Ignite Creation Date:
2024-05-06 @ 3:55 PM
Last Modification Date:
2024-10-26 @ 2:00 PM
Study NCT ID:
NCT04806282
Status:
RECRUITING
Last Update Posted:
2024-03-28
First Post:
2021-03-18
Brief Title:
Deep Phenotyping of Hearing Instability Disorders Cohort Establishment Biomarker Identification Development of Novel Phenotyping Measures and Discovery of Therapeutic Targets
Sponsor:
National Institute on Deafness and Other Communication Disorders NIDCD
Organization:
National Institutes of Health Clinical Center CC
Study Overview
Official Title:
Deep Phenotyping of Hearing Instability Disorders Cohort Establishment Biomarker Identification Development of Novel Phenotyping Measures and Discovery of Therapeutic Targets
Status:
RECRUITING
Status Verified Date:
2024-10-18
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief 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
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
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:
Title
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 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
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
5 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
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
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
1 Identification of potential targets for therapeutics based on longitudinal immunetranscriptional 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
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 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
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
5 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
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
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
1 Identification of potential targets for therapeutics based on longitudinal immunetranscriptional 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 Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None
Secondary IDs
| Secondary ID | Type | Domain | Link |
|---|---|---|---|
| 000141-DC | None | None | None |