ORPHA:100084
Middle ear neuroendocrine tumor
Publications
46
49.1th percentile
Trials
0
Interventional, condition-specific
Researchers
246
Distinct authors in sample
Gene link
—
Readiness
1/6
Stages with a signal
Clinical definition (Orphanet)
Middle ear neuroendocrine tumor is a rare, otorhinolaryngologic tumor characterized by a mixed glandular and non-glandular histological features and positive immunostaining for pancytokeratin, vimentin, synaptophysin and islet-1 protein. Common signs and symptoms are hearing loss, mass, pain, discharge, equilibrium disturbances, tinnitus and nerve paralysis.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0015071
- UMLS:C4305468
Additional Mondo synonyms (7)
middle ear NET · middle ear neuroendocrine neoplasm · middle ear neuroendocrine tumor · middle ear neuroendocrine tumor, well differentiated, low or intermediate grade · neuroendocrine neoplasm of middle ear · neuroendocrine tumor of middle ear · neuroendocrine tumour of middle ear
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.”
1/6 stages with a signal
Research-stage checklist from open sources (GenCC, literature, Monarch when enriched, ClinicalTrials.gov). Not a prognosis or care recommendation.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
46 matched papers (39 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialNot found
No matched interventional trial under our ClinicalTrials.gov rules
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)
Not enriched in this build — Monarch phenotype joins were not run for this record.
Animal models (Monarch / Alliance)
Not enriched in this build.
Literature
Is anyone studying this?
46
46 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
46 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).
39 in the last 10 years · high confidence · 49.1th percentile (publications denominator)
Phrase hits: 46 · MeSH hits: 0
Who's working on it?
246
Distinct author names in 46 sampled papers — named people below.
Who's working on it?
No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.
- 01La Rosa S3 papers · 2026
Institute of Pathology, University Hospital and University of Lausanne, Lausanne, Switzerland. stefano.larosa@chuv.ch.
Papers in Europe PMC - 02Appetecchia M2 papers · 2026
Oncological Endocrinology Unit, IRCCS Regina Elena National Cancer Institute, Rome, Italy.
Papers in Europe PMC - 03Bishop JA2 papers · 2026
Department of Pathology, UT Southwestern Medical Center, Dallas, TX.
Papers in Europe PMC - 04Mete O2 papers · 2022
Department of Pathology, University Health Network, University of Toronto, 200 Elizabeth Street, 11th floor, Toronto, ON, M5G 2C4, Canada. ozgur.mete2@uhn.ca.
Papers in Europe PMC - 05Uccella S2 papers · 2022
Unit of Pathology, Department of Medicine and Surgery, University of Insubria, Varese, Italy.
Papers in Europe PMC - 06Abdrakhmanova D1 paper · 2025
Astana Medical University, Department of Otolaryngology, Republic of Kazakhstan abdrakhmanovadina585@gmail.com.
Papers in Europe PMC - 07Abita P1 paper · 2021Papers in Europe PMC
- 08Ahmed ZM1 paper · 2025
Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, University of Maryland, Baltimore, MD 21201, USA.
Papers in Europe PMC - 09Alabood S1 paper · 2024
Department of Otorhinolaryngology Head and Neck Surgery, Security Forces Hospital Program, Salah Ad Din Al Ayyubi Rd, Aldhubbat Dist, Riyadh 12211, Saudi Arabia.
Papers in Europe PMC - 10Alcantara Dzib JR1 paper · 2026
Department of Pathology, Unidad Médica de Alta Especialidad, Hospital de Especialidades Centro Médico Nacional "Ignacio García Téllez", Instituto Mexicano del Seguro Social, Mérida, MEX.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
0
interventional trials for this specific condition
No interventional trial testing a treatment matched this specific condition name on ClinicalTrials.gov (observational studies and pan-disease registries are listed separately when present).
Data as of 27 July 2026
No matched interventional trials. This is true for 73% of diseases in the trials denominator (5114 of 7003). Here are the researchers publishing on it.
high confidence · 36.5th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.
See who's working on it — people publishing on this disease are often the practical next contact when no trial is listed.
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This 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.
"Middle ear neuroendocrine tumor" OR "middle ear NET" OR "middle ear neuroendocrine neoplasm" OR "middle ear neuroendocrine tumor, well differentiated, low or intermediate grade" OR "neuroendocrine neoplasm of middle ear" OR "neuroendocrine neoplasm of the middle ear" OR "neuroendocrine tumor of middle ear" OR "neuroendocrine tumor of the middle ear" OR "neuroendocrine tumour of middle ear" OR "neuroendocrine tumour of the middle ear"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Middle ear neuroendocrine tumor" OR "middle ear NET" OR "middle ear neuroendocrine neoplasm" OR "middle ear neuroendocrine tumor, well differentiated, low or intermediate grade" OR "neuroendocrine neoplasm of middle ear" OR "neuroendocrine neoplasm of the middle ear" OR "neuroendocrine tumor of middle ear" OR "neuroendocrine tumor of the middle ear" OR "neuroendocrine tumour of middle ear" OR "neuroendocrine tumour of the middle ear"
Study-type breakdown: 0 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase; with hits: phrase
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 2026-07-27T07:05:10.660Z