{"orpha":71276,"name":"Silent sinus syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["CMA grade 3","Chronic maxillary atelectasis grade III","Imploding antrum syndrome"],"definition":"A rare otorhinolaryngologic disease characterized by adult-onset progressive enophthalmos due to collapse of some or all of the maxillary sinus walls and remodeling of the orbital floor.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-71276/","orphanet_url":"https://www.orpha.net/en/disease/detail/71276","names":{"fr":{"name":"Syndrome du sinus silencieux","synonyms":["Atélectasie maxillaire chronique de grade III","CMA grade 3","Collapsus progressif du sinus maxillaire","Syndrome \"silent sinus\""],"definition":"Maladie othorinolaryngologique rare caractérisée par une énophtalmie progressive chez l'adulte due à l'effondrement de l'une ou de plusieurs parois du sinus maxillaire et au remodelage du plancher orbital."},"de":{"name":"Silent-sinus-Syndrom","synonyms":["Chronische maxilläre Atelektase Grad III","Enophthalmus und Hypotropie durch Kieferhöhlenatelektase","Syndrom des implodierenden Antrums"],"definition":"Eine seltene otorhinolaryngologische Erkrankung, die durch einen fortschreitenden Enophthalmus im Erwachsenenalter gekennzeichnet ist, der durch den Kollaps einiger oder aller Kieferhöhlenwände und die Umformung des Orbitabodens entsteht."},"es":{"name":"Síndrome del seno silente","synonyms":["AMC grado III","Atelectasia maxilar crónica grado III","Síndrome de implosión maxilar"],"definition":"Es una enfermedad otorrinolaringológica poco frecuente caracterizada por enoftalmos progresivo de inicio en la edad adulta debido al colapso de algunas o de todas las paredes del seno maxilar y a la remodelación del suelo orbitario."},"it":{"name":"Sindrome del seno silente","synonyms":["CMA grado 3","Sindrome da collasso dell'antro","Atelettasia mascellare cronica di grado III"]},"nl":{"name":"Silent sinus-syndroom","synonyms":["CMA graad 3","Chronische maxillaire atelectase graad III","Imploding antrum-syndroom"],"definition":"Een zeldzame otorinolaryngologische aandoening, gekenmerkt door in de volwassenheid optredende progressieve enoftalmie als gevolg van collaps van enkele of alle wanden van sinus maxillaris (bovenkaaksholte) en remodellering van de orbitabodem."},"pt":{"name":"Síndrome seio silencioso","synonyms":["Síndrome de implosão"],"definition":"Doença otorrinolaringológica rara caracterizada por enoftalmia progressiva de início na idade adulta, devida ao colapso de algumas ou de todas as paredes do seio maxilar e remodelação do pavimento orbitário."},"pl":{"name":"Zespół cichej zatoki","synonyms":["CMA grade 3","Chronic maxillary atelectasis grade III","Imploding antrum syndrome"]},"cs":{"name":"Silent sinus syndrom","synonyms":["Syndrom zhroucení předsíně"]},"uk":{"name":"Синдром мовчазного синуса","synonyms":["Синдром вибухового антруму"],"definition":"A rare otorhinolaryngologic disease characterized by adult-onset progressive enophthalmos due to collapse of some or all of the maxillary sinus walls and remodeling of the orbital floor."},"zh":{"name":"隐匿性鼻窦综合征","synonyms":["隐匿性鼻窦综合征"]},"tr":{"name":"Sessiz sinüs sendromu","synonyms":["İç içe geçmiş antrum sendromu"]}},"xrefs":[{"source":"ICD-10","code":"J34.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CA0B","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1204931989"},{"source":"MONDO","code":"0019108","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3698095","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10075540","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":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":558.0,"area":"Worldwide","status":"Validated","source":"37219052[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"37219052[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0000490","label":"Deeply set eye","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000478","label":"Abnormality of the eye","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000504","label":"Abnormality of vision","frequency":"Occasional (29-5%)","criteria":null}]}}