RARE DISEASE
Sudden sensorineural hearing loss
Sudden sensorineural hearing loss
Sudden sensorineural hearing loss
Synonyms: Secondary acute sensorineural hearing loss
Synonyms: Secondary acute sensorineural hearing loss
Synonyms: Secondary acute sensorineural hearing loss
Drug discovery
7
drugs
With orphan designations
Overview
Sudden sensorineural hearing loss (SSNHL) is an otologic emergency characterized by rapid-onset hearing loss ≥30 dB across three contiguous frequencies within 72 hours, typically unilateral. Most cases (85–90%) are idiopathic, though viral infections, vascular compromise, and autoimmune etiologies are implicated. Diagnosis requires audiometry and exclusion of conductive causes. Immediate high-dose corticosteroids are the cornerstone of treatment, with intratympanic steroids or hyperbaric oxygen as adjuncts. Prognosis improves with early intervention, but 30–50% have permanent deficits requiring auditory rehabilitation [1][3][9][15].
Categories: rare otorhinolaryngological diseases
Research Papers
1,671 drug discovery papers about Sudden sensorineural hearing loss, with 3 first-in-class and 11 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
1,671 drug discovery papers about Sudden sensorineural hearing loss, with 3 first-in-class and 11 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2026-09-25 | Intratympanic steroids as primary treatment for sudden sensorineural hearing loss
Sudden sensorineural hearing loss (SSNHL) is an otological emergency which demands immediate treatment. It is a rare condition which goes unnoticed because of lack of awareness about this condition among the people and healthcare professionals. SSNHL is considered to be idiopathic but some researchers have reported viral infections, vascular insufficiency, intra labyrinthine membrane rupture etc. as possible etiologies. There are various treatments modalities described in literature such as are steroids, antivirals, vasodilators, hyperbaric oxygen therapy etc. Steroids are one of the most common treatment modalities used for SSNHL. Intratympanic administration of steroids is beneficial because they help in hearing loss recovery while bypassing the side effects of systemic steroids. Our study is a retrospective study of the 15 cases of SSNHL treated by intratympanic steroids in the ENT Department of Burnpur Hospital from 2022-2025. The study reported more number of males with SSNHL. Most of the cases were seen in the age group of 61-70 years. Hearing recovery was seen in 73.3% of the cases. It is concluded that Intratympanic administration of steroids are a novel modality of treatment for cases of SSNHL and awareness of this condition must be created among the public and medical practitioners.
2026-09-25 | Audiological outcomes and related clinical indicators of intratympanic steroid injection therapy in patients with sudden sensorineural hearing loss and alcohol consumption.
Sudden sensorineural hearing loss (SSNHL) is an acute otologic disorder with heterogeneous clinical presentations and diverse hearing recovery outcomes. Alcohol consumption has been implicated in vascular and metabolic alterations that may affect inner ear function; however, its influence on hearing outcomes following intratympanic steroid injection (ITSI) remains unclear. This study aimed to evaluate the audiological outcomes and identify factors associated with hearing recovery in SSNHL patients with alcohol consumption treated with ITSI. This retrospective study analyzed 103 patients with SSNHL and active alcohol consumption who were treated with ITSI at a tertiary medical center. Alcohol intake was quantified based on the reported daily number of beer bottles consumed. Patients were stratified into ≤1 bottle/day and >1 bottle/day groups. Audiological outcomes were evaluated before and after ITSI, and hearing recovery was categorized according to Siegel's criteria. Factors associated with hearing recovery were examined using univariate and multivariate logistic regression models. The average age of the patients was 52.66 ± 15.41 years, with mean gains of 15.62 ± 19.15 dB in mean hearing level, 15.93 ± 29.83 dB in SRT, and 17.98 ± 33.46% in SDS. Complete or partial recovery was achieved in 29 patients (28.16%). No significant association was observed between alcohol consumption level and either hearing gains or recovery rates. Tinnitus, vertigo, aural fullness, higher HbA1c levels, and a profound audiometric configuration emerged as significant predictors of poorer hearing recovery in the univariate analysis. Multivariate analysis demonstrated that vertigo (OR = 0.1732), aural fullness (OR = 0.1832), elevated HbA1c (OR = 0.1661), and a profound pure-tone audiometry pattern (OR = 0.0419) remained independently associated with reduced odds of hearing recovery. In patients with SSNHL and alcohol consumption, ITSI was associated with overall audiological improvement across multiple hearing parameters. Prognosis appeared to be more closely related to clinical characteristics, metabolic status, and audiometric configuration. Careful assessment of these variables may therefore assist clinicians in risk stratification, prognostic counseling, and the development of more individualized management strategies for this patient population.
2026-09-24 | Therapeutic Effect of Intratympanic Methylprednisolone in Sudden Sensorineural Hearing Loss: A Prospective Observational Study
Introduction: Sudden sensorineural hearing loss (SSNHL) is an otological emergency requiring prompt treatment. Intratympanic steroid therapy delivers a high concentration of steroid directly into the inner ear while minimizing systemic adverse effects. Despite its routine use, outcomes of intratympanic methylprednisolone therapy have not been documented in our setting. This study evaluated its therapeutic effect in SSNHL patients treated in routine clinical practice. Methods: This hospital-based prospective observational analytical study was conducted in the Department of ENT at Lumbini Medical College from June 2025 to May 2026. Forty patients with SSNHL who received intratympanic methylprednisolone (40 mg in 1 mL) as part of routine care were included. Each received four injections over 15 days. Hearing thresholds were assessed using pure tone audiometry before treatment and at one month after treatment, using 3-frequency (500, 1000, 2000 Hz) and 4-frequency (500, 1000, 2000, 4000 Hz) averages. Improvement was defined as a hearing gain of ≥10 dB in the 3-frequency PTA. Results: Improvement was observed in 35 patients (87.5%). The mean 3-frequency PTA improved from 60.3 ± 6.6 dB to 49.8 ± 10.2 dB (mean gain 10.4 ± 7.8 dB; p < 0.001). The mean 4-frequency PTA improved from 62.0 ± 6.0 dB to 52.3 ± 9.9 dB (mean gain 9.7 ± 6.5 dB; p < 0.001). No vertigo, perforation, or infection occurred. Conclusions: Intratympanic methylprednisolone therapy resulted in significant hearing improvement in patients with SSNHL treated in routine clinical practice, with a favorable safety profile.
2026-09-24 | The inner ear immune microenvironment in sensorineural hearing loss: mechanisms and therapeutic perspectives.
Sensorineural hearing loss (SNHL) affects over 1.5 billion individuals worldwide and remains the most prevalent sensory deficit in humans. Although classical pathological models have emphasized hair cell death from oxidative stress and mitochondrial dysfunction, accumulating evidence over the past decade has redefined the cochlea as an immunologically active tissue rather than an immune-privileged organ. Resident macrophages populate the spiral ligament, stria vascularis, spiral ganglion, and modiolus, and they orchestrate both protective and damaging responses to acoustic, ototoxic, metabolic, and immunological stress. Recent single-cell transcriptomic data have identified disease-specific macrophage subsets, including a proinflammatory CD74+CD14+ population enriched in age-related and noise-induced hearing loss. Molecular drivers of cochlear inflammation include cytokines such as TNF-alpha, IL-1beta, and IL-6, danger-associated molecular patterns including HMGB1 and HSP70, NLRP3 inflammasome activation, complement signaling, and disruption of the blood-labyrinth barrier. These mechanisms converge across diverse SNHL etiologies, from noise-induced synaptopathy and inflammaging in presbycusis, to ototoxicity, autoimmune inner ear disease, sudden idiopathic SNHL, and viral or post-viral hearing loss. Therapeutically, the field is shifting from broad-spectrum corticosteroids toward immune-targeted strategies, including cytokine-directed biologics, NLRP3 inhibitors, complement-targeted agents, macrophage reprogramming, mesenchymal stem cell-derived exosomes, intratympanic nanoparticle delivery systems, and gene therapy approaches with regulated immune profiles. Perilymph sampling now offers a window into the cochlear microenvironment in living patients, enabling biomarker-guided precision medicine. This review synthesizes the cellular, molecular, and clinical evidence supporting the cochlear immune microenvironment as a unifying axis in SNHL pathogenesis, and outlines therapeutic perspectives that may transform the standard of care over the next decade.
2026-09-15 | Bilateral sudden sensorineural hearing loss following indirect lightning exposure successfully treated with hyperbaric oxygen therapy: a case report.
Lightning injuries may result in a wide spectrum of neurological and otological complications. Sudden sensorineural hearing loss (SSNHL) following indirect lightning exposure is rare, and evidence regarding the role of hyperbaric oxygen therapy (HBOT) remains limited. We report a case of severe bilateral SSNHL following indirect lightning exposure with substantial recovery after HBOT. A 26-year-old man developed sudden bilateral hearing loss after a lightning strike hit a lightning rod approximately 20 m from his location. He experienced transient syncope and limb weakness consistent with keraunoparalysis. Audiometry demonstrated severe bilateral sensorineural hearing loss, with pure-tone averages (PTA) of 70 dB HL in the right ear and 75 dB HL in the left ear. Because of persistent hearing loss despite systemic corticosteroid treatment, HBOT was initiated approximately 105 days after injury. A total of 30 HBOT sessions were administered together with intratympanic steroid injections. After 20 sessions, PTAs improved to 39 dB HL and 42 dB HL. Following completion of treatment, PTAs improved to 15 dB HL and 12 dB HL, with speech discrimination scores of 96% bilaterally. Severe bilateral SSNHL following indirect lightning exposure is rarely reported. Proposed mechanisms include cochlear injury, microvascular compromise, and oxidative damage to inner-ear structures. This case demonstrates that indirect lightning exposure may result in both keraunoparalysis and severe bilateral SSNHL. Substantial hearing recovery was observed despite initiation of HBOT more than three months after injury, suggesting a potential role for HBOT as part of a multidisciplinary treatment approach in selected cases.
proteins
2026-09-03 | [Analysis of short-term efficacy and influencing factors of severe to profound sudden sensorineural hearing loss in children].
Objective:To investigate the short-term efficacy and prognostic factors of severe to profound sudden sensorineural hearing loss（SSNHL） in pediatric patients. Methods:A retrospective analysis was conducted on the clinical data of 87 children with severe to profound SSNHL who were admitted to our department between January 2014 and December 2024. Hearing changes were evaluated 10 days after treatment initiation. Pearson's chi-square test was used to compare intergroup differences in categorical variables, including gender, affected side, vertigo, tinnitus, early combined use of batroxobin, degree of hearing loss, and upper respiratory tract infection. The Mann-Whitney U test was used to analyze intergroup differences in continuous variables, including age and duration of hearing loss. Univariate and multivariate logistic regression analyses assessed the short-term therapeutic efficacy and its influencing factors in severe and profound SSNHL. Grouped stacked bar charts and grouped box plots explored the relationship between significant influencing factors and therapeutic response rate. Results:Pearson's chi-square test and Mann-Whitney U test revealed that gender, age, duration of hearing loss, and early combined use of batroxobin were significantly associated with the therapeutic response rate of severe and profound SSNHL in children（P<0.05）. No statistically significant differences in therapeutic response rate were observed among groups with affected side, tinnitus, vertigo, degree of hearing loss, or upper respiratory tract infection（P>0.05）. Univariate logistic regression analysis indicated that age, gender, duration of hearing loss, and early combined use of batroxobin were influencing factors for the therapeutic efficacy of severe and profound SSNHL in children. Multivariate logistic regression analysis further demonstrated that duration of hearing loss was negatively correlated with the response rate, whereas female gender, older age, and early combined use of batroxobin were positively correlated with the response rate. Grouped stacked bar charts showed that the response rate was higher in females, and higher in patients with early batroxobin administration. Grouped box plots indicated that a shorter duration of hearing loss was associated with a more concentrated distribution of response rates, and older age was also correlated with a more concentrated distribution of response rates. Conclusion:Gender, age, early combined use of batroxobin, and duration of hearing loss are independent influencing factors for the short-term treatment of severe and profound SSNHL in children.
2026-03-24 | Apolipoprotein AI is a protective factor for idiopathic sudden sensorineural hearing loss: a two-sample mendelian randomisation study.
Observational studies have suggested an association between cardiometabolic factors and idiopathic sudden sensorineural hearing loss (ISSNHL), but the causal relationship remains unclear. We aimed to assess the causal effect of cardiometabolic factors on the risk of ISSNHL using a two-sample Mendelian randomisation (MR) approach. Two-sample MR study. We used summary-level data from genome-wide association studies (GWAS). Genetic instruments for cardiometabolic factors were obtained from GWAS. Summary statistics for ISSNHL were derived from a publicly available GWAS database. Genetically predicted higher ApoAI levels were significantly associated with an decreased risk of ISSNHL (IVW OR = 0.82, 95% CI: 0.68-0.98, P = 0.03). No evidence of directional pleiotropy was detected in MR-Egger regression (P = 0.30), and Cochran's Q test indicated no significant heterogeneity (P = 0.94). MR-PRESSO detected no outliers. This study provides evidence supporting a potential causal relationship between higher ApoAI levels and decreased risk of ISSNHL. Further biological and clinical studies are warranted to elucidate the underlying mechanisms.
2023-06-29 | Neurofilament Expression as a Biomarker of Post-COVID-19 Sudden Sensorineural Hearing Loss
Sudden sensorineural hearing loss (SSHL) affects a patient's quality of life and requires rapid treatment. The etiology is viral, vascular, and autoimmune, even though, in most cases, it remains idiopathic SSHL. Since 2019, several different complications have been identified following COVID-19 infection. The post-COVID-19 ENT manifestations reported in the literature are sore throat, headache, pharyngeal erythema, nasal obstruction, rhinorrhea, upper respiratory tract infection, and tonsil enlargement. Cases of SSHL, vestibular neuronitis, and audio-vestibular disorders (such as tinnitus, dizziness, and vertigo) have also been reported, albeit in a smaller percentage of patients. We reported our experience of a case of post-COVID-19 SSHL in the absence of any other type of post-COVID symptoms or brain and internal auditory canal magnetic resonance imaging and magnetic resonance angiography modifications. We aimed to identify a serological biomarker of sudden sensorineural hearing loss, and we also dosed and monitored the value of the serum neurofilament light (NfL). the best of our knowledge, this is the first report that associates SSHL and the serological increase in NfL as a potential biomarker of neuronal-disease-related damage.
2023-06-29 | Analysis of Clinical Efficacy and Influencing Factors of Nerve Growth Factor (NGF) Treatment for Sudden Sensorineural Hearing Loss.
Objective: This study aims to examine the clinical efficacy and prognostic factors associated with nerve growth factor (NGF) treatment for sudden sensorineural hearing loss (SSHL). Materials and methods: A retrospective analysis was conducted on the clinical data of 101 patients with moderate or more severe SSHL who underwent secondary treatment at Sun Yat-sen Memorial Hospital of Sun Yat-sen University between January 2019 and July 2020. Prior to treatment, all patients were assessed using Pure Tone Audiometry (PTA), auditory brainstem response, otoacoustic emission, temporal bone computed tomography, or inner ear magnetic resonance imaging. Fifty-seven patients received conventional systemic treatment and served as the control group, while 44 patients received NGF in conjunction with conventional systemic treatment, forming the experimental group. PTA results were compared between the two groups before treatment and at 1 week, 2 weeks, and 1 month post-treatment. Additionally, the impact of age, sex, affected side, hypertension, and other factors on patient prognosis was analyzed. Results: Both groups demonstrated significant PTA improvements following treatment, with a statistically significant difference (P < .05). The hearing recovery effective rate in the control group was 42.1%, while that of the experimental group reached 70.5%, with a statistically significant difference between the groups (P < .05). Most patients experienced notable hearing improvements 1 week after treatment, with some patients still showing progress 2 weeks post-treatment. Multifactor analysis revealed that hypertension and onset days were associated with treatment outcomes. Conclusion: Secondary treatment remains clinically significant for patients with SSHL who have not achieved a satisfactory response or show no clear improvement following initial treatment. The presence of hypertension and delayed treatment are negative factors related to treatment efficacy.
2021-09-09 | Effects of Insulin-Like Growth Factor (IGF-1) in Patients with Sensorineural Hearing Loss.
(1) To test the effect of local administration of insulin-like growth factor-1 (IGF-1) in patients with sensorineural hearing loss (SNHL). (2) To test the effect of local administration of IGF-1 in patients with ototoxicity. Forty patients with SNHL were included in the study. Their hearing thresholds at different frequencies (0.5, 1, 2, and 4 kHz) along with the average hearing threshold were noted. The patients were then randomly allocated to 2 groups and were treated with IGF-1 via one of the following routes: (1) intratympanic injection and (2) Gelfoam. Patients were followed-up at weekly intervals for 6 weeks but follow-up PTA was done at 3 weeks, 6 weeks, and 6 months only. Forty patients (25 male, 15 female) participated in the study. Their age ranged from 13 to 63 years, with a mean of 31.3 years. Nineteen (47.5%) patients exhibited some degree of recovery after 6 months of follow-up, while 21 (52.5%) did not exhibit any recovery. Fourteen (35%) patients showed slight recovery (SR), 1 (4%) patient showed marked recovery, and complete recovery was observed in 4 (10%) patients. Twelve of the 20 patients who underwent treatment using Gelfoam showed improvement in hearing (measured as a reduction in hearing threshold), while only 7 of the 20 patients who underwent intratympanic injection showed such improvement. Among adverse reactions, the most common was pain (88%) which typically did not last beyond 3 days. Other adverse reactions observed were dizziness (24%) and headache (20%). One patient suffered from acute suppurative otitis media (ASOM) and had a perforation in the tympanic membrane. However, this was treated successfully with medications. Intratympanic IGF-1 is a novel drug that has shown early promise in controlling and reversing SNHL.
antibodies
2023-11-27 | A Comparative Study Using Vestibular Mapping in Sudden Sensorineural Hearing Loss With and Without Vertigo.
To investigate the impairment patterns in peripheral vestibular organs in sudden sensorineural hearing loss (SSNHL) with and without vertigo. Retrospective study. Single tertiary medical center. Data from 165 SSNHL patients in a tertiary referral center from January 2017 to December 2022 were retrospectively analyzed. All patients underwent a video head impulse test, vestibular evoked myogenic potential test, and pure-tone audiometry. Hierarchical cluster analysis was performed to investigate vestibular impairment patterns. The prognosis of the hearing was determined using American Academy of Otolaryngology-Head and Neck Surgery recommendations. After excluding patients with vestibular schwannoma and Meniere's disease, 152 patients were included in this study. A total of 73 of 152 patients were categorized as SSNHL with vertigo (SSNHL_V) and showed an independent merge of the posterior semicircular canal (PSCC) in cluster analysis. A total of 79 of 152 patients were categorized as SSNHL without vertigo (SSNHL_N) and showed an independent merge of saccule in cluster analysis. The PSCC (56.2%) and saccule (20.3%) were the most frequently impaired vestibular organs in SSNHL_V and SSNHL_N, respectively. In terms of prognosis, 106 of 152 patients had partial/no recovery and showed an independent merge of the PSCC in cluster analysis. A total of 46 of 152 patients had a complete recovery and showed an independent merge of the saccule in cluster analysis. A tendency of isolated PSCC dysfunction was seen in SSNHL_V and partial/no recovery. A tendency of isolated saccular dysfunction was seen in SSNHL_N and complete recovery. Different treatments might be needed in SSNHL depending on the presence of vertigo.
2021-11-25 | Biologics for Immune-Mediated Sensorineural Hearing Loss.
Immune-mediated hearing losses include autoimmune inner ear disease, sudden sensorineural hearing loss, and Meniere's disease. Standard therapy for an acute decline in hearing is timely use of corticosteroids. Although 60% to 70% of patients are initially corticosteroid-responsive, that responsiveness is lost over time. In corticosteroid-resistant patients, increased expression of interleukin (IL)-1 is observed, and these patients may benefit from IL-1 inhibition. Autoinflammatory diseases are characterized by dysregulation of the innate immune response, clinically include sensorineural hearing loss, and benefit from IL-1 inhibition, thereby further establishing the relationship of IL-1 with immune-mediated sensorineural hearing loss.
2021-01-27 | Lipopolysaccharide decreases cochlear blood flow dose dependently in a guinea pig animal model via TNF signaling
ABSTRACT Objective To evaluate the effect of Lipopolysaccharide (LPS), a bacterial endotoxin on cochlear microcirculation, and its mode of action. Methods Twenty‐five Dunkin‐Hartley guinea pigs were divided into five groups of five animals each. After surgical preparation, cochlear microcirculation was quantified by in vivo fluorescence microscopy. Placebo or LPS (1 mg, 10 µg, and 100 ng) was applied topically, and microcirculation was measured before and twice after application. A fifth group was pretreated with etanercept, a tumor necrosis factor (TNF) antagonist, and afterward the lowest LPS concentrations that yielded significant results (10 µg) were applied. Results In the groups that had been treated with 1 mg and 10 µg LPS, a significant drop in cochlear microcirculation was observed after 30 (.791 ± .089 Arbitrary Units (AU), compared to baseline, and .888 ± .071AU) and 60 (.756 ± .101 AU and .817 ± .124 AU, respectively) minutes. The groups that had been treated with 100 ng LPS and that had been pretreated with etanercept showed no significant change in cochlear blood flow compared to placebo. Conclusion Lipopolysaccharide shows a dose‐dependent effect on cochlear microcirculation; this effect can already be observed after 30 min. Pretreatment with etanercept can abrogate this effect, indicating that TNF mediates the effect of LPS on cochlear microcirculation.
2020-02-10 | Intracochlear Perfusion of Tumor Necrosis Factor-Alpha Induces Sensorineural Hearing Loss and Synaptic Degeneration in Guinea Pigs
Tumor necrosis factor-alpha (TNF-alpha) is a proinflammatory cytokine that plays a prominent role in the nervous system, mediating a range of physiologic and pathologic functions. In the auditory system, elevated levels of TNF-alpha have been implicated in several types of sensorineural hearing loss, including sensorineural hearing loss induced by vestibular schwannoma, a potentially fatal intracranial tumor that originates from the eighth cranial nerve; however, the mechanisms underlying the tumor's deleterious effects on hearing are not well understood. Here, we investigated the effect of acute elevations of TNF-alpha in the inner ear on cochlear function and morphology by perfusing the cochlea with TNF-alpha in vivo in guinea pigs. TNF-alpha perfusion did not significantly change thresholds for compound action potential (CAP) responses, which reflect cochlear nerve activity, or distortion product otoacoustic emissions, which reflect outer hair cell integrity. However, intracochlear TNF- alpha perfusion reduced CAP amplitudes and increased the number of inner hair cell synapses without paired post-synaptic terminals, suggesting a pattern of synaptic degeneration that resembles that observed in primary cochlear neuropathy. Additionally, etanercept, a TNF-alpha-blocker, protected against TNF-alpha-induced synaptopathy when administered systemically prior to intracochlear TNF-alpha perfusion. Findings motivate further investigation into the deleterious effects of chronically elevated intracochlear levels of TNF-alpha, and the potential for etanercept to counter these effects.
2018-02-17 | The Role of Tumor Necrosis Factor Alpha (TNFα) in Hearing Loss and Vestibular Schwannomas
The aim of this review is to highlight relevant literature on the role of tumor necrosis factor alpha (TNFα) in sensorineural hearing loss (SNHL) and vestibular schwannomas (VS). A comprehensive review of publically available databases including PubMed was performed. The mechanism by which hearing loss occurs in VS is still unknown and likely multifactorial. Genetic differences between VSs and tumor-secreted proteins may be responsible, at least in part, for VS-associated SNHL. TNFα has pleotropic roles in promoting inflammation, maintaining cellular homeostasis, inducing apoptosis, and mediating ototoxicity in patients with sporadic VS. TNFα-targeted therapies have shown efficacy in both animal models of sensorineural hearing loss and clinical trials in patients with immune-mediated hearing loss. Efforts are underway to develop nanotechnology-based methods to target TNFα and other pathogenic molecules in VS. Development of molecularly targeted therapies against TNFα represents an important area of research in ameliorating VS-associated hearing loss.
cell therapies
2026-07-01 | Intratympanic injection of human umbilical cord mesenchymal stem cell derived small extracellular vesicles for refractory sudden sensorineural hearing loss: a Phase 1 trial.
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).
2026-03-26 | Clinical Outcomes of Platelet-Rich Plasma in Otology: A Systematic Review of Tympanoplasty, Myringoplasty, and Hearing Loss Management.
Platelet-rich plasma (PRP), an autologous concentrate of platelets and growth factors, has been explored as an adjunct in otologic procedures, including tympanoplasty, myringoplasty, and treatment of sudden sensorineural hearing loss (SNHL). This systematic review evaluated reported clinical outcomes associated with PRP in these settings. A literature search was conducted in March 2025 across PubMed, Scopus, Web of Science, EMBASE, and the Cochrane Library. Eligible studies were English-language human studies (2015-2025) with original clinical outcome data from randomized or comparative observational designs. Due to methodological heterogeneity, findings were synthesized narratively. Six studies (n = 349 patients) were included: five addressing tympanic membrane repair and one addressing idiopathic sudden SNHL. In myringoplasty/tympanoplasty-related studies, PRP was generally associated with higher graft uptake and/or faster healing than comparator approaches; where reported, closure outcomes included 85.7% versus 60% in one comparative study, and several studies reported between-group differences with p < 0.05. Reported postoperative complications were low, and no major PRP-related safety concerns were identified in the included studies. In SNHL, one comparative study suggested hearing improvement with intratympanic PRP that was comparable to, or greater than, steroid therapy, but evidence remains limited to short-term follow-up and a small number of studies. Current evidence supports PRP as a feasible and apparently safe adjunct in otologic practice, particularly for tympanic membrane repair. However, certainty regarding the magnitude of benefit and long-term auditory outcomes remains limited by small sample sizes, heterogeneity in PRP preparation/application, and variability in study design. Standardized protocols and larger, well-designed randomized trials are needed.
2026-03-01 | Role of platelet-rich plasma in management of sensorineural hearing loss diseases.
Sensorineural deafness is a form of loss of hearing. It happens from damage to the cochlea. Platelet-rich plasma (PRP) is an autologous management derived from a case's blood and was observed to have pro-nerve regenerative characteristics. Intratympanic injection of PRP improves the cells of the hair of the inner ear, therefore enhancing the hearing. To show the effectiveness and safety of PRP in the management of sensorineural hearing loss (SNHL) diseases. 150 patients who had sensorineural hearing loss because of different otological diseases, such as acoustic trauma, presbycusis, idiopathic sudden SNHL, in addition to Meniere's disease (MD). All cases received 4 times of intratympanic injections of PRP with one-week intervals between them. In our research, 150 cases with SNHL diseases, as acoustic trauma, MD, presbycusis, and idiopathic sudden sensorineural hearing loss (ISSNHL) (68 (46.3%) males and 82 (54.6%) females) with age ranges from 18 to 78 years, received intratympanic injections of PRP. 50 patients with ISSNHL: 39 of them showed hearing improvement, with an average of 28 dB in pure tone audiometry (PTA) and 26% in speech discrimination. In acoustic trauma, 18 patients, 12 of them, showed hearing improvement, averaging 30 dB in PTA and 28% in speech discrimination. In presbycusis, 58 patients, 42 of them, showed an improvement in hearing of an average of 25 dB and 24% in speech discrimination. In Meniere's disease, 24 patients, 16 of them, showed an improvement in hearing of an average of 15 dB and 5% in speech discrimination. Platelet-rich plasma appears effective and safe for the management of sensorineural hearing loss with no systemic side effects as an oral steroid and at low cost.
2026-02-16 | Sudden Unilateral Hearing Loss and Vertigo in an Adolescent: Viral Labyrinthitis Versus Idiopathic Sudden Sensorineural Hearing Loss.
Labyrinthitis and idiopathic sudden sensorineural hearing loss (SSNHL) are uncommon in children and are both considered otologic emergencies, as delayed diagnosis can lead to permanent disability. We describe a 15-year-old boy with a history of focal cortical dysplasia and a single remote seizure who presented with abrupt left-sided hearing loss, tinnitus, vertigo, nausea, and headache. Neurologic examination revealed no focal deficits; however, marching Romberg testing demonstrated instability, and otoscopy revealed only a small left middle-ear effusion. Laboratory studies, including complete blood count and C-reactive protein, were unremarkable. Non-contrast computed tomography (CT) of the head showed no acute intracranial abnormality. Magnetic resonance imaging (MRI) of the brain and internal auditory canals with and without contrast demonstrated normal labyrinthine structures and stable right frontal cortical dysplasia. The working diagnosis, established by pediatric neurology and otolaryngology, was acute viral labyrinthitis or idiopathic SSNHL with vestibular involvement. The patient received high-dose systemic corticosteroids, vestibular suppressants, antiemetics, and empiric oral acyclovir. Vertigo and nausea improved substantially, but profound left-sided hearing loss persisted at discharge. Outpatient audiometry and possible intratympanic steroid therapy were planned. This case emphasizes the diagnostic overlap between labyrinthitis and SSNHL in adolescents, the limited role of routine head CT for peripheral vestibulocochlear presentations, and the importance of early guideline-directed corticosteroid therapy. It also illustrates that MRI may be normal in clinically significant labyrinthitis and that careful follow-up is required to determine the need for salvage intratympanic steroid treatment.
2025-05-09 | Intratympanic steroid via saturated gelfoam vs. intratympanic injection for sudden sensorineural hearing loss.
The aim of this study was to investigate the efficacy of a novel intratympanic steroid (ITS) therapy via saturated Gelfoam® (GSD) in sudden sensorineural hearing loss (SSNHL) by comparing with ITS injection therapy. The patients with SSNHL who received initial ITS therapy with no prior systemic steroid treatment were divided into two groups: the ITS via injection group (Group 1) and the ITS via GSD group (Group 2). The groups were compared in terms of the patients' demographic data, side of ear involvement, vertigo and tinnitus symptoms, comorbidities, initiation time of therapy, hearing level (HL) before and after therapy, word recognition score (WRS) before and after therapy, hearing gain (HG), WRS gain (WRSG), recovery rate (RR), tolerability of the applications and complications. HL and WRS scores were similar among the groups before therapy. Regarding the hearing outcomes after therapy HG and WRSG between the groups were not significant. In the comparison of RR, Group 2's score (70 %) was significantly higher than that of Group 1 (42.4 %) (p = 0.028). In terms of the tolerability of applications, ITS via GSD application was significantly more tolerable than ITS via injection (p = 0.046). Regarding complications, no otorrhea was observed in any patient, and tympanic membrane perforation was observed in 1 patient (3.33 %) in Group 2, whereas no perforation was observed in Group 1. Intratympanic GSD is a simple, effective and tolerable technique in the therapy of SSNHL, with success rates similar to those of ITS injection therapy and significant improvements in the RR scores.
other
2026-08-06 | Acupuncture-related therapy for sudden sensorineural hearing loss: clinical efficacy and central mechanistic insights
Sudden sensorineural hearing loss (SSNHL) is a common otolaryngological emergency, but its pathogenesis remains incompletely understood. Although acupuncture has recently become widely used as a complementary therapy for SSNHL in clinical practice, a comprehensive review of its central mechanisms is still lacking. Based on literature from 2020 to 2025 in databases such as PubMed, Web of Science, Embase, China National Knowledge Infrastructure (CNKI) and Wanfang Data (Wanfang), this narrative review synthesizes the latest research into the central mechanisms of acupuncture-related treatment for SSNHL, focusing on three key areas: auditory pathway plasticity, activation of the emotional regulation network, and functional integration of neurovascular units. Preliminary evidence suggests that acupuncture-related therapy may promote hearing recovery and alleviate associated symptoms via multiple target pathways. The proposed central mechanisms include modulation of functional connectivity in the temporal auditory cortex, regulation of limbic–subcortical emotional pathways, and enhancement of neurovascular coupling. This review provides a theoretical basis for a deeper understanding of the potential roles of acupuncture-related treatment in SSNHL, although further rigorous studies are needed to confirm these mechanistic links.
2024-12-20 | Treatment of sudden sensorineural hearing loss with tinnitus with Fu subcutaneous needling: A case report.
Fu's subcutaneous needling (FSN) is a special acupuncture method that uses FSN to sweep the subcutaneous tissue around or adjacent to the limbs to disperse the pain. Sudden sensorineural hearing loss (SSHL) is a kind of otological emergency with sudden onset within 72 hours, with unilateral hearing loss, and hearing loss of ≥20 dB in 2 connected frequencies, and most of the patients have no obvious triggers. Most patients with SSHL are accompanied by tinnitus, vertigo, and nausea and vomiting. The patient suffered from hearing loss with tinnitus. Although received relevant treatment, the hearing loss and tinnitus did not improve significantly. SSHL with tinnitus. FSN treatment. A point 5 cm lateral to the point of tension and stiffness of the left trapezius muscle was selected on the affected back as the needle insertion point, and the condition of the neck muscles was improved through the FSN sweeping movement with neck reperfusion activities. The patient received treatment twice a week, and 4 weeks of treatment as a phase. At the end of each phase, a pure tone threshold audiometry, tinnitus evaluation scale Tinnitus Handicap Inventory assessment, and neck muscle palpation were performed. The patient's hearing improved and the Tinnitus Handicap Inventory score decreased. FSN has a good therapeutic effect on SSHL with tinnitus.
2024-01-17 | NcRNA: key and potential in hearing loss
Hearing loss has an extremely high prevalence worldwide and brings incredible economic and social burdens. Mechanisms such as epigenetics are profoundly involved in the initiation and progression of hearing loss and potentially yield definite strategies for hearing loss treatment. Non-coding genes occupy 97% of the human genome, and their transcripts, non-coding RNAs (ncRNAs), are widely participated in regulating various physiological and pathological situations. NcRNAs, mainly including micro-RNAs (miRNAs), long-stranded non-coding RNAs (lncRNAs), and circular RNAs (circRNAs), are involved in the regulation of cell metabolism and cell death by modulating gene expression and protein-protein interactions, thus impacting the occurrence and prognosis of hearing loss. This review provides a detailed overview of ncRNAs, especially miRNAs and lncRNAs, in the pathogenesis of hearing loss. We also discuss the shortcomings and issues that need to be addressed in the study of hearing loss ncRNAs in the hope of providing viable therapeutic strategies for the precise treatment of hearing loss.
2023-06-15 | Single-cell sequencing: a powerful technique to understand the pathophysiology of auditory disorders
Deciphering the inner ear transcriptome has allowed major breakthroughs in therapeutic development for auditory disorders. Single-cell sequencing (SCS) technologies, through the analysis of cellular heterogeneity and the identification of novel gene markers, can significantly enhance our understanding about auditory disorders. The application of SCS has allowed the identification of gene targets specific to the inner ear cellular subtypes such as supporting cells, hair cells, stria vascularis cells, and spiral ganglion neuron cells. SCS has provided new insights into the molecular mechanisms underlying the pathophysiology of auditory disorders such as noise-trauma induced hearing loss, ototoxicity, sudden sensorineural hearing loss (SSNHL), vestibular schwannomas and acute otitis media, leading to the discovery of novel therapeutic targets. The objective of this perspective article is to discuss recent advancements regarding the utilization of SCS for inner ear research. With global initiatives striving to overcome the increasing burden of hearing loss, the SCS technique holds a great potential in revolutionizing the management of auditory disorders, paving the way to develop effective treatment modalities.
2022-09-02 | Role of microRNAs as novel diagnostic biomarkers and potential therapeutic targets for hearing disorders (Review)
The ear is a complex structure consisting of the outer, middle and inner ear. For the proper function of the auditory system, the development of all these parts needs to be highly organized. Any alterations in this chain of events can lead to hearing loss or other impairments, such as dizziness, tinnitus and loss of balance. Of note, several external and internal elements, including noise exposure, ototoxic drugs, aging, as well as environmental or genetic factors, have been widely associated with hearing loss. Over the years, a growing body of evidence has indicated that microRNAs (miRNAs/miRs) play a critical role in ear development and auditory function, suggesting that an altered pattern of miRNA expression may be involved in the progression of these pathological conditions. On these bases, the present review article aimed to summarize the role of miRNAs in inner ear development and hearing disorders. Although further studies are warranted to provide more in‑depth knowledge of the implicated mechanisms of action, according to the current data, miRNAs may be used as novel diagnostic biomarkers for the early detection of hearing loss. In addition, miRNAs and related signaling pathways may represent novel potential therapeutic targets for the treatment of hearing loss, ear inflammation and vestibular schwannoma.
small molecules
2026-09-25 | Intratympanic steroids as primary treatment for sudden sensorineural hearing loss
Sudden sensorineural hearing loss (SSNHL) is an otological emergency which demands immediate treatment. It is a rare condition which goes unnoticed because of lack of awareness about this condition among the people and healthcare professionals. SSNHL is considered to be idiopathic but some researchers have reported viral infections, vascular insufficiency, intra labyrinthine membrane rupture etc. as possible etiologies. There are various treatments modalities described in literature such as are steroids, antivirals, vasodilators, hyperbaric oxygen therapy etc. Steroids are one of the most common treatment modalities used for SSNHL. Intratympanic administration of steroids is beneficial because they help in hearing loss recovery while bypassing the side effects of systemic steroids. Our study is a retrospective study of the 15 cases of SSNHL treated by intratympanic steroids in the ENT Department of Burnpur Hospital from 2022-2025. The study reported more number of males with SSNHL. Most of the cases were seen in the age group of 61-70 years. Hearing recovery was seen in 73.3% of the cases. It is concluded that Intratympanic administration of steroids are a novel modality of treatment for cases of SSNHL and awareness of this condition must be created among the public and medical practitioners.
2026-09-25 | Audiological outcomes and related clinical indicators of intratympanic steroid injection therapy in patients with sudden sensorineural hearing loss and alcohol consumption.
Sudden sensorineural hearing loss (SSNHL) is an acute otologic disorder with heterogeneous clinical presentations and diverse hearing recovery outcomes. Alcohol consumption has been implicated in vascular and metabolic alterations that may affect inner ear function; however, its influence on hearing outcomes following intratympanic steroid injection (ITSI) remains unclear. This study aimed to evaluate the audiological outcomes and identify factors associated with hearing recovery in SSNHL patients with alcohol consumption treated with ITSI. This retrospective study analyzed 103 patients with SSNHL and active alcohol consumption who were treated with ITSI at a tertiary medical center. Alcohol intake was quantified based on the reported daily number of beer bottles consumed. Patients were stratified into ≤1 bottle/day and >1 bottle/day groups. Audiological outcomes were evaluated before and after ITSI, and hearing recovery was categorized according to Siegel's criteria. Factors associated with hearing recovery were examined using univariate and multivariate logistic regression models. The average age of the patients was 52.66 ± 15.41 years, with mean gains of 15.62 ± 19.15 dB in mean hearing level, 15.93 ± 29.83 dB in SRT, and 17.98 ± 33.46% in SDS. Complete or partial recovery was achieved in 29 patients (28.16%). No significant association was observed between alcohol consumption level and either hearing gains or recovery rates. Tinnitus, vertigo, aural fullness, higher HbA1c levels, and a profound audiometric configuration emerged as significant predictors of poorer hearing recovery in the univariate analysis. Multivariate analysis demonstrated that vertigo (OR = 0.1732), aural fullness (OR = 0.1832), elevated HbA1c (OR = 0.1661), and a profound pure-tone audiometry pattern (OR = 0.0419) remained independently associated with reduced odds of hearing recovery. In patients with SSNHL and alcohol consumption, ITSI was associated with overall audiological improvement across multiple hearing parameters. Prognosis appeared to be more closely related to clinical characteristics, metabolic status, and audiometric configuration. Careful assessment of these variables may therefore assist clinicians in risk stratification, prognostic counseling, and the development of more individualized management strategies for this patient population.
2026-09-24 | Therapeutic Effect of Intratympanic Methylprednisolone in Sudden Sensorineural Hearing Loss: A Prospective Observational Study
Introduction: Sudden sensorineural hearing loss (SSNHL) is an otological emergency requiring prompt treatment. Intratympanic steroid therapy delivers a high concentration of steroid directly into the inner ear while minimizing systemic adverse effects. Despite its routine use, outcomes of intratympanic methylprednisolone therapy have not been documented in our setting. This study evaluated its therapeutic effect in SSNHL patients treated in routine clinical practice. Methods: This hospital-based prospective observational analytical study was conducted in the Department of ENT at Lumbini Medical College from June 2025 to May 2026. Forty patients with SSNHL who received intratympanic methylprednisolone (40 mg in 1 mL) as part of routine care were included. Each received four injections over 15 days. Hearing thresholds were assessed using pure tone audiometry before treatment and at one month after treatment, using 3-frequency (500, 1000, 2000 Hz) and 4-frequency (500, 1000, 2000, 4000 Hz) averages. Improvement was defined as a hearing gain of ≥10 dB in the 3-frequency PTA. Results: Improvement was observed in 35 patients (87.5%). The mean 3-frequency PTA improved from 60.3 ± 6.6 dB to 49.8 ± 10.2 dB (mean gain 10.4 ± 7.8 dB; p < 0.001). The mean 4-frequency PTA improved from 62.0 ± 6.0 dB to 52.3 ± 9.9 dB (mean gain 9.7 ± 6.5 dB; p < 0.001). No vertigo, perforation, or infection occurred. Conclusions: Intratympanic methylprednisolone therapy resulted in significant hearing improvement in patients with SSNHL treated in routine clinical practice, with a favorable safety profile.
2026-09-24 | The inner ear immune microenvironment in sensorineural hearing loss: mechanisms and therapeutic perspectives.
Sensorineural hearing loss (SNHL) affects over 1.5 billion individuals worldwide and remains the most prevalent sensory deficit in humans. Although classical pathological models have emphasized hair cell death from oxidative stress and mitochondrial dysfunction, accumulating evidence over the past decade has redefined the cochlea as an immunologically active tissue rather than an immune-privileged organ. Resident macrophages populate the spiral ligament, stria vascularis, spiral ganglion, and modiolus, and they orchestrate both protective and damaging responses to acoustic, ototoxic, metabolic, and immunological stress. Recent single-cell transcriptomic data have identified disease-specific macrophage subsets, including a proinflammatory CD74+CD14+ population enriched in age-related and noise-induced hearing loss. Molecular drivers of cochlear inflammation include cytokines such as TNF-alpha, IL-1beta, and IL-6, danger-associated molecular patterns including HMGB1 and HSP70, NLRP3 inflammasome activation, complement signaling, and disruption of the blood-labyrinth barrier. These mechanisms converge across diverse SNHL etiologies, from noise-induced synaptopathy and inflammaging in presbycusis, to ototoxicity, autoimmune inner ear disease, sudden idiopathic SNHL, and viral or post-viral hearing loss. Therapeutically, the field is shifting from broad-spectrum corticosteroids toward immune-targeted strategies, including cytokine-directed biologics, NLRP3 inhibitors, complement-targeted agents, macrophage reprogramming, mesenchymal stem cell-derived exosomes, intratympanic nanoparticle delivery systems, and gene therapy approaches with regulated immune profiles. Perilymph sampling now offers a window into the cochlear microenvironment in living patients, enabling biomarker-guided precision medicine. This review synthesizes the cellular, molecular, and clinical evidence supporting the cochlear immune microenvironment as a unifying axis in SNHL pathogenesis, and outlines therapeutic perspectives that may transform the standard of care over the next decade.
2026-09-15 | Bilateral sudden sensorineural hearing loss following indirect lightning exposure successfully treated with hyperbaric oxygen therapy: a case report.
Lightning injuries may result in a wide spectrum of neurological and otological complications. Sudden sensorineural hearing loss (SSNHL) following indirect lightning exposure is rare, and evidence regarding the role of hyperbaric oxygen therapy (HBOT) remains limited. We report a case of severe bilateral SSNHL following indirect lightning exposure with substantial recovery after HBOT. A 26-year-old man developed sudden bilateral hearing loss after a lightning strike hit a lightning rod approximately 20 m from his location. He experienced transient syncope and limb weakness consistent with keraunoparalysis. Audiometry demonstrated severe bilateral sensorineural hearing loss, with pure-tone averages (PTA) of 70 dB HL in the right ear and 75 dB HL in the left ear. Because of persistent hearing loss despite systemic corticosteroid treatment, HBOT was initiated approximately 105 days after injury. A total of 30 HBOT sessions were administered together with intratympanic steroid injections. After 20 sessions, PTAs improved to 39 dB HL and 42 dB HL. Following completion of treatment, PTAs improved to 15 dB HL and 12 dB HL, with speech discrimination scores of 96% bilaterally. Severe bilateral SSNHL following indirect lightning exposure is rarely reported. Proposed mechanisms include cochlear injury, microvascular compromise, and oxidative damage to inner-ear structures. This case demonstrates that indirect lightning exposure may result in both keraunoparalysis and severe bilateral SSNHL. Substantial hearing recovery was observed despite initiation of HBOT more than three months after injury, suggesting a potential role for HBOT as part of a multidisciplinary treatment approach in selected cases.
proteins
2026-09-03 | [Analysis of short-term efficacy and influencing factors of severe to profound sudden sensorineural hearing loss in children].
Objective:To investigate the short-term efficacy and prognostic factors of severe to profound sudden sensorineural hearing loss（SSNHL） in pediatric patients. Methods:A retrospective analysis was conducted on the clinical data of 87 children with severe to profound SSNHL who were admitted to our department between January 2014 and December 2024. Hearing changes were evaluated 10 days after treatment initiation. Pearson's chi-square test was used to compare intergroup differences in categorical variables, including gender, affected side, vertigo, tinnitus, early combined use of batroxobin, degree of hearing loss, and upper respiratory tract infection. The Mann-Whitney U test was used to analyze intergroup differences in continuous variables, including age and duration of hearing loss. Univariate and multivariate logistic regression analyses assessed the short-term therapeutic efficacy and its influencing factors in severe and profound SSNHL. Grouped stacked bar charts and grouped box plots explored the relationship between significant influencing factors and therapeutic response rate. Results:Pearson's chi-square test and Mann-Whitney U test revealed that gender, age, duration of hearing loss, and early combined use of batroxobin were significantly associated with the therapeutic response rate of severe and profound SSNHL in children（P<0.05）. No statistically significant differences in therapeutic response rate were observed among groups with affected side, tinnitus, vertigo, degree of hearing loss, or upper respiratory tract infection（P>0.05）. Univariate logistic regression analysis indicated that age, gender, duration of hearing loss, and early combined use of batroxobin were influencing factors for the therapeutic efficacy of severe and profound SSNHL in children. Multivariate logistic regression analysis further demonstrated that duration of hearing loss was negatively correlated with the response rate, whereas female gender, older age, and early combined use of batroxobin were positively correlated with the response rate. Grouped stacked bar charts showed that the response rate was higher in females, and higher in patients with early batroxobin administration. Grouped box plots indicated that a shorter duration of hearing loss was associated with a more concentrated distribution of response rates, and older age was also correlated with a more concentrated distribution of response rates. Conclusion:Gender, age, early combined use of batroxobin, and duration of hearing loss are independent influencing factors for the short-term treatment of severe and profound SSNHL in children.
2026-03-24 | Apolipoprotein AI is a protective factor for idiopathic sudden sensorineural hearing loss: a two-sample mendelian randomisation study.
Observational studies have suggested an association between cardiometabolic factors and idiopathic sudden sensorineural hearing loss (ISSNHL), but the causal relationship remains unclear. We aimed to assess the causal effect of cardiometabolic factors on the risk of ISSNHL using a two-sample Mendelian randomisation (MR) approach. Two-sample MR study. We used summary-level data from genome-wide association studies (GWAS). Genetic instruments for cardiometabolic factors were obtained from GWAS. Summary statistics for ISSNHL were derived from a publicly available GWAS database. Genetically predicted higher ApoAI levels were significantly associated with an decreased risk of ISSNHL (IVW OR = 0.82, 95% CI: 0.68-0.98, P = 0.03). No evidence of directional pleiotropy was detected in MR-Egger regression (P = 0.30), and Cochran's Q test indicated no significant heterogeneity (P = 0.94). MR-PRESSO detected no outliers. This study provides evidence supporting a potential causal relationship between higher ApoAI levels and decreased risk of ISSNHL. Further biological and clinical studies are warranted to elucidate the underlying mechanisms.
2023-06-29 | Neurofilament Expression as a Biomarker of Post-COVID-19 Sudden Sensorineural Hearing Loss
Sudden sensorineural hearing loss (SSHL) affects a patient's quality of life and requires rapid treatment. The etiology is viral, vascular, and autoimmune, even though, in most cases, it remains idiopathic SSHL. Since 2019, several different complications have been identified following COVID-19 infection. The post-COVID-19 ENT manifestations reported in the literature are sore throat, headache, pharyngeal erythema, nasal obstruction, rhinorrhea, upper respiratory tract infection, and tonsil enlargement. Cases of SSHL, vestibular neuronitis, and audio-vestibular disorders (such as tinnitus, dizziness, and vertigo) have also been reported, albeit in a smaller percentage of patients. We reported our experience of a case of post-COVID-19 SSHL in the absence of any other type of post-COVID symptoms or brain and internal auditory canal magnetic resonance imaging and magnetic resonance angiography modifications. We aimed to identify a serological biomarker of sudden sensorineural hearing loss, and we also dosed and monitored the value of the serum neurofilament light (NfL). the best of our knowledge, this is the first report that associates SSHL and the serological increase in NfL as a potential biomarker of neuronal-disease-related damage.
2023-06-29 | Analysis of Clinical Efficacy and Influencing Factors of Nerve Growth Factor (NGF) Treatment for Sudden Sensorineural Hearing Loss.
Objective: This study aims to examine the clinical efficacy and prognostic factors associated with nerve growth factor (NGF) treatment for sudden sensorineural hearing loss (SSHL). Materials and methods: A retrospective analysis was conducted on the clinical data of 101 patients with moderate or more severe SSHL who underwent secondary treatment at Sun Yat-sen Memorial Hospital of Sun Yat-sen University between January 2019 and July 2020. Prior to treatment, all patients were assessed using Pure Tone Audiometry (PTA), auditory brainstem response, otoacoustic emission, temporal bone computed tomography, or inner ear magnetic resonance imaging. Fifty-seven patients received conventional systemic treatment and served as the control group, while 44 patients received NGF in conjunction with conventional systemic treatment, forming the experimental group. PTA results were compared between the two groups before treatment and at 1 week, 2 weeks, and 1 month post-treatment. Additionally, the impact of age, sex, affected side, hypertension, and other factors on patient prognosis was analyzed. Results: Both groups demonstrated significant PTA improvements following treatment, with a statistically significant difference (P < .05). The hearing recovery effective rate in the control group was 42.1%, while that of the experimental group reached 70.5%, with a statistically significant difference between the groups (P < .05). Most patients experienced notable hearing improvements 1 week after treatment, with some patients still showing progress 2 weeks post-treatment. Multifactor analysis revealed that hypertension and onset days were associated with treatment outcomes. Conclusion: Secondary treatment remains clinically significant for patients with SSHL who have not achieved a satisfactory response or show no clear improvement following initial treatment. The presence of hypertension and delayed treatment are negative factors related to treatment efficacy.
2021-09-09 | Effects of Insulin-Like Growth Factor (IGF-1) in Patients with Sensorineural Hearing Loss.
(1) To test the effect of local administration of insulin-like growth factor-1 (IGF-1) in patients with sensorineural hearing loss (SNHL). (2) To test the effect of local administration of IGF-1 in patients with ototoxicity. Forty patients with SNHL were included in the study. Their hearing thresholds at different frequencies (0.5, 1, 2, and 4 kHz) along with the average hearing threshold were noted. The patients were then randomly allocated to 2 groups and were treated with IGF-1 via one of the following routes: (1) intratympanic injection and (2) Gelfoam. Patients were followed-up at weekly intervals for 6 weeks but follow-up PTA was done at 3 weeks, 6 weeks, and 6 months only. Forty patients (25 male, 15 female) participated in the study. Their age ranged from 13 to 63 years, with a mean of 31.3 years. Nineteen (47.5%) patients exhibited some degree of recovery after 6 months of follow-up, while 21 (52.5%) did not exhibit any recovery. Fourteen (35%) patients showed slight recovery (SR), 1 (4%) patient showed marked recovery, and complete recovery was observed in 4 (10%) patients. Twelve of the 20 patients who underwent treatment using Gelfoam showed improvement in hearing (measured as a reduction in hearing threshold), while only 7 of the 20 patients who underwent intratympanic injection showed such improvement. Among adverse reactions, the most common was pain (88%) which typically did not last beyond 3 days. Other adverse reactions observed were dizziness (24%) and headache (20%). One patient suffered from acute suppurative otitis media (ASOM) and had a perforation in the tympanic membrane. However, this was treated successfully with medications. Intratympanic IGF-1 is a novel drug that has shown early promise in controlling and reversing SNHL.
antibodies
2023-11-27 | A Comparative Study Using Vestibular Mapping in Sudden Sensorineural Hearing Loss With and Without Vertigo.
To investigate the impairment patterns in peripheral vestibular organs in sudden sensorineural hearing loss (SSNHL) with and without vertigo. Retrospective study. Single tertiary medical center. Data from 165 SSNHL patients in a tertiary referral center from January 2017 to December 2022 were retrospectively analyzed. All patients underwent a video head impulse test, vestibular evoked myogenic potential test, and pure-tone audiometry. Hierarchical cluster analysis was performed to investigate vestibular impairment patterns. The prognosis of the hearing was determined using American Academy of Otolaryngology-Head and Neck Surgery recommendations. After excluding patients with vestibular schwannoma and Meniere's disease, 152 patients were included in this study. A total of 73 of 152 patients were categorized as SSNHL with vertigo (SSNHL_V) and showed an independent merge of the posterior semicircular canal (PSCC) in cluster analysis. A total of 79 of 152 patients were categorized as SSNHL without vertigo (SSNHL_N) and showed an independent merge of saccule in cluster analysis. The PSCC (56.2%) and saccule (20.3%) were the most frequently impaired vestibular organs in SSNHL_V and SSNHL_N, respectively. In terms of prognosis, 106 of 152 patients had partial/no recovery and showed an independent merge of the PSCC in cluster analysis. A total of 46 of 152 patients had a complete recovery and showed an independent merge of the saccule in cluster analysis. A tendency of isolated PSCC dysfunction was seen in SSNHL_V and partial/no recovery. A tendency of isolated saccular dysfunction was seen in SSNHL_N and complete recovery. Different treatments might be needed in SSNHL depending on the presence of vertigo.
2021-11-25 | Biologics for Immune-Mediated Sensorineural Hearing Loss.
Immune-mediated hearing losses include autoimmune inner ear disease, sudden sensorineural hearing loss, and Meniere's disease. Standard therapy for an acute decline in hearing is timely use of corticosteroids. Although 60% to 70% of patients are initially corticosteroid-responsive, that responsiveness is lost over time. In corticosteroid-resistant patients, increased expression of interleukin (IL)-1 is observed, and these patients may benefit from IL-1 inhibition. Autoinflammatory diseases are characterized by dysregulation of the innate immune response, clinically include sensorineural hearing loss, and benefit from IL-1 inhibition, thereby further establishing the relationship of IL-1 with immune-mediated sensorineural hearing loss.
2021-01-27 | Lipopolysaccharide decreases cochlear blood flow dose dependently in a guinea pig animal model via TNF signaling
ABSTRACT Objective To evaluate the effect of Lipopolysaccharide (LPS), a bacterial endotoxin on cochlear microcirculation, and its mode of action. Methods Twenty‐five Dunkin‐Hartley guinea pigs were divided into five groups of five animals each. After surgical preparation, cochlear microcirculation was quantified by in vivo fluorescence microscopy. Placebo or LPS (1 mg, 10 µg, and 100 ng) was applied topically, and microcirculation was measured before and twice after application. A fifth group was pretreated with etanercept, a tumor necrosis factor (TNF) antagonist, and afterward the lowest LPS concentrations that yielded significant results (10 µg) were applied. Results In the groups that had been treated with 1 mg and 10 µg LPS, a significant drop in cochlear microcirculation was observed after 30 (.791 ± .089 Arbitrary Units (AU), compared to baseline, and .888 ± .071AU) and 60 (.756 ± .101 AU and .817 ± .124 AU, respectively) minutes. The groups that had been treated with 100 ng LPS and that had been pretreated with etanercept showed no significant change in cochlear blood flow compared to placebo. Conclusion Lipopolysaccharide shows a dose‐dependent effect on cochlear microcirculation; this effect can already be observed after 30 min. Pretreatment with etanercept can abrogate this effect, indicating that TNF mediates the effect of LPS on cochlear microcirculation.
2020-02-10 | Intracochlear Perfusion of Tumor Necrosis Factor-Alpha Induces Sensorineural Hearing Loss and Synaptic Degeneration in Guinea Pigs
Tumor necrosis factor-alpha (TNF-alpha) is a proinflammatory cytokine that plays a prominent role in the nervous system, mediating a range of physiologic and pathologic functions. In the auditory system, elevated levels of TNF-alpha have been implicated in several types of sensorineural hearing loss, including sensorineural hearing loss induced by vestibular schwannoma, a potentially fatal intracranial tumor that originates from the eighth cranial nerve; however, the mechanisms underlying the tumor's deleterious effects on hearing are not well understood. Here, we investigated the effect of acute elevations of TNF-alpha in the inner ear on cochlear function and morphology by perfusing the cochlea with TNF-alpha in vivo in guinea pigs. TNF-alpha perfusion did not significantly change thresholds for compound action potential (CAP) responses, which reflect cochlear nerve activity, or distortion product otoacoustic emissions, which reflect outer hair cell integrity. However, intracochlear TNF- alpha perfusion reduced CAP amplitudes and increased the number of inner hair cell synapses without paired post-synaptic terminals, suggesting a pattern of synaptic degeneration that resembles that observed in primary cochlear neuropathy. Additionally, etanercept, a TNF-alpha-blocker, protected against TNF-alpha-induced synaptopathy when administered systemically prior to intracochlear TNF-alpha perfusion. Findings motivate further investigation into the deleterious effects of chronically elevated intracochlear levels of TNF-alpha, and the potential for etanercept to counter these effects.
2018-02-17 | The Role of Tumor Necrosis Factor Alpha (TNFα) in Hearing Loss and Vestibular Schwannomas
The aim of this review is to highlight relevant literature on the role of tumor necrosis factor alpha (TNFα) in sensorineural hearing loss (SNHL) and vestibular schwannomas (VS). A comprehensive review of publically available databases including PubMed was performed. The mechanism by which hearing loss occurs in VS is still unknown and likely multifactorial. Genetic differences between VSs and tumor-secreted proteins may be responsible, at least in part, for VS-associated SNHL. TNFα has pleotropic roles in promoting inflammation, maintaining cellular homeostasis, inducing apoptosis, and mediating ototoxicity in patients with sporadic VS. TNFα-targeted therapies have shown efficacy in both animal models of sensorineural hearing loss and clinical trials in patients with immune-mediated hearing loss. Efforts are underway to develop nanotechnology-based methods to target TNFα and other pathogenic molecules in VS. Development of molecularly targeted therapies against TNFα represents an important area of research in ameliorating VS-associated hearing loss.
cell therapies
2026-07-01 | Intratympanic injection of human umbilical cord mesenchymal stem cell derived small extracellular vesicles for refractory sudden sensorineural hearing loss: a Phase 1 trial.
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).
2026-03-26 | Clinical Outcomes of Platelet-Rich Plasma in Otology: A Systematic Review of Tympanoplasty, Myringoplasty, and Hearing Loss Management.
Platelet-rich plasma (PRP), an autologous concentrate of platelets and growth factors, has been explored as an adjunct in otologic procedures, including tympanoplasty, myringoplasty, and treatment of sudden sensorineural hearing loss (SNHL). This systematic review evaluated reported clinical outcomes associated with PRP in these settings. A literature search was conducted in March 2025 across PubMed, Scopus, Web of Science, EMBASE, and the Cochrane Library. Eligible studies were English-language human studies (2015-2025) with original clinical outcome data from randomized or comparative observational designs. Due to methodological heterogeneity, findings were synthesized narratively. Six studies (n = 349 patients) were included: five addressing tympanic membrane repair and one addressing idiopathic sudden SNHL. In myringoplasty/tympanoplasty-related studies, PRP was generally associated with higher graft uptake and/or faster healing than comparator approaches; where reported, closure outcomes included 85.7% versus 60% in one comparative study, and several studies reported between-group differences with p < 0.05. Reported postoperative complications were low, and no major PRP-related safety concerns were identified in the included studies. In SNHL, one comparative study suggested hearing improvement with intratympanic PRP that was comparable to, or greater than, steroid therapy, but evidence remains limited to short-term follow-up and a small number of studies. Current evidence supports PRP as a feasible and apparently safe adjunct in otologic practice, particularly for tympanic membrane repair. However, certainty regarding the magnitude of benefit and long-term auditory outcomes remains limited by small sample sizes, heterogeneity in PRP preparation/application, and variability in study design. Standardized protocols and larger, well-designed randomized trials are needed.
2026-03-01 | Role of platelet-rich plasma in management of sensorineural hearing loss diseases.
Sensorineural deafness is a form of loss of hearing. It happens from damage to the cochlea. Platelet-rich plasma (PRP) is an autologous management derived from a case's blood and was observed to have pro-nerve regenerative characteristics. Intratympanic injection of PRP improves the cells of the hair of the inner ear, therefore enhancing the hearing. To show the effectiveness and safety of PRP in the management of sensorineural hearing loss (SNHL) diseases. 150 patients who had sensorineural hearing loss because of different otological diseases, such as acoustic trauma, presbycusis, idiopathic sudden SNHL, in addition to Meniere's disease (MD). All cases received 4 times of intratympanic injections of PRP with one-week intervals between them. In our research, 150 cases with SNHL diseases, as acoustic trauma, MD, presbycusis, and idiopathic sudden sensorineural hearing loss (ISSNHL) (68 (46.3%) males and 82 (54.6%) females) with age ranges from 18 to 78 years, received intratympanic injections of PRP. 50 patients with ISSNHL: 39 of them showed hearing improvement, with an average of 28 dB in pure tone audiometry (PTA) and 26% in speech discrimination. In acoustic trauma, 18 patients, 12 of them, showed hearing improvement, averaging 30 dB in PTA and 28% in speech discrimination. In presbycusis, 58 patients, 42 of them, showed an improvement in hearing of an average of 25 dB and 24% in speech discrimination. In Meniere's disease, 24 patients, 16 of them, showed an improvement in hearing of an average of 15 dB and 5% in speech discrimination. Platelet-rich plasma appears effective and safe for the management of sensorineural hearing loss with no systemic side effects as an oral steroid and at low cost.
2026-02-16 | Sudden Unilateral Hearing Loss and Vertigo in an Adolescent: Viral Labyrinthitis Versus Idiopathic Sudden Sensorineural Hearing Loss.
Labyrinthitis and idiopathic sudden sensorineural hearing loss (SSNHL) are uncommon in children and are both considered otologic emergencies, as delayed diagnosis can lead to permanent disability. We describe a 15-year-old boy with a history of focal cortical dysplasia and a single remote seizure who presented with abrupt left-sided hearing loss, tinnitus, vertigo, nausea, and headache. Neurologic examination revealed no focal deficits; however, marching Romberg testing demonstrated instability, and otoscopy revealed only a small left middle-ear effusion. Laboratory studies, including complete blood count and C-reactive protein, were unremarkable. Non-contrast computed tomography (CT) of the head showed no acute intracranial abnormality. Magnetic resonance imaging (MRI) of the brain and internal auditory canals with and without contrast demonstrated normal labyrinthine structures and stable right frontal cortical dysplasia. The working diagnosis, established by pediatric neurology and otolaryngology, was acute viral labyrinthitis or idiopathic SSNHL with vestibular involvement. The patient received high-dose systemic corticosteroids, vestibular suppressants, antiemetics, and empiric oral acyclovir. Vertigo and nausea improved substantially, but profound left-sided hearing loss persisted at discharge. Outpatient audiometry and possible intratympanic steroid therapy were planned. This case emphasizes the diagnostic overlap between labyrinthitis and SSNHL in adolescents, the limited role of routine head CT for peripheral vestibulocochlear presentations, and the importance of early guideline-directed corticosteroid therapy. It also illustrates that MRI may be normal in clinically significant labyrinthitis and that careful follow-up is required to determine the need for salvage intratympanic steroid treatment.
2025-05-09 | Intratympanic steroid via saturated gelfoam vs. intratympanic injection for sudden sensorineural hearing loss.
The aim of this study was to investigate the efficacy of a novel intratympanic steroid (ITS) therapy via saturated Gelfoam® (GSD) in sudden sensorineural hearing loss (SSNHL) by comparing with ITS injection therapy. The patients with SSNHL who received initial ITS therapy with no prior systemic steroid treatment were divided into two groups: the ITS via injection group (Group 1) and the ITS via GSD group (Group 2). The groups were compared in terms of the patients' demographic data, side of ear involvement, vertigo and tinnitus symptoms, comorbidities, initiation time of therapy, hearing level (HL) before and after therapy, word recognition score (WRS) before and after therapy, hearing gain (HG), WRS gain (WRSG), recovery rate (RR), tolerability of the applications and complications. HL and WRS scores were similar among the groups before therapy. Regarding the hearing outcomes after therapy HG and WRSG between the groups were not significant. In the comparison of RR, Group 2's score (70 %) was significantly higher than that of Group 1 (42.4 %) (p = 0.028). In terms of the tolerability of applications, ITS via GSD application was significantly more tolerable than ITS via injection (p = 0.046). Regarding complications, no otorrhea was observed in any patient, and tympanic membrane perforation was observed in 1 patient (3.33 %) in Group 2, whereas no perforation was observed in Group 1. Intratympanic GSD is a simple, effective and tolerable technique in the therapy of SSNHL, with success rates similar to those of ITS injection therapy and significant improvements in the RR scores.
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2026-08-06 | Acupuncture-related therapy for sudden sensorineural hearing loss: clinical efficacy and central mechanistic insights
Sudden sensorineural hearing loss (SSNHL) is a common otolaryngological emergency, but its pathogenesis remains incompletely understood. Although acupuncture has recently become widely used as a complementary therapy for SSNHL in clinical practice, a comprehensive review of its central mechanisms is still lacking. Based on literature from 2020 to 2025 in databases such as PubMed, Web of Science, Embase, China National Knowledge Infrastructure (CNKI) and Wanfang Data (Wanfang), this narrative review synthesizes the latest research into the central mechanisms of acupuncture-related treatment for SSNHL, focusing on three key areas: auditory pathway plasticity, activation of the emotional regulation network, and functional integration of neurovascular units. Preliminary evidence suggests that acupuncture-related therapy may promote hearing recovery and alleviate associated symptoms via multiple target pathways. The proposed central mechanisms include modulation of functional connectivity in the temporal auditory cortex, regulation of limbic–subcortical emotional pathways, and enhancement of neurovascular coupling. This review provides a theoretical basis for a deeper understanding of the potential roles of acupuncture-related treatment in SSNHL, although further rigorous studies are needed to confirm these mechanistic links.
2024-12-20 | Treatment of sudden sensorineural hearing loss with tinnitus with Fu subcutaneous needling: A case report.
Fu's subcutaneous needling (FSN) is a special acupuncture method that uses FSN to sweep the subcutaneous tissue around or adjacent to the limbs to disperse the pain. Sudden sensorineural hearing loss (SSHL) is a kind of otological emergency with sudden onset within 72 hours, with unilateral hearing loss, and hearing loss of ≥20 dB in 2 connected frequencies, and most of the patients have no obvious triggers. Most patients with SSHL are accompanied by tinnitus, vertigo, and nausea and vomiting. The patient suffered from hearing loss with tinnitus. Although received relevant treatment, the hearing loss and tinnitus did not improve significantly. SSHL with tinnitus. FSN treatment. A point 5 cm lateral to the point of tension and stiffness of the left trapezius muscle was selected on the affected back as the needle insertion point, and the condition of the neck muscles was improved through the FSN sweeping movement with neck reperfusion activities. The patient received treatment twice a week, and 4 weeks of treatment as a phase. At the end of each phase, a pure tone threshold audiometry, tinnitus evaluation scale Tinnitus Handicap Inventory assessment, and neck muscle palpation were performed. The patient's hearing improved and the Tinnitus Handicap Inventory score decreased. FSN has a good therapeutic effect on SSHL with tinnitus.
2024-01-17 | NcRNA: key and potential in hearing loss
Hearing loss has an extremely high prevalence worldwide and brings incredible economic and social burdens. Mechanisms such as epigenetics are profoundly involved in the initiation and progression of hearing loss and potentially yield definite strategies for hearing loss treatment. Non-coding genes occupy 97% of the human genome, and their transcripts, non-coding RNAs (ncRNAs), are widely participated in regulating various physiological and pathological situations. NcRNAs, mainly including micro-RNAs (miRNAs), long-stranded non-coding RNAs (lncRNAs), and circular RNAs (circRNAs), are involved in the regulation of cell metabolism and cell death by modulating gene expression and protein-protein interactions, thus impacting the occurrence and prognosis of hearing loss. This review provides a detailed overview of ncRNAs, especially miRNAs and lncRNAs, in the pathogenesis of hearing loss. We also discuss the shortcomings and issues that need to be addressed in the study of hearing loss ncRNAs in the hope of providing viable therapeutic strategies for the precise treatment of hearing loss.
2023-06-15 | Single-cell sequencing: a powerful technique to understand the pathophysiology of auditory disorders
Deciphering the inner ear transcriptome has allowed major breakthroughs in therapeutic development for auditory disorders. Single-cell sequencing (SCS) technologies, through the analysis of cellular heterogeneity and the identification of novel gene markers, can significantly enhance our understanding about auditory disorders. The application of SCS has allowed the identification of gene targets specific to the inner ear cellular subtypes such as supporting cells, hair cells, stria vascularis cells, and spiral ganglion neuron cells. SCS has provided new insights into the molecular mechanisms underlying the pathophysiology of auditory disorders such as noise-trauma induced hearing loss, ototoxicity, sudden sensorineural hearing loss (SSNHL), vestibular schwannomas and acute otitis media, leading to the discovery of novel therapeutic targets. The objective of this perspective article is to discuss recent advancements regarding the utilization of SCS for inner ear research. With global initiatives striving to overcome the increasing burden of hearing loss, the SCS technique holds a great potential in revolutionizing the management of auditory disorders, paving the way to develop effective treatment modalities.
2022-09-02 | Role of microRNAs as novel diagnostic biomarkers and potential therapeutic targets for hearing disorders (Review)
The ear is a complex structure consisting of the outer, middle and inner ear. For the proper function of the auditory system, the development of all these parts needs to be highly organized. Any alterations in this chain of events can lead to hearing loss or other impairments, such as dizziness, tinnitus and loss of balance. Of note, several external and internal elements, including noise exposure, ototoxic drugs, aging, as well as environmental or genetic factors, have been widely associated with hearing loss. Over the years, a growing body of evidence has indicated that microRNAs (miRNAs/miRs) play a critical role in ear development and auditory function, suggesting that an altered pattern of miRNA expression may be involved in the progression of these pathological conditions. On these bases, the present review article aimed to summarize the role of miRNAs in inner ear development and hearing disorders. Although further studies are warranted to provide more in‑depth knowledge of the implicated mechanisms of action, according to the current data, miRNAs may be used as novel diagnostic biomarkers for the early detection of hearing loss. In addition, miRNAs and related signaling pathways may represent novel potential therapeutic targets for the treatment of hearing loss, ear inflammation and vestibular schwannoma.
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Drug Discovery Landscape
7 orphan drug designations for Sudden sensorineural hearing loss.
7 orphan drug designations for Sudden sensorineural hearing loss.
Drug | Therapy type | Orphan designation | Clinical trial status | Sponsor |
|---|---|---|---|---|
6-fluoro-9-methyl-9H-pyrido[3,4-b]-indole | small molecules | FDA | AudioCure Pharma GmbH | |
pioglitazone | small molecules | FDA | Strekin AG | |
6-fluoro-9-methyl-9H-pyrido[3,4-b]-indole | small molecules | EMA | AudioCure Pharma GmbH | |
Pioglitazone hydrochloride | small molecules | EMA | Regiomedica GmbH | |
R-azasetron besylate | small molecules | EMA | Sensorion | |
(UDU-stereoisomer of c-UJUun UNU-terminal UkUnhibitor) | small molecules | FDA | Altamira Therapeutics Inc. | |
H-D-Asp-D-Gln-D-Ser-D-Arg-D-Pro-D-Val-D-Gln-D-Pro-D-Phe-D-Leu-D-Asn-D-Leu-D-Thr-D-Thr-D-Pro-D-Arg-D-Lys-D-Pro-D-Arg-D-Pro-D-Pro-D-Arg-D-Arg-D-Arg-D-Gln-D-Arg-D-Arg-D-Lys-D-Lys-D-Arg-D-Gly-NH2 | peptides | EMA | Auris Medical Limited |
“Terminated” – programs with discontinued clinical trials, or with no updates reported for 5 years after completion or 12 years after the last trial started.
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