Background Age-related hearing loss (ARHL) is the most prevalent sensory impairment worldwide, affecting more than one-third of adults aged 65 years or older. Early detection and intervention are essential for improving quality of life and reducing dementia-related risks. However, conventional one-size-fits-all screening strategies may fail to detect early frequency-specific declines. Aims The proposed cross-sectional study aimed to identify the critical age ranges during which hearing thresholds increased most markedly at different frequencies. Hearing thresholds at seven standard frequencies (0.125u20138 kHz) were analyzed among outpatients with symmetric bilateral hearing to inform more targeted clinical screening. Materials and Methods We retrospectively analyzed pure-tone audiometry data from both ears of 11,507 patients who visited our otolaryngology clinic between 2021 and 2025. Pearson correlation and LOESS regression were used for statistical analysis. Results Significant positive correlations between age and hearing thresholds were observed across all frequencies (p u0026lt; 0.001). The critical age ranges associated with the greatest threshold differences were 43u201352 years for high frequencies, 48u201357 years for mid frequencies, and 63u201372 years for low frequencies. Conclusions This large-scale study identified distinct frequency-specific critical age ranges for ARHL among Chinese outpatients. The findings identify ages 43u201352 as a critical period for high-frequency hearing decline, providing a clear basis for targeted screening and timely clinical management.
Purpose The purpose of this study was to perform a meta-analysis comparing the performance of 10-minute and 4-hour delayed-enhanced Three-Dimensional Fluid-Attenuated Inversion Recovery Magnetic Resonance Imaging (3D-FLAIR MRI) for the detection of inner ear abnormalities in sudden sensorineural hearing loss (SSNHL). Methods A comprehensive literature search was performed independently by two researchers, covering databases including PubMed, Web of Science, Science Direct and CNKI up to June 4, 2025. Original research studies comparing 10-minute and 4-hour delayed-enhanced 3D-FLAIR MRI in evaluating SSNHL were included. The Joanna Briggs Institute Critical Appraisal Checklist for Studies Reporting Prevalence Data (JBI) was used for quality assessment. All literature was independently screened and data were extracted, followed by meta-analysis and subgroup analysis using RevMan 5.4 to compare the performance of 10-minute and 4-hour delayed-enhanced 3D-FLAIR MRI. Results Four studies including 211 affected ears in 210 SSNHL patients met inclusion criteria for the systematic review. All patients underwent 10-minute and 4-hour delayed-enhanced 3D-FLAIR MRI scanning. The meta-analysis showed a statistically significant difference in detection rates between the 10-minute and 4-hour delays (risk difference [RD], -0.24; 95% CI, -0.38 to -0.10; I2=63%; p=0.0006). Subgroup analyses indicated that study type, region and Gd dose may be the sources of heterogeneity. Conclusion A 4-hour delay reveals 24% more abnormal findings than a 10-minute delay in Gd-enhanced 3D-FLAIR MRI. Therefore, we recommend a 4-hour delay for the clinical evaluation of SSNHL patients.
Mitochondrial translation depends on coordinated contributions from the mitochondrial and nuclear genomes and is essential for oxidative phosphorylation. Disruption of this system can cause isolated sensorineural hearing loss or auditory impairment within multisystem disease. This review examines how mitochondrial rRNA variants, nuclear-encoded mitoribosomal protein defects, and environmental exposures converge on mitochondrial translation to impair hearing. The mitochondrial 12S rRNA 1555Au0026gt;G and 1494Cu0026gt;T mutations alter decoding-site architecture and increase susceptibility to aminoglycoside ototoxicity, whereas pathogenic variants in nuclear mitoribosomal protein genes can compromise subunit assembly, stability, or abundance. Nuclear modifiers, particularly TRMU, and secondary mitochondrial tRNA variants illustrate how RNA modification and tRNA function influence the consequences of a primary rRNA defect. We propose mitochondrial translational reserve as a testable framework for incomplete penetrance and cochlear vulnerability, while recognizing that its quantitative relationship to hearing outcomes remains unresolved. Current clinical applications include molecular diagnosis, genotype-informed aminoglycoside prescribing, cascade testing of maternal relatives, and individualized hearing rehabilitation. Patient-derived auditory models and genetic rescue studies provide opportunities to clarify disease mechanisms and evaluate interventions, although modifier correction and pharmacological otoprotection remain experimental. Integrating molecular mechanisms with clinical assessment can strengthen prevention of avoidable ototoxicity and guide care for mitochondrial hearing loss.
Objectives: In sensorineural hearing loss treatment, transtympanic membrane (TTM) steroid injections help in recovery. However, steroid dosage distribution and patient recovery rates remains underexplored. Our systematic review compiles steroid regimens and their hearing recovery rates. Data Sources: PubMed, OVID, and Google Scholar. Review Methods: Search terms included TTM steroid and hearing loss. Inclusion criteria were measurements for human hearing recovery, full-text articles, and studies published on or before 1/20/2025. Exclusion criteria included reviews. Primary outcomes were hearing and patient recovery rates. For analysis, we focused on dose levels and simultaneous systemic steroids, and employed descriptive and exploratory study-level analyses Results: 29 articles were included. Of studies reporting gender, the distribution was 54.6% (635) male and 45.4% (528) female. 1,294 total patients received TTM steroids with distributions being: 26.0% (n=336) received 4 mg/mL dexamethasone, 25.2% (n=326) received 5 mg/mL dexamethasone, 2.7% (n=35) received 8 mg/mL dexamethasone, 3.5% (n=45) used 10 mg/mL dexamethasone, 3.1% (n=40) received 24 mg/mL dexamethasone, 31.8% (n=411) received 40 mg/mL methylprednisolone, 2.6% (n=34) received 62.5 mg/mL methylprednisolone, and 5.2% (n=67) received 125 mg/mL methylprednisolone. Hearing recovery ranged from 6.4-41 decibels (dB). The exploratory analysis did not identify significant differences in hearing recovery; however, methodological variance limits direct cross-study comparison and limits dose equivalence. Conclusion: Variability exists in regimen and outcomes reported. The findings of this study-level exploratory analysis should not be interpreted as evidence that a dose-response relationship does not exist. Therefore, this study provides a framework for researchers and clinicians to understand steroid regimens used to date.
Objective To report the first Chinese case of complex mosaic neurofibromatosis type 2 (NF2)u2014recently reclassified as NF2-related schwannomatosisu2014in a patient presenting with concurrent severe visual impairment and an intracochlear schwannoma, and to evaluate the clinical efficacy and strategic priority of tumor-preserving in situ cochlear implantation (CI). Methods The patientu2019s clinical presentation, extensive neurosurgical history, genetic profile, and perioperative audiological data were retrospectively reviewed. Additionally, we analyzed the surgical approachu2014which involved localized microdrilling of the obstructed cochlear turnu2014and the subsequent one-year follow-up outcomes of auditory-verbal rehabilitation. Results A 52-year-old female presented with bilateral vestibular schwannomas and secondary profound sensorineural hearing loss. Peripheral blood genetic testing revealed no pathogenic NF2 mutations, strongly indicating somatic mosaicism. The patient also suffered from a concurrent profound visual deficit due to bilateral optic atrophy and exhibited a right-sided intracochlear schwannoma. Successful tumor-preserving in situ cochlear implantation (CI) was performed on her right ear by bypassing the tumor obstruction along the cochlear trajectory. At the one-year postoperative follow-up, the patient achieved significant gains in aided hearing thresholds, along with a disyllabic word recognition score of 22%. Furthermore, her Category of Auditory Performance (CAP) score markedly improved from 0 to 6, and her Speech Intelligibility Rating (SIR) score reached level 5. Conclusion Cochlear implantation (CI) with tumor preservation in situ represents a safe, viable, and highly effective hearing reconstruction strategy for complex NF2 patients with intracochlear schwannomas. For patients afflicted with dual sensory impairment (dual sensory loss), a successful cochlear implant serves as a critical standalone interface that significantly facilitates auditory-verbal rehabilitation and restores social autonomy, even within an intricate oncological landscape.
