An observational study in Meniere's Disease, Enlarged Vestibular Aqueduct Syndrome and Autoimmune Inner Ear Disease, sponsored by National Institute on Deafness and Other Communication Disorders (NIDCD). Recruiting at 1 site in United States. Open to participants aged 18 Years to 80 Years, including healthy volunteers. Per ClinicalTrials.gov, last updated 2026-06-29.
Sponsored by National Institute on Deafness and Other Communication Disorders (NIDCD) · Observational
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.
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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:
Exploratory objective:
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
74 studies on the registry are indexed under Meniere Disease; 12 are open to participants now.
This study's planned enrollment of 210 is above the median of 100 across 10 observational studies indexed under Meniere Disease.
Browse Meniere Disease studies →National Institute on Deafness and Other Communication Disorders (NIDCD) is the lead sponsor of 37 studies on the registry; 7 are open to participants now.
Counted across the registry records on this site, refreshed daily.
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.
Affected Adults
In order to be eligible to participate in this study as a subject with HI, an individual must meet all of the following criteria:
All genders, aged 18-80 years
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 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.
Unaffected Adults
In order to be eligible to participate in this study as a healthy volunteer, an individual must meet all of the following criteria:
EXCLUSION CRITERIA:
Affected and unaffected individuals who meet any of the following criteria will be excluded from participation in this study:
History or diagnosis of a central nervous system disorder, including but not limited to:
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.
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Judged by the study doctor to matter for your health or for the study. It is a doctor’s decision, so ask the study team if you are unsure whether something counts.
Able to have children. For women this usually means from the first period until menopause, unless permanently unable to through surgery; for men, unless permanently sterile. Read who the line is about — participants, women, men, or their partners.
Criteria about it usually ask for contraception during the study and for a time after it.
The American Psychiatric Association’s manual of mental health diagnoses. DSM-5 (2013, revised as DSM-5-TR in 2022) replaced DSM-IV, and some studies still name the older edition. “Meets DSM criteria for …” means diagnosed by that edition’s diagnostic criteria, usually by a clinician in an interview.
Informed consent
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Clinically significant
Judged by the study doctor to matter for your health or for the study. It is a doctor’s decision, so ask the study team if you are unsure whether something counts.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Reproductive potential
Able to have children. For women this usually means from the first period until menopause, unless permanently unable to through surgery; for men, unless permanently sterile. Read who the line is about — participants, women, men, or their partners.
Criteria about it usually ask for contraception during the study and for a time after it.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
DSM diagnostic criteria
The American Psychiatric Association’s manual of mental health diagnoses. DSM-5 (2013, revised as DSM-5-TR in 2022) replaced DSM-IV, and some studies still name the older edition. “Meets DSM criteria for …” means diagnosed by that edition’s diagnostic criteria, usually by a clinician in an interview.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Documented hearing instability
Phenomic features
To identify phenotype features associated with hearing fluctuation
Time frame: Day 90, 180, 270, 360, and 450, respectively +/- 14 days
Subdivisions of phenomic features
To Identify a variety of phenomic features of patients with HI that allow assignment to clinical subdivisions.
Time frame: Day 90, 180, 270, 360, and 450, respectively +/- 14 days
Started Aug 2021 (actual). Primary completion is estimated for Dec 2030, 112 months after the start.
How these figures are worked out, and which studies are left out of them.
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National Institute on Deafness and Other Communication Disorders (NIDCD) →