Deep Phenotyping of Hearing Instability Disorders: Cohort Establishment Biomarker Identification Development of Novel Phenotyping Measures and Discovery of Therapeutic Targets

  • End date
    Dec 31, 2030
  • participants needed
  • sponsor
    National Institute on Deafness and Other Communication Disorders (NIDCD)
Updated on 22 July 2021



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.


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.


Adults ages 18 65 who have symptoms consistent with possible HI.


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.




Deep Phenotyping of Hearing Instability Disorders: Cohort Establishment, Biomarker Identification, Development of Novel Phenotyping Measures and Discovery of Therapeutic Targets

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.


Primary objective: To develop a cohort of patients with hearing instability (HI).

Secondary objectives:

  1. To correlate evidence of HI with changes in phase-shift distortion product otoacoustic emissions (DPOAEs).
  2. To compare phase-shift DPOAEs to existing measures of auditory function including word recognition score (WRS), standard DPOAEs, and electrocochleography (ECochG) over time.
  3. To compare phase-shift DPOAEs to vestibular measures including cervical and ocular vestibular evoked myogenic potentials (VEMPs) over time.
  4. 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.

Exploratory objective:

  1. 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.
  2. To perform transcriptional and immunoprofiling of PBMCs at time points associated with hearing fluctuation from phenotyped patient cohorts.

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

  1. Identification of potential targets for therapeutics based on longitudinal immune/transcriptional profiling of patients with hearing instability.
  2. Identification of subtype-specific immune cell distributions
  3. Identification of immune profiles as well as immune cell transcriptional profiles related to hearing fluctuation

Study Population:

This study will ascertain patients who reside within the United States and exhibit HI. Subjects will undergo an informed consent process . Patients of all genders and all ethnicities will be recruited.

Description of Sites/Facilities Enrolling Participants:

This will be a single site study that will be conducted at the NIH Clinical Center.

Study Duration:

10 years

Participant Duration:

15 months

Condition Autoimmune Inner Ear Disease, Meniere s Disease, Enlarged Vestibular Aqueduct Syndrome
Clinical Study IdentifierNCT04806282
SponsorNational Institute on Deafness and Other Communication Disorders (NIDCD)
Last Modified on22 July 2021


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If you are confirmed eligible after full screening, you will be required to understand and sign the informed consent if you decide to enroll in the study. Once enrolled you may be asked to make scheduled visits over a period of time.

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