[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"autoimmune-inner-ear-disease\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:autoimmune-inner-ear-disease":27},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,2,0,[8,45],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":13,"acronym":4,"eligibilityCriteria":14,"healthyVolunteers":15,"sex":16,"minAge":17,"maxAge":18,"enrollmentInfo":19,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":23,"conditions":24,"keywords":28,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":33,"lastUpdatePostDateStruct":34,"startDateStruct":37,"completionDateStruct":39,"leadSponsor":41,"locationsCount":44},"100424391","deep-phenotyping-of-hearing-instability-disorders-cohort-establishment-biomarker-identification-development-of-novel-phenotyping-measures-and-discovery-of-therapeutic-targets-100424391",false,"NCT04806282","Deep Phenotyping of Hearing Instability Disorders: Cohort Establishment, Biomarker Identification, Development of Novel Phenotyping Measures, and Discovery of Therapeutic Targets","* NCLUSION CRITERIA:\n\nAffected Adults\n\nIn order to be eligible to participate in this study as a subject with HI, an individual must meet all of the following criteria:\n\n1. Provision of signed and dated informed consent.\n2. Stated willingness to comply with all study procedures and availability for the duration of the study\n3. All genders, aged 18-80 years\n\n   Diagnosed with hearing instability, defined as documented hearing instability on serial audiometry with sensorineural hearing loss (SNHL) greater than 30 dB HL at one or more frequencies on at least one hearing test. Inclusion will require documentation of clinically significant change in hearing (either worsening or improvement) between at least 2 hearing tests or documentation of a sudden change in hearing. Clinically significant change in hearing will be defined by a change of10 dB at any three frequencies, 15 dB at any two frequencies, or at least 20 dB at one frequency. A sudden change in hearing will be defined as at least a 30 dB difference at 3 consecutive frequencies in the affected ear as compared to the contralateral ear.\n4. No air-bone gaps in excess of 10 dB for 500-4000 Hz indicative of conductive HL in at least 1 ear.\n5. For females of reproductive potential: Negative pregnancy test at start of study\n\nUnaffected Adults\n\nIn order to be eligible to participate in this study as a healthy volunteer, an individual must meet all of the following criteria:\n\n1. Provision of signed and dated informed consent.\n2. Stated willingness to comply with all study procedures and availability for the duration of the study\n3. All genders, aged 18-80 years\n4. No air-bone gaps in excess of 10 dB for 500-4000Hz indicative of conductive HL in at least one ear.\n5. Normal middle ear function as indicated by normal 226 Hz tympanograms bilaterally, defined as middle ear pressure between plus minus 100 decaPascals, and peak compensated static compliance between 0.3-1.8 milliliters\n6. For females of reproductive potential: Negative pregnancy test at start of study\n\nEXCLUSION CRITERIA:\n\nAffected and unaffected individuals who meet any of the following criteria will be excluded from participation in this study:\n\n1. Presence of non-MRI compatible devices (cardiac pacemaker, meta\\\u003Cspecific devices (e.g., cardiac pacemaker)\n2. Pregnancy or lactation\n3. Known allergic reactions to gadolinium\n4. Febrile illness within 2 weeks that could affect immune profiling\\*\n5. Evidence of active outer or middle ear disease or anomaly (e.g. otitis media, stenotic ear canal, otorrhea)\n6. History of chronic, as defined by fluid in the middle ear for more than 4 months, or recurrent otitis media, as defined by more than 4 episodes of acute otitis media in one year.\n7. Current PE tubes\n8. Bilateral profound (Pure tone average (PTA) \\> 90 dB HL) sensorineural hearing loss\n9. History or diagnosis of a central nervous system disorder, including but not limited to:\n\n   1. Intracranial tumors\n   2. Cerebrovascular disease\n   3. Degenerative CNS disorder\n   4. CNS trauma\n   5. Encephalitis\n   6. Meningitis\n10. Unable to discontinue medications that can interfere with vestibular test results for the 48 hours immediately preceding a vestibular study session. These include any and all anti-dizziness medications (such as Antivert), alcohol, caffeine, prescription pain medications (such as Percocet), prescription headache medications (such as Imitrex), sleeping pills (such as Ambien), anti-seizure medications (such as Topamax), and\u002For antihistamines (such as Benadryl).\n11. Current diagnosis from the Diagnostic and Statistical Manual of Mental Disorders (DSM IV) of schizophrenia, bipolar disorder, or psychosis.\n12. Unstable intercurrent illness that in the judgment of the PI could prevent or confound collection of data.\n\nProspective study subjects who are cognitively impaired and lack consent capacity, will not be enrolled.\n\n\\*Participants suspected of having COVID-19 will be moved to the designated COVID-19 unit and tested for SARS CoV-2 and Respiratory Pathogen Panel per guidance from NIH CC Clinical Practice Safety Guidelines. Possible COVID-19 infections identified by phone screen will not be eligible for study protocol until infection resolved.",true,"ALL","18 Years","80 Years",{"count":20,"type":21},210,"ESTIMATED","OBSERVATIONAL","Background:\n\nDisorders of hearing instability (HI) are poorly characterized and ineffectively treated. HI can cause fluctuations in hearing thresholds and speech understanding. Researchers want to use a specialized form of magnetic resonance imaging (MRI) and blood tests to learn more about HI.\n\nObjective:\n\nTo characterize a cohort of people with HI and to correlate HI with other data, including hearing evaluations, as well as radiologic and immunologic biomarkers of inflammation over time.\n\nEligibility:\n\nAdults ages 18-80 who have symptoms consistent with possible HI.\n\nDesign:\n\nParticipants will be screened with a medical and hearing history and medical record review.\n\nParticipants will have physical exams. Their head and neck will be examined. They will have blood drawn.\n\nParticipants will have hearing tests. They will wear headphones or foam earplugs. They will listen to different tones. They may describe what they hear.\n\nParticipants will have balance tests. They will wear goggles as they watch moving lights or while cold or warm air is blown into their ears. They will sit in a spinning chair in a quiet, dark booth. From a reclining position, they will raise their head while clicking sounds are played into their ears.\n\nParticipants will have MRIs of the inner ear and brain. The MRI scanner is a metal cylinder surrounded by a strong magnetic field. During the MRIs, participants will lie on a table that slides in and out of the scanner. Soft padding or a coil will be placed around their head. They will get a contrast agent through an intravenous catheter.\n\nParticipation will last up to 15 months.\n\n...",[25,26,27],"Meniere's Disease","Enlarged Vestibular Aqueduct Syndrome","Autoimmune Inner Ear Disease",[29,30,31],"Biomarkers","immunoprofiling","Auditory","RECRUITING","2026-06-26",{"date":35,"type":36},"2026-06-29","ACTUAL",{"date":38,"type":36},"2021-08-17",{"date":40,"type":21},"2030-12-31",{"name":42,"class":43},"National Institute on Deafness and Other Communication Disorders (NIDCD)","NIH",1,{"id":46,"slug":47,"hasResults":11,"nctId":48,"briefTitle":49,"officialTitle":50,"acronym":4,"eligibilityCriteria":51,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":52,"enrollmentInfo":53,"targetDuration":4,"studyType":55,"phases":56,"briefSummary":58,"conditions":59,"keywords":4,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":60,"lastUpdatePostDateStruct":61,"startDateStruct":63,"completionDateStruct":65,"leadSponsor":67,"locationsCount":44},"100330858","phase-2-effects-of-anakinra-in-subjects-with-corticosteroid-resistantintolerant-menieres-disease-and-autoimmune-inner-ear-disease-100330858","NCT03587701","Effects of Anakinra in Subjects With Corticosteroid-resistant\u002FIntolerant Meniere's Disease and Autoimmune Inner Ear Disease","A Phase II Placebo Controlled Study of the Effects of Anakinra (Kineret) in Subjects With Corticosteroid-Resistant\u002FIntolerant Meniere's Disease (CR-MD) and Corticosteroid-Resistant\u002FIntolerant Autoimmune Inner Ear Disease Disease (CR-AIED)","Inclusion Criteria:\n\n* meet diagnostic criteria for Autoimmune Inner Ear Disease (AIED) with active deterioration in one ear as defined below\n* SNHL of greater than 30dB at one or more frequencies in the active ear. The contralateral ear may have any hearing threshold that is either stable or worsening, but may not exhibit improvement in response to corticosteroids even if the more active ear declines. Active deterioration is defined as a 15dB decline at one frequency (excluding 250 or 8kHz as a sole indicator) on their audiogram, or 10dB at 2+ frequencies developing in \\> or equal 3 days but \\\u003C or equal 90 days, or if the hearing loss evolved in less than 3 days, the patient displayed features suggestive of an autoimmune disorder\n* Capable of understanding and giving informed consent\n* Have 2 sequential audiograms following the decline in hearing \\>or equal 28 days apart with no demonstrable improvement in hearing following corticosteroid therapy (\\\u003Cor equal 5dB of improvement at any individual frequency (250, 500, 1000, 2000 and 4000Hz) and \\\u003C12% improvement in WRS)\n* Patients must have been treated with oral or IT prednisone with no demonstrable improvement or attempted to be treated and deemed intolerant. Steroid intolerance is defined as unable or unwilling to take corticosteroids because of a prior untoward effect or a current medical condition that would negatively be impacted by steroid use\n* Must have completed steroid therapy, including any intratympanic steroid therapy\n* Meniere's disease defined according to the AAO criteria, with the additional requirement of corticosteroid resistance AND no new diuretics may be instituted or dose increased from 28 days prior to trial enrollment\n* Patients must be fluent in English as all word recognition scores are based on testing in English\n* Patients may have other autoimmune disease(s) Females of childbearing potential, must be practicing a method of birth control considered effective and medically acceptable by the investigator for a minimum of 2 months prior to the study and at least 2 months after last administration of study drug\n* Males similarly must be practicing a method of birth control considered effective and medically acceptable by the investigator for a minimum of 2 months prior to the study and at least 2 months after last administration of study drug\n\nExclusion Criteria:\n\n* Evidence of retrocochlear pathology (vestibular schwannoma) or inner ear malformation (Mondini Malformation or Enlarged Vestibular Aqueduct) based on imaging\n* Prior treatment with gentamicin for Meniere's Disease\n* Most recent decline in hearing occurring \\>90 days prior to trial enrollment\n* Positive test for Muckle-Wells mutation\n* Concurrently receiving methotrexate, TNF-antagonist therapy or any other immunosuppressive therapy\n* Steroid-dependent hearing loss\n* Any immunodeficiency syndrome\n* Active or chronic infections\n* Currently receiving, or having received treatment for a malignancy in the past 3 years\n* Chronic renal insufficiency (a creatinine clearance of \\\u003C49mL\u002Fmin) or chronic renal failure\n* Neutropenia prior to treatment with anakinra\n* Receipt of live vaccine \\\u003C3 months prior to enrollment\n* Previous treatment with an IL-1 antagonist for any clinical indication\n* First-degree relative with a diagnosis of a CAPS disease\n* History of active narcotic abuts, including prescription narcotics\n* Pregnant or lactating females\n* Non-English speaking patients\n* Seropositive for Hepatitis B, C, HIV or tuberculosis on screening, with the exception of presence of antibodies to Hepatitis B in subjects reporting prior vaccination, and presence of positive skin testing for TB in subjects who received BCG in the past\n* History of suicidal ideation, significant depression that resulted in hospitalization, or answers \"yes\" during C-SSR screening\n* Prior participation in any type of clinical trial using a medication that inhibits interleukin-1 activity\n* Known hypersensitivity to E.coli derived products","75 Years",{"count":54,"type":21},57,"INTERVENTIONAL",[57],"PHASE2","A Phase II randomized, placebo controlled study design of anakinra (Kineret) in corticosteroid-resistant or intolerant Meniere's disease (CR-MD)and corticosteroid-resistant or intolerant autoimmune inner ear disease (CR-AIED) patients. Patients will be randomized by a 2:1 allocation to anakinra or placebo for 42 continuous days. After day 42, a second placebo-controlled period will begin for an additional 42 days. This will be followed by a 264 day observation period, during which, hearing declines may be re-treated with anakinra after 30 days following the initial drug.",[27,25],"2026-04-14",{"date":62,"type":36},"2026-04-15",{"date":64,"type":36},"2019-01-03",{"date":66,"type":21},"2026-12-31",{"name":68,"class":69},"Northwell Health","OTHER"]