{"orpha":252175,"name":"Vestibular schwannoma","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Acoustic neurilemoma","Acoustic neurinoma","Acoustic neuroma"],"definition":"Vestibular schwannoma is a rare tumor of the posterior fossa originating in the Schwann cells of the vestibular transitional zone of the vestibulocochlear nerve that can be benign, small, slow growing and asymptomatic or large, faster growing and aggressive and potentially fatal, presenting with symptoms of hearing and balance impairment, vertigo, ataxia, headache and fifth, sixth or seventh cranial nerve dysfunction and facial numbness.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-252175/","orphanet_url":"https://www.orpha.net/en/disease/detail/252175","names":{"fr":{"name":"Neurinome de l'acoustique","synonyms":["Schwannome vestibulaire"],"definition":"Le neurinome de l'acoustique est une tumeur rare de la fosse postérieure, issue des cellules de Schwann de la zone transitionnelle vestibulaire du nerf vestibulo-cochléaire. Il peut être petit, bénin, lentement progressif et asymptomatique, ou large, rapidement progressif, agressif et potentiellement fatal. Il se manifeste par des troubles auditifs et de l'équilibre, des vertiges, une ataxie, des céphalées, une atteinte du cinquième, sixième ou septième nerf crânien et un engourdissement du visage."},"de":{"name":"Schwannom, vestibuläres","synonyms":["Akustikusneurinom","Vestibularis-Schwannom"],"definition":"Das vestibuläre Schwannom ist ein seltener Tumor der hinteren Schädelgrube, der in den Schwann-Zellen der vestibulären Übergangszone des Nervus vestibulocochlearis entsteht. Er kann gutartig, klein, langsam wachsend und asymptomatisch oder groß, schnell wachsend, aggressiv und lebensbedrohlich sein und Symptome wie Hör- und Gleichgewichtsstörungen, Schwindel, Ataxie, Kopfschmerzen, Funktionsstörungen des fünften, sechsten oder siebten Hirnnervs und Taubheitsgefühl im Gesicht hervorrufen."},"es":{"name":"Schwannoma vestibular","synonyms":["Neurilemoma acústico","Neurinoma acústico","Neuroma acústico"],"definition":"El schwannoma vestibular es un tumor poco frecuente de la fosa posterior que se origina en las células de Schwann de la zona de transición vestibular del nervio vestibulococlear. Puede ser benigno, pequeño, de crecimiento lento y asintomático o grande, de crecimiento rápido y agresivo y potencialmente letal, presentándose con síntomas de deterioro auditivo y del equilibrio, vértigo, ataxia, dolor de cabeza y disfunción de los pares craneales quinto, sexto o séptimo y entumecimiento facial."},"it":{"name":"Schwannoma vestibolare","synonyms":["Neurilemmoma del nervo acustico","Neurinoma del nervo acustico","Neuroma del nervo acustico"],"definition":"Lo schwannoma vestibolare è un tumore raro della fossa posteriore che origina nelle cellule di Schwann dell'area transizionale vestibolare del nervo vestibolo-cocleare. Può essere benigno, di piccole dimensioni, a progressione lenta e asintomatico, oppure di grandi dimensioni, a progressione veloce, aggressivo e potenzialmente letale, con disturbi dell'udito e dell'equilibrio, vertigini, atassia, cefalea, disfunzione del quinto, sesto o settimo nervo cranico e intorpidimento facciale."},"nl":{"name":"Vestibulair schwannoom","synonyms":["Akoestisch neurilemoom","Akoestisch neurinoom","Akoestisch neuroom"],"definition":"Vestibulair schwannoom is een zeldzame tumor van de achterste schedelgroeve (<i>fossa cranii posterior</i>) die ontstaat in de schwanncellen van de vestibulaire transitiezone van de gehoor- en evenwichtszenuw (nervus vestibulocochlearis), en ofwel goedaardig, klein en traag groeiend kan zijn, ofwel groot, sneller groeiend, agressief en mogelijks fataal. Symptomen zijn gehoor- en evenwichtsstoornissen, draaiduizeligheid (vertigo), ataxie, hoofdpijn en stoornissen aan de vijfde, zesde of zevende hersenzenuw, alsook gevoelloosheid aan het gezicht."},"pt":{"name":"Schwannoma vestibular","synonyms":["Neurilemoma do acústico","Neurinoma do acústico","Neuroma do acústico"]},"pl":{"name":"Nerwiak osłonkowy przedsionkowy","synonyms":["Neurilemmoma nerwu słuchowego","Nerwiak nerwu słuchowego"]},"cs":{"name":"Vestibulární schwannom","synonyms":["Neurilemom akustiku","Neurinom akustiku","Neurom akustiku"]},"uk":{"name":"Вестибулярна шваннома","synonyms":["Акустична невринома","Акустична невринома","Акустична неврома"],"definition":"Vestibular schwannoma is a rare tumor of the posterior fossa originating in the Schwann cells of the vestibular transitional zone of the vestibulocochlear nerve that can be benign, small, slow growing and asymptomatic or large, faster growing and aggressive and potentially fatal, presenting with symptoms of hearing and balance impairment, vertigo, ataxia, headache and fifth, sixth or seventh cranial nerve dysfunction and facial numbness."},"zh":{"name":"前庭神经鞘瘤","synonyms":["听神经鞘瘤"],"definition":"前庭神经鞘瘤是一种罕见的后颅窝肿瘤，起源于前庭蜗神经的前庭移行区的施旺细胞，可以是良性的、小的、生长缓慢的、无症状的或大的、生长较快的、侵袭性的和潜在致命的，表现为听力和平衡障碍、眩晕、共济失调、头痛和第五、第六或第七颅神经功能障碍和面部麻木。"},"tr":{"name":"Vestibüler schwannom","synonyms":["Akustik nöroma","Akustik nörinom","Akustik nörilemoma"]}},"xrefs":[{"source":"ICD-10","code":"D33.3","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2A02.3","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"873486320"},{"source":"MONDO","code":"0001569","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D009464","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0027859","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"223","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10000523","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":252164,"name":"Benign schwannoma","classifications":[181,202]},{"orpha":98061,"name":"Rare otorhinolaryngologic tumor","classifications":[200,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.0,"area":"Denmark","status":"Validated","source":"20871439[PMID]_24655069[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.2,"area":"United States","status":"Validated","source":"National Cancer OTHERitute[OTHER]_23432451[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.8,"area":"Specific population","status":"Validated","source":"21897393[PMID]"},{"type":"Annual incidence","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}