Auditory Brainstem Implant for Bilateral Hearing Loss

Overseen By William Shapiro

Age: < 65

Sex: Any

Trial Phase: Academic

Sponsor: NYU Langone Health

Disqualifiers: Significant CI progress, Brain anomalies, Seizures, MRI need, others

No Placebo Group All trial participants will receive the active study treatment (no placebo)

Approved in 2 Jurisdictions This treatment is already approved in other countries

Compensation: Varies

Number of Visits: 1

20 Participants Needed

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial tests a special device called the Nucleus ABI541 Auditory Brainstem Implant. It aims to help children with severe hearing loss in both ears who haven't benefited from cochlear implants. The trial includes two groups: children born deaf or who lost hearing early (before learning to speak), and those who lost hearing after learning to speak but no longer benefit from cochlear implants. Children with little to no success with cochlear implants and conditions like cochlear nerve issues or ear malformations might be suitable candidates. As an unphased trial, this study offers children a unique opportunity to potentially benefit from an innovative device not yet widely available.

Will I have to stop taking my current medications?

The trial information does not specify whether you need to stop taking your current medications. It's best to discuss this with the trial coordinators or your doctor.

What prior data suggests that the Nucleus ABI541 Auditory Brainstem Implant is safe for children?

Research has shown that the Nucleus ABI541 Auditory Brainstem Implant is safe. The FDA approved this implant, an updated version of the earlier ABI24M device, indicating a strong safety record. Studies also confirm the safety of the Nucleus 24 ABI System, providing reasonable assurance for its use. Although specific numbers on side effects are not provided, the approval and research findings suggest that the implant is generally well-tolerated. ^{1 2 3 4 5}

Why are researchers excited about this trial?

The Nucleus ABI541 Auditory Brainstem Implant is unique because it directly stimulates the brainstem, bypassing the damaged auditory nerves that traditional cochlear implants rely on. This feature makes it particularly promising for individuals with bilateral hearing loss who cannot benefit from cochlear implants, which are the current standard of care. Researchers are excited about this treatment because it offers the potential for sound perception in children who are pre-lingually or post-lingually deaf, providing a new avenue for auditory development and communication skills.

What evidence suggests that the Nucleus ABI541 Auditory Brainstem Implant is effective for bilateral hearing loss?

Research shows that the Nucleus ABI541 Auditory Brainstem Implant can help children with severe hearing loss. This trial studies the implant in two groups: children with pre-lingual deafness and those with post-lingual deafness. Studies have found that similar implants effectively aid people with hearing loss from brain tumors or genetic conditions. These implants send sound signals directly to the brain, bypassing damaged inner ear parts. In children without these specific conditions, the implant has shown potential to improve hearing. Although research on children without certain conditions continues, early results are promising. ^{2 6 7 8 9}

Who Is on the Research Team?

John T. Roland, Jr., MD

Principal Investigator

NYU Langone Health

Are You a Good Fit for This Trial?

This trial is for children with severe to profound hearing loss who have no cochleae or auditory nerves. It includes those aged 18 months-5 years born with hearing loss and those under 21 who lost hearing later but can't benefit from cochlear implants due to specific inner ear issues, ossification after meningitis, or other damage. Participants need strong family support and must be able to travel for the study.

Inclusion Criteria

I have bone growth after meningitis.

If you have a functioning cochlea and have used a cochlear implant consistently for more than 6 months without significant improvement, you may not qualify for the study.

My child was born with hearing loss and has inner ear issues confirmed by MRI or CT.

Exclusion Criteria

You are unable to complete certain tests and procedures, have medical conditions that make anesthesia risky, need brainstem irradiation, have unrealistic expectations about the treatment, are unwilling to sign a consent form, or unwilling to go to follow-up appointments.

My MRI shows my inner ear and hearing nerves are normal, or I have a condition or surgical issue affecting my cochlear implant's performance that can be fixed.

My child is showing improvement with their cochlear implant.

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Surgical Implantation

Surgical implantation of the Nucleus ABI541 Auditory Brainstem Implant in children

1 week

1 visit (in-person)

Post-operative Evaluation

Post-operative evaluations conducted at initial activation and at 1, 3, 6, 12, 18, 24, 30, and 36-month intervals post-activation

36 months

Multiple visits (in-person)

Follow-up

Participants are monitored for safety and effectiveness after treatment

5 years

What Are the Treatments Tested in This Trial?

Interventions

• Nucleus ABI541 Auditory Brainstem Implant

Trial Overview The Nucleus ABI541 Auditory Brainstem Implant (ABI) is being tested in children without neurofibromatosis type 2 (NF2). The study measures how well the implant works over time by comparing it to normal hearing and traditional cochlear implants. This research will help design future studies on ABIs in kids.

How Is the Trial Designed?

Group I: Pre-lingual Deafness Experimental Treatment 1 Intervention

Surgical implantation of the Nucleus ABI541 Auditory Brainstem Implant in prelingiustically deaf children ages 18 months - 5 years

Group II: Post-Lingual Deafness Experimental Treatment 1 Intervention

Surgical implantation of the Nucleus ABI541 Auditory Brainstem Implant in postlinguistically deaf children \< 21 years of age

Nucleus ABI541 Auditory Brainstem Implant is already approved in United States, European Union for the following indications:

Approved in United States as Nucleus ABI541 for:

• Neurofibromatosis Type 2 (NF2)
• Non-functional auditory nerves due to congenital or acquired etiologies

Approved in European Union as Nucleus ABI541 for:

• Neurofibromatosis Type 2 (NF2)
• Non-functional auditory nerves due to congenital or acquired etiologies such as auditory nerve aplasia, auditory nerve hypoplasia, head trauma, non-NF2 tumors, severe cochlear ossification

Who Is Running the Clinical Trial?

NYU Langone Health

Lead Sponsor

Trials

1,431

Recruited

838,000+

Published Research Related to This Trial

Auditory brainstem implantation (ABI) was successfully performed in 11 prelingual deaf children, leading to significant improvements in their ability to recognize and discriminate sounds within just a few months after the procedure.

Most children showed progress in auditory-verbal skills, with some able to identify sounds and words by 6 to 9 months post-implantation, although those with additional challenges, like Attention Deficit Hyperactivity Disorder, experienced slower progress.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Preliminary results of auditory brainstem implantation in prelingually deaf children with inner ear malformations including severe stenosis of the cochlear aperture and aplasia of the cochlear nerve. Sennaroglu, L., Ziyal, I., Atas, A., et al.[2022]

Multichannel auditory brainstem implants (ABIs) are safe for patients with neurofibromatosis type II (NF2) and do not increase surgical complications, as shown in a study of 18 patients implanted between 1994 and 2003.

Most patients experienced improved communication and access to environmental sounds post-implantation, although some variability in auditory performance was noted, highlighting the importance of patient education for maximizing benefits.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Auditory brainstem implantation in patients with neurofibromatosis type 2. Kanowitz, SJ., Shapiro, WH., Golfinos, JG., et al.[2006]

The auditory brainstem implant (ABI) is primarily used for patients with neurofibromatosis 2 (NF2) who have lost auditory nerve function due to bilateral vestibular schwannomas, allowing them to perceive sound sensations, mainly aiding in lipreading.

Out of five patients who received the ABI in the Czech Republic since 1999, one patient has achieved significant benefits by understanding speech without lipreading, while others primarily use the device to assist with lipreading, highlighting varying levels of efficacy among users.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

[Use of the auditory brainstem neuroprosthesis in the Czech Republic]. Skrivan, J., Zvĕrina, E., Betka, J., et al.[2006]

In two adult patients with postlingual sensorineural deafness, the combination of a cochlear implant (CI) and a contralateral auditory brainstem implant (ABI) showed that bilateral sound detection improved auditory outcomes compared to unilateral devices, particularly in sound localization and speech discrimination.

The study found that using a CI linked to a contralateral routing of sound (CROS) hearing aid system provided similar benefits to the CI-ABI combination, suggesting that for patients with only one functioning auditory nerve, a CI-CROS may be a viable alternative to ABI.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Cochlear implantation and contralateral auditory brainstem implantation. Sinnathuray, AR., Meller, R., Cosso, M., et al.[2012]

In a study of 12 children with cochlear nerve deficiency, using both a cochlear implant (CI) in one ear and an auditory brainstem implant (ABI) in the other led to significant improvements in auditory perception skills compared to using either device alone.

The results showed that bimodal stimulation (CI + ABI) significantly enhanced auditory performance, as indicated by improved scores in auditory perception tests, suggesting that this combined approach should be recommended for children with cochlear nerve deficiencies.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Bimodal stimulation in children with inner ear malformation: One side cochlear implant and contralateral auditory brainstem implant. Batuk, MO., Cinar, BC., Yarali, M., et al.[2021]

In a study of 24 patients with neurofibromatosis 2 (NF2) who received bilateral auditory brainstem implants (ABIs), 75% showed improved audiometric outcomes after receiving a second implant, indicating that bilateral ABIs can enhance hearing performance for some patients.

Indications for a second implant included poor performance from the first implant and severe nonauditory symptoms, suggesting that patients with limited success from unilateral ABIs may benefit from additional surgical intervention.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Bilateral Auditory Brainstem Implants in Patients With Neurofibromatosis 2. Noonan, KY., Rock, J., Barnard, Z., et al.[2021]

The auditory brainstem implant (ABI) effectively stimulates the auditory pathway in bilaterally deafened rats, as evidenced by increased Fos-like immunoreactivity in key auditory brainstem nuclei, indicating neural activation.

The study suggests that the ABI can convey auditory information in a way that is organized for speech recognition, demonstrating its potential as a treatment for patients with severe hearing loss due to nerve damage.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Distribution of Fos-like immunoreactivity in the auditory pathway evoked by bipolar electrical brainstem stimulation. Takagi, H., Saito, H., Nagase, S., et al.[2007]

Citations

1. clinicaltrials.gov clinicaltrials.gov/study/NCT02310399

Auditory Brainstem Implant (ABI) in Children With No ... The purpose of this study is to evaluate the clinical safety and efficacy of the Nucleus™ 24 Auditory Brainstem Implant (ABI) in pediatric patients who do not ...

2. clinicaltrials.gov clinicaltrials.gov/study/NCT01864291

Auditory Brainstem Implant (ABI) in Pediatric Non- ... For many years, ABIs have improved the hearing of patients who are deaf due to brain tumors associated with a genetic syndrome called Neurofibromatosis Type 2 ( ...

3. pmc.ncbi.nlm.nih.gov pmc.ncbi.nlm.nih.gov/articles/PMC7077937/

Auditory Brainstem Implantation for Adults With ... When compared with no intervention, auditory brainstem implantation provides some benefit for completely deaf adults with NF2 or severe inner ear abnormalities ...

4. withpower.com withpower.com/trial/phase-hearing-loss-4-2014-8f4a6

Auditory Brainstem Implant for Bilateral Hearing Loss This trial is testing a special device that helps children with severe hearing loss by sending sound signals directly to their brain.

5. centerwatch.com centerwatch.com/clinical-trials/listings/NCT02310399/auditory-brainstem-implant-abi-in-children-with-no-cochleae-or-auditory-nerves

Auditory Brainstem Implant (ABI) in Children With No ... This study is a feasibility study of the Nucleus 24 ABI in children without NF2. It will be conducted as a repeated-measures, single subject ...

6. accessdata.fda.gov accessdata.fda.gov/scripts/cdrh/cfdocs/cfpma/pma.cfm?id=P000015S012

Premarket Approval (PMA) - FDA Approval for the Nucleus ABI541 Auditory Brainstem Implant, a modification to the existing ABI24M device, which is intended to restore a level of auditory ...

7. clinicaltrials.gov clinicaltrials.gov/study/NCT01736267

Auditory Brainstem Implant (ABI) in Adult Non- ... The purpose of this research study is to determine whether Auditory Brainstem Implant (ABI) can improve hearing in persons who are deaf in both ears and are not ...

8. accessdata.fda.gov accessdata.fda.gov/cdrh_docs/pdf/P000015B.pdf

Auditory Brainstem Implant - accessdata.fda.gov The results of these activities are summarized below and demonstrate, with a reasonable degree of assurance, that the Nucleus 24 ABI System is safe and.

9. mss-p-007-delivery.stylelabs.cloud mss-p-007-delivery.stylelabs.cloud/api/public/content/72a28a0e1192416aacb63efe2583e220?v=910a4d90

Cochlear™ Nucleus® ABI541 auditory brainstem implant A Cochlear Nucleus implant is a medical treatment for moderate to profound hearing loss. Inside each Cochlear Nucleus implant is a magnet. To ensure MRI ...