Ear, Nose, and Throat Diseases

Nervous System Diseases

Symptoms and General Pathology

All Conditions

Hearing Loss

Deafness

Hearing Loss, Bilateral

Hearing Disorders

Ear Diseases

Otorhinolaryngologic Diseases

Sensation Disorders

Neurologic Manifestations

All subjects will be part of a single arm involving placement of the ABI541 Auditory Brainstem Implant (ABI) device. The Nucleus 24 was discontinued and is no longer available.

Nucleus ABI541 Auditory Brainstem Implant (ABI) surgery followed by device activation, testing, and clinical assessment for five years following surgery.

Nucleus 24 (discontinued) and ABI541 Auditory Brainstem Implants (ABI)

Daniel J Lee, MD FACS

The purpose of this research study is to determine whether Auditory Brainstem Implant (ABI) can improve hearing in children who are deaf and cannot receive a cochlear implants.

The goal of this MEEI Auditory Brainstem Implant (ABI) research study is to find new ways to improve hearing in children who are deaf and cannot receive a cochlear implant. The ABI is a surgically placed bionic implant that converts sounds into electrical signals that are directly transmitted to the cochlear nucleus, the first auditory center of the brain. 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 (NF2). However, a number of recent studies suggest that deaf patients who do not have NF2 and are not eligible for a cochlear implant may also benefit from placement of an ABI. These preliminary studies suggest that these non-NF2 or "nontumor" patients may actually have better outcomes after ABI surgery than patients suffering from NF2. Children appear to be particularly good candidates because of their developmental plasticity and in many studies, outcomes are more favorable in children that adults. Patients who do not have NF2 and are deaf due to abnormalities in their hearing nerves or inner ears from congenital malformations, infection, disease, or injury are not cochlear implant candidates and there are no other options to improve hearing in these cases except for the ABI. Thus, the purpose of our study is to carefully analyze whether ABI surgery improves the hearing and quality of life of non-NF2 children based on subjective and objective measures of their hearing before and after ABI surgery. In particular, we plan to study ABI outcomes in non-NF2 pediatric patients, characterize the parameters used on their devices, and determine the safety profile of ABIs in these patients.

Auditory Brainstem Implant (ABI) in Pediatric Non-Neurofibromatosis Type 2 Subjects

Study of the Nucleus 24 and ABI541 Auditory Brainstem Implants in Pediatric Non-Neurofibromatosis Type 2

We will track any major and minor complications peri-operatively and post-operatively in all study participants. Major complications include mortality, cerebellar contusion, facial palsy, meningitis, lower cranial neuropathy, hydrocephalus, pseudomeningocele and CSF leak. Minor complications include transient hydrocephalus, wound seroma, minor infections, balance problems, transient nerve palsies, transient dysphonia or difficulty swallowing, headache, flap problems, and non-auditory side effects. These complications will be tracked through serial clinical exams as well as patient recorded questionnaires to determine the safety profile of ABI surgery.

We will complete audiometric threshold testing and speech based tests to determine performance before and after ABI surgery.

The measure data value was calculated as mean threshold shift in dB HL from pre-surgery (or baseline) to postoperatively (following activation of the ABI) across subjects analyzed. Only those subjects that had auditory perception abilities were included. 2/9 subjects did not have auditory perception and are not using their devices.

The outcome measure time frame varies across subjects based on available followup data as this study was stopped early.

Audiometric testing will include:

• Behavioral pure tone threshold average (PTA) in both aided (with ABI) and unaided (without ABI)

We will measure and record the specific programmed parameters of the ABI device. Intraoperatively, evoked Auditory Brainstem Response (eABRs) will be recorded and saved. These tracings and data will be compared to the settings on activation to determine whether the same electrodes are appropriately positioned. By following these measures, we will be able to capture how electrophysiologic parameters vary (if at all) over time.

The outcome measure data are the number of subjects whose eABR morphology was similar on the day of activation (4 to 8 weeks following surgery) as recorded intraoperatively during ABI surgery.

Massachusetts Eye and Ear Infirmary

Principal Investigator

Implantation with Auditory Brainstem Implant (ABI) device and clinical follow-up

Non-NF2 ABI Surgery

Age, Categorical

Age, Continuous

Sex: Female, Male

Race and Ethnicity were not collected from any participant.

Race and Ethnicity Not Collected

United States

Region of Enrollment

As all participants have (1) absent cochleae or absent cochlear nerves or (2) anatomic conditions of the inner ear that preclude the use of a traditional cochlear implant array, it was expected that participants would have profound hearing loss at baseline and no residual hearing. Therefore, it was hypothesized that sound detection would be at the upper limit of clinical testing.

The decibel unit is plotted on a logarithmic scale; higher values represents worse hearing. 120 dB HL represents the upper limit of clinical testing.

Sound detection via pure-tone threshold audiometry

Dr. Daniel Lee

Major Complications

Minor Complications

Number of Participants With Major or Minor ABI Complications

2/9 subjects required revision surgery for device issues and were not included in this analysis as they did not complete the study. An additional 2/9 subjects did not have auditory perception and are not using their device and were also not included in this analysis.

Change in Pure Tone Threshold (dB HL) Using the ABI

Number of Participants With Similar eABR Morphology on the Day of ABI Activation Compared to Intraoperative Responses

Overall Study

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

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

Surgical implantation of the Nucleus ABI541 Auditory Brainstem Implant device

Nucleus ABI541 Auditory Brainstem Implant

Susan B Waltzman, PhD

Kristin Montella

John T. Roland, MD

Susan B. Waltzman, PhD

John T. Roland, Jr., MD

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 experiment in order to accommodate the known variability in cochlear implant clinical results. Data obtained in the course of this study will be compared with the existing published outcome data from children with normal hearing and with cochlear implants (CIs). These comparisons may provide additional useful information about the progress of ABI subjects' performance over time. Also, this may allow the results of the current study to be utilized to design future pivotal studies of the device in the pediatric population.

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 have Neurofibromatosis Type 2 (NF2); specifically, children with total hearing loss due to severe cochlear anomalies, cochlear nerve disorders or failed cochlear implantation. These conditions can include: developmental or acquired cochlear nerve deficiency (CND), cochlear aplasia, post-meningitis cochlear ossification, or cochlear malformation. This study proposes to implant up to 10 pre-linguistic young children (18 mos to 5 yrs. of age) and up to 10 post-linguistic children (\<21 yrs of age) with the Nucleus 24 Multichannel ABI in an attempt to demonstrate safety of the surgical procedure and device stimulation and the potential for auditory benefit beyond that experienced with a CI. These children would not qualify for the Nucleus ABI device, as their etiology is not an approved indication such as neurofibromatosis Type 2(NF2). Further, these children do not benefit from conventional hearing aids or cochlear implants.

Two groups of children will be included: Group 1 will include prelingiustic deaf children ages 18 months - 5 years and Group 2 will include postlinguistic deaf children \< 21 years of age. Post-operative evaluations will be conducted at the initial activation and at 1, 3, 6, 12, 18, 24, 30, and 36-month intervals post-activation in order to demonstrate the emergence of open-set speech perception abilities prior to study cessation. Experience in the CI literature has shown that a minimum of 2 years of device usage is required for children with pre-lingual hearing impairment to develop some open-set speech perception abilities. While Group 2 is post-linguistic and some may have had significant hearing prior to becoming deaf, the same time intervals will be used for consistency. Blinding or masking procedures are not included in the design, as the presence/absence of a brainstem implant cannot be easily concealed from the device recipients and/or clinical investigators.

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

Study participants will be monitored on a case-by-case basis for ongoing or unanticipated medical complications. Adverse events will be tracked on a case-by-case basis and recorded at the time of occurrence and followed up at resolution. Any adverse events will be reported to the NYUSOM IRB. Should there be concern for the safety of the subjects because of their participation in the study by the investigators or the IRB, the study would be halted at least temporarily and a detailed discussion with the investigators and the IRB would be undertaken.

The following audiometry and speech perception tests will be administered as age-appropriate:

* Detection audiogram, aided and unaided individual ears
* IT-MAIS or MAIS
* LING 6 sound test
* ESP Low Verbal or Standard- MLV @ 50dB HL
* PB-k words and phonemes- MLV @ 50dB HL
* MLNT- recorded @ 60dB SPL
* LNT- recorded @ 60dB SPL
* HINT-C- 2 lists of 10 @ 60dB SPL
* CNC- 50 words @ 60dB SPL

Each child will receive speech/language intervention using the Comprehensive Assessment of Spoken Language (CASL, Elizabeth Carrow-Woolfolk, 1999) The following tests will be administered when appropriate:

* The Oral and Written Language Scales (OWLS)
* Goldman-Fristoe Test of Articulation
* The Peabody Picture Vocabulary Test (PPVT)
* The Rossetti Infant-Toddler Language Scale
* Pre-School Language Scale, (PLS-5)
* The Expressive Vocabulary Test, 2nd Ed
* The Peabody Picture Vocabulary Test, 4th Ed

NYU Langone Health

All subjects will be part of a single arm involving placement of the Nucleus ABI541 Auditory Brainstem Implant (ABI) device. The Nucleus 24 was discontinued and is no longer available.

Daniel J Lee, MD, FACS

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 candidates for cochlear implants.

The goal of this MEEI Auditory Brainstem Implant (ABI) research study is to find new ways to improve hearing in patients who are deaf and cannot receive a cochlear implant. The ABI is a surgically placed bionic implant that converts sounds into electrical signals that are directly transmitted to the cochlear nucleus, the first auditory center of the brain. 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 (NF2). However, a number of recent studies suggest that deaf patients who do not have NF2 and are not eligible for a cochlear implant may also benefit from placement of an ABI. These preliminary studies suggest that these non-NF2 or "nontumor" patients may actually have better outcomes after ABI surgery than patients suffering from NF2. Patients who do not have NF2 and are deaf due to damage to the hearing nerves or inner ears from infection, disease or injury are not cochlear implant candidates and there are no other options to improve hearing in these cases except for the ABI. Thus, the purpose of our study is to carefully analyze whether ABI surgery improves the hearing and quality of life of non-NF2 patients based on subjective and objective measures of their hearing before and after ABI surgery. In particular, we plan to study ABI outcomes in non-NF2 patients, characterize the parameters used on their devices, and determine the safety profile of ABIs in these patients.

Auditory Brainstem Implant (ABI) in Adult Non-Neurofibromatosis Type 2 Subjects

Study of Nucleus 24 and ABI541 Auditory Brainstem Implants (ABI) in Adult Non-Neurofibromatosis Type 2 Subjects

Average pure tone threshold measured 12 months post-operatively. Measured in dB HL, where a lower threshold indicates more sensitive hearing and a higher threshold indicates less sensitive hearing.

We only included data from subjects that had auditory percept of any magnitude following ABI surgery (1/3 subjects).

Director of Auditory Brainstem Implant Progra, Department of Otolaryngology, Massachusetts Eye and Ear Infirmary

Placement of an Auditory Brainstem Implant (ABI) device

Pure Tone Threshold Average (dB)

We only included data from subjects that had auditory percept of any magnitude following ABI surgery (1/3 subjects).

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Auditory Brainstem Implant (ABI) in Pediatric Non-Neurofibromatosis Type 2 Subjects	NCT01864291	Bilateral Heari...	Nucleus 24 (dis...	18 Months - 17 Years	Massachusetts Eye and Ear Infirmary
Auditory Brainstem Implant (ABI) in Children With No Cochleae or Auditory Nerves	NCT02310399	Hearing Loss, B...	Nucleus ABI541 ...	18 Months - 21 Years	NYU Langone Health
Auditory Brainstem Implant (ABI) in Children With No Cochleae or Auditory Nerves	NCT02310399	Hearing Loss, B...	Nucleus ABI541 ...	18 Months - 21 Years	NYU Langone Health
Auditory Brainstem Implant (ABI) in Adult Non-Neurofibromatosis Type 2 Subjects	NCT01736267	Bilateral Heari...	Nucleus 24 (dis...	18 Years -	Massachusetts Eye and Ear Infirmary

Spots Global Cancer Trial Database for abi

Every month we try and update this database with for abi cancer trials from around the world to help patients and their families find trials that might be right for them.
We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.

Spots Global Cancer Trial Database for abi

Every month we try and update this database with for abi cancer trials from around the world to help patients and their families find trials that might be right for them. We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.

Every month we try and update this database with for abi cancer trials from around the world to help patients and their families find trials that might be right for them.
We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.