Search Results (105)

Background: Bortezomib, a proteasome inhibitor used in multiple myeloma (MM), is associated with several adverse effects, most notably peripheral neuropathy. Ototoxicity, however, remains a rare and underrecognized complication. Case presentation: We report the case of a 74-year-old man with MM who developed sudden unilateral sensorineural hearing loss following subcutaneous bortezomib administration. Audiometry confirmed severe right-sided hearing loss. MRI of the internal auditory canal was normal. Given the absence of other ototoxic agents, bortezomib was identified as the likely causative drug. The patient was treated with intratympanic dexamethasone injections, achieving partial hearing recovery. Subsequent chemotherapy re-exposure triggered another hearing decline, which again improved after repeated intratympanic treatment. Conclusions: Bortezomib-related ototoxicity is a rare but potentially reversible adverse event. This case suggests that early intratympanic corticosteroid therapy may mitigate cochlear injury, allowing continuation of chemotherapy for patients responding well to bortezomib. Full article

Background/Objectives: Patients with Idiopathic sudden sensorineural hearing loss (ISSNHL) are often devastated by the unknown etiology coupled with the unknown pathway to recovery. The aim of this study was to evaluate whether abnormalities on the video Head Impulse Test (vHIT) performed early in the course of ISSNHL are associated with poorer hearing recovery. Methods: Forty-four patients with ISSNHL were prospectively enrolled between 2019 and 2022 following exclusion of differential diagnoses on clinical and MRI evaluation. vHIT was performed within 1–14 days of symptom onset and within 48 h of hospitalization. Recovery at six months was assessed both as a dichotomous outcome and by change in pure tone average (PTA). Group differences were analyzed using Fisher’s exact and Mann–Whitney U tests. A two-predictor logistic regression model examined the association between vHIT results, dizziness, and recovery. Results: Twelve patients exhibited abnormal vHIT findings. Abnormal vHIT was strongly associated with the presence of dizziness and with markedly poorer hearing recovery at six months. Patients with normal vHIT demonstrated substantially greater improvement in PTA thresholds compared with those showing abnormal results. Logistic regression further confirmed that abnormal vHIT was an independent predictor of reduced likelihood of hearing recovery, whereas dizziness alone did not independently influence outcomes. Conclusions: Our findings suggest that abnormal vHIT results in ISSNHL patients are linked to poor hearing recovery, which can enhance patient counseling regarding expectations. Although promising as a prognostic tool, we acknowledge our limited sample size and recommend validation in larger prospective cohorts. Full article

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

Background: Idiopathic sudden sensorineural hearing loss (ISSNHL) is an abrupt unilateral hearing loss of unknown origin. Combination therapy with hyperbaric oxygen (HBO), systemic steroids (SS), and prostaglandin E1 (PGE1) has been used in Japan; however, its prognostic factors remain unclear. Objective: To evaluate the efficacy of HBO combined with SS and PGE1 and to identify prognostic factors for hearing recovery in patients with ISSNHL. Methods: This retrospective study included 116 patients treated within 14 days of ISSNHL onset. Sixty patients received HBO, SS, and PGE1 (HBO group), and 56 received SS and PGE1 alone (No-HBO group). Hearing outcomes were assessed using PTA (arithmetic mean hearing at 250–4000 Hz) and graded by Siegel’s criteria. Prognostic factors were analyzed by multivariate logistic regression. Results: The HBO group showed significantly better hearing grade outcomes (p = 0.007) and greater PTA improvement (p = 0.003) than the No-HBO group. Vertigo and higher initial PTA were identified as independent predictors of poor hearing outcomes. Patients without vertigo showed significantly greater improvement at 2000 Hz (p = 0.009). Receiver operating characteristic analysis revealed an optimal initial PTA cutoff of ≥90.5 dB for predicting poor hearing outcome. Conclusions: HBO combined with SS and PGE1 significantly improves hearing outcomes in ISSNHL. However, the presence of vertigo and severe initial hearing loss remain poor prognostic indicators. These findings suggest that while the addition of HBO may enhance hearing outcomes, prognosis remains limited in severe cases. Further prospective studies are needed to confirm these results. Full article

Background and Objectives: Labyrinthitis is an inflammatory inner ear disorder often resulting in acute vertigo and hearing loss. While typically self-limiting, some cases lead to persistent deficits. This study examined incidence trends of acute labyrinthitis before and after 2020 and compared hearing outcomes between these periods. Materials and Methods: This retrospective cohort study included 126 patients diagnosed with acute labyrinthitis at a tertiary medical center between January 2014 and May 2024. Patients were divided into pre-2020 (2014–2019) and post-2020 (2020–2024) cohorts. Poisson regression analyzed incidence trends, while audiometric outcomes were compared in 79 patients with complete 3-month follow-up data. Hearing recovery was assessed using pure-tone averages (PTA) across 500–4000 Hz, and predictors of persistent impairment were identified through multivariable logistic regression. Results: The cohort had a mean age of 47.8 years with female predominance (63.5%). Annual case counts increased significantly post-2020 (19.8 vs. 6.5 cases/year; CRR 3.05, 95% CI 2.07–4.57, p < 0.001). Substantial hearing improvement occurred across all frequencies (median AC improvement 13.8 dB). Hearing recovery was comparable between periods, with similar PTA improvements (−16.7 vs. −15.3 dB, p = 0.73) and equivalent distributions across World Health Organization (WHO) hearing categories (p = 0.64). Baseline hearing level was the strongest predictor of persistent impairment (OR 1.56 per 5 dB increase, 95% CI 1.25–1.95, p < 0.001), while age, sex, and diagnostic period showed no significant association. Among post-2020 patients, only 12.6% had confirmed coronavirus disease 19 (COVID-19), and no reliable association with hearing outcomes could be established due to substantial missing data. Conclusions: A significant increase in hospital-diagnosed labyrinthitis cases occurred following 2020, yet hearing outcomes at 3-month follow-up remained consistent with the pre-pandemic period. Baseline hearing level was the primary determinant of recovery, unaffected by demographic factors or diagnostic period. These findings suggest that while pandemic-related factors may have influenced case frequency, they did not alter auditory prognosis or recovery patterns. Full article

Objectives: Patients with severe to profound sudden sensorineural hearing loss (SSNHL) generally experience poorer hearing recovery; however, the associated risk factors have not been identified. This study synthesizes current evidence to explore prognostic risk factors in this patient group. Methods: Databases were systematically searched through PubMed, Embase, Web of Science, and the Cochrane Library, from their inception to 18 October 2025. Three researchers independently extracted and recorded patient information and relevant data from all selected studies. Any inconsistencies were clarified through discussion or by consulting a fourth researcher. Results: The study included 2632 patients from 15 articles published between 2002 and 2025 and evaluated 8 prognostic risk factors. The results showed that profound hearing loss (OR = 4.68; 95% CI: 3.57–6.13; p < 0.001) and vertigo (OR = 1.95; 95% CI: 1.28–2.98; p = 0.002) were correlated with poorer hearing recovery. Subgroup analyses based on different prognostic criteria confirmed the consistent impact of hearing loss severity on poor outcomes. The remaining 6 risk factors did not show statistically meaningful associations. Conclusions: Profound hearing loss and vertigo are significantly associated with poorer prognosis in patients with severe to profound SSNHL. These findings may help identify high-risk patients early and inform the design of personalized therapeutic approaches in clinical settings. Full article

A 70-year-old Japanese woman with longstanding hearing loss and asthma developed floating sensations, left finger numbness, and postural instability one day after influenza vaccination, leading to hospital admission. Neurological examinations showed hearing loss, hyperreflexia, left-predominant ataxia, bilateral mild bathyanesthesia, and inability to tandem gait. Cerebrospinal fluid (CSF) analysis showed no pleocytosis or malignant cells, but revealed positive oligoclonal bands and elevated myelin basic protein. Despite no contrast agent use due to asthma, brain magnetic resonance imaging (MRI) revealed pontine hyperintensities on diffusion-weighted imaging (DWI) and T2-fluid attenuated inversion recovery (T2-FLAIR) sequences, along with hyperperfusion on arterial spin labeling (ASL) imaging. Serum anti-aquaporin-4 antibodies (AQP4-Ab) were negative by ELISA. Given the temporal proximity to vaccination and elevated demyelination markers, brainstem-type acute disseminated encephalomyelitis (ADEM) was initially suspected. Symptoms nearly resolved after two cycles of methylprednisolone pulse therapy. Notably, hyperperfusion gradually improved on ASL imaging. Post-discharge, a cell-based assay confirmed the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) by detecting positive anti-AQP4-Ab. She has been relapse-free for about a year without any immunosuppressants or biologics. Although contrast-enhanced MRI remains the gold standard modality for lesion evaluation due to its high sensitivity, hyperperfusion on ASL may provide a useful alternative in patients for whom contrast agents are contraindicated, such as those with asthma or impaired renal function. Full article

Objective: We aimed to explore the efficacy of repetitive transcranial magnetic stimulation (rTMS) for idiopathic sudden sensorineural hearing loss (ISSNHL) and evaluate the correlation between treatment courses of rTMS and hearing outcomes. Methods: A prospective observational study was conducted at West China Fourth Hospital, Sichuan University, from January 2018 to January 2025. The study enrolled 339 patients (342 affected ears) diagnosed with ISSNHL. Among them, 67 patients (group A) received standard therapy combined with rTMS, while the control group (group B) received conventional therapy only. To verify the correlation between different treatment courses of rTMS and hearing outcomes, patients in Group A were divided into Group A1 (treatment courses ≤ 10) and Group A2 (treatment courses > 10). Hearing thresholds and clinical characteristics were evaluated at admission, discharge day and 6 months post-treatment. The SDRG’s criteria were used for the grading of hearing recovery. Results: Tinnitus (79.89% vs. 75.32%, p = 0.361) and sleep disorders (33.70% vs. 41.14%, p = 0.178) were highly prevalent among patients in group A and group B. 1 Hz rTMS significantly improved these symptoms (PSQI: 52.32% vs. 44.44%, p = 0.032; THI: 16.67 ± 19.41 vs. 8.22 ± 12.77, p = 0.002). Compared to high-tone hearing loss patients, those with low-tone loss in groupA2 showed a more rapid improvement (250 Hz: 17.66 ± 16.59 vs. 14.09 ± 15.58, p = 0.041; 500 Hz: 21.20 ± 18.03 vs. 17.31 ± 16.24, p = 0.036) than grouA1, with benefits sustained at 6-month follow-up (250 Hz: 27.79 ± 18.74 vs. 22.71 ± 18.31, p = 0.012; 500 Hz: 31.89 ± 19.73 vs. 26.49 ± 20.08, p = 0.013). Conclusions: rTMS at 1 Hz, administered in courses > 10 sessions, demonstrated both short-term and long-term beneficial effects in the ISSNHL. Those with low-tone hearing loss exhibit better recovery but a higher chance of relapse than high-tone loss patients. As a non-invasive approach with minimal side effects, rTMS is suitable for routine ISSNHL treatment. Full article

Background and Objectives: This study aimed to investigate the influence of continuous positive airway pressure in patients with obstructive sleep apnoea on hearing and the possibility of recovering cochlear receptor cells. Materials and Methods: Forty-two patients with obstructive sleep apnoea (OSA) were assigned to the study group. Patients underwent pure-tone audiometry and transient-evoked (TEOAE) and distortion-product (DPOAE) otoacoustic emissions before starting continuous positive airway pressure (CPAP) therapy and six months after CPAP therapy. Subjects were further divided into the following two groups: those who adequately used the therapy and those who did not adhere to treatment recommendations. Results: There is no significant difference in hearing thresholds for specific frequencies after six months of CPAP therapy. There is no significant difference in TEOAE and DPOAE SNR values at any frequency after 6 months of CPAP therapy. There is no significant difference in hearing threshold results for specific frequencies as a function of subject co-operation with treatment. After therapy, there is a significant difference in the SNR values in TEOAEs at 2 kHz and 4 kHz in subjects of the OSA target group, depending on co-operation, being higher in co-operative subjects, while there are no significant differences at other frequencies. There is a significant difference in the SNR results in DPOAEs, where they are higher in co-operative subjects at 1000, 6000, 7000 and 8000 Hz. Conclusions: The use of continuous positive airway pressure as a therapy for OSA has no effect on hearing and cochlear receptor cell recovery. Co-operation with CPAP treatment does not affect hearing threshold, but does affect cochlear receptor cell function, which is better at mid and higher frequencies in those who co-operate. These findings underscore the clinical significance of treatment adherence. Consistent adherence is associated with measurable improvements in hearing, particularly at higher frequencies, which cannot typically be evaluated in routine clinical practice using standard pure-tone audiometry. Our results emphasise the importance of promoting compliance with CPAP therapy not only for cardiovascular and neurological protection, but also for maintaining hearing health. Full article

Introduction: Noggin encoding (NOG) gene plays a critical role in early embryogenesis and development of bones, joints, cartilage, eyes, and neural tissue. The NOG gene encodes the noggin protein. Noggin is the only secreted inhibitor of bone morphogenetic protein (BMP) that is associated with abnormal phenotypes in humans. The most commonly observed manifestations of NOG gene mutations include bilateral conductive hearing loss, proximal symphalangism, broad thumbs, hyperopia, and a distinct facial appearance. This genetic disorder was first reported in 1990 by Teunissen and Cremers. Since then, various phenotypic presentations of NOG mutation have been reported, leading to the introduction of the term NOG-related symphalangism spectrum disorder (NOG-SSD). Case report: In this report, we describe a family (mother and daughter) with bilateral mixed hearing loss. Both patients had hyperopia, distinct facial appearance with hemicylindrical nose, broad thumbs, and syndactyly of the second and third toes. Genetic testing confirmed a NOG gene mutation. Bilateral stapedotomy was successfully performed, resulting in significant hearing improvement. However, due to sensorineural component of hearing loss, complete hearing recovery was only achieved with the use of hearing aids. Discussion: The etiology of the sensorineural component of hearing loss in NOG-SSD remains unclear. In animal models, the NOG gene is essential for inner ear development, while in humans, only middle ear malformations have been reported. The phenotypic variability observed in individuals with NOG mutations is very wide, suggesting that the sensorineural component of hearing loss could represent one of the possible manifestations. Conclusions: Conductive hearing loss is the primary manifestation of the NOG-SSD, and all previously reported cases of NOG gene mutations have presented exclusively with conductive hearing loss. It is possible that additional genetic factors, not necessarily directly related to the NOG gene but present in this family, contribute to the development of the sensorineural component of hearing loss, although thorough genetic testing did not reveal any additional mutation. This is, to our knowledge, the first report of mixed hearing loss associated with a NOG mutation confirmed preoperatively. Further studies are needed to determine whether the sensorineural component represents a primary manifestation or arises from secondary mechanisms. Full article

Background: Meniere’s disease (MD) is a chronic inner ear disorder affecting approximately 0.2% of the population, with 30% of patients remaining refractory to conventional treatments. The pathophysiology involves endolymphatic hydrops, suggesting that agents affecting fluid homeostasis might provide therapeutic benefit. Sodium-glucose cotransporter 2 (SGLT-2) inhibitors, originally developed for diabetes, offer unique mechanisms including natriuresis and osmotic diuresis that may address the underlying fluid imbalance in MD. Methods: We conducted a retrospective observational study at the Korea University Anam Hospital, analyzing the medical records of patients with definite MD (Bárány Society criteria) who received off-label empagliflozin 10 mg daily between January 2023 and December 2023. Six patients (3 men, 3 women; mean age 55.8 years) with treatment-resistant MD were identified who had failed conventional therapy for at least 3 months. Primary outcomes included changes in pure tone threshold average (PTA), low-frequency threshold average (LFA), vertigo episode frequency, and vertigo severity using visual analog scale (VAS) scores, assessed at baseline and after 3 months of treatment. Results: All patients demonstrated clinically significant improvements in both auditory and vestibular symptoms. Mean PTA improved from 31.4 dB to 20.8 dB (improvement of 10.6 dB, p < 0.05). Low-frequency hearing showed more substantial recovery, with LFA improving from 37.2 dB to 15.6 dB (improvement of 21.6 dB, p < 0.01). Vertigo frequency decreased dramatically from 1.6 episodes per month to 0.1 episodes per month, with four patients experiencing a complete resolution of vertigo episodes. VAS scores for vertigo severity decreased from 5.2 to 0.5. Treatment was well-tolerated, with only minor adverse effects reported in two patients: transient polyuria in one patient and 5 kg weight loss in another, both consistent with the known pharmacological profile of SGLT-2 inhibitors. Conclusions: This preliminary study suggests a potential clinical benefit of repurposing SGLT-2 inhibitors for treatment-resistant MD. However, the retrospective design and inherent limitations prevent definitive conclusions about causality. The significant improvements observed in both hearing thresholds and vestibular symptoms warrant further investigation through randomized controlled trials with objective outcome measures to establish the true efficacy of this therapeutic approach. Full article

Background and Objectives: Otitis media with effusion (OME), frequently associated with obstructive adenoid hypertrophy (OAH), is a leading cause of paediatric hearing loss. Clinically distinguishing effusion types (serous vs. mucoid) and predicting postoperative hearing recovery are unresolved challenges. This study evaluated the utility of preoperative blood inflammatory markers in predicting effusion characteristics and short-term hearing outcomes following adenoidectomy with tympanostomy tube (TT) insertion. Materials and Methods: In this retrospective cohort study, 232 children under 12 years old in 2024 and undergoing adenoidectomy (with or without TT insertion) were categorised into serous OME (n = 42), mucoid OME (n = 78), and non-effusion (n = 112) groups. Preoperative blood sample analyses assessed neutrophil, lymphocyte, eosinophil, basophil, and platelet counts, along with derived indices, including neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), eosinophil-to-basophil ratio (EBR), mean platelet volume (MPV), and systemic immune–inflammation index (SII). Hearing was evaluated at 2 weeks and 1 month postoperatively. Statistical analyses used SPSS v.28, with significance set at p < 0.05. Result: Mucoid OME patients exhibited significantly elevated neutrophil counts, platelet counts, eosinophils, NLR, and SII compared to those in serous OME and non-effusion groups (p < 0.05). All serous OME children achieved normal hearing by the first follow-up, whereas 15.4% of mucoid OME cases had transient mild hearing loss persisting after 2 weeks (p = 0.008; OR=15.97) but resolving by 1 month. Preoperative neutrophil count independently predicted delayed hearing recovery (p = 0.021). Conclusions: Systemic inflammatory markers, particularly neutrophil count, NLR, and SII, effectively differentiate mucoid OME from other effusion types and correlate with short-term hearing recovery. Neutrophil count may serve as a prognostic tool for surgical planning and patient counselling. Prospective studies are warranted to validate these findings in broader paediatric populations. Full article

Objectives: This study aimed to evaluate the clinical and hearing outcomes of patients with cholesteatomatous chronic otitis media using the ChOLE classification system and to assess its utility in predicting recurrence, guiding surgical approach, and anticipating hearing recovery. Materials and Methods: This retrospective study included 130 patients (141 ears) who underwent surgery for cholesteatoma between 2011 and 2020. Data were collected from surgical notes, imaging studies, and audiological evaluations. Patients were classified according to the ChOLE criteria, which incorporate cholesteatoma extension (Ch), ossicular chain status (O), and life-threatening complications (L). Surgical procedures and functional outcomes were compared across different stages and classification groups. Hearing outcomes were assessed in the early (3rd month) and late (2nd year) postoperative period. Results: Among the 130 patients (141 ears) evaluated, Stage II was the most common ChOLE stage (74.4%), followed by Stage I (17.7%) and Stage III (7.8%). The most frequently observed cholesteatoma extent was Ch3. A statistically significant association was found between surgical technique and ChOLE stage (p = 0.001): canal wall-down (CWD) tympanomastoidectomy was performed in 91% of Stage III cases and 84% of Stage II cases, whereas 76% of Stage I cases underwent canal wall-up (CWU) procedures. The overall recurrence rate was 29.5% in the CWU group and 16.4% in the CWD group, although this difference did not reach statistical significance (p = 0.792). However, ossicular chain status (O) showed a strong association with both early and late hearing outcomes. At 2 years, conductive hearing success was achieved in 90.9% of O0 patients, compared to 14% of O3b and 0% of O4 patients (p = 0.001). With regard to cholesteatoma extent (Ch), a statistically significant correlation was observed with early air–bone gap (ABG) closure success (p = 0.008) and late air conduction thresholds (p = 0.015). Similarly, ChOLE stage was significantly associated with early conductive hearing success (p = 0.012) and late ABG closure (p = 0.015). Overall, long-term hearing outcomes were superior to early results. Hearing success increased from 19.1% to 24.8% for air conduction thresholds and from 23% to 31.2% for hearing gain when comparing early and late follow-up periods. Conclusions: The ChOLE classification proved useful in guiding surgical strategy and predicting functional outcomes in cholesteatoma surgery. Advanced stage and greater cholesteatoma extension were associated with more extensive surgical procedures and poorer hearing results. Long-term audiological follow-up provided more accurate insights into surgical success. The standardized ChOLE system facilitates consistent reporting and meaningful comparison across institutions and studies. Full article

Background/Objectives: Sudden sensorineural hearing loss (SSNHL) represents a challenging clinical entity with variable prognosis. Audiometric curve configuration has been proposed as a predictor of recovery. This study aimed to evaluate the association between audiogram morphology at onset and hearing outcome in patients with idiopathic unilateral SSNHL treated with standardized therapy. Methods: We retrospectively analyzed 156 patients with idiopathic SSNHL. Hearing thresholds at key frequencies were measured at baseline and 4 weeks post-treatment. Patients were categorized into upsloping, flat, downsloping, or U-shaped audiogram subgroups. Recovery was classified into four levels. Comparisons were made across subgroups for audiometric and laboratory data using ANOVA and chi-square tests. Results: Baseline PTA values were comparable across audiogram subgroups (p = 0.12). Hearing recovery differed significantly according to audiogram configuration (chi-square, p < 0.001), with upsloping and U-shaped patterns showing the best outcomes. Flat and downsloping curves were associated with poorer recovery, lower HDL, and elevated NLR values. Conclusions: Audiogram configuration is a relevant prognostic marker in SSNHL. Patterns linked to adverse metabolic and inflammatory profiles may benefit from tailored treatment strategies in a personalized medicine framework. Full article

Objectives: Lermoyez syndrome (LS) is a rare variant of endolymphatic hydrops with a unique clinical presentation characterized by reversible sensorineural hearing loss preceding vertigo. This review aims to synthesize available literature on LS to clarify its clinical characteristics, diagnostic approach, management strategies, and outcomes, and to highlight the distinguishing features from Menière’s disease (MD). Methods: A systematic literature review according to PRISMA guidelines was conducted from 1919 to 2025. The extracted data included demographics, symptom profiles, audiovestibular testing, imaging findings, treatment approaches, and patient outcomes. Results: A total of 23 studies were identified, reporting 53 individual cases of LS. Patients ranged from 27 to 85 years of age, with a mean age of 50.34 years and a male predominance (64.1%). The hallmark of LS across cases was a reproducible clinical pattern of unilateral low-frequency hearing loss followed by vertigo and subsequent auditory recovery. Audiometry typically confirmed reversible sensorineural hearing loss, while vestibular tests and imaging were often unremarkable, primarily used to exclude alternative diagnoses. Treatment approaches varied and were often based on MD protocols, including dietary modifications, vasodilators, diuretics, and vestibular suppressants. Prognosis was generally favorable, with most patients experiencing both hearing recovery and symptom resolution. Conclusions: LS remains a clinically distinct but underrecognized inner ear disorder. Its defining feature—the paradoxical improvement in hearing after vertigo—distinguishes it from Menière’s disease and should prompt clinicians to consider LS in differential diagnosis. Due to the rarity of LS and the lack of standardized guidelines, diagnosis and treatment rely on careful clinical assessment and individualized management strategies. Full article