{"orpha":90059,"name":"Sudden sensorineural hearing loss","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["Secondary acute sensorineural hearing loss"],"definition":"A rare otorhinolaryngologic condition characterized by sudden hearing loss of at least 30 decibels across three contiguous frequencies, within 72 hours or less, due to acute damage to the cochlear hair cells, resulting from acoustic trauma or mechanical trauma during surgery, or occurring without any known cause. The hearing loss may affect one or both ears. Typical concomitant symptoms are aural fullness, tinnitus, and vertigo. Patients may fully recover or remain with hearing loss of variable intensity.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90059/","orphanet_url":"https://www.orpha.net/en/disease/detail/90059","names":{"fr":{"name":"Perte auditive neurosensorielle brutale","synonyms":["Perte auditive neurosensorielle aigüe brutale"],"definition":"Maladie otorhinolaryngologique rare caractérisée par une perte auditive soudaine d'au moins 30 décibels sur trois fréquences contiguës, en 72 heures ou moins, due à une lésion aiguë des cellules ciliées de la cochlée, résultant d'un traumatisme acoustique ou mécanique au cours d'une intervention chirurgicale, ou survenant sans cause apparente. La perte auditive peut toucher une seule ou les deux oreilles. Les symptômes concomitants typiques sont la plénitude de l'oreille, les acouphènes et les vertiges. Les patients peuvent se rétablir complètement ou garder une altération auditive de l'intensité variable."},"de":{"name":"Plötzliche Schallempfindungsschwerhörigkeit","synonyms":["Sekundärer akuter sensorineuraler Hörverlust"],"definition":"Ein seltener Zustand in der Hals-Nasen-Ohren-Heilkunde, der durch einen plötzlichen Hörverlust von mindestens 30 Dezibel bei drei zusammenhängenden Frequenzen innerhalb von 72 Stunden oder weniger gekennzeichnet ist, der auf eine akute Schädigung der Haarzellen der Cochlea zurückzuführen ist, die durch ein akustisches Trauma oder ein mechanisches Trauma während eines chirurgischen Eingriffs verursacht wird oder ohne bekannte Ursache auftritt. Der Hörverlust kann ein oder beide Ohren betreffen. Typische Begleitsymptome sind Ohrensausen, Tinnitus und Schwindel. Die Patienten können sich vollständig erholen oder mit einem Hörverlust unterschiedlicher Intensität zurückbleiben."},"es":{"name":"Hipoacusia neurosensorial súbita","synonyms":["Hipoacusia neurosensorial aguda secundaria"],"definition":"Es un trastorno otorrinolaringológico poco frecuente caracterizado por hipoacusia súbita de al menos 30 decibelios en tres frecuencias consecutivas en menos de 72 horas, debido a un daño agudo en las células ciliadas de la cóclea, como resultado de un trauma acústico o de un trauma mecánico durante la cirugía, o que ocurre sin ninguna causa conocida. La pérdida auditiva puede afectar a uno o a ambos oídos. Los síntomas concomitantes típicos son plenitud ótica, tinnitus y vértigo. Los pacientes pueden recuperarse por completo o permanecer con una hipoacusia de intensidad variable."},"it":{"name":"Perdita di udito neurosensoriale improvvisa","synonyms":["Perdita di udito neurosensoriale acuta secondaria"]},"nl":{"name":"Plotseling sensorineuraal gehoorverlies","synonyms":["Secundair acuut sensorineuraal gehoorverlies"],"definition":"Een zeldzame otorinolaryngologische aandoening, gekenmerkt door plots gehoorverlies van minstens 30 decibel over drie aangrenzende frequenties heen, binnen 72 uur of minder, als gevolg van acute beschadiging van cochleaire haarcellen door akoestisch trauma of mechanisch trauma tijdens chirurgie, of zonder gekende oorzaak. Gehoorverlies kan één of beide oren aantasten. Typische concomitante symptomen zijn vol gevoel van oor, tinnitus, en vertigo. Patiënten kunnen volledig herstellen of gehoorverlies van variabele intensiteit overhouden."},"pt":{"name":"Perda auditiva neurossensorial de início súbito","synonyms":["Perda auditiva neurossensorial aguda secundária"],"definition":"Patologia otorrinolaringológica rara caracterizada por perda auditiva súbita de pelo menos 30 decibéis em três frequências contíguas, dentro de 72 horas ou menos, devido a lesão aguda das células ciliadas da cóclea, resultante de trauma acústico ou mecânico durante cirurgia ou ocorrendo sem qualquer causa. A perda auditiva pode afetar um ou ambos os ouvidos. Os sintomas concomitantes típicos são plenitude auricular, zumbidos e vertigens. Os doentes podem recuperar-se totalmente ou permanecer com perda auditiva de intensidade variável."},"pl":{"name":"Ostra czuciowo-nerwowa utrata słuchuna skutek ostrego urazu akustycznego lub nagła głuchota lub operacja powodująca uraz akustyczny","synonyms":["Nagła głuchota czuciowo-nerwowa"]},"cs":{"name":"Akutní senzorineurální ztráta sluchu způsobená akutním akustickým traumatem anebo náhlou hluchotou nebo akustickým traumatem indukovaným operací","synonyms":["Secondary acute sensorineural hearing loss"]},"uk":{"name":"Раптова нейросенсорна приглухуватість","synonyms":["Вторинна гостра сенсоневральна приглухуватість"],"definition":"A rare otorhinolaryngologic condition characterized by sudden hearing loss of at least 30 decibels across three contiguous frequencies, within 72 hours or less, due to acute damage to the cochlear hair cells, resulting from acoustic trauma or mechanical trauma during surgery, or occurring without any known cause. The hearing loss may affect one or both ears. Typical concomitant symptoms are aural fullness, tinnitus, and vertigo. Patients may fully recover or remain with hearing loss of variable intensity."},"zh":{"name":"急性听觉损伤、突发性耳聋或手术所致听觉损伤致急性感音神经性听觉丧失"},"tr":{"name":"Akut akustik travma veya ani sağırlık veya cerrahiye bağlı akustik travma nedeniyle akut sensörinöral işitme kaybı"}},"xrefs":[{"source":"ICD-10","code":"H83.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"AB51.1","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0043373","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4275242","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12927","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98036,"name":"Rare otorhinolaryngologic disease"},"parents":[{"orpha":68361,"name":"Rare deafness","classifications":[200]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":40.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2018[OTHER]"}],"phenotypes":null}