Auditory Brainstem Implant (ABI) in Pediatric Non-Neurofibromatosis Type 2 Subjects
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.
Bilateral Hearing Loss for Causes Other Than Tumors
Device: Nucleus 24 Auditory Brainstem Implant (ABI)
|Study Design:||Endpoint Classification: Safety/Efficacy Study
Intervention Model: Single Group Assignment
Masking: Open Label
Primary Purpose: Treatment
|Official Title:||Study of the Nucleus 24 Auditory Brainstem Implant in Pediatric Non-Neurofibromatosis Type 2|
- ABI Complications [ Time Frame: 5 years from date of surgery ] [ Designated as safety issue: Yes ]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.
- Audiologic Performance with ABI [ Time Frame: 5 years from date of surgery ] [ Designated as safety issue: No ]
We will complete audiologic and speech based tests to determine performance before and after ABI surgery. In particular, pre- and post-operative behavioral audiometry and speech perception testing will be used.
Audiometric testing will include:
- Detection audiogram, aided and unaided individual ears
- IT-MAIS or MAIS
- LING 6 sound test (MLV)
- ESP Low Verbal or Standard-Recorded if possible or MLV @ 60dB HL
- PB-k words and phonemes- Recorded @ 60dB HL
- CNC- 50 words @ 65dB SPL
Speech and language assessment: MacArthur-Bates Communicative Development Inventories (CDIs) (56), Pre-School Language Scale, , Fourth Edition (PLS-5) Clinical Evaluation of Language Fundamentals Preschool, Second Edition (CELF-P2), Clinical Evaluation of Language Fundamentals, Fourth Edition (CELF-4), and the Goldman-Fristoe Test of Articulation.
- ABI Electrophysiologic Measures [ Time Frame: 5 years from date of surgery ] [ Designated as safety issue: No ]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. In addition, we will record stimulation thresholds for each electrode and track those parameters over time. By following these measure, we will be able to capture how electrophysiologic parameters vary (if at all) over time.
|Study Start Date:||May 2013|
|Estimated Study Completion Date:||November 2022|
|Estimated Primary Completion Date:||November 2022 (Final data collection date for primary outcome measure)|
Experimental: Non-NF2 ABI surgery
All subjects will be part of a single arm involving placement of the Nucleus 24 Auditory Brainstem Implant (ABI) device.
Device: Nucleus 24 Auditory Brainstem Implant (ABI)
Nucleus 24 Auditory Brainstem Implant (ABI) surgery followed by device activation, testing, and clinical assessment for five years following surgery.
Other Name: ABI, Nucleus 24, Cochlear Americas
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.
Please refer to this study by its ClinicalTrials.gov identifier: NCT01864291
|Contact: Daniel J Lee, MD, FACSfirstname.lastname@example.org|
|Contact: Barbara S Herrmann, PhD, CCC-Aemail@example.com|
|United States, Massachusetts|
|Massachusetts Eye and Ear Infirmary||Recruiting|
|Boston, Massachusetts, United States, 02114|
|Contact: Daniel J Lee, MD, FACS 617-573-3130 firstname.lastname@example.org|
|Contact: Barbara S Herrmann, PhD, CCC-A 617-573-3266 email@example.com|
|Principal Investigator: Daniel J Lee, MD, FACS|
|Sub-Investigator: Barbara S Herrmann, PhD, CCC-A|