Hearing and Balance Disorders in Peripheral Neuropathy

Charcot-Marie-Tooth Disease Clinical Trial

Official title:

Hearing and Balance Disorders in Peripheral Neuropathy

Clinical Trial Details — Status: Terminated

Administrative data

• NCT number: NCT05827419
• Other study ID #: TM001
• Status: Terminated
• Start date: June 1, 2018
• Est. completion date: March 10, 2023

Study information

• Verified date: October 2018
• Source: Nemours Children's Clinic
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Terminated
• Enrollment: 1
• Est. completion date: March 10, 2023
• Est. primary completion date: March 10, 2023
• Accepts healthy volunteers: No
• Gender: All
• Age group: 5 Years to 21 Years

Eligibility

Inclusion Criteria:

• Individuals diagnosed with Charcot-Marie Tooth Exclusion Criteria: -

Related Conditions & MeSH terms

• Charcot-Marie-Tooth Disease
• Hereditary Sensory and Motor Neuropathy
• Nerve Compression Syndromes
• Peripheral Nervous System Diseases
• Tooth Diseases

Locations

Country	Name	City	State
United States	Nemours	Wilmington	Delaware

Sponsors (1)

Lead Sponsor	Collaborator
Nemours Children's Clinic

Country where clinical trial is conducted

United States, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Prevalence of auditory disorders in children diagnosed with Charcot-Marie-Tooth Characteristics.	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
Primary	Prevalence of vestibular disorders in children diagnosed with Charcot-Marie-Tooth	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
Primary	Progression of hearing loss	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