Autoimmune inner ear disease

Summary

Autoimmune inner ear disease (MONDO:0022518) is a disease and 5 clinical trials. Top therapeutic interventions include anakinra, golimumab, and gevokizumab. A subtype of autoimmune disease of ear, nose and throat — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Clinical trials: 5

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	autoimmune inner ear disease
Mondo ID	MONDO:0022518
ICD-11	1392879169
UMLS	C0395947
MedGen	583162
Is cancer (heuristic)	no

Also known as: AIED

Disease family

This is a subtype of autoimmune disease of ear, nose and throat. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › immune system disorder › autoimmune disease › autoimmune disease of ear, nose and throat › autoimmune inner ear disease

Related subtypes (6): Cogan syndrome, IgG4-related submandibular gland disease, eosinophilic angiocentric fibrosis, IgG4-related dacryoadenitis and sialadenitis, autoimmune uveitis, autoimmune retinopathy

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 5.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE1/PHASE2	2
PHASE2	2
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT03587701	PHASE2	RECRUITING	Effects of Anakinra in Subjects With Corticosteroid-resistant/Intolerant Meniere’s Disease and Autoimmune Inner Ear Disease
NCT01267994	PHASE1/PHASE2	COMPLETED	A Clinical Trial of Anakinra for Steroid-Resistant Autoimmune Inner Ear Disease
NCT01526174	PHASE1/PHASE2	TERMINATED	Intratympanic Injection for Autoimmune Inner Ear Disease
NCT01950312	PHASE2	COMPLETED	The Effects of Gevokizumab in Corticosteroid-resistant Subjects With Autoimmune Inner Ear Disease
NCT04806282	Not specified	RECRUITING	Deep Phenotyping of Hearing Instability Disorders: Cohort Establishment, Biomarker Identification, Development of Novel Phenotyping Measures, and Discovery of Therapeutic Targets

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
ANAKINRA	4	2
GOLIMUMAB	4	1
GEVOKIZUMAB	3	1
CHEMBL5220618	0	2