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Hearing and Balance Disorders in Peripheral Neuropathy

Sponsor
Nemours Children's Clinic (Other)
Overall Status
Terminated
CT.gov ID
NCT05827419
Collaborator
(none)
1
Enrollment
1
Location
57.3
Actual Duration (Months)
0
Patients Per Site Per Month

Study Details

Study Description

Brief Summary

The long term goal of this proposal is to precisely characterize the auditory and vestibular abilities of children with Charcot-Marie-Tooth (CMT) and how these abilities evolve during the progression of the disorder. This information will be used to refine the management methods for hearing loss and vestibular disorders in these patients. Given that the phenotypic severity is variable within the CMT patient population, we predict that not all CMT patients will present with auditory and vestibular dysfunction. We will therefore collect specimens (i.e., buccal swabs and saliva) from study participants so that their DNA can be isolated and used to determine the genetic basis for auditory and vestibular dysfunction in peripheral neuropathies.

Condition or Disease Intervention/Treatment Phase

    Detailed Description

    Aim #1: Evaluate the prevalence of auditory and vestibular disorders in children diagnosed with Charcot-Marie-Tooth.

    We hypothesize that a significant number of children with Charcot-Marie-Tooth (CMT) will develop auditory and vestibular neuropathies, due to the progression of the disorder. We will examine the characteristics of auditory and vestibular abilities of children and how these relate to the type of CMT.

    Aim #2: Examine the progression of hearing and vestibular abilities during the course of the disorder.

    We hypothesize that the hearing and vestibular abilities of some children with CMT will deteriorate with time. We will repeat the auditory and vestibular testing on a yearly basis to precisely describe the progression of these abilities.

    Study Design

    Study Type:
    Observational
    Actual Enrollment :
    1 participants
    Observational Model:
    Case-Only
    Time Perspective:
    Prospective
    Official Title:
    Hearing and Balance Disorders in Peripheral Neuropathy
    Actual Study Start Date :
    Jun 1, 2018
    Actual Primary Completion Date :
    Mar 10, 2023
    Actual Study Completion Date :
    Mar 10, 2023

    Outcome Measures

    Primary Outcome Measures

    1. Prevalence of auditory disorders in children diagnosed with Charcot-Marie-Tooth Characteristics. [2 years]

      We will evaluate how many children with CMT have present hearing loss. The presence of hearing loss will be based on the results of the following tests (passed or failed): otoacoustic emissions, auditory brainstem responses, auditory event related potentials, audiometry and speech perception

    2. Prevalence of vestibular disorders in children diagnosed with Charcot-Marie-Tooth [2 years]

      We will evaluate how many children with CMT have present vestibular disorder. The presence of vestibular disorder will be based on the results of the following test (passed or failed): video head impulse test

    3. Progression of hearing loss [2 years]

      We will follow subjects longitudinally to evaluate the progression of hearing loss. The degree of hearing loss will be monitor over time to evaluate if changes are noticed. All tests listed in Outcome 1 will be repeated and compared to initial values to define the presence or absence of changes in the degree of hearing loss.

    Eligibility Criteria

    Criteria

    Ages Eligible for Study:
    5 Years to 21 Years
    Sexes Eligible for Study:
    All
    Accepts Healthy Volunteers:
    No
    Inclusion Criteria:
    • Individuals diagnosed with Charcot-Marie Tooth
    Exclusion Criteria:

    Contacts and Locations

    Locations

    Site City State Country Postal Code
    1 Nemours Wilmington Delaware United States 19803

    Sponsors and Collaborators

    • Nemours Children's Clinic

    Investigators

    • Principal Investigator: Thierry Morlet, PhD, Nemours

    Study Documents (Full-Text)

    None provided.

    More Information

    Publications

    None provided.
    Responsible Party:
    Nemours Children's Clinic
    ClinicalTrials.gov Identifier:
    NCT05827419
    Other Study ID Numbers:
    • TM001
    First Posted:
    Apr 25, 2023
    Last Update Posted:
    Apr 25, 2023
    Last Verified:
    Oct 1, 2018
    Studies a U.S. FDA-regulated Drug Product:
    No
    Studies a U.S. FDA-regulated Device Product:
    No
    Additional relevant MeSH terms:
    Tooth Diseases
    Peripheral Nervous System Diseases
    Charcot-Marie-Tooth Disease
    Nerve Compression Syndromes
    Hereditary Sensory and Motor Neuropathy

    Study Results

    No Results Posted as of Apr 25, 2023