Auditory Neural Function in Implanted Patients With Usher Syndrome

NCT04906135 | Completed

• 2 other identifiers: 2018H0344R21DC019458
• Study Type: interventional
• Target: 29
• Locations: 1 country
• Sites: 4

Brief Summary

Usher syndrome (USH) causes extensive degeneration in the cochlear nerve (CN), especially in CN fibers innervating the base of the cochlea. As the first step toward developing evidence-based practice for managing implant patients with USH, this study evaluates local neural health, as well as the neural encoding of temporal and spectral cues at the CN in implanted patients with USH. Aim 1 will determine local CN health in patients with USH by assessing the sensitivity of the electrically evoked compound action potential to changes in interphase gap and pulse polarity. Aim 2 will determine group differences in neural encoding of temporal and spectral cues at the CN between patients with USH and patients with idiopathic hearing loss. Aim 3 will use supervised machine learning techniques to develop an objective tool for assessing the electrode-neuron interface at individual electrode locations.

Conditions

Usher Syndrome; Cochlear Implantation

Keywords

Auditory Electrophysiology

Outcome Measures

Primary Outcomes (1)

• The electrically evoked compound action potential

  The primary outcome measure is the neural response generated by the electrically-stimulated cochlear nerve, which can be considered a health-related, biomedical outcome.

  Outcome measures will be obtained from Day 1 through study completion, an average of 2 years.

Study Arms (2)

Usher Syndrome

EXPERIMENTAL

Adult and pediatric cochlear implant users with Usher syndrome

Other: Experimental manipulation of stimulation parameters

Idiopathic Hearing Loss

ACTIVE COMPARATOR

Adult and pediatric cochlear implant users with idiopathic hearing loss

Other: Experimental manipulation of stimulation parameters

Interventions

Experimental manipulation of stimulation parameters OTHER

This translational mechanistic study involves changing stimulation parameters (i.e., experimental manipulation) for measuring the electrically evoked compound action potential in order to understand the pathophysiology of the auditory system in patients with Usher syndrome.

Idiopathic Hearing Loss; Usher Syndrome

Eligibility Criteria

• Age: 1 Year - 85 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

• Minimum of 6 months of listening experience with cochlear implant
• Diagnosed with Usher syndrome or idiopathic hearing loss

You may not qualify if:

• Severe medical comorbidities
• Electrode malposition or migration as determined based on imaging results

Sponsors & Collaborators

• Ohio State University: lead
• National Institute on Deafness and Other Communication Disorders (NIDCD): collaborator

Study Sites (4)

Boston children's Hospital

Boston, Massachusetts, 02453, United States

Location

Gina Hounam

Columbus, Ohio, 43205-2664, United States

Location

The Ohio State University

Columbus, Ohio, 43210, United States

Location

Vanderbilt University Medical Center

Nashville, Tennessee, 37232-8718, United States

Location

MeSH Terms

Conditions

Usher Syndromes

Condition Hierarchy (Ancestors)

Deaf-Blind Disorders; Deafness; Hearing Loss; Hearing Disorders; Ear Diseases; Otorhinolaryngologic Diseases; Hearing Loss, Sensorineural; Sensation Disorders; Neurologic Manifestations; Nervous System Diseases; Blindness; Vision Disorders; Retinitis Pigmentosa; Retinal Dystrophies; Retinal Degeneration; Retinal Diseases; Eye Diseases; Abnormalities, Multiple; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Eye Diseases, Hereditary; Genetic Diseases, Inborn; Signs and Symptoms; Pathological Conditions, Signs and Symptoms

Study Officials

• Shuman He, MD, PhD

  Ohio State University

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: BASIC SCIENCE
• Intervention Model: PARALLEL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Professor

Study Record Dates

• First Submitted: May 24, 2021
• First Posted: May 28, 2021
• Study Start: October 1, 2021
• Primary Completion: March 31, 2025
• Study Completion: July 11, 2025
• Last Updated: July 29, 2025

Record last verified: 2025-07

Locations

US (4)