Case report: endolymphatic sac tumor masquerading as Meniere's disease.

Marked enhancement of bone conduction subsequent to TORP implantation following oval window reinforcement: A case report and comprehensive narrative review.

The development of inner ear gene therapy for DFNB9: From bench to bedside.

Patient-derived TWNK variants recapitulate multisystem Perrault syndrome pathology in a mouse model.

[Elevated ABR thresholds in the presence of otoacoustic emissions: case series].

Precise and efficient DNA base editing restores normal hearing in adult DFNB9 mouse model.

Neural Hearing Loss: Mechanisms, Diagnosis and Treatment Horizons.

A Diphtheria Toxin-Induced Mouse Model of Auditory Neuropathy: From Cochlear Synaptopathy to Neuronal Degeneration.

Case Report: SLC52A2 variants cause Brown-Vialetto-Van Laere syndrome type 2, characterized by pure red cell aplastic anemia: clinical and genetic features of three Chinese children.

Patient-Reported Outcomes and Long-Term Toxicity in stage I TGCT: A Retrospective Single-Center Cohort (1994-2023).

Neoadjuvant Therapy in Cisplatin-Ineligible Muscle-Invasive Bladder Cancer: Recent Progress, Challenges, and Future Directions in the Era of TAR-200 and Enfortumab Vedotin Plus Pembrolizumab.

Factors Predicting Subsequent Changes in Hearing Thresholds in Neonates with Initial Hearing Impairment.

Cortical processing of speech sounds in individuals with cochlear hearing loss and auditory neuropathy.

Impacts of heminode disruption on auditory processing of noisy sound stimuli.

A rare constellation of bilateral progressive visual and auditory loss in neurofibromatosis type 2: a multimodal diagnostic approach.

Cumulative diabetes-related metabolic burden and the risk of hearing loss: A population-based study.

Auditory dysfunction and perinatal risk factors in high-risk infants: Insights from brainstem evoked response audiometry.

The Silent Complication: Auditory Dysfunction in Pediatric Patients with Type 1 Diabetes.

[Clinical diagnosis and condition monitoring of auditory neuropathy based on vestibular evoked myogenic potentials].

Retrocochlear Auditory Dysfunctions (RADs) and Their Treatment: A Narrative Review.

Bortezomib-Induced Sensorineural Hearing Loss May Be Reversible with Intratympanic Dexamethasone.

A novel minimally invasive stereotaxic technique to target inner ear neurons in the mouse.

Evaluating Cochlear Implantation Outcomes in Charcot-Marie-Tooth Disease: A Case Series Analysis of Genetic Profiles and Intervention Timing.

Hearing Loss as a Neurological Manifestation of Diabetes.

Identification of a Novel Likely Pathogenic Variant of DIAPH3 Associated With New Phenotype of Sensorineural Hearing Loss.

Hearing loss and cochlear implantation in Chudley McCullough syndrome: A case series.

A Novel Exon Duplication in the SACS Gene in Charlevoix-Saguenay Ataxia and a Summary of Polish Cases.

Mitochondrial tRNA-Derived Diseases.

Auditory Function and Natural History of 645 Auditory Neuropathy Patients Over a 27-Year Span in China.

MSC-sEV Promote Regeneration of Cochlear Spiral Ganglion Neurons and Myelin Sheaths in 3D Culture System.

Neural stem cell-loaded biohybrid hydrogel improves cochlear implants by electrode-neural coupling and neural regeneration.

Riboflavin Transporter Deficiency as a Cause of Progressive Encephalopathy.

Identification of novel pathogenic variants in the PHYH gene and extending the phenotypic range in Refsum disease.

A review of occupational noise-induced hearing loss: focus on mechanisms and preventive measures.

Language and Memory Skills in Pediatric Cochlear Implant Users With Auditory Neuropathy Spectrum Disorder.

Characteristic cortical alterations in auditory neuropathy: An EEG study.

Efficacy and Safety of Disitamab Vedotin Combined with Gemcitabine as Neoadjuvant Therapy in Muscle-invasive Bladder Cancer: An Open-label, Multicenter, Single-arm, Phase 2 Trial.

Mitochondrial DNA Depletion Syndrome 1 (MTDPS1)-A Novel Cause of Premature Ovarian Insufficiency.

DRP2 Mutation in Familial Autism Spectrum Disorder: A Case Report of 2 Consanguineous Patients.

ASSR and ABR tests in early diagnosis of hearing loss: A STROBE observational study.

Distinct genetic patterns and natural history of OPA1-related auditory neuropathy in Chinese population.

[Application of cochlear microphonics combined with otoacoustic emission in early differential and localization diagnosis of auditory neuropathy].

A multicenter study reveals a novel pathogenic splice-site founder variant in OTOF.

Functional, sustained recovery of hearing in Otoferlin-deficient mice using DB-OTO, a hair-cell-specific AAV-based gene therapy.

Management of isolated neurosarcoidosis mimicking cerebral meningiomatosis with multiple cranial neuropathies: An uncommon clinical scenario.

Extreme lateral infrajugular transtubercular exposure for resection of a glomus jugulare: Microsurgical operative video.

Development and validation of nomogram prediction model for diabetic hearing impairment based on the levels of lncRNA MALAT1, miR-199b and AGEs in peripheral blood.

Reversible Profound Sensorineural Hearing Loss Complicating Cryptococcal Meningitis in an Immunocompetent Adult: Diagnostic and Therapeutic Insights.

Treating Hearing Loss: From Cochlear Implantation to Gene Therapy.

Real-world economic burden of disease recurrence in patients with muscle-invasive bladder cancer: A population-level claims-based analysis.

Nasopharyngeal Giant Carcinoma Mimicking Intracranial Lesions: A Case Report and Literature Review.

Mitochondrial Neurogastrointestinal Encephalomyopathy Presenting with Peripheral Neuropathy and Hearing Loss.

Hummingbird sign in a patient with DNMT1-related disorder.

Recessive variants in TWNK cause syndromic and non-syndromic post-synaptic auditory neuropathy through MtDNA replication defects.

Impact of Population Pharmacogenomics on Cisplatin-Induced Neurotoxicities in Testicular Cancer Survivors.

[Gene therapy for inner ear disorders: a step into the future].

[Analysis of clinical manifestations and genetic characteristics of a late-onset auditory neuropathy pedigree caused by a mitochondrial MT-TS1 gene mutation m.7471dup].

Mitophagy Activation by N-Acetylcysteine Protects against Mic60 Deficiency-Induced Auditory Neuropathy.

Bibliometrics in thyroid eye disease.

Further expanding the clinical spectrum of giant cell arteritis: Non-classical organ involvement and uncommon complications.

Retinal Degeneration Diagnosed at 12 and 13 Months and Sensorineural Hearing Loss in Two Unrelated Female Infants With PRS Deficiency.

Extraocular features of Leber hereditary optic neuropathy: A scoping review.

Natural history of SPTBN4-related neurodevelopmental disorder with hypotonia, neuropathy, and deafness.

Utility of auditory steady-state response in differentiating pediatric auditory neuropathy spectrum disorders: with normal auditory nerve diameter versus with auditory nerve hypoplasia.

Bilateral retinal dystrophy and unilateral hearing loss caused by mosaic phosphoribosyl pyrophosphate synthetase 1 deficiency: expanding the spectrum of an ultrarare neurometabolic disorder.

Temporal and Spectral Cues for Phoneme Perception in School-Age Children and Adults.

Diagnostic Quandary of Granulomatosis With Polyangiitis Presenting First in the Petrous Apex.

Late Toxicity and Long-Term Quality of Life in Survivors of Cancer of the Major Salivary Glands More Than 5 Years After Diagnosis: A Multi-National Study.

Nationwide Phenotypic and Genotypic Characterisation of 103 Patients With SH3TC2 Gene-Related Demyelinating Peripheral Neuropathy.

Transmembrane Protein 43: Molecular and Pathogenetic Implications in Arrhythmogenic Cardiomyopathy and Various Other Diseases.

Riboflavin Transporter Deficiency Type 2: Expanding the Phenotype of the Lebanese Founder Mutation p.Gly306Arg in the SLC52A2 Gene.

Teprotumumab for Thyroid Eye Disease: Mechanism, Clinical Efficacy, and Current Challenges.

Pseudo Foster-Kennedy Syndrome in an amblyopic patient: a case report.

Is Contralateral Suppression of Otoacoustic Emission Observable in Unilateral Cochlear Implant Users With Auditory Neuropathy Spectrum Disorder?

Evaluating the N1-P2 interpeak latency of the eCAP and its intertrial variability as potential indicators of neural synchrony in the cochlear nerve of cochlear implant users.

The impact of type 1 diabetes mellitus on hearing function in children: A systematic review.

Comparative Outcomes of Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder and Sensorineural Hearing Loss: A Systematic Review and Meta-Analysis.

Long-Term Survival in Brown-Vialetto-Van Laere Syndrome: A Case Report Highlighting Respiratory Care.

Early-Onset Hearing Loss in Leber's Hereditary Optic Neuropathy: A Case Report.

Bilateral Sensorineural Hearing Loss in a Patient with Primary Ciliary Dyskinesia and Concomitant SH3TC2 Gene Mutation.

Charcot-Marie-Tooth disease type 1E: clinical natural history and molecular impact of PMP22 variants.

Neurological involvement in 51 cystinosis patients: A single-center experience.

Cochlear Nerve Atrophy in Postlingual ANSD: Diagnostic Clue and Implications for Cochlear Implantation.

Cisplatin-Induced APE2 Overexpression Disrupts MYH9 Function and Causes Hearing Loss.

A novel BCAP31 variant associated with nonsyndromic auditory neuropathy spectrum disorder: mitochondrial dysfunction, cisplatin sensitivity, and amenability to mitochondrial transplantation.

Cobalt toxicity from third-body wear following revision of fractured ceramic-on-ceramic hip bearings with a metal articulation : the fractured ceramic syndrome.

MPO-ANCA-Associated Hypertrophic Pachymeningitis Mimicking IgG4-Related Disease: A Case Report and Literature Review.

Next-Generation Sequencing to Detect Mutations for the Molecular Diagnosis of Auditory Neuropathy Spectrum Disorder in a Chinese Series.

Auditory neuropathy spectrum disorder and related auditory features in patients with hearing loss associated with the MT-TS1 m.7471dup variant.

Outcomes of Patients With International Germ Cell Cancer Collaborative Group Good Prognosis Metastatic Seminoma Undergoing De-Escalation Chemotherapy: A Single-Center Retrospective Study.

Unraveling the complex genetic landscape of OTOF-related hearing loss: a deep dive into cryptic variants and haplotype phasing.

Incidence of Cochlear Nerve Deficiency in Unilateral Pediatric Auditory Neuropathy Spectrum Disorder.

Phenotypic Heterogeneity in Genetic and Acquired Pediatric Cerebellar Disorders.

Exploring the cognitive impacts of diabetic neuropathy: a comprehensive review.

Thyroid eye disease (Graves' orbitopathy): clinical presentation, epidemiology, pathogenesis, and management.

Cochlear Amyloid-β42 Accumulation Drives Progressive Auditory Neuropathy in 5XFAD Mice: A Potential Biomarker for Early Alzheimer's Disease.

Cerebellar Ataxia-deafness-narcolepsy (ADCA) syndrome. Description of a variable family phenotype.

Unmasking vestibular schwannoma: A series of unusual cases.

[Ototoxicity of systemic treatments for malignant tumours, principles of diagnostic and management].

Pathogenesis and research progress of OTOF gene related auditory neuropathy: a retrospective review.

Targeted spiral ganglion neuron degeneration in parvalbumin-Cre neonatal mice.

[Distribution of G6PD mutations and phenotypic characteristics in individuals with auditory neuropathy].

Brown-Vialetto-Van Laere syndrome patients with unusual phenotypes from Indian ethnicity: Functional analysis of clinical variants in SLC52A2 and SLC52A3 genes.

[The natural history of the relationship between OTOF mutation-related genotypes and audiological phenotypes].

Auditory Characteristics in Children With Enlarged Vestibular Aqueduct.

Rare Case with Pathogenic Variant in DHX16 Gene Causing Neuromuscular Disease and Oculomotor Anomalies.

[The algorithm for clinical and audiological observation of children born preterm].

Rapid detection of the GJB2 c.235delC mutation based on CRISPR-Cas13a combined with lateral flow dipstick.

Hypocalcemia Induced Optic Neuropathy in a Patient with Undiagnosed Hypocalcemia, Deafness, and Renal Syndrome.

From Profiles to Structure in Cochlear Nerve: A Clinical Study of Auditory Neuropathy.

Advances in Understanding the Molecular Dynamics of Autosomal Dominant Auditory Neuropathy: Unveiling a Novel DIAPH3 Gene Variant Associated With Sensorineural Hearing Loss and Bilateral Vestibular Aqueduct Enlargement.

Hearing loss and small and large fibre neuropathy associated with the heterozygous variants c.20A>T in HBB and del-3.7 in HBA.

[Perioperative management of cochlear implantation and analysis on the influencing factors of efficacy in patients diagnosed as hereditary syndromic hearing loss].

Auditory Neuropathy Caused by a Structural Variation in the OTOF Gene, Identified Using Oxford Nanopore Adaptive Sampling.

Deciphering Auditory Hyperexcitability in Otogl Mutant Mice Unravels an Auditory Neuropathy Mechanism.

The Role of Visual Electrophysiology in Systemic Hereditary Syndromes.

A Case Report of Auditory Neuropathy Due to TWNK Gene Mutations.

Diffusion-Weighted Magnetic Resonance Imaging: A Diagnostic Tool for Auditory (Axonal) Neuropathy.

Heterozygous PNPT1 Variants Cause a Sensory Ataxic Neuropathy.

Audiological findings in Brown Vialetto-Van-Laere Syndrome: A scoping review.

Osteosarcoma: A comprehensive review of model systems and experimental therapies.

A Case of Bilateral Sudden Deafness Caused by Wernicke Encephalopathy.

Factors associated with longitudinal progression of the cumulative burden of morbidity and overall mortality after cisplatin-based chemotherapy for testicular cancer.

Expanded Clinical Phenotype and the Role of Untargeted Metabolomics Analysis in Confirming the Diagnosis of Sodium-Dependent Multivitamin Transporter Deficiency.

Canadian Association of Radiologists Central Nervous System Diagnostic Imaging Referral Guideline.

Genetic etiology of Perrault syndrome in Iranian families: first report from Iran and literature review.

Outcomes of Cochlear Implantation in Adolescents With Auditory Neuropathy Spectrum Disorder: Scoping Review and Case Report.

Modeling of auditory neuropathy spectrum disorders associated with the TEME43 variant reveals impaired gap junction function of iPSC-derived glia-like support cells.

Chronic Myeloid Leukemia Presenting With Bilateral Optic Neuropathy and Sensorineural Hearing Loss as the First Clinical Presentation: A Case Report.

Relationships between hearing, auditory processing, and communication in children diagnosed with auditory neuropathy spectrum disorder.

Identification of genetic mechanisms of non-isolated auditory neuropathy with various phenotypes in Chinese families.

Audiological Features in Patients with Rheumatoid Arthritis: A Systematic Review.

An Update of Phenotypic-Genotypic IMNEPD Cases and a Bioinformatics Analysis of the New PTRH2 Gene Variants.

Phosphoribosyl pyrophosphate synthetase 1 (PRPS1) associated retinal degeneration: an international study.

[Clinical characteristics and pathogenic variant analysis in a pedigree with syndromic hearing loss caused by likely pathogenic variants in the NARS2 gene].

Sensory neuropathy in patients with Pompe disease: a case series in Iran.

Clinical Reasoning: Clinical Manifestations and Diagnostic Challenges in a 16-Year-Old With Early-Onset Ataxia.

Current trends and hotspots of etiology of auditory neuropathy in the past 30 years: A bibliometric analysis.

Heterogeneous Group of Genetically Determined Auditory Neuropathy Spectrum Disorders.

Systemic Therapy for Stage I-III Anal Squamous Cell Carcinoma: ASCO Guideline.

Canonical MAPK signaling in auditory neuropathy.

Neural tracking of the speech envelope predicts binaural unmasking.

Sphenoidal pneumosinus dilatans associated compressive optic neuropathy: A case series of four adolescent patients.

How hidden is hidden hearing loss? Self-reported listening problems in charcot Marie tooth disease.

Conductive Hearing Loss Due to Hypertrophic Neuropathy.

Novel AIFM1 Variant in 2 Siblings With Sensorineural Hearing Loss and Cerebellar Ataxia.

A Novel MYH14 Variant Presenting as a New Phenotype of MYH14-Associated Neuromuscular Disorders-Clinicohistologic Findings and Review of the Literature.

Disordered Electron Transfer: New Forms of Defective Steroidogenesis and Mitochondriopathy.

Radiotherapy with cetuximab or durvalumab for locoregionally advanced head and neck cancer in patients with a contraindication to cisplatin (NRG-HN004): an open-label, multicentre, parallel-group, randomised, phase 2/3 trial.

Middle ear cholesteatoma and facial nerve hypertrophy mimicking schwannoma in Charcot-Marie-Tooth disease: A case report.

Genetic landscape of primary mitochondrial diseases in children and adults using molecular genetics and genomic investigations of mitochondrial and nuclear genome.

Late side effects of testicular cancer and treatment: a comprehensive review.

Garcin Syndrome in a Patient with Hypertrophic Pachymeningitis Following Otitis Media with Antineutrophil Cytoplasmic Antibody (ANCA)-associated Vasculitis (OMAAV).

Postoperative Auditory Progress in Cochlear-Implanted Children With Auditory Neuropathy.

Polygenic Risk Scores and Hearing Loss Phenotypes in Children.

CGG Repeat Expansion in NOTCH2NLC Causing Overlapping Oculopharyngodistal Myopathy and Neuronal Intranuclear Inclusion Disease With Diffusion Weighted Imaging Abnormality in the Cerebellum.

Auditory and vestibular function in mitochondrial patients harbouring the m.3243A>G variant.

Riboflavin transporter deficiency, the search for the undiagnosed: a retrospective data mining study.

Thiamine-Responsive Megaloblastic Anemia Syndrome Mimicking Myelodysplastic Neoplasm.

Early cochlear implantation: exploring electrophysiological thresholds and their role in pre-behavioural recommendations.

Dissociation between caloric test and the video head impulse test in individuals with auditory neuropathy spectrum disorders.

Advances in understanding cisplatin-induced toxicity: Molecular mechanisms and protective strategies.

Good cochlear implantation outcomes in subjects with mono-allelic WFS1-associated sensorineural hearing loss - a case series.

Characterization of Two Novel PNKP Splice-Site Variants in a Proband With Microcephaly, Intellectual Disability, and Multiple Malformations.

Whole blood viscosity and its association with the presence and severity of hearing loss and other microangiopathies in Indian patients with type 2 diabetes mellitus.

Characteristics of autosomal dominant WFS1-associated optic neuropathy and its comparability to OPA1-associated autosomal dominant optic atrophy.

Carbon Monoxide Poisoning (Reprinted from the 2023 Hyperbaric Indications Manual 15th edition).

Dilated cardiomyopathy revealing Refsum disease: a case report.

Detailed characterization of auditory neuropathy in perrault syndrome with TWNK variants.

Stub1 promotes degradation of the activated Diaph3: A negative feedback regulatory mechanism of the actin nucleator.

Objective Determination of Site-of-Lesion in Auditory Neuropathy.

Middle-ear osteolytic transthyretin amyloidosis: A CARE case report.

Hearing loss secondary to variants in the OTOF gene.

Masseter and cervical vestibular evoked myogenic potentials in individuals with auditory neuropathy.

Eligibility Criteria for Different Platinum-Based Chemotherapy Regimens in Metastatic Urothelial Carcinoma.

Two novel compound heterozygous HOXB1 variants in congenital facial palsy: A case report and a brief review of the literature.

Electrocochleography-Based Tonotopic Map: I. Place Coding of the Human Cochlea With Hearing Loss.

Cochlear implants for hereditary ATTR amyloidosis: a case report of two cases.

Identification and Characterization of Novel Founder Mutations in NDRG1: Refining the Genetic Landscape of Charcot-Marie-Tooth Disease Type 4D in Bulgaria.

A deep phenotyping study in mouse and iPSC models to understand the role of oligodendroglia in optic neuropathy in Wolfram syndrome.

Etiologic Diagnosis of Genetic Hearing Loss in an Ethnically Diverse Deafness Cohort.

Measurement of thresholds using Chirp-ABR in children with auditory neuropathy spectrum disorder and sensorineural hearing loss.

Hidden hearing loss in a Charcot-Marie-Tooth type 1A mouse model.

Severe Respiratory and Swallowing Disorders in Infantile-Onset Multisystem Neurologic, Endocrine, and Pancreatic Disease Type 1: Two Cases.

Novel heterozygous OPA3 variant in a family with congenital cataracts, sensorineural hearing loss and neuropathy, without optic atrophy and comparison of pathogenic and population variants.

Case report on a de novo variant in the X-linked PRPS1 gene presenting with retinal dystrophy, severe tremors, and ataxia in a female patient.

Associations between hearing loss, peripheral neuropathy, balance, and survival in older primary care patients.

Case Report: Brucellosis Mimicking Tuberculous Meningitis in a Child.

Expanding the genetic and phenotypic landscape of replication factor C complex-related disorders: RFC4 deficiency is linked to a multisystemic disorder.

Novel Variant of FDXR as a Molecular Etiology of Postlingual Post-synaptic Auditory Neuropathy Spectrum Disorder via Mitochondrial Dysfunction: Reiteration of the Correlation between Genotype and Cochlear Implantation Outcomes.

Frequency and severity of autonomic dysfunction assessed by objective hemodynamic responses and patient-reported symptoms in individuals with myasthenia gravis.

Cochlear reimplantation outcomes over 20 years: Expertise in reimplantation surgery and auditory-speech rehabilitation.

A systematic review of acoustic change complex (ACC) measurements and applicability in children for the assessment of the neural capacity for sound and speech discrimination.

Revisiting the transient-evoked otoacoustic emissions passing criteria used for newborn hearing screening.

Phenotypic variability related to dominant UCHL1 mutations: about three families with optic atrophy and ataxia.

Phenotype and prognostic factors in geriatric and non-geriatric patients with transthyretin cardiomyopathy.

Prognostic Value of Electrophysiological and MRI Findings for Pediatric Cochlear Implant Outcomes: A Systematic Review.

Diabetes mellitus, hearing loss, and therapeutic interventions: A systematic review of insights from preclinical animal models.

Exploring the evidence for mitochondrial dysfunction and genetic abnormalities in the etiopathogenesis of tropical ataxic neuropathy.