phosphatidylinositol-4,5-bisphosphate binding”, “phosphatidylinositol-3,5-bisphosphate phosphatase activity”. KEGG is significantly enriched in signaling pathways including “Wnt signaling pathway”, “Hippo signaling pathway”, “Cushing syndrome”, and “Nucleocytoplasmic transport”. The functional annotations of Reactome were significantly enriched in biomolecular pathways including “tRNA processing”, “Cellular response to heat stress”, “Extra-nuclear estrogen signaling”, “Metabolism of non-coding RNA”, and “CTNNB1 T41 mutants aren’t phosphorylated”. DO is significantly enriched in diseases or pathological conditions such as “hepatitis”, “bacterial infectious disease”, “cystic fibrosis”, and “vasculitis”. Conclusions:THAP2, ZNF225, MED12, and RNF141 may serve as potential candidate biomarker for ARHL. Additionally, lncRNA MSTRG.150961.7, lncRNA MSTRG.336598.1, and lncRNA ENSG00000273493 may play significant roles in the pathogenesis of this condition. Full article
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18 pages, 12725 KB  
Article
Dicer Deletion in the Ear Can Cut Most Neurons and Their Innervation of Hair Cells to Project to the Ear and the Brainstem
by Ebenezer N. Yamoah, Gabriela Pavlinkova, Jeong Han Lee, Jennifer Kersigo, Marsha L. Pierce and Bernd Fritzsch
Int. J. Mol. Sci. 2026, 27(1), 539; https://doi.org/10.3390/ijms27010539 - 5 Jan 2026
Viewed by 623
Abstract
Dicer is crucial for the generation of microRNAs (miRNAs), which are essential for regulating gene expression and keeping neuronal health. Dicer’s conditional deletion cuts all spiral ganglion neurons but spares a small fraction of vestibular ganglion neurons, innervating the utricle and part of [...] Read more.
Dicer is crucial for the generation of microRNAs (miRNAs), which are essential for regulating gene expression and keeping neuronal health. Dicer’s conditional deletion cuts all spiral ganglion neurons but spares a small fraction of vestibular ganglion neurons, innervating the utricle and part of the saccule. Hair cells develop in the utricle, saccule, posterior crista, and the cochlea in Pax2Cre; Dicerf/f. Cochlear hair cells develop at the base and expand the OHC and IHC in the middle, or split into a base/middle and the apex. In contrast, Foxg1Cre; Dicerf/f cuts all canal cristae and cochlea hair cells, leaving a reduced utricle and an exceedingly small saccule. Likewise, Foxg1Cre; Gata3f/f shows no cochlear hair cells and is absent in the horizontal and reduced in the posterior crista. In contrast, the utricle, saccule, and anterior crista are nearly normal, underscoring the intricate regulatory networks involved in hair cell and neuronal development. The central projections have been described as the topology of various null deletions. Still, without spiral ganglion neurons, fibers from Dicer null mice navigate to the cochlear nuclei and expand into the vestibular nuclei to innervate the caudal brainstem. Beyond a ramification around the CN, no fibers expand to reach the cerebellum, likely due to Pax2 and Foxg1 that cut these neurons. Genetic alterations, such as Dicer deletion, can lead to hearing loss and impairments in auditory signal processing, illustrating the critical role of microRNAs in the development and function of auditory and vestibular neurons. Further studies on this topic could help in understanding potential therapeutic targets for hearing loss associated with neuronal degradation of miRNA. Full article
(This article belongs to the Section Molecular Biology)
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12 pages, 1543 KB  
Case Report
Cochlear Implantation in Narrow Duplicated Internal Auditory Canal: Case Report and Systematic Review
by Eleonora Lovati, Davide Soloperto, Michele Pellegrino, Elisabetta Genovese and Daniele Marchioni
J. Otorhinolaryngol. Hear. Balance Med. 2026, 7(1), 2; https://doi.org/10.3390/ohbm7010002 - 31 Dec 2025
Viewed by 302
Abstract
Background: Narrow duplicated internal auditory canal (IAC) is a rare congenital malformation frequently associated with severe-to-profound sensorineural hearing loss. Case Presentation: We present a one-year-old girl with bilateral narrow duplicated IAC and profound hearing loss evaluated through CT/MRI and electrically evoked auditory brainstem [...] Read more.
Background: Narrow duplicated internal auditory canal (IAC) is a rare congenital malformation frequently associated with severe-to-profound sensorineural hearing loss. Case Presentation: We present a one-year-old girl with bilateral narrow duplicated IAC and profound hearing loss evaluated through CT/MRI and electrically evoked auditory brainstem response (EABR). Methods: We conducted a systematic review (1990–2023), identifying 59 published cases of which 24 were bilateral. The mean age at diagnosis was 10.34 years, and 25 cases presented additional inner ear malformations. Only seven patients underwent cochlear implantation, and EABR was performed in four cases. Outcomes of cochlear implantation were heterogeneous. Discussion: In our case, EABR showed a reproducible wave V on the right side, supporting candidacy for cochlear implantation which led to positive early auditory responses. Conclusions: This case and review highlight the role of EABR in identifying residual cochlear nerve functionality and guiding candidacy for cochlear implantation in narrow duplicated IAC. Full article
(This article belongs to the Special Issue Etiology, Diagnosis, and Treatment of Congenital Hearing Loss)
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23 pages, 1227 KB  
Review
Genetics of Waardenburg Syndrome in Africa: A Systematic Review
by Elvis Twumasi Aboagye, Ramses Peigou Wonkam, Carmen de Kock, Collet Dandara and Ambroise Wonkam
Int. J. Mol. Sci. 2026, 27(1), 127; https://doi.org/10.3390/ijms27010127 - 22 Dec 2025
Viewed by 501
Abstract
Waardenburg syndrome (WS) represents a group of genetic conditions characterized by auditory and pigmentation defects. Pathogenic variants in PAX3, MITF, SOX10, EDN3, EDNRB, SNAI2, and KITLG genes have been associated with WS across multiple populations; a comprehensive [...] Read more.
Waardenburg syndrome (WS) represents a group of genetic conditions characterized by auditory and pigmentation defects. Pathogenic variants in PAX3, MITF, SOX10, EDN3, EDNRB, SNAI2, and KITLG genes have been associated with WS across multiple populations; a comprehensive study of WS in Africa has not yet been reported. We conducted a systematic review of clinical expressions and genetics of WS across Africa. The Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were followed, and the study protocol was registered on PROSPERO, the International Prospective Register of Systematic Reviews (2025 CRD420250655744). A literature search was performed on Google Scholar, PubMed, Scopus, Directory of Open Access Journals (DOAJ), Global Index Medicus, African-Wide Information, ScienceDirect, Connecting Repositories (CORE), and the Web of Science databases. We reviewed a total of 15 articles describing 84 WS cases, which showed no gender bias and a mean age at reporting of 17.5 years. Congenital, sensorineural, and profound hearing loss was described in most cases (66.7%; n = 56/84). WS type 2 (WS2), with characteristically no dystopia canthorum, is the predominant subtype (36.9%; n = 31/84). Pathogenic variants in four WS known genes, i.e., PAX3 (13 families), SOX10 (7 families), EDNRB (4 families), and EDN3 (1 family), were reported in Morocco, Tunisia, and South Africa. One candidate gene (PAX8) was described in one family in Ghana. Two non-syndromic hearing loss (NSHL) genes (BDP1 and MYO6) were reported in two separate families in South Africa, suggesting a possible phenotypic expansion. The highest number of WS cases was described in South Africa (38.1%; n = 32/84) and Tunisia (26.2%; n = 22/84). Gene variants were missense (27/43), deletion (7/43), splicing (5/43), nonsense (2/43), indel (1/43), and duplication (1/43), chiefly segregating in an autosomal dominant inheritance mode. There was no functional data to support the pathogenicity of putative causative variants. This review showed that WS2 is the most common in Africa. Variants in PAX3 and SOX10 were the predominant genetic causes. This study emphasizes the need to further investigate in-depth clinical characterization, molecular landscape, and the pathobiology of WS in Africa. Full article
(This article belongs to the Special Issue Hearing Loss: Recent Progress in Molecular Genomics)
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24 pages, 4537 KB  
Review
Cochlear Homeostasis in Sensorineural Hearing Loss: Mechanisms, Implications, and Therapeutic Prospects
by Srdjan M. Vlajkovic, Haruna Suzuki-Kerr and Bryony A. Nayagam
Int. J. Mol. Sci. 2026, 27(1), 102; https://doi.org/10.3390/ijms27010102 - 22 Dec 2025
Viewed by 808
Abstract
Cochlear homeostasis is critical for the preservation of hearing sensitivity by maintaining optimal cochlear fluid composition, sustaining electrochemical gradients, and supporting the function of sensory and supporting cells in the cochlea. Sensorineural hearing loss, resulting from the damage or loss of sensory hair [...] Read more.
Cochlear homeostasis is critical for the preservation of hearing sensitivity by maintaining optimal cochlear fluid composition, sustaining electrochemical gradients, and supporting the function of sensory and supporting cells in the cochlea. Sensorineural hearing loss, resulting from the damage or loss of sensory hair cells, auditory neurons and other cochlear cells and structures, is intimately linked to disruptions in the homeostatic environment. In this narrative review, we explore the cellular and molecular pathways underpinning cochlear homeostasis in health and disease and examine the mechanisms by which failed homeostasis leads to sensorineural hearing loss. We further discuss current research avenues and emerging therapeutic strategies to restore or compensate for the loss of homeostatic balance. These interventions suggest a future where regenerative healing is possible, ultimately leading to permanent repair and functional recovery. Full article
(This article belongs to the Special Issue Inner Ear Disorders: From Molecular Mechanisms to Treatment)
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13 pages, 1912 KB  
Article
Vibro-Acoustic Radiation Analysis for Detecting Otitis Media with Effusion
by Gyuyoung Yi, Jonghoon Jeon, Kyunglae Gu, Junhong Park and Jae Ho Chung
Appl. Sci. 2026, 16(1), 4; https://doi.org/10.3390/app16010004 - 19 Dec 2025
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Abstract
Otitis media with effusion (OME) is a common middle ear disease characterized by fluid accumulation without acute infection, leading to conductive hearing loss. Conventional diagnostic tools, such as tympanometry and otoscopy, have limited sensitivity and rely on expert interpretation. This study investigates vibro-acoustic [...] Read more.
Otitis media with effusion (OME) is a common middle ear disease characterized by fluid accumulation without acute infection, leading to conductive hearing loss. Conventional diagnostic tools, such as tympanometry and otoscopy, have limited sensitivity and rely on expert interpretation. This study investigates vibro-acoustic radiation (VAR) as a novel, non-invasive, and objective method for OME detection. VAR signals were obtained from 36 OME patients (43 ears) and 15 normal ears using bone-conduction excitation and stereo microphones, and the frequency response functions were analyzed. OME increases the mechanical loading of the tympanic membrane and ossicular chain, thereby modifying sound transmission across the middle ear. Using a simplified theoretical model, we estimated acoustic parameters of the ear canal, eardrum, and middle ear, including specific acoustic impedance and resonance frequency ranges, to interpret changes in VAR. VAR analysis revealed significantly reduced signal amplitude in the 8–10 kHz range in OME ears compared with normal ears (p < 0.05). A classification algorithm based on these features achieved 86.7% accuracy, 85.0% sensitivity, and 80.0% specificity, with an area under the ROC curve of 0.986. These findings suggest that VAR has strong potential as a non-invasive diagnostic tool for OME, warranting validation in larger clinical studies. Full article
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