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
1,659 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:
1,659 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:
2026-08-12 | Efficacy of Intratympanic versus Systemic Corticosteroids in Sudden Sensorineural Hearing Loss: A Systematic Review.
Sudden sensorineural hearing loss (SSNHL) is an otologic emergency that typically requires prompt treatment with corticosteroids, administered either systemically or intratympanically. The optimal route for initial therapy remains uncertain due to heterogeneity in efficacy and safety profiles. We conducted a systematic review of randomized controlled trials (RCTs) published between 2011 and 2025, comparing intratympanic and systemic corticosteroids as primary treatment for SSNHL in adults. Following PRISMA 2020 and Cochrane Handbook guidelines, we screened studies without language restrictions. Two independent reviewers performed study selection, data extraction, and risk of bias assessment using the RoB 2.0 tool. Outcomes were synthesized narratively, focusing on changes in pure-tone average (PTA), complete/functional recovery, speech discrimination, and safety. Trials with combination/salvage strategies, adjuncts, or formulation comparisons were synthesized separately. Seventeen RCTs were included. Efficacy results were heterogeneous; however, most studies indicated that intratympanic corticosteroids are at least as effective as systemic therapy. In severe hearing loss, intratympanic treatment achieved higher success rates (up to 70.6%) and greater PTA improvement compared to systemic administration. Adverse events differed by route: systemic corticosteroids were associated with metabolic disturbances, while intratympanic therapy caused localized, self-limiting events. Overall, 47.1% of studies had high risk of bias, and 52.9% showed some concerns. Intratympanic corticosteroids appear to be a non-inferior alternative to systemic steroids for initial SSNHL treatment, with potential advantages in cases of severe hearing loss or systemic contraindications. Nonetheless, methodological limitations across studies warrant cautious interpretation. Future high-quality trials should address efficacy, functional recovery, and safety more rigorously.
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.
2026-08-06 | Hyperhomocysteinemia in sudden sensorineural hearing loss: Evidence from a prospective clinical study.
To compare baseline and post-treatment homocysteine levels in patients with sudden sensorineural hearing loss (SSNHL) and to evaluate the potential role of homocysteine in the pathogenesis of the disease as well as its association with hearing recovery. A total of 40 patients diagnosed with SSNHL and 40 age- and sex-matched healthy controls were included in this prospective observational study. Serum homocysteine levels were measured at baseline and at the 4-6-week follow-up visit after treatment in the SSNHL group, and once at enrollment in the control group. Hearing thresholds were assessed using pure tone audiometry. Treatment response was classified according to Siegel criteria. Statistical analyses included group comparisons, correlation analyses, and multivariate logistic regression. Baseline homocysteine levels were significantly higher in the SSNHL group compared with controls. No significant change in homocysteine levels was observed after treatment, although hearing thresholds improved significantly. Homocysteine levels were not significantly associated with hearing recovery. In multivariate logistic regression analysis, baseline homocysteine was independently associated with SSNHL (OR = 1.742, 95% CI: 1.351-2.245, p < 0.001). ROC curve analysis demonstrated good discriminative ability for baseline homocysteine in distinguishing SSNHL patients from controls (AUC = 0.809, 95% CI: 0.712-0.905, p < 0.001). Homocysteine may play a role in the pathophysiology of SSNHL as a potential vascular risk marker. However, the lack of significant post-treatment reduction and its absence of association with hearing recovery suggest that it may function more as a predisposing factor rather than a prognostic biomarker. Further large-scale prospective studies are needed to evaluate the potential therapeutic implications of homocysteine-lowering strategies in SSNHL.
2026-08-03 | Differentiating hidden meniere's disease from typical sudden sensorineural hearing loss: clinical significance of acute low-tone hearing loss.
This study aimed to elucidate the clinical heterogeneity of sudden sensorineural hearing loss (SSNHL) by analyzing audiometric profiles over a 12-year period. Specifically, the study sought to identify a subgroup with acute low-tone hearing loss (ALHL) as a potential manifestation of hidden Meniere's disease (MD) and to differentiate its pathophysiological characteristics from typical SSNHL. A retrospective review was conducted on 620 patients treated for SSNHL at a tertiary referral center between 2004 and 2016. Patients were stratified into an ALHL group (n = 80), suspected of hidden MD, and a typical SSNHL group (n = 540) based on frequency-specific audiometric configurations. Clinical characteristics, hearing recovery outcomes, and the prevalence of vertigo and metabolic comorbidities (diabetes and hypertension) were compared between groups. The ALHL group exhibited significantly milder initial low-tone hearing loss (50.5 ± 15.1 dBHL) and superior recovery (27.9 ± 17.2 dBHL) compared with the severe pan-frequency loss in typical SSNHL (initial: 83.0 ± 23.6 dBHL; post-treatment: 48.7 ± 27.9 dBHL). A marked divergence in comorbidities was observed. The ALHL group demonstrated a high prevalence of vertigo (87.5%) but significantly lower metabolic risk (diabetes, 12.5%; hypertension, 28.8%). Conversely, typical SSNHL displayed higher metabolic risk (diabetes, 26.5%; hypertension, 41.1%) with less frequent vertigo (29.1%). Sudden hearing loss associated with MD represents a distinct clinical entity within SSNHL: low-frequency impairment, high vertigo prevalence, and low risk of metabolic diseases. These findings suggest a hydropic etiology for this ALHL pattern, distinct from the viral or vascular causes of typical SSNHL. Therefore, clinical differentiation is imperative to implement targeted therapeutic strategies addressing endolymphatic hydrops in patients with ALHL in MD within SSNHL.
2026-08-03 | Clinical effectiveness of salvage intratympanic corticosteroids for idiopathic sudden sensorineural hearing loss: a real-world observational series.
To describe real-world hearing outcomes after salvage intratympanic corticosteroid (ITC) therapy in patients with idiopathic sudden sensorineural hearing loss (ISSNHL) and incomplete recovery after systemic corticosteroids, emphasizing clinically meaningful recovery. Prospective observational study at a single tertiary center. Patients met ISSNHL diagnostic criteria (≥ 30 dB loss in ≥ 3 consecutive frequencies within 72 h) and received salvage ITC (methylprednisolone 40 mg/mL, weekly for 3 weeks) after absent or incomplete recovery on follow-up audiometry (day 10-15). Primary outcome was PTA4 change from pre-salvage audiogram to last available audiogram (minimum follow-up 1 month). Recovery was categorized using Siegel criteria. Non-parametric methods and Hodges-Lehmann estimators were used. Of 83 ISSNHL patients, 41 received salvage ITC and were analyzed. In the salvage cohort, 63.4% were women; mean age was 53.3 years (95% CI 46.7-59.9); median follow-up was 11 months (IQR 3-15). Median PTA gain attributed to ITC rescue was 5.0 dB (IQR - 3.8 to 18.8; padj = 0.043). After salvage ITC, 71% showed ≤ 15 dB improvement; 29% achieved ≥ 15 dB. By Siegel criteria, normal hearing was achieved in 7%, mild-to-moderate loss in 34%, and severe loss in 59%. Salvage ITC was associated with a statistically significant but clinically modest hearing gain and a low proportion of complete recovery. Standardized, patient-relevant outcome definitions are needed to support realistic counseling.
2026-08-12 | Efficacy of Intratympanic versus Systemic Corticosteroids in Sudden Sensorineural Hearing Loss: A Systematic Review.
Sudden sensorineural hearing loss (SSNHL) is an otologic emergency that typically requires prompt treatment with corticosteroids, administered either systemically or intratympanically. The optimal route for initial therapy remains uncertain due to heterogeneity in efficacy and safety profiles. We conducted a systematic review of randomized controlled trials (RCTs) published between 2011 and 2025, comparing intratympanic and systemic corticosteroids as primary treatment for SSNHL in adults. Following PRISMA 2020 and Cochrane Handbook guidelines, we screened studies without language restrictions. Two independent reviewers performed study selection, data extraction, and risk of bias assessment using the RoB 2.0 tool. Outcomes were synthesized narratively, focusing on changes in pure-tone average (PTA), complete/functional recovery, speech discrimination, and safety. Trials with combination/salvage strategies, adjuncts, or formulation comparisons were synthesized separately. Seventeen RCTs were included. Efficacy results were heterogeneous; however, most studies indicated that intratympanic corticosteroids are at least as effective as systemic therapy. In severe hearing loss, intratympanic treatment achieved higher success rates (up to 70.6%) and greater PTA improvement compared to systemic administration. Adverse events differed by route: systemic corticosteroids were associated with metabolic disturbances, while intratympanic therapy caused localized, self-limiting events. Overall, 47.1% of studies had high risk of bias, and 52.9% showed some concerns. Intratympanic corticosteroids appear to be a non-inferior alternative to systemic steroids for initial SSNHL treatment, with potential advantages in cases of severe hearing loss or systemic contraindications. Nonetheless, methodological limitations across studies warrant cautious interpretation. Future high-quality trials should address efficacy, functional recovery, and safety more rigorously.
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.
2026-08-06 | Hyperhomocysteinemia in sudden sensorineural hearing loss: Evidence from a prospective clinical study.
To compare baseline and post-treatment homocysteine levels in patients with sudden sensorineural hearing loss (SSNHL) and to evaluate the potential role of homocysteine in the pathogenesis of the disease as well as its association with hearing recovery. A total of 40 patients diagnosed with SSNHL and 40 age- and sex-matched healthy controls were included in this prospective observational study. Serum homocysteine levels were measured at baseline and at the 4-6-week follow-up visit after treatment in the SSNHL group, and once at enrollment in the control group. Hearing thresholds were assessed using pure tone audiometry. Treatment response was classified according to Siegel criteria. Statistical analyses included group comparisons, correlation analyses, and multivariate logistic regression. Baseline homocysteine levels were significantly higher in the SSNHL group compared with controls. No significant change in homocysteine levels was observed after treatment, although hearing thresholds improved significantly. Homocysteine levels were not significantly associated with hearing recovery. In multivariate logistic regression analysis, baseline homocysteine was independently associated with SSNHL (OR = 1.742, 95% CI: 1.351-2.245, p < 0.001). ROC curve analysis demonstrated good discriminative ability for baseline homocysteine in distinguishing SSNHL patients from controls (AUC = 0.809, 95% CI: 0.712-0.905, p < 0.001). Homocysteine may play a role in the pathophysiology of SSNHL as a potential vascular risk marker. However, the lack of significant post-treatment reduction and its absence of association with hearing recovery suggest that it may function more as a predisposing factor rather than a prognostic biomarker. Further large-scale prospective studies are needed to evaluate the potential therapeutic implications of homocysteine-lowering strategies in SSNHL.
2026-08-03 | Differentiating hidden meniere's disease from typical sudden sensorineural hearing loss: clinical significance of acute low-tone hearing loss.
This study aimed to elucidate the clinical heterogeneity of sudden sensorineural hearing loss (SSNHL) by analyzing audiometric profiles over a 12-year period. Specifically, the study sought to identify a subgroup with acute low-tone hearing loss (ALHL) as a potential manifestation of hidden Meniere's disease (MD) and to differentiate its pathophysiological characteristics from typical SSNHL. A retrospective review was conducted on 620 patients treated for SSNHL at a tertiary referral center between 2004 and 2016. Patients were stratified into an ALHL group (n = 80), suspected of hidden MD, and a typical SSNHL group (n = 540) based on frequency-specific audiometric configurations. Clinical characteristics, hearing recovery outcomes, and the prevalence of vertigo and metabolic comorbidities (diabetes and hypertension) were compared between groups. The ALHL group exhibited significantly milder initial low-tone hearing loss (50.5 ± 15.1 dBHL) and superior recovery (27.9 ± 17.2 dBHL) compared with the severe pan-frequency loss in typical SSNHL (initial: 83.0 ± 23.6 dBHL; post-treatment: 48.7 ± 27.9 dBHL). A marked divergence in comorbidities was observed. The ALHL group demonstrated a high prevalence of vertigo (87.5%) but significantly lower metabolic risk (diabetes, 12.5%; hypertension, 28.8%). Conversely, typical SSNHL displayed higher metabolic risk (diabetes, 26.5%; hypertension, 41.1%) with less frequent vertigo (29.1%). Sudden hearing loss associated with MD represents a distinct clinical entity within SSNHL: low-frequency impairment, high vertigo prevalence, and low risk of metabolic diseases. These findings suggest a hydropic etiology for this ALHL pattern, distinct from the viral or vascular causes of typical SSNHL. Therefore, clinical differentiation is imperative to implement targeted therapeutic strategies addressing endolymphatic hydrops in patients with ALHL in MD within SSNHL.
2026-08-03 | Clinical effectiveness of salvage intratympanic corticosteroids for idiopathic sudden sensorineural hearing loss: a real-world observational series.
To describe real-world hearing outcomes after salvage intratympanic corticosteroid (ITC) therapy in patients with idiopathic sudden sensorineural hearing loss (ISSNHL) and incomplete recovery after systemic corticosteroids, emphasizing clinically meaningful recovery. Prospective observational study at a single tertiary center. Patients met ISSNHL diagnostic criteria (≥ 30 dB loss in ≥ 3 consecutive frequencies within 72 h) and received salvage ITC (methylprednisolone 40 mg/mL, weekly for 3 weeks) after absent or incomplete recovery on follow-up audiometry (day 10-15). Primary outcome was PTA4 change from pre-salvage audiogram to last available audiogram (minimum follow-up 1 month). Recovery was categorized using Siegel criteria. Non-parametric methods and Hodges-Lehmann estimators were used. Of 83 ISSNHL patients, 41 received salvage ITC and were analyzed. In the salvage cohort, 63.4% were women; mean age was 53.3 years (95% CI 46.7-59.9); median follow-up was 11 months (IQR 3-15). Median PTA gain attributed to ITC rescue was 5.0 dB (IQR - 3.8 to 18.8; padj = 0.043). After salvage ITC, 71% showed ≤ 15 dB improvement; 29% achieved ≥ 15 dB. By Siegel criteria, normal hearing was achieved in 7%, mild-to-moderate loss in 34%, and severe loss in 59%. Salvage ITC was associated with a statistically significant but clinically modest hearing gain and a low proportion of complete recovery. Standardized, patient-relevant outcome definitions are needed to support realistic counseling.
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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 | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
6-fluoro-9-methyl-9H-pyrido[3,4-b]-indole | small molecules | FDA | 2021-05-10 | — | AudioCure Pharma GmbH |
pioglitazone | small molecules | FDA | 2019-12-12 | — | Strekin AG |
6-fluoro-9-methyl-9H-pyrido[3,4-b]-indole | small molecules | EMA | 2018-12-14 | — | AudioCure Pharma GmbH |
Pioglitazone hydrochloride | small molecules | EMA | 2017-01-12 | — | Regiomedica GmbH |
R-azasetron besylate | small molecules | EMA | 2016-11-18 | — | Sensorion |
(UDU-stereoisomer of c-UJUun UNU-terminal UkUnhibitor) | small molecules | FDA | 2006-03-28 | — | 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 | 2005-06-16 | — | Auris Medical Limited |
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