Hearing and Balance Disorders in Peripheral Neuropathy

April 21, 2023 updated by: Nemours Children's Clinic
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.

Study Overview

Status

Terminated

Conditions

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 Type

Observational

Enrollment (Actual)

1

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

  • United States
    • Delaware
      • Wilmington, Delaware, United States, 19803
        • Nemours

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

  • Child
  • Adult

Accepts Healthy Volunteers

No

Sampling Method

Non-Probability Sample

Study Population

Children and young adults between the ages of 5 and 21 years diagnosed with Charcot-Marie-Tooth will be included.

Description

Inclusion Criteria:

  • Individuals diagnosed with Charcot-Marie Tooth

Exclusion Criteria:

-

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Prevalence of auditory disorders in children diagnosed with Charcot-Marie-Tooth Characteristics.
Time Frame: 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 years
Prevalence of vestibular disorders in children diagnosed with Charcot-Marie-Tooth
Time Frame: 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
2 years
Progression of hearing loss
Time Frame: 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.
2 years

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Nemours Children's Clinic

Investigators

  • Principal Investigator: Thierry Morlet, PhD, Nemours

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

June 1, 2018

Primary Completion (Actual)

March 10, 2023

Study Completion (Actual)

March 10, 2023

Study Registration Dates

First Submitted

June 25, 2018

First Submitted That Met QC Criteria

April 21, 2023

First Posted (Actual)

April 25, 2023

Study Record Updates

Last Update Posted (Actual)

April 25, 2023

Last Update Submitted That Met QC Criteria

April 21, 2023

Last Verified

October 1, 2018

More Information

Terms related to this study

Additional Relevant MeSH Terms

Other Study ID Numbers

  • TM001

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

Clinical Trials on Charcot-Marie-Tooth Disease

Search Similar Trials

Sponsors and Collaborators

Medical Conditions

Drug Interventions

CROs by country

CROs in Serbia

Conditions

Rare Diseases

Drug Interventions

Dietary Supplements

Sponsor/Collaborators

Locations

3
Subscribe