Auditory Neural Function in Implanted Patients With Usher Syndrome

Completed · Not applicable

Conditions studied: Usher Syndrome, Cochlear Implantation

In brief

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.

Key facts

Study ID

NCT04906135

Run by

Ohio State University

People needed

29

Starts

2021-10-01

Expected to finish

2025-07-11

Last updated by the study team

2025-07-29

Who can join

Age: 1 and older, up to 85. Sex: any. Healthy volunteers: not accepted.

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

Where it is running

• Boston children's Hospital — Boston, Massachusetts, United States
• Gina Hounam — Columbus, Ohio, United States
• The Ohio State University — Columbus, Ohio, United States
• Vanderbilt University Medical Center — Nashville, Tennessee, United States

Full record on ClinicalTrials.gov

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