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idiopathic bilateral vestibulopathy - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

idiopathic bilateral vestibulopathy

MONDO:0015743

Overview

Idiopathic bilateral vestibulopathy is a rare otorhinolaryngologic disease characterized by dysfunction of both peripheral labyrinths or of the eighth nerves, which presents with persistent unsteadiness of gait (particularly in darkness, during eye closure or under impaired visual conditions, or when standing/walking on uneven, soft or wobbly ground) and oscillopsia associated with head movements. The disease may be progressive, presenting no episodes of vertigo, or sequential, presenting recurrent episodes of vertigo.

ResearchLimited published research (2 articles)

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Postural instability and Oscillopsia; and common findings: Gait imbalance, Vertigo, Abnormal vestibulo-ocular reflex, and Abnormal vestibulo-spinal reflex. 10 total HPO annotations.

Organ System	Phenotype Count	Example Features
Brain and nerves	3	Gait imbalance, Anxiety, Memory problems (memory impairment)

Prognosis

Phenotype severity distribution: 2 very common features, 4 common features.

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for idiopathic bilateral vestibulopathy.

2 publications have been identified in PubMed for idiopathic bilateral vestibulopathy. Research spans Case Report / Case Series (50%) and Epidemiology / Natural History (50%).

Recent Publications

Kongsvad MSN (2025). [PMID: 41257360](https://pubmed.ncbi.nlm.nih.gov/41257360/). *Ugeskrift for laeger*. [Case Report / Case Series]
Loos E (2025). [PMID: 40033069](https://pubmed.ncbi.nlm.nih.gov/40033069/). *Scientific reports*. [Epidemiology / Natural History]

Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 6:56 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:171684

GARD

Genetic and Rare Diseases Info Center

GARD:20124

Questions for Your Care Team

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Kisho

idiopathic bilateral vestibulopathy

Overview

Clinical Presentation

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Latest News & Developments

Frequency and phenotype of GAA-FGF14 disease in bilateral vestibulopathy syndromes: insights from repeat expansion carriers, including a case of co-occurrence with RFC1-related CANVAS.

idiopathic bilateral vestibulopathy

Overview

Clinical Presentation

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Latest News & Developments

Frequency and phenotype of GAA-FGF14 disease in bilateral vestibulopathy syndromes: insights from repeat expansion carriers, including a case of co-occurrence with RFC1-related CANVAS.