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

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	Link
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 Adult Non-Neurofibromatosis Type 2 Subjects	NCT01736267	Bilateral Heari...	Nucleus 24 (dis...	18 Years -	Massachusetts Eye and Ear Infirmary

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