raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:171684

Idiopathic bilateral vestibulopathy

Type
Disease
Group
Disorder
Status
active

Idiopathic bilateral vestibulopathy is a rare otorhinolaryngologic disease characterized by dysfunction of both peripheral labyrinths or of the eighth cranial 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.

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Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enIdiopathic bilateral vestibulopathy
frVestibulopathie bilatérale idiopathique
deVestibulopathie, bilaterale idiopathische
esVestibulopatía bilateral idopática
itVestibulopatia idiopatica bilaterale
nlIdiopathische bilaterale vestibulopathie
ptVestibulopatia bilateral idiopática
plIdiopatyczna obustronna westibulopatia
csIdiopatická bilaterální vestibulopatie
ukІдіопатична двостороння вестибулопатія
zh特发性双侧前庭病变
trİdiyopatik bilateral vestibülopati

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10H81.8NTBTattributedvalidated
ICD-11AB34.0Especificvalidated
MONDO0015743Evalidated
UMLSC4545229Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

11 annotations · validated 2024-07-18
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0034773Oscillopsia
HP:0002172Postural instability
Frequent (79-30%)
HP:0007670Abnormal vestibulo-ocular reflex
HP:6001104Abnormal vestibulo-spinal reflex
HP:0002141Gait imbalance
HP:0002321Vertigo
Occasional (29-5%)
HP:0000739Anxiety
HP:0031987Diminished ability to concentrate
HP:0002354Memory impairment
HP:5200273Sadness
Excluded (0%)
HP:0000407Sensorineural hearing impairment

Run the differential on the 2 obligate / very frequent terms

Annotation source: 38178884[PMID]_31426838[PMID]_29081426[PMID]

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Sources for this card