Auditory Neural Function in Implanted Patients With Usher Syndrome

Brief Title

Auditory Neural Function in Implanted Patients With Usher Syndrome

Official Title

Auditory Neural Function in Implanted Patients With Usher Syndrome

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.
    

Detailed Description

      Usher syndrome (USH) is an autosomal recessive disorder characterized by hearing loss, visual
      impairment, and in some cases, vestibular dysfunction. It is the leading cause of hereditary
      deaf-blindness in humans. USH causes extensive degeneration in the cochlear nerve (CN),
      especially in CN fibers innervating the base of the cochlea. Whereas there is no treatment
      for arresting this degenerative process or for restoring visual loss, the restoration of
      auditory input is possible with cochlear implantation. Due to the progressive deterioration
      in vision, using visual cues for communication will eventually become impossible. Therefore,
      the importance of optimizing auditory inputs through cochlear implants (CIs) for patients
      with USH is paramount. However, patients with USH have much higher rates of neurological,
      mental, or behavioral disorders than the general CI patient population, which limits their
      ability to provide reliable behavioral responses or sufficient verbal descriptions of their
      auditory perception, especially for pediatric patients. In addition, optimal programming
      parameters for CI users with CN damage differ from those used in typical CI users due to
      declined CN responsiveness to electrical stimulation. As a result, the clinical programming
      process in implanted patients with USH can be extremely challenging. To date, auditory neural
      encoding of electrical stimulation in patients with USH has not been systematically
      evaluated. Consequently, the field lacks evidence-based practice guidelines for managing
      implanted patients with USH. For patients who cannot provide reliable feedback, clinicians
      rely on a "trial-and-error" approach for adjusting CI programming settings, which ultimately
      may not result in appropriate programming maps for individual patients. Therefore, there is
      an urgent need to develop objective clinical tools for optimizing CI settings for these
      patients. As the first step toward developing evidence-based practice for managing 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. Results of this study have high
      scientific significance because they will establish how CN degeneration affects neural
      encoding and processing of electrical stimulation, and identify tests that distinguish the
      loss of spiral ganglion neurons from the loss of peripheral axons. Results of this study also
      have high clinical significance because they will 1) lay the groundwork for developing
      effective, evidence-based clinical practice guidelines for managing patients with USH, and 2)
      yield an objective tool for assessing the site-specific electrode-neuron interface in all CI
      users, which is foundational for creating optimal programming maps for individual patients.
    


Study Type

Interventional


Primary Outcome

The electrically evoked compound action potential


Condition

Usher Syndrome

Intervention

Experimental manipulation of stimulation parameters

Study Arms / Comparison Groups

 Usher Syndrome
Description:  Adult and pediatric cochlear implant users with Usher syndrome

Publications

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information


Recruitment Status

Other

Estimated Enrollment

44

Start Date

October 1, 2021

Completion Date

March 2023

Primary Completion Date

December 2022

Eligibility Criteria

        Inclusion Criteria:

          -  Minimum of 6 months of listening experience with cochlear implant

          -  Diagnosed with Usher syndrome or idiopathic hearing loss

        Exclusion Criteria:

          -  Severe medical comorbidities

          -  Electrode malposition or migration as determined based on imaging results
      

Gender

All

Ages

1 Year - 85 Years

Accepts Healthy Volunteers

No

Contacts

Shuman He, MD, PhD, , 

Location Countries

United States

Location Countries

United States

Administrative Informations


NCT ID

NCT04906135

Organization ID

2018H0344

Secondary IDs

R21DC019458

Responsible Party

Principal Investigator

Study Sponsor

Ohio State University

Collaborators

 National Institute on Deafness and Other Communication Disorders (NIDCD)

Study Sponsor

Shuman He, MD, PhD, Principal Investigator, Ohio State University


Verification Date

November 2021