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ORPHA:90059

Sudden sensorineural hearing loss

high confidenceDisorder

Also known as: Secondary acute sensorineural hearing loss

Publications

4,559

92.4th percentile

Trials

32

Interventional, condition-specific

Researchers

877

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare otorhinolaryngologic condition characterized by sudden hearing loss of at least 30 decibels across three contiguous frequencies, within 72 hours or less, due to acute damage to the cochlear hair cells, resulting from acoustic trauma or mechanical trauma during surgery, or occurring without any known cause. The hearing loss may affect one or both ears. Typical concomitant symptoms are aural fullness, tinnitus, and vertigo. Patients may fully recover or remain with hearing loss of variable intensity.

How rare: 1-5 / 10 000 — about one to five people per ten thousand (still uncommon, but less ultra-rare).

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (1)

sudden sensorineural hearing loss

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

3/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    4,559 matched papers (3,117 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationPresent

    1 FDA · 3 EMA designations (1 FDA orphan-indication approval) — e.g. pioglitazone Source

  6. Interventional trialPresent

    32 matched on ClinicalTrials.gov (13 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

4

Designations · 1 with FDA orphan-indication approval

  • FDA pioglitazoneSudden sensorineural hearing loss · 2019-12-12 · Not FDA Approved for Orphan Indication
  • EMA R-azasetron besilateTreatment of sudden sensorineural hearing loss · 18/11/2016 · PositiveEMA designation
  • EMA 6-fluoro-9-methyl-9H-pyrido[3,4-b]-indoleTreatment of sudden sensorineural hearing loss · 14/12/2018 · PositiveEMA designation
  • EMA pioglitazone hydrochlorideTreatment of sudden sensorineural hearing loss · 12/01/2017 · PositiveEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

20

Drugs / clinical candidates · MONDO_0043373

CTD chemicals (MyDisease.info)

13 associated chemicals · 8 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Methylprednisolone · therapeutic
  • Cocaine · marker/mechanism
  • Dantrolene · marker/mechanism
  • Lamivudine · marker/mechanism
  • Mefloquine · marker/mechanism
  • Methadone · marker/mechanism
  • Naproxen · marker/mechanism
  • Nevirapine · marker/mechanism
  • peginterferon alfa-2a · marker/mechanism
  • Ribavirin · marker/mechanism
  • Stavudine · marker/mechanism
  • Tacrolimus · marker/mechanism

Pathways: One carbon pool by folate; Metabolic pathways; Carbon metabolism; Antifolate resistance; Metabolism; Metabolism of folate and pterines; Metabolism of water-soluble vitamins and cofactors; Metabolism of vitamins and cofactors

MyDisease.info · MONDO:0043373

Literature

Is anyone studying this?

4,559

4,559 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

4,559 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

3,117 in the last 10 years · high confidence · 92.4th percentile (publications denominator)

Phrase hits: 4,553 · MeSH hits: 36

Open Europe PMC search

Who's working on it?

877

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Gao Y9 papers · 2026

    Senior Department of Otolaryngology Head and Neck Surgery, Sixth Medical Center of the PLA General Hospital, Beijing 100853, China.

    Papers in Europe PMC
  2. 02
    Wang Y8 papers · 2026

    Department of Otorhinolaryngology and Head and Neck Surgery, BenQ Medical Center, The Affiliated BenQ Hospital of Nanjing Medical University, Nanjing, Jiangsu, China.

    Papers in Europe PMC
  3. 03
    Zhang J8 papers · 2026

    Department of Otolaryngology-Head and Neck Surgery, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, China.

    Papers in Europe PMC
  4. 04
    Zhang Q8 papers · 2026

    Department of Radiology Department, BenQ Medical Center, The Affiliated BenQ Hospital of Nanjing Medical University, JiangSu, China.

    Papers in Europe PMC
  5. 05
    Li H7 papers · 2026

    Department of Ophthalmology, Shanghai Sixth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. lky0110@126.com.

    Papers in Europe PMC
  6. 06
    Li J7 papers · 2026

    Departmemt of Otorhinolaryngology, The Second Hospital of Dalian Medical University, Dalian, China.

    Papers in Europe PMC
  7. 07
    Chen G5 papers · 2026

    Faculty of Chinese Medicine, Macau University of Science and Technology, Taipa, Macao SAR, China.

    Papers in Europe PMC
  8. 08
    Feng Y5 papers · 2026

    Department of Otolaryngology-Head and Neck Surgery, Shanghai Sixth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. ymfeng@sjtu.edu.cn.

    Papers in Europe PMC
  9. 09
    Fu Y5 papers · 2026

    Department of Otorhinolaryngology, The Quzhou Affiliated Hospital of Wenzhou Medical University, Quzhou People's Hospital, Quzhou, China.

    Papers in Europe PMC
  10. 10
    Wang J5 papers · 2026

    Department of Otolaryngology, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

32

interventional trials for this specific condition

32 interventional trials matched this specific condition name; 13 currently recruiting in our sample.

Data as of 11 September 2026

32 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 96.2th percentile).

high confidence · 96.2th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

32 interventional trials matched after quoted-phrase search and title/condition post-filter.

Observational and natural-history studies

11 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 9 · after dedupe 9 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 9 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (9)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Sudden sensorineural hearing loss — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Sudden sensorineural hearing loss" OR "Secondary acute sensorineural hearing loss"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Hearing Loss, Sudden

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Sudden sensorineural hearing loss" OR "Secondary acute sensorineural hearing loss" OR "Hearing Loss, Sudden"

Interventional trials matched via: both, phrase, mesh (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 32 interventional · 11 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T03:36:11.065Z