Implanting Auditory Brainstem Devices in Children Without Cochleae or Auditory Nerves

Auditory Brainstem Implant (ABI) in Children With No Cochleae or Auditory Nerves

This study is testing a special hearing device in children who can't hear at all due to problems with their ears or hearing nerves to see if it helps them hear better.

Quick facts

• Phase: Not applicable
• Study type: Interventional
• Enrollment: 20 (estimated)
• Ages: 18 Months to 21 Years
• Sex: All
• Sponsor: NYU Langone Health (Academic / other)
• Locations: 1 site (New York, New York)
• Trial ID: NCT02310399 on ClinicalTrials.gov

What this trial studies

This study evaluates the safety and effectiveness of the Nucleus 24 Auditory Brainstem Implant (ABI) in children who have total hearing loss due to severe cochlear anomalies or cochlear nerve disorders. It includes two groups: pre-linguistic children aged 18 months to 5 years and post-linguistic children under 21 years. The study will monitor the children's auditory performance over time through a series of evaluations after the implant activation. The goal is to gather data that may inform future studies and improve treatment options for this unique patient population.

Who should consider this trial

Good fit: Ideal candidates are children aged 18 months to 21 years with severe hearing loss due to cochlear anomalies or cochlear nerve disorders who do not benefit from cochlear implants.

Not a fit: Patients with hearing loss due to tumors or those who have functional cochleae and can benefit from conventional hearing aids or cochlear implants may not receive benefit from this study.

Why it matters

Potential benefit: If successful, this study could provide a new auditory solution for children who currently have no effective means of hearing.

How similar studies have performed: While there have been studies on cochlear implants, this approach with the ABI in this specific pediatric population is novel and has not been extensively tested.

Eligibility criteria

Inclusion Criteria:

Group 1: Prelinguistic hearing loss (birth-5 years; age at implantation of ABI 18 months-5 years)

• Pre-linguistic hearing loss (birth-5 yrs.; age at ABI 18 months-5yrs) with both:

* MRI +/- CT evidence of one of the following:

  * Cochlear nerve deficiency
  * Cochlear aplasia or severe hypoplasia
  * Severe inner ear malformation
  * Post-meningitis ossification
* When a cochlea is present or patent, lack of significant benefit from CI despite consistent use (\>6 mo.)

  * No or limited speech perception ability (limited to pattern perception on closed set testing materials using the CI)
  * Lack of progress in auditory skills development

Group 2: Post-linguistic hearing loss (\<21 yrs. of age)

* Post-linguistic hearing loss (\<21 yrs. of age) with:

  * Loss or lack of benefit from appropriate CI without the possibility for revision or contralateral implantation. Examples might include:

    * Post-meningitis ossification
    * Bilateral temporal bone fractures with cochlear nerve avulsion
    * Failed revision CI without benefit
  * Previously developed open set speech perception and auditory-oral language skills
* No medical contraindications
* Willing to receive the appropriate meningitis vaccinations
* No or limited cognitive/developmental delays.
* Strong family support
* Reasonable expectations from parents/guardians including a thorough understanding:

  * of potential benefits and limitations of ABI
  * of parental role in rehabilitation
  * that the child may not develop spoken language as a primary communication mode or even sufficient spoken language to make significant academic progress in an aural/oral environment
* Involvement in a rehabilitation program that emphasizes development of auditory skills with or without the use of supplementary visual communication.
* Able to comply with study requirements including travel to investigation sites.
* Informed consent for the procedure from the child's parents/legal guardian.

Exclusion Criteria:

For both Groups 1 and 2:

* Pre- or post-linguistic child currently making significant progress with CI Even for the very young children (18 months of age with 6 months of use), nearly all children with a good auditory signal from their CI will show evidence of improvement in these metrics over time.
* MRI evidence of one of the following:

  * normal cochlea and cochlear nerves or NF2
  * brainstem or cortical anomaly that makes implantation unfeasible
* Clear surgical reason for poor CI performance that can be remediated with revision CI or contralateral surgery rather than ABI.
* Intractable seizures or progressive, deteriorating neurological disorder
* Patients with evidence of Chiari malformation, hydrocephalus, spina bifid a
* Patients with any foreseeable need for a future MRI scan
* Unable to participate in behavioral testing and mapping with their CI. If this appears to be an age effect, ABI will be delayed until we can be assured that the child will be able to participate, as reliable objective measures of mapping are currently not available for mapping these devices.
* Unable to tolerate general anesthesia (cardiac, pulmonary, bleeding diathesis, etc.).
* Need for brainstem irradiation
* Unrealistic expectations on the part of the subject/family regarding the possible benefits, risks and limitations inherent to the procedure and ABI device.
* Unwilling to sign the informed consent.
* Unwilling to make necessary follow-up appointments.

Where this trial is running

New York, New York

• NYU Cochlear Implant Center — New York, New York, United States (Recruiting)

Study contacts

• Principal investigator: John T. Roland, Jr., MD — NYU Langone Health
• Study coordinator: William Shapiro

Conditions Hearing Loss, Bilateral; Auditory Brainstem Implant; Deafness; Hearing Loss; ABI