{"orpha":171684,"name":"Idiopathic bilateral vestibulopathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-171684/","orphanet_url":"https://www.orpha.net/en/disease/detail/171684","names":{"fr":{"name":"Vestibulopathie bilatérale idiopathique","definition":"La vestibulopathie bilatérale idiopathique est une maladie oto-rhino-laryngologique rare caractérisée par une atteinte des deux labyrinthes périphériques ou des huitièmes nerfs crâniens. Elle se manifeste par une démarche déséquilibrée persistante (surtout à l'obscurité, les yeux fermés ou en cas d'altération des conditions visuelles ; ou en se levant/marchant sur un sol inégal, mou ou bancal), ainsi qu'une oscillopsie associée à des mouvements de la tête. La maladie peut être progressive, ne présentant pas d'épisodes de vertige, ou être séquentielle et présenter des épisodes récurrents de vertige."},"de":{"name":"Vestibulopathie, bilaterale idiopathische","definition":"Die idiopathische bilaterale Vestibulopathie ist eine seltene Hals-Nasen-Ohren-Krankheit, die durch Dysfunktion sowohl der peripheren Labyrinthe als auch der achten Schädelnerven gekennzeichnet ist. Sie geht mit anhaltender Gangunsicherheit (insbesondere in der Dunkelheit, beim Schließen der Augen oder unter eingeschränkten Sehbedingungen, beim Stehen/Wandern auf unebenem, weichem oder wackeligem Boden) und Oszillopsie im Zusammenhang mit Kopfbewegungen einher. Die Krankheit kann progressiv sein, mit Episoden ohne Schwindel, oder sequentiell mit wiederkehrenden Schwindelanfällen."},"es":{"name":"Vestibulopatía bilateral idopática","definition":"La vestibulopatía bilateral idiopática es una enfermedad otorrinolaringológica poco frecuente caracterizada por la disfunción de ambos laberintos periféricos o de los nervios del octavo par craneal, que se presenta con inestabilidad persistente de la marcha (particularmente en la oscuridad, durante el cierre ocular o en condiciones visuales deficientes, o al caminar/permanecer de pie en un terreno irregular, blando o inestable) y oscilopsia asociada con los movimientos de la cabeza. La enfermedad puede ser progresiva, sin episodios de vértigo, o secuencial, presentando episodios de vértigo recurrentes."},"it":{"name":"Vestibulopatia idiopatica bilaterale","definition":"La vestibulopatia idiopatica bilaterale è una malattia di interesse otorinolaringoiatrico, caratterizzata dalla disfunzione dei labirinti periferici o dell'ottavo nervo cranico. La malattia esordisce con un'instabilità persistente dell'andatura (soprattutto al buio, quando si chiudono gli occhi, in condizioni di scarsa visibilità oppure quando il paziente è in posizione eretta o cammina su una superficie sconnessa, morbida o traballante) e oscilloscopia associata ai movimenti della testa. La malattia può essere progressiva, in assenza di vertigini, oppure sequenziale, con episodi ricorrenti di vertigine."},"nl":{"name":"Idiopathische bilaterale vestibulopathie","definition":"Idiopathische bilaterale vestibulopathie is een zeldzame otorinolaryngologische ziekte die wordt gekarakteriseerd door dysfunctie van beide perifere labyrinten of van de acht hersenzenuwen, en die zich uit met aanhoudende onstabiele gang (vooral in het donker, bij het sluiten van de ogen of bij verminderde visuele condities, of bij het staan/stappen op oneffen, zachte of wankele ondergrond) en oscillopsie geassocieerd met hoofdbewegingen. De ziekte kan progressief zijn, zonder episodes van vertigo, of sequentieel, met terugkerende episodes van vertigo."},"pt":{"name":"Vestibulopatia bilateral idiopática"},"pl":{"name":"Idiopatyczna obustronna westibulopatia"},"cs":{"name":"Idiopatická bilaterální vestibulopatie"},"uk":{"name":"Ідіопатична двостороння вестибулопатія","definition":"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."},"zh":{"name":"特发性双侧前庭病变","definition":"特发性双侧前庭病变是一种罕见的耳鼻咽喉疾病，其主要临床表现为骨迷路、膜迷路或听神经功能障碍，表现为持续的步态不稳（在黑暗、闭眼或视力障碍情况下加重，或在不平、柔软或不稳定的地面行走或站立），振动幻视与头部运动有关，不伴有眩晕，可呈进行性发展。"},"tr":{"name":"İdiyopatik bilateral vestibülopati"}},"xrefs":[{"source":"ICD-10","code":"H81.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"AB34.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1394072237"},{"source":"MONDO","code":"0015743","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4545229","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98036,"name":"Rare otorhinolaryngologic disease"},"parents":[{"orpha":98036,"name":"Rare otorhinolaryngologic disease","classifications":[200]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-07-18","source":"38178884[PMID]_31426838[PMID]_29081426[PMID]","terms":[{"hpo":"HP:0034773","label":"Oscillopsia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002172","label":"Postural instability","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0007670","label":"Abnormal vestibulo-ocular reflex","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:6001104","label":"Abnormal vestibulo-spinal reflex","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002141","label":"Gait imbalance","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002321","label":"Vertigo","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000739","label":"Anxiety","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031987","label":"Diminished ability to concentrate","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002354","label":"Memory impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:5200273","label":"Sadness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000407","label":"Sensorineural hearing impairment","frequency":"Excluded (0%)","criteria":null}]}}