Meniere's DiseaseNCT04806282
Deep Phenotyping of Hearing Instability Disorders: Cohort Establishment, Biomarker Identification, Development of Novel Phenotyping Measures, and Discovery of Therapeutic Targets
An observational study of Meniere's Disease and Enlarged Vestibular Aqueduct Syndrome, run by National Institute on Deafness and Other Communication Disorders (NIDCD) at 1 site in United States.
Trial summary
Background: Disorders 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. Read more about this study
- Who is it for?
- Ages 18 to 80 yearsHealthy volunteers can take part
- Conditions the study is for
- Meniere's Disease, Enlarged Vestibular Aqueduct Syndrome, Autoimmune Inner Ear Disease
- How many people
- 210 participants
- Where
- 1 siteUnited States
- Study dates
- 17 Aug 2021to 31 Dec 2030
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 to 80 years
- All sexes
- Healthy volunteers accepted
- Adult, Older adult
Study population: This study will enroll up to a total of 150 patients with HI. The enrollment target is between 10-30 patients per clinical subtype for at least 2 clinical subtypes of HI (i.e., without or with EH, without or with significant immune cell, cytokine or FLAIR positivity). 20 cytokines/chemokines in patients with Meniere s disease that revealed statistically higher levels of IL-1Beta compared to healthy controls (Flook et al., 2019), a sample size of 40-50 patients would be required for 80% power to detect an immune subtype associated with IL-1Beta.
Who may be able to join
None listed.
Who cannot join
- Affected and unaffected individuals who meet any of the following criteria will be excluded from participation in this study:
- Presence of non-MRI compatible devices (cardiac pacemaker, meta<specific devices (e.g., cardiac pacemaker)
- Pregnancy or lactation
- Known allergic reactions to gadolinium
- Febrile illness within 2 weeks that could affect immune profiling*
- Evidence of active outer or middle ear disease or anomaly (e.g. otitis media, stenotic ear canal, otorrhea)
Show all 21Show fewer
- 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.
- Current PE tubes
- Bilateral profound (Pure tone average (PTA) > 90 dB HL) sensorineural hearing loss
- History or diagnosis of a central nervous system disorder, including but not limited to:
- Intracranial tumors
- Cerebrovascular disease
- Degenerative CNS disorder
- CNS trauma
- Encephalitis
- Meningitis
- 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/or antihistamines (such as Benadryl).
- Current diagnosis from the Diagnostic and Statistical Manual of Mental Disorders (DSM IV) of schizophrenia, bipolar disorder, or psychosis.
- Unstable intercurrent illness that in the judgment of the PI could prevent or confound collection of data.
- Prospective study subjects who are cognitively impaired and lack consent capacity, will not be enrolled.
- 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.
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
United States1 site, 1 recruiting
- Recruiting
National Institutes of Health Clinical Center
Bethesda, Maryland, 20892
For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR), 800-411-1222 ext. TTY8664111010, prpl@cc.nih.gov
About this study
Background:
Disorders 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.
Read more
Objective:
To 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.
Eligibility:
Adults ages 18-80 who have symptoms consistent with possible HI.
Design:
Participants will be screened with a medical and hearing history and medical record review.
Participants will have physical exams. Their head and neck will be examined. They will have blood drawn.
Participants will have hearing tests. They will wear headphones or foam earplugs. They will listen to different tones. They may describe what they hear.
Participants 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.
Participants 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.
Participation will last up to 15 months.
...
Read the detailed description
Study Description:
Disorders of hearing instability remain poorly characterized, phenotyped and ineffectively treated, and may result in sudden changes in hearing. These disorders include, but are not limited to, sudden sensorineural hearing loss (SSNHL) as well as hearing fluctuation, including, but not limited to, autoimmune inner ear disease (AIED), Meniere s disease (MD) and enlarged vestibular aqueduct syndrome (EVAS). While this group of disorders is likely to be clinically and etiologically heterogeneous, a common feature is fluctuation of hearing thresholds and speech understanding as measured by word recognition scores (WRS). This protocol seeks to ascertain a cohort of patients with hearing fluctuation to correlate these main phenotypic features with other phenomic data including audiometric indicators of endolymphatic hydrops as well as radiologic and immunologic biomarkers of inflammation over time. The overall hypothesis is that phenomic data will enable stratification of the phenotype of patients with hearing instability disorders. Healthy volunteers will be recruited to establish the normative range of endolymph and perilymph on contrast-enhanced delayed FLAIR MRI to allow for quantitative computational analysis of contrastenhanced delayed FLAIR MRI images.
Objectives:
Primary objective: To develop a cohort of patients with hearing instability (HI).
Secondary objectives:
- To correlate evidence of HI with changes in phase-shift distortion product otoacoustic emissions (DPOAEs).
- To compare phase-shift DPOAEs to existing measures of auditory function including word recognition score (WRS), standard DPOAEs, and electrocochleography (ECochG) over time.
- To compare phase-shift DPOAEs to vestibular measures including cervical and ocular vestibular evoked myogenic potentials (VEMPs) over time.
- To correlate auditory and vestibular indicators of hearing instability and endolymphatic hydrops (EH) on MRI with quantitative differences in immunologic markers of inflammation over time.
- Establish the normative range of endolymph and perilymph volume on contrast-enhanced delayed FLAIR MRI to allow for quantitative computational analysis of contrast-enhanced delayed FLAIR MRI images
Exploratory objective:
- To stratify patients with hearing instability (HI) by differences in cytokine levels, which will inform the identification of clinical subtypes of HI and potentially identify therapeutic targets for future treatment with targeted agents.
- To perform transcriptional and immunoprofiling of PBMCs at time points associated with hearing fluctuation from phenotyped patient cohorts.
Endpoints:
Primary Endpoint: Identification of phenomic features associated with HI.
Secondary Endpoint:
(1) Identification of a variety of phenomic features of patients with HI that allow assignment to clinical subdivisions.
Exploratory Endpoints
- Identification of potential targets for therapeutics based on longitudinal immune/transcriptional profiling of patients with hearing instability.
- Identification of subtype-specific immune cell distributions
- Identification of immune profiles as well as immune cell transcriptional profiles related to hearing fluctuation
- Keywords
- Biomarkers, immunoprofiling, Auditory
- Medical subject headings
- Meniere Disease
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| 1/All Patients | Documented hearing instability |
What is being measured
The primary outcome is the main question the study is designed to answer.
Phenomic features
To identify phenotype features associated with hearing fluctuation
Measured: Day 90, 180, 270, 360, and 450, respectively +/- 14 days
Secondary outcomes (1)
Subdivisions of phenomic features
To Identify a variety of phenomic features of patients with HI that allow assignment to clinical subdivisions.
Measured: Day 90, 180, 270, 360, and 450, respectively +/- 14 days
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted19 Mar 2021
- Study start17 Aug 2021
- Primary completion31 Dec 2030 (estimated)
- Study completion31 Dec 2030 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (1)Papers the sponsor has linked to this study.
- Other trials for Meniere's DiseaseStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 210 participants (estimated)
Sponsor and investigators
- Lead sponsor
- National Institute on Deafness and Other Communication Disorders (NIDCD)
- Sponsor type
- US National Institutes of Health
- Responsible party
- Sponsor
- Investigators
- Michael Hoa, M.D., Principal investigator, National Institute on Deafness and Other Communication Disorders (NIDCD)
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04806282
- Sponsor's protocol ID
- 10000141
- Other IDs
- 000141-DC
- Status last verified
- 25 Jun 2026
- First submitted
- 18 Mar 2021
- Last updated
- 29 Jun 2026
- Results posted
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
Publications
Telischi J, Strepay D, Li B, Chisholm J, Cheng H, Brewer C, Hsu LY, Butman J, Hoa M. MRI-based measurement of inner ear fluids reveals increased endolymph volume variability in patients with endolymphatic hydrops and hearing instability. Sci Rep. 2025 Jul 2;15(1):22560. doi: 10.1038/s41598-025-06083-w.
PubMed 40596438
Study at a glance
From the registry record, last updated by the sponsor on 29 Jun 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Deep Phenotyping of Hearing Instability Disorders: Cohort Establishment, Biomarker Identification, Development of Novel Phenotyping Measures, and Discovery of Therapeutic Targets
- ClinicalTrials.gov ID
- NCT04806282
- Acronym / sponsor ID
- 10000141
- Keywords
- Biomarkers, immunoprofiling, Auditory
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 210 participants (estimated)
- Lead sponsor
- National Institute on Deafness and Other Communication Disorders (NIDCD)US National Institutes of Health
- Study start
- 17 Aug 2021 (actual)
- Primary completion
- 31 Dec 2030 (estimated)
- Study completion
- 31 Dec 2030 (estimated)
- First posted
- 19 Mar 2021 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Background:
Disorders 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.
Objective:
To 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.
Eligibility:
Adults ages 18-80 who have symptoms consistent with possible HI.
Design:
Participants will be screened with a medical and hearing history and medical record review.
Participants will have physical exams. Their head and neck will be examined. They will have blood drawn.
Participants will have hearing tests. They will wear headphones or foam earplugs. They will listen to different tones. They may describe what they hear.
Participants 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.
Participants 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.
Participation will last up to 15 months.
...
Detailed description
Study Description:
Disorders of hearing instability remain poorly characterized, phenotyped and ineffectively treated, and may result in sudden changes in hearing. These disorders include, but are not limited to, sudden sensorineural hearing loss (SSNHL) as well as hearing fluctuation, including, but not limited to, autoimmune inner ear disease (AIED), Meniere s disease (MD) and enlarged vestibular aqueduct syndrome (EVAS). While this group of disorders is likely to be clinically and etiologically heterogeneous, a common feature is fluctuation of hearing thresholds and speech understanding as measured by word recognition scores (WRS). This protocol seeks to ascertain a cohort of patients with hearing fluctuation to correlate these main phenotypic features with other phenomic data including audiometric indicators of endolymphatic hydrops as well as radiologic and immunologic biomarkers of inflammation over time. The overall hypothesis is that phenomic data will enable stratification of the phenotype of patients with hearing instability disorders. Healthy volunteers will be recruited to establish the normative range of endolymph and perilymph on contrast-enhanced delayed FLAIR MRI to allow for quantitative computational analysis of contrastenhanced delayed FLAIR MRI images.
Objectives:
Primary objective: To develop a cohort of patients with hearing instability (HI).
Secondary objectives:
- To correlate evidence of HI with changes in phase-shift distortion product otoacoustic emissions (DPOAEs).
- To compare phase-shift DPOAEs to existing measures of auditory function including word recognition score (WRS), standard DPOAEs, and electrocochleography (ECochG) over time.
- To compare phase-shift DPOAEs to vestibular measures including cervical and ocular vestibular evoked myogenic potentials (VEMPs) over time.
- To correlate auditory and vestibular indicators of hearing instability and endolymphatic hydrops (EH) on MRI with quantitative differences in immunologic markers of inflammation over time.
- Establish the normative range of endolymph and perilymph volume on contrast-enhanced delayed FLAIR MRI to allow for quantitative computational analysis of contrast-enhanced delayed FLAIR MRI images
Exploratory objective:
- To stratify patients with hearing instability (HI) by differences in cytokine levels, which will inform the identification of clinical subtypes of HI and potentially identify therapeutic targets for future treatment with targeted agents.
- To perform transcriptional and immunoprofiling of PBMCs at time points associated with hearing fluctuation from phenotyped patient cohorts.
Endpoints:
Primary Endpoint: Identification of phenomic features associated with HI.
Secondary Endpoint:
(1) Identification of a variety of phenomic features of patients with HI that allow assignment to clinical subdivisions.
Exploratory Endpoints
- Identification of potential targets for therapeutics based on longitudinal immune/transcriptional profiling of patients with hearing instability.
- Identification of subtype-specific immune cell distributions
- Identification of immune profiles as well as immune cell transcriptional profiles related to hearing fluctuation
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| 1/All Patients | – | –Documented hearing instability |
Who may qualify
- Ages
- 18 to 80 years
- Sex
- All
- Healthy volunteers
- Accepted
- Age groups
- Adult, Older adult
- Study population
- This study will enroll up to a total of 150 patients with HI. The enrollment target is between 10-30 patients per clinical subtype for at least 2 clinical subtypes of HI (i.e., without or with EH, without or with significant immune cell, cytokine or FLAIR positivity). 20 cytokines/chemokines in patients with Meniere s disease that revealed statistically higher levels of IL-1Beta compared to healthy controls (Flook et al., 2019), a sample size of 40-50 patients would be required for 80% power to detect an immune subtype associated with IL-1Beta.
Key inclusion criteria (0 listed)
Key exclusion criteria (21 listed)
- Affected and unaffected individuals who meet any of the following criteria will be excluded from participation in this study:
- Presence of non-MRI compatible devices (cardiac pacemaker, meta<specific devices (e.g., cardiac pacemaker)
- Pregnancy or lactation
- Known allergic reactions to gadolinium
- Febrile illness within 2 weeks that could affect immune profiling*
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
Phenomic features
Time frame: Day 90, 180, 270, 360, and 450, respectively +/- 14 days
To identify phenotype features associated with hearing fluctuation
1 secondary outcome
Subdivisions of phenomic features
Time frame: Day 90, 180, 270, 360, and 450, respectively +/- 14 days
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 29 Jun 2026.
- Derived from this study: PubMed 40596438Telischi J, Strepay D, Li B, Chisholm J, Cheng H, Brewer C, Hsu LY, Butman J, Hoa M. MRI-based measurement of inner ear fluids reveals increased endolymph volume variability in patients with endolymphatic hydrops and hearing instability. Sci Rep. 2025 Jul 2;15(1):22560. doi: 10.1038/s41598-025-06083-w.
- NIH Clinical Center Detailed Web PageLink provided by the sponsor.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Michael Hoa, M.D. principal investigator
National Institute on Deafness and Other Communication Disorders (NIDCD)
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Meniere's Disease and Enlarged Vestibular Aqueduct Syndrome, run by National Institute on Deafness and Other Communication Disorders (NIDCD) at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 0 inclusion and 21 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 29 Jun 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Anna E Clements Centeno ((301) 451-1215, anna.clements@nih.gov), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Phenomic features (time frame: Day 90, 180, 270, 360, and 450, respectively +/- 14 days). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT04806282, which also lists 1 linked publication.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 29 Jun 2026.