Enlarged Vestibular Aqueduct Registry
EVAR
Enlarged Vestibular Aqueduct Patient Registry at University Hospitals Rainbow Babies and Children's Hospital
1 other identifier
observational
165
1 country
1
Brief Summary
People with the congenital anatomical malformation known as "Enlarged Vestibular Aqueduct" (EVA) may experience decline in hearing ability. This is an online registry to gather patient experiences with EVA, with the goal of providing future researchers with the information necessary to better evaluate and make recommendations for patients with EVA. Data will be obtained from volunteers who enroll and submit their information on a website, https://eva.uhhospitals.org, and a limited data set may be provided to researchers interested in analyzing repository data. The limited data set would not contain any identifiable information except for ages, dates such as date of diagnosis, or city/state of residence. EVA Research Project Website: http://rainbow.org/EVAResearch EVA Patient Registry Website: https://eva.uhhospitals.org
Trial Health
Trial Health Score
Automated assessment based on enrollment pace, timeline, and geographic reach
participants targeted
Target at P50-P75 for all trials
Started Jun 2021
1 active site
Health score is calculated from publicly available data and should be used for screening purposes only.
Trial Relationships
Click on a node to explore related trials.
Study Timeline
Key milestones and dates
First Submitted
Initial submission to the registry
June 3, 2016
CompletedFirst Posted
Study publicly available on registry
June 14, 2016
CompletedStudy Start
First participant enrolled
June 29, 2021
CompletedPrimary Completion
Last participant's last visit for primary outcome
June 2, 2023
CompletedStudy Completion
Last participant's last visit for all outcomes
June 2, 2023
CompletedDecember 6, 2023
November 1, 2023
1.9 years
June 3, 2016
November 29, 2023
Conditions
Keywords
Outcome Measures
Primary Outcomes (1)
Self-reported lifelong hearing loss progression, subjectively as a questionnaire response and as hearing thresholds (dB) change in clinical documentation.
Patients will be asked about the progression of hearing loss, including the date and age at which hearing loss progressed. This will be clinically corroborated by evaluating hearing threshold changes via clinical documentation (e.g. audiogram, OAE, ABR)
Through study completion, an average of one year
Secondary Outcomes (7)
Age in years at diagnosis of hearing loss, subjectively as a questionnaire response and in clinical documentation.
Through study completion, an average of one year
Age in years at first CT or MRI used to diagnose Enlarged Vestibular Aqueduct, subjectively as a questionnaire response and in clinical documentation.
Through study completion, an average of one year
Hearing loss progression after flying on an airplane, subjectively as a questionnaire response and as hearing thresholds (dB) change in clinical documentation
Within four weeks of flight travel
Use of hearing aids, subjectively as a questionnaire response or as recorded in prior clinical documentation.
Through study completion, an average of one year
Hearing loss progression after documented head trauma, subjectively as a questionnaire response or as recorded in prior clinical documentation.
Within four weeks of head trauma
- +2 more secondary outcomes
Study Arms (1)
Diagnosed with EVA
Self-reported Enlarged Vestibular Aqueduct (EVA), or, when available, radiological diagnosis of EVA will be used to define the cohort.
Eligibility Criteria
The study population includes those people who report having a diagnosis of Enlarged Vestibular Aqueduct. The cohort will be defined by voluntary submission of information to a website, eva.uhhospitals.org.
You may qualify if:
- Self-reported diagnosis of Enlarged Vestibular Aqueduct OR
- Radiological diagnosis of Enlarged Vestibular Aqueduct
You may not qualify if:
- None
Contact the study team to confirm eligibility.
Sponsors & Collaborators
Study Sites (1)
University Hospitals Cleveland Medical Center
Cleveland, Ohio, 44040, United States
Related Publications (5)
Valvassori GE, Clemis JD. The large vestibular aqueduct syndrome. Laryngoscope. 1978 May;88(5):723-8. doi: 10.1002/lary.1978.88.5.723.
PMID: 306012BACKGROUNDGopen Q, Zhou G, Whittemore K, Kenna M. Enlarged vestibular aqueduct: review of controversial aspects. Laryngoscope. 2011 Sep;121(9):1971-8. doi: 10.1002/lary.22083. Epub 2011 Aug 16.
PMID: 22024854BACKGROUNDGriffith AJ, Wangemann P. Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, and mouse models. Hear Res. 2011 Nov;281(1-2):11-7. doi: 10.1016/j.heares.2011.05.009. Epub 2011 Jun 6.
PMID: 21669267BACKGROUNDMadden C, Halsted M, Benton C, Greinwald J, Choo D. Enlarged vestibular aqueduct syndrome in the pediatric population. Otol Neurotol. 2003 Jul;24(4):625-32. doi: 10.1097/00129492-200307000-00016.
PMID: 12851556BACKGROUNDDewan K, Wippold FJ 2nd, Lieu JE. Enlarged vestibular aqueduct in pediatric sensorineural hearing loss. Otolaryngol Head Neck Surg. 2009 Apr;140(4):552-8. doi: 10.1016/j.otohns.2008.12.035.
PMID: 19328346BACKGROUND
Related Links
MeSH Terms
Conditions
Condition Hierarchy (Ancestors)
Study Officials
- PRINCIPAL INVESTIGATOR
Todd D Otteson, MD, MPH
University Hospitals Cleveland Medical Center
- PRINCIPAL INVESTIGATOR
Cliff Megerian, MD
University Hospitals Cleveland Medical Center
- STUDY DIRECTOR
Benjamin A Reinthal
University Hospitals Cleveland Medical Center
- STUDY DIRECTOR
Mustafa S Ascha, MS
University Hospitals Cleveland Medical Center
Study Design
- Study Type
- observational
- Observational Model
- CASE ONLY
- Time Perspective
- PROSPECTIVE
- Target Duration
- 1 Year
- Sponsor Type
- OTHER
- Responsible Party
- SPONSOR
Study Record Dates
First Submitted
June 3, 2016
First Posted
June 14, 2016
Study Start
June 29, 2021
Primary Completion
June 2, 2023
Study Completion
June 2, 2023
Last Updated
December 6, 2023
Record last verified: 2023-11
Data Sharing
- IPD Sharing
- Will share
- Time Frame
- Data will be available one year after enrollment is to start, on June 2, 2017. The study would initially continue for four years, and continue based on enrollment numbers and data requests.
- Access Criteria
- Background information: * Corresponding researcher * Name * Title * Institution * Department * Address * Contact phone number * Contact email address * Principal Investigator * Name * Title * Institution * Department * Address * Contact phone number * Contact email address * Origin of the protocol (e.g. investigator-initiated, industry, federal agency, cooperative group, other) Study information: * Full title of study * Whether the research has IRB approval * Background/rationale/significance * Purpose/Hypothesis Document Upload: * Approved IRB Protocol Application * Signed CV for each investigator listed on the IRB application * Signed Data Use Agreement
A limited data set will be available to researchers who demonstrate IRB approval for a study that would require the use of data points collected by the Enlarged Vestibular Aqueduct Registry. This dataset will include information only in accordance with HIPAA definitions of a limited dataset, for example, excluding names and addresses. Data will be available one year after enrollment is to start, on June 2, 2017. The data may be obtained by submission of a request along with proof of investigator institutional review board approval, forms for which will be available through the registry web site.