The role of DTL in maintaining survival of cochlear hair cell and hearing function.

Rare adverse events after COVID-19 vaccination among Swedish older adults-evidence from a nationwide register-based study.

Assessing the Role of Nystagmus Slow Phase Velocity as a Prognostic Indicator in Idiopathic Sudden Sensorineural Hearing Loss: A Prospective Study: Erratum.

Newborn hearing screening in infants at risk of hearing loss: diagnostic outcomes, false-negative results, and the role of surveillance.

Aymé-Gripp syndrome is an ultra-rare autosomal dominant multisystem disorder caused by pathogenic variants in the MAF gene, typically affecting the N-terminal transactivation domain. It is characterized by craniofacial dysmorphism, early-onset cataracts, sensorineural hearing loss, developmental delay or intellectual disability, and variable neurological or skeletal anomalies. Here, we report two unrelated Turkish patients harboring heterozygous MAF variants within the glycogen synthase kinase 3 recognition motif, evaluated using clinical, neuroimaging, and molecular approaches. Targeted next-generation sequencing (NGS) and parental segregation analyses by NGS and Sanger sequencing were performed, and a literature review of cases published between January 2015 and April 2026 was conducted. Both patients presented with craniofacial and neurodevelopmental features. However, one patient showed no clinically detectable ocular abnormalities or hearing impairment at the time of evaluation. The detected variant in this patient was inherited from his asymptomatic father with low-level mosaicism (16% variant allele frequency in blood and 22% in buccal mucosa), representing the first reported case suggestive of paternal germline mosaicism in Aymé-Gripp syndrome. Literature review (n = 38) revealed consistent findings of sensorineural hearing loss (94.5%), cataracts (78.3%), developmental delay/intellectual disability (100%), epilepsy (68.5%), skeletal anomalies (72.7%), and cardiac involvement (55.1%). Additional features, including non-cataract ocular abnormalities, renal involvement, dermatologic findings, and hematological manifestations, have also been reported. All variants clustered within residues 54-69 of the transactivation domain. These findings provide clinically and molecularly relevant insights into Aymé-Gripp syndrome and highlight the importance of molecular diagnosis and the detection of parental mosaicism for accurate recurrence risk assessment and genetic counseling.

Neuritin protects spiral ganglion neurons via NKA-Ca²⁺ homeostasis and CaMKII/MAPK suppression.

An injectable bioadhesive hyaluronic acid hydrogel formulated with drug-loaded microparticle cross-linkers for co-delivery of dexamethasone and lidocaine to the inner ear.

Guideline-Integrated Large Language Models Improve Decision Support for Acute Ear, Nose and Throat Emergencies.

A Tumor-Bearing Mouse Model of Cisplatin-Induced Hearing Loss for Preclinical Otoprotectant Evaluation.

Dimethyl fumarate alleviates oxidative stress and inflammation in noise-induced hearing loss by activating Nrf2/HO-1 signaling in cochlear hair cells.

We aimed to determine whether computed tomography (CT)-based radiomic features of the inner ear can distinguish the affected side from the contralateral normal-hearing side in patients with idiopathic sudden sensorineural hearing loss (ISSNHL) using a fully automated three-dimensional (3D) segmentation model. Deep learning-based inner ear segmentation followed by radiomics was hypothesized to reveal subtle structural differences associated with ISSNHL. This retrospective study included 318 patients who underwent 420 temporal bone CT scans. An independent test set consisted of 42 inner ear volumes from 21 patients with unilateral ISSNHL, including affected and contralateral normal-hearing sides. Inner ear structures were manually annotated by experienced otologists. A SwinUNETR-based 3D segmentation model was trained using an 8:1:1 dataset split with on-the-fly augmentation. Segmentation performance was evaluated using the Dice similarity coefficient(DSC), Intersection over Union (IoU), accuracy, precision, recall, and F1-score. A total of 1316 radiomic features were extracted from the automatically generated segmentation masks using PyRadiomics, encompassing original, wavelet, and Laplacian of Gaussian (LoG) derived feature classes. Group differences between the ISSNHL-affected and normal-hearing sides were assessed using Welch's t-test or the Mann-Whitney U test, with false discovery rate (FDR) correction applied. The segmentation model demonstrated high and stable performance, with comparable accuracy for ISSNHL-affected and normal-hearing sides. No significant differences were observed in radiomic features between the groups after correction. Principal component analysis and uniform manifold approximation and projection revealed no distinct clustering; the nine shape features with the lowest p-value features exhibited overlapping distributions. CT-based morphological radiomic features did not identify measurable structural differences between the affected and contralateral sides in ISSNHL, supporting the functional or microstructural nature of its underlying pathophysiology. Although automated 3D segmentation using SwinUNETR achieved highly accurate inner ear delineation, CT-derived radiomics demonstrated limited discriminatory value for ISSNHL. Alternative imaging biomarkers, functional imaging approaches, or deep learning-based representation features may be necessary for etiological assessment or prognostication.

Idiopathic sudden sensorineural hearing loss (ISSNHL) is an otologic emergency. While systemic corticosteroids are first-line therapy, intratympanic corticosteroid (ITC) injection has emerged as an important modality. The optimal timing of ITC-as primary therapy, combined initial treatment, or salvage therapy-remains controversial. To critically synthesize current evidence regarding ITC efficacy at different treatment timings and provide evidence-based clinical recommendations. A structured search of PubMed, Embase, and the Cochrane Library was conducted from database inception to December 2025 using terms sudden sensorineural hearing loss, ISSNHL, sudden deafness, intratympanic injection, ITC, intratympanic dexamethasone, and intratympanic methylprednisolone. The search identified 321 records, and additional articles were found in the reference lists of key reviews, meta-analyses, and guidelines. Studies were selected using predefined criteria, prioritizing randomized controlled trials, systematic reviews, meta-analyses, clinical practice guidelines, and relevant observational studies in adults with ISSNHL. Eligible studies evaluated ITC as primary monotherapy, combined initial therapy, or salvage therapy, and reported quantitative audiometric outcomes. Exclusion criteria included non-idiopathic sudden hearing loss, pediatric populations, non-corticosteroid intratympanic therapies, animal or laboratory-only studies, case reports, conference abstracts without full text, duplicate publications, and studies without clinically interpretable hearing outcomes. After screening, 25 studies were included. Salvage ITC provides significant benefit (OR: 6.04; 95% CI: 3.26-11.2; NNT≈3) with Grade A evidence. Combined initial therapy is superior to systemic steroids alone (OR: 2.50; 95% CI: 1.95-3.21), particularly for severe hearing loss. ITC monotherapy is non-inferior to systemic steroids (PTA difference: 2.0 dB). A stratified approach based on treatment timing is recommended. Salvage ITC should be offered to all patients with incomplete recovery. Combined therapy should be considered for patients with severe hearing loss. ITC monotherapy is appropriate when systemic steroids are contraindicated.

BackgroundA hearing impairment is associated with reduced work participation. Research suggests a lack of focus on vocational rehabilitation among a growing population of employees with hearing impairment.ObjectiveThis study aimed to explore the lived experience of people with hearing impairments managing their everyday work situation and conditions that promote work participation.MethodsTen participants - six woman and four men - participated in two audio-recorded focus group interviews, with five participants in each group. The mean age was 57 years (range: 45-70 years). Five participants reported having a sensorineural hearing impairment, two had combined of neurological and conductive hearing impairments, and three were unsure of their diagnostic classification. Eight participants had an acquired hearing impairment, whereas two participants had congenital hearing impairment. The audio-recorded interviews were transcribed and analysed by systematic text condensation.ResultsThe analysis resulted in four intertwined concepts: control; disclosure; support and understanding; and accommodation and technical aids. Further reflection on what it means for participants to be at the 'top of things' revealed three overarching interpretive themes: chasing consistency, managing inconsistency and expecting normalcy.ConclusionsThis study emphasises the importance of for professionals, employer and the employees themselves recognising the vast complexity and dynamic challenges associated of having a hearing impairment in working life. A stronger emphasis on vocational rehabilitation has the potential to enhance work participation for individuals with hearing impairment, with substantial benefits for both individual well-being and broader societal welfare.

Refractory sudden sensorineural hearing loss (RSSNHL) remains a therapeutic challenge with limited effective treatment options. Human umbilical cord mesenchymal stem cell-derived small extracellular vesicles (hucMSC-sEVs) have demonstrated otoprotective and regenerative potential in preclinical studies. This Phase 1 trial evaluated the safety of intratympanic hucMSC-sEVs in patients with RSSNHL. This single-center, open-label, single-arm clinical study enrolled RSSNHL patients between June and September 2025 (ChiCTR2500103765). Participants received intratympanic hucMSC-sEVs for five consecutive days across four dose cohorts, ranging from 8 × 10⁸ to 1 × 10¹¹ particles/mL. Follow-up assessments were conducted at 1, 2, 4, and 8 weeks after treatment, during which adverse events (AEs) were recorded, and hearing outcomes were evaluated. Twelve of the thirteen enrolled patients completed the 8-week follow-up. Intratympanic administration of hucMSC-sEVs was well tolerated across all dose levels, with no dose-limited toxicity or serious AEs. The most common AEs were ear pain (5/12), ear fullness (3/12), and transient dizziness (2/12), all characteristic of intratympanic injection and resolving without intervention. In this Phase 1 study, intratympanic hucMSC-sEVs were well tolerated and showed preliminary signals of hearing improvement. Responses were observed only in patients receiving lower dose levels (8 × 10⁸ and 4 × 10⁹ particles/mL) and in those treated within a shorter disease window (≤ 22 days). These exploratory findings require confirmation in larger controlled trials. Trial Registration This study was registered on June 5,2025, at Chinese Clinical Trial Registry (ChiCTR2500103765).

Detection of cochlear microphonic for differential diagnosis between auditory neuropathy mice and noise-induced sensorineural hearing loss mice.

Dermoid cysts are congenital lesions that typically occur along the midline. They usually remain asymptomatic unless ruptured, causing meningitis, hydrocephalus, or cranial nerve deficits. This case presents an exceptionally rare scenario of bilateral, symmetric intradiploic dermoid cysts of the occipital bone, which initially manifested as sensorineural hearing loss (an unusual symptom). A 21-year-old woman presented with right-sided hearing loss. Imaging showed bilateral, symmetric intradiploic cystic lesions within the occipital bone. The right-sided lesion had ruptured into the posterior fossa, releasing lipid-rich contents over the cerebellum. A T1-hyperintense signal along the right vestibular aqueduct suggested the migration of debris into the inner ear. Both lesions were resected with maximal safe resection, targeting the ruptured component and preventing future complications. Histopathological analysis confirmed that both lesions were dermoid cysts. Although typically associated with chemical meningitis, dermoid cyst rupture may rarely present solely with sensorineural hearing loss when fatty debris migrates into the vestibular aqueduct. Bilateral, symmetric intradiploic occipital lesions are rare and may reflect developmental anomalies at paired ossification centers. Surgical removal of the antigenic source, even without acute complications, may prevent delayed sequelae. A tailored resection approach that preserves vital structures, including the transverse sinus, is safe and effective. https://thejns.org/doi/10.3171/CASE2691.

This study evaluated the indications for cochlear implant (CI) that extend beyond the current criteria of the Health Implementation Communiqué (HIC) of the Turkish Social Security Institution. A retrospective review was performed on 27 patients who underwent CI, even though they did not meet the HIC criteria. All cases were approved by the Scientific Advisory Board on Auditory Implants of the Ministry of Health. Demographic, clinical, and audiological data, including pre- and post-operative pure-tone averages (PTA) and speech discrimination scores (SDS), were analyzed. The cohort included 15 females and 12 males, with a median age of 17 years. Etiologies comprised congenital hearing loss (n=14), idiopathic sudden sensorineural hearing loss (n=3), post-meningitic hearing loss (n=2), Menière's disease (n=1), and other acquired causes. Exclusion from the HIC criteria was mainly due to age restrictions for bilateral CI, audiological thresholds outside defined limits, single-sided deafness, SDS above 30%, or a gap of more than four years between chronological and language age. Audiological outcomes from 22 patients revealed a median PTA with the CI alone of 35 dB hearing level and a median SDS of 58%, with significant improvement compared to baseline (p<0.001). While most patients demonstrated substantial benefit, 14.8% (cases 6, 17, 21, 25) exhibited poor performance (SDS <30%). Case analyses underscored the impact of etiology and duration of auditory deprivation on outcomes. CI beyond conventional reimbursement criteria can provide meaningful functional gains. Individualized, evidence-based, multidisciplinary evaluation supports broader access to hearing rehabilitation and is consistent with global trends in personalized auditory care.

Congenital middle ear anomalies are primarily managed surgically, but data on facial nerve anomalies and bilateral cases are limited. To report endoscopic outcomes for congenital middle ear anomalies, focusing on facial nerve anomalies and bilateral consistency. Retrospective review of 33 patients (41 ears) from 2021 to 2025. Anomalies were intraoperatively classified using the Teunissen system. All underwent endoscopic, class-specific ossicular reconstruction. Facial nerve anomalies recorded. Audiometric outcomes (ABG, 8 kHz) were analyzed. Complications recorded. The cohort had 57.6% unilateral and 42.4% bilateral cases. Facial nerve anomalies in 41.5% of ears. In all eight patients who underwent staged bilateral surgery, the ossicular and facial nerve patterns were identical between the two ears. Mean ABG improved from 39.7 ± 9.5 dB to 14.5 ± 5.4 dB (p < 0.001). Successful hearing (ABG ≤ 20 dB) achieved in 87.8% (Class I 80%, II 80%, III 95.2%, IV 80%). No sensorineural hearing loss (SNHL) or facial palsy. Congenital middle ear anomalies are frequently associated with facial nerve malformations and may exhibit bilateral symmetry in staged cases, offering preoperative insight. An endoscopic, classification-based approach yielded encouraging hearing outcomes and a favorable safety profile, even with complex facial nerve anomalies. These findings are preliminary, limited by retrospective design and sample size.

This systematic review aimed to assess and integrate research on risk factors for congenital hearing loss (CHL), emphasizing genetic, infectious, perinatal, environmental, and sociodemographic influences. The review was prospectively registered with PROSPERO (CRD42022372879) and conducted according to PRISMA 2020 and PRISMA-S guidelines. A comprehensive search was performed across PubMed, Embase, Scopus, and Google Scholar using MeSH terms and free-text keywords related to CHL and its risk factors. Observational studies (cohort, case-control, cross-sectional) involving children with CHL and assessing genetic, infectious, perinatal, or environmental exposures were included. Data extraction was done independently by two reviewers, covering study characteristics, diagnostic methods, and measures of association (odds ratio, relative risk). Risk of bias was evaluated using the Newcastle-Ottawa scale for cohort/case-control studies and the Joanna Briggs Institute checklist for cross-sectional studies. Genetic factors such as GJB2 mutations, a positive family history, and consanguinity were consistently associated with CHL. Infectious etiologies, particularly congenital cytomegalovirus, were prominent across studies, with TORCH infections also commonly implicated. Perinatal risk factors, including neonatal intensive care unit admission, low birth weight, and hyperbilirubinemia, were frequently reported in affected children. Environmental exposures, especially to ototoxic medications, were noted as significant contributors, often acting synergistically with other risk factors like infections or genetic conditions. Sensorineural hearing loss, predominantly bilateral, emerged as the most common type reported. CHL is a multifactorial condition, with genetic and infectious causes being most prevalent. Targeted screening and preventive strategies addressing these risk domains are crucial for early detection and management.

To ascertain the diagnostic yield of MRIs to diagnose vestibular schwannoma based on the patient's presenting symptoms and age. A retrospective cohort study at a single centre. All MRIs ordered by otolaryngologists from 01/01/2022 to 31/12/2023 for the purpose of diagnosing a vestibular schwannoma were included. Data on presenting symptoms, audiogram results, demographics and management outcomes were extracted from clinical records. 1814 MRIs were included. The overall diagnostic yield of vestibular schwannomas was 1.65% (30 cases). Mild asymmetrical sensorineural hearing loss alone (defined as < 15 dB difference between ears at two consecutive frequencies) was not associated with vestibular schwannoma; no patients with this finding were diagnosed (p = 0.025). On multivariate analysis, vertigo (odds ratio [OR] 4.03, p < 0.001) and severe asymmetrical sensorineural hearing loss (OR 5.26, p = 0.002) were independently associated with a diagnosis. Unilateral tinnitus (OR 2.10, p = 0.062), sudden onset asymmetrical hearing loss (OR 0.57, p = 0.46), mild asymmetrical hearing loss (OR 1.03, p = 0.98), or age (OR 0.74, p = 0.48) were not significantly associated with a vestibular schwannoma. Incidental findings were common (23%) and most patients (80%) were managed conservatively with serial MRIs. There was no correlation between age and likelihood of diagnosis. The diagnostic yield of MRIs for vestibular schwannomas is low. Patients with isolated mild asymmetrical hearing loss alone may not warrant further investigation. Presenting with unilateral tinnitus was a poor discriminator for a diagnosis of vestibular schwannomas. However, patients with unexplained vertigo should be considered for an MRI. Further research is needed to refine current guidelines.