Enlarged Vestibular Aqueduct Registry

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Brief Title

Enlarged Vestibular Aqueduct Registry

Official Title

Enlarged Vestibular Aqueduct Patient Registry at University Hospitals Rainbow Babies and Children's Hospital

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
    

Detailed Description

      Study design

      The present study would employ a prospective questionnaire and retrospective chart review to
      develop a centralized registry of EVA patient information. The questionnaire would be
      administered using custom-designed software and an internet web site that uses secure
      information transfer protocol and encryption. Given authorization for the release of
      Protected Health Information (PHI), respective clinicians would be contacted for medical
      records that would be entered alongside corresponding self-reported data. Interested
      researchers will be allowed to obtain data, given their respective institution's
      Institutional Review Board (IRB) approval and documentation thereof. The study would
      initially continue for four years, and continue based on enrollment numbers and data
      requests.

      Study procedure

      Collection methods would be chosen by the participant, and can include one or both of a
      questionnaire and/or Authorization for the Release of PHI and relevant medical records
      retrieval.

      Implementation of this project would involve three separate components: participant data
      collection and interaction, (if authorized) PHI retrieval, and data provision to interested
      researchers.

      Questionnaire Data Collection

      Upon providing an electronic signature, participants will be taken to a page where they can
      begin the questionnaire. Patients who begin the questionnaire will be presented with web
      pages asking for history relating to EVA. Please note that these web pages all offer the
      option to exit the questionnaire. Patients who elect to provide the Authorization for the
      Release of PHI will be provided the form to print and mail to the study center (University
      Hospitals Case Medical Center).

      Clinical Data Retrieval and Data Quality

      For those participants who do provide Authorization for the Release of PHI, the clinicians
      whose information was included on the authorization form will be contacted and information
      requested. This process would involve a transfer of the Authorization for the Release of PHI
      in one of two ways: either by mail, or via fax. Clinicians who are willing to provide
      information will be instructed to transmit, via fax or mail, health data to the
      otolaryngology department at University Hospitals.

      Health data that is received will be reviewed and input into forms similar to those that the
      patients fill. Such a mechanism will provide redundancy to data provided by participants,
      thus ensuring the quality of this data.

      Providing Data to Researchers

      The data that is collected will cover a range of topics related to EVA, and will be held with
      the aim of providing subsets of the data to interested researchers who have IRB approval from
      their own institutions. A form on the website will list fields for interested researchers to
      complete, including upload of an IRB protocol. Interested researchers would also be required
      to complete a Data Use Agreement.

      Informed consent procedure

      Consent will be obtained electronically and stored separately from patient data, such that
      identifiable information will only be linked to healthcare data using a patient
      identification (ID) number.

      Potential participants who elect to visit the EVAR website would be presented with an option
      to participate in the EVAR. From there, participants would be directed to an informed consent
      web page that contains all of the same information that would be in a written informed
      consent, a blank version of which is provided in attached documents. The web site would have
      a field where participants can type their name and the date to signify consent, and another
      field where participants (if applicable) can type their name and the date to signify assent.
      Completion of such a field would be considered equivalent to written consent, and is referred
      to in this document as an "electronic signature".

      Only after completing an electronic consent would potential participants be allowed to share
      questionnaire or authorization information. This means that before seeing the questionnaire
      or authorization for the release of protected health information, the participant would be
      required to provide electronic consent.

      The consent document itself would be provided to all potential participants, in the form of a
      downloadable Portable Document Format (PDF) file and as a separate html web page that does
      not have a section where consent may be entered. This twofold approach will ensure that
      potential participants can both view the consent in their web browser using the html version,
      and print the PDF consent in the format that it was accepted by the University Hospitals IRB.

      Authorization to Release PHI Procedure

      After providing an electronic signature for the informed consent, participants will have the
      opportunity to complete an Authorization to Release PHI form. Please see attachments for an
      example of this form.

      Participants who provide the information necessary to fill this form would be given the
      option of printing the form for themselves, or receiving the form in the mail with return
      postage. In either case, the form would be populated with participant-provided data and
      provided such that only a participant signature and date would be needed to complete the
      form. For participants who elect to receive the form in the mail, two copies would be
      provided to allow the participant to keep one. In either case, participants would be asked to
      sign and date the document and mail it to the Otolaryngology Department at University
      Hospitals Case Medical Center (UHCMC). Original documents would be copied and, along with the
      copy, stored in a locked cabinet that is in a normally locked office on the UHCMC campus.

      Data security

      Data transfer will occur using secure internet protocol, using computers physically located
      on a University Hospitals campus.Participant information will be maintained in a secure,
      HIPAA-compliant server located on a University Hospitals campus. Only database developers and
      study personnel will have access to this data. For patients who provide Authorization to
      release PHI, data will only be received via fax, mail, or through University Hospitals email,
      and will be stored on secure University Hospitals servers.

      Identifiable information will be protected from improper use or disclosure by storing data on
      a secure server located on the University Hospitals campus. When submitting data over the
      internet, participants will be connecting directly with University Hospitals servers using
      secure https protocol such that there will be no data access to anyone outside of the
      participant and server.

      For patients who provide Authorization to release PHI, data will only be received via fax,
      mail, or through University Hospitals email.

      Identifiable information will not be reused or disclosed to any other person or entity
      outside University Hospitals other than those identified in the protocol, except as required
      by law, for authorized oversight of this research study, or as specifically approved for use
      in another study by an IRB. Any access to the website or data itself will be reviewed on a
      monthly basis.

      Statistical analysis and sample size calculations

      Data on such a wide variety of patient experience measures is unavailable to determine effect
      sizes and subsequent minimum sample sizes, particularly for an end-point with likely high
      variability such as self-reported hearing loss. For this reason, and to ensure greatest
      possible discrimination of effects, the EVA registry will be open to as many people as are
      willing to enroll. Previously, a mailing list for interested participants was created, and 53
      people signed up over the course of three weeks. Sizes of online special interest groups
      (such as the Yahoo group, http://health.groups.yahoo.com/group/LVAS) have between 1000 and
      3000 registered users. Given mailing list interest, and other online community sizes, the
      investigators expect 300 participants to enroll.

      Statistical methods will be left to researchers who request this data, but the data has been
      organized to facilitate descriptive and model-based statistical characterization of the EVA
      population. Hearing loss progression may be evaluated in multiple dimensions, particularly in
      relation to events such as head trauma or flying. For example, such categories of treatment
      (head trauma, flying) are amenable to analysis with respect to outcomes (hearing loss
      progression) by such tests as the Chi-square test. Other researchers may be interested in
      understanding the time taken to diagnose EVA, in which case the interval between date of
      birth and date of first imaging study can be modeled with respect to other available data
      points.

      Data elements to be collected:

      Part 1 - Demographics:

        -  Relationship of respondent to patient

        -  Patient:

        -  Date of Birth

        -  Gender

        -  City

        -  State

        -  Guardian:

        -  Date of Birth

        -  Gender

        -  City

        -  State

        -  Contact:

        -  Email

        -  Phone

      Part 2 - Initial Findings

        -  Age at diagnosis of hearing loss

        -  Diagnostic method

        -  Audiogram, Otoacoustic Emissions Testing, Auditory Brainstem Response, or other?

        -  Age at first imaging study

        -  First image type

        -  Computed Tomography (CT) or Magnetic Resonance Imaging (MRI)?

        -  Imaging results

        -  Which ear has EVA

        -  Left, right, or both

        -  Diameter of the vestibular aqueduct

      Part 3 - Progression

        -  Has there been progression of hearing loss?

        -  Date and age of progression of hearing loss diagnosis - also, commands?

        -  Events that contributed to hearing loss, open text box

        -  Did the family notice hearing loss or its event

        -  Was the hearing loss recognized coincidentally on a hearing screen/routine audiogram?

        -  Was the patient treated for hearing loss?

        -  Check boxes for treatment with steroids or other

      Part 4 - Trauma and Flying

        -  Has the patient had any documented head trauma?

        -  Date and age of trauma - also, commands?

        -  Was there any associated hearing loss?

        -  What additional testing was performed, if any?

        -  Was additional imaging done?

        -  Has the patient flown?

        -  Was there any subjective worsening of hearing?

        -  Was additional testing required after flying?

      Part 5 - Family History

        -  Is there any family history of hearing loss?

        -  Is there any family history of EVA?

        -  Has any genetics testing been completed?

        -  If so, what type of testing?

        -  At what age?

        -  What were the results?

        -  Does the patient have Pendred syndrome?

      Part 6 - Aids

        -  Does the patient receive speech therapy?

        -  If so, how often?

        -  At what age did speech therapy begin?

        -  Does the patient use a Frequency Modulation (FM) system at school?

        -  Does the patient use a hearing aid?

        -  If so, in which ear?

        -  Starting at what year of age?

        -  Does the patient have a cochlear implant?

        -  If so, in which ear?

        -  What year was the operation performed?

        -  If two implants, were they done at the same time?

        -  At what age was the operation performed?

        -  How long after diagnosis of hearing loss was the operation performed?

        -  How long after diagnosis of EVA was the operation performed?

      Part 7 - Vertigo

        -  Has the patient ever had balance problems or vertigo?

        -  If so, how frequently?

        -  How severe?

        -  Are there triggering activities?

        -  At what age was the first episode?

        -  How long after diagnosis did vertigo occur?
    


Study Type

Observational [Patient Registry]


Primary Outcome

Self-reported lifelong hearing loss progression, subjectively as a questionnaire response and as hearing thresholds (dB) change in clinical documentation.

Secondary Outcome

 Age in years at diagnosis of hearing loss, subjectively as a questionnaire response and in clinical documentation.

Condition

Enlarged Vestibular Aqueduct


Study Arms / Comparison Groups

 Diagnosed with EVA
Description:  Self-reported Enlarged Vestibular Aqueduct (EVA), or, when available, radiological diagnosis of EVA will be used to define the cohort.

Publications

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information



Estimated Enrollment

300

Start Date

June 2016

Completion Date

June 2023

Primary Completion Date

June 2020

Eligibility Criteria

        Inclusion Criteria:

          -  Self-reported diagnosis of Enlarged Vestibular Aqueduct OR

          -  Radiological diagnosis of Enlarged Vestibular Aqueduct

        Exclusion criteria:

          -  None
      

Gender

All

Ages

N/A - 90 Years

Accepts Healthy Volunteers

Accepts Healthy Volunteers

Contacts

Todd D Otteson, MD, MPH, 7403905102, [email protected]tals.org

Location Countries

United States

Location Countries

United States

Administrative Informations


NCT ID

NCT02798783

Organization ID

04-16-06


Responsible Party

Sponsor-Investigator

Study Sponsor

Todd Otteson, MD, MPH, FAAP, FACS


Study Sponsor

Todd D Otteson, MD, MPH, Principal Investigator, University Hospitals Cleveland Medical Center


Verification Date

April 2019