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ORPHA:252175

Vestibular schwannoma

high confidenceSubtype of disorder

Also known as: Acoustic neurilemoma · Acoustic neurinoma · Acoustic neuroma

Publications

20,415

96.7th percentile

Trials

66

Interventional, condition-specific

Researchers

1,070

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

Vestibular schwannoma is a rare tumor of the posterior fossa originating in the Schwann cells of the vestibular transitional zone of the vestibulocochlear nerve that can be benign, small, slow growing and asymptomatic or large, faster growing and aggressive and potentially fatal, presenting with symptoms of hearing and balance impairment, vertigo, , headache and fifth, sixth or seventh cranial nerve dysfunction and facial numbness.

How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (11)

acoustic neurilemmoma · acoustic neurilemoma · acoustic neurinoma · acoustic neuroma · acoustic schwannoma · neurilemoma, acoustic · neurinoma of the acoustic nerve · neuroma, acoustic · vestibular neurilemmoma · vestibular schwannoma · vestibular schwannoma (disease)

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.”

2/6 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

    20,415 matched papers (9,138 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 designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    66 matched on ClinicalTrials.gov (16 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)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

20,415

20,415 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

20,415 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).

9,138 in the last 10 years · high confidence · 96.7th percentile (publications denominator)

Phrase hits: 20,415 · MeSH hits: 56

Open Europe PMC search

Who's working on it?

1,070

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
    Carlson ML34 papers · 2026

    Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, USA.

    Papers in Europe PMC
  2. 02
    Link MJ24 papers · 2026

    Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, USA.

    Papers in Europe PMC
  3. 03
    Lohse CM24 papers · 2026

    Department of Quantitative Health Sciences, Mayo Clinic, Rochester, Minnesota, USA.

    Papers in Europe PMC
  4. 04
    Marinelli JP16 papers · 2026

    Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, USA.

    Papers in Europe PMC
  5. 05
    Khandalavala KR12 papers · 2026

    Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, USA.

    Papers in Europe PMC
  6. 06
    Tombers NM10 papers · 2026

    Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota.

    Papers in Europe PMC
  7. 07
    Saoji AA9 papers · 2026

    Departments of1Otolaryngology-Head and Neck Surgery.

    Papers in Europe PMC
  8. 08
    Yost KJ8 papers · 2026

    3Quantitative Health Sciences, Mayo Clinic, Rochester, Minnesota.

    Papers in Europe PMC
  9. 09
    Zalaquett NG8 papers · 2026

    Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, MN.

    Papers in Europe PMC
  10. 10
    Ahmed AK7 papers · 2026

    Department of Neurosurgery, Johns Hopkins University School of Medicine, United States.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

66

interventional trials for this specific condition

66 interventional trials matched this specific condition name; 16 currently recruiting in our sample. 6 trials are registered for schwannoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

66 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 97.9th percentile).

high confidence · 97.9th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: schwannoma

6

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Observational and natural-history studies

19 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 7 · after dedupe 7 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 7 · 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 (7)

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 Vestibular schwannoma — 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.

"Vestibular schwannoma" OR "Acoustic neurilemoma" OR "Acoustic neurinoma" OR "Acoustic neuroma" OR "acoustic neurilemmoma" OR "acoustic schwannoma" OR "neurilemoma, acoustic" OR "neurinoma of the acoustic nerve" OR "neurinoma of acoustic nerve" OR "neuroma, acoustic" OR "vestibular neurilemmoma" OR "vestibular schwannoma (disease)"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Neuroma, Acoustic

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

"Vestibular schwannoma" OR "Acoustic neurilemoma" OR "Acoustic neurinoma" OR "Acoustic neuroma" OR "acoustic neurilemmoma" OR "acoustic schwannoma" OR "neurilemoma, acoustic" OR "neurinoma of the acoustic nerve" OR "neurinoma of acoustic nerve" OR "neuroma, acoustic" OR "vestibular neurilemmoma" OR "vestibular schwannoma (disease)"

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

Study-type breakdown: 66 interventional · 19 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"schwannoma"

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 2026-07-27T11:00:09.201Z