Bilateral Hearing Loss for Causes Other Than TumorsABINCT01864291
Auditory Brainstem Implant (ABI) in Pediatric Non-Neurofibromatosis Type 2 Subjects
A study testing Nucleus 24 (discontinued) and ABI541 Auditory Brainstem Implants (ABI) in people with Bilateral Hearing Loss for Causes Other Than Tumors, run by Massachusetts Eye and Ear Infirmary at 1 site in United States.
Trial summary
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. Read more about this study
- Who is it for?
- Ages 18 months to 17 years
- Conditions the study is for
- Bilateral Hearing Loss for Causes Other Than Tumors
- What is being tested?
- Nucleus 24 (discontinued) and ABI541 Auditory Brainstem Implants (ABI)
- Type of treatment
- Medical device
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 9 participants
- Where
- 1 siteUnited States
- Study dates
- May 2013to 25 Apr 2019
- Phase
- Interventional
- Are there results?
- Yes, posted in the registrySummary in the doctors' view
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 months to 17 years
- All sexes
- No healthy volunteers
- Child
Who may be able to join
- Pre-linguistic hearing loss (birth-5 yrs.; age at ABI implantation 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
Show all 26Show fewer
- When a cochlea is present or patent with a normally appearing cochlear nerve, 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
- Post-linguistic hearing loss (<18 yrs. of age) with both:
- 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 which would be expected to interfere with the child's ability to cooperate in testing and/or programming of the device, in developing speech and oral language, or which would make an implant and subsequent emphasis on aural/oral communication not in the child's best interest
- Strong family support including language proficiency of the parent(s) in the child's primary mode of communication as well as written and spoken English.
- Reasonable expectations from parents 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 an educational 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 and clinic visits.
- Informed consent for the procedure from the child's parents.
Who cannot join
- Pre- or post-linguistic child currently making significant progress with CI - This will be considered if a child is progressing along the expected speech reception hierarchy (SRI-Q) as detailed by Wang et al (50). 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 have reached ceiling effects on the IT-MAIS and have pattern perception beyond chance using the ESP (50). Moreover, there will 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
Show all 14Show fewer
- 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.
- Lack of potential for spoken language development. This will be considered the case when evidence of the following exist:
- Severe psychomotor retardation, autism, cerebral palsy, or developmental delays beyond speech that would preclude usage of the device and oral educational development. Autism is a special case where there is the potential for delayed presentation. When early signs are considered present, we will request a comprehensive developmental assessment for further evaluation prior to considering routine evaluation.
- 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 that are inherent to the procedure and prosthetic device.
- Unwilling to sign the informed consent.
- Unwilling to make necessary follow-up appointments.
Trial locations and contacts
1 site in 1 country.
United States1 site
Massachusetts Eye and Ear Infirmary
Boston, Massachusetts, 02114
About this study
Official title: Study of the Nucleus 24 and ABI541 Auditory Brainstem Implants in Pediatric Non-Neurofibromatosis Type 2
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.
Read the detailed description
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.
- Being tested
- Nucleus 24 (discontinued) and ABI541 Auditory Brainstem Implants (ABI)
- Keywords
- Auditory Brainstem Implant, ABI, Nucleus 24, Deafness, Hearing loss, Non-tumor, Non-NF2, Pediatric, ABI541
- Medical subject headings
- Deafness, Hearing Loss
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Nucleus 24 (discontinued) and ABI541 Auditory Brainstem Implants (ABI)Device
Also called ABI, Nucleus 24, ABI541, Nucleus Profile, Cochlear Americas
Nucleus ABI541 Auditory Brainstem Implant (ABI) surgery followed by device activation, testing, and clinical assessment for five years following surgery.
| Study group | Role | What they receive |
|---|---|---|
| Non-NF2 ABI surgery | Experimental | 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. Receives: Device: Nucleus 24 (discontinued) and ABI541 Auditory Brainstem Implants (ABI) |
What is being measured
The primary outcome is the main question the study is designed to answer.
Number of Participants With Major or Minor ABI Complications
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.
Measured: 1 year from date of surgery
Secondary outcomes (2)
Change in Pure Tone Threshold (dB HL) Using the ABI
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)
Measured: 1-4 years from date of surgery
Number of Participants With Similar eABR Morphology on the Day of ABI Activation Compared to Intraoperative Responses
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.
Measured: 4 to 8 weeks after surgery (day of activation under anesthesia)
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted29 May 2013 (estimated)
- Study startMay 2013
- Primary completion25 Apr 2019
- Study completion25 Apr 2019
Why it stopped: Closed by IRB
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Posted resultsParticipant flow, outcome measures and adverse events reported by the sponsor.
- Publications (7)Papers the sponsor has linked to this study.
- Other trials for Bilateral Hearing Loss for Causes Other Than TumorsStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase N/A
- Primary purpose
- Treatment
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Enrolment
- 9 participants (actual)
Sponsor and investigators
- Lead sponsor
- Massachusetts Eye and Ear Infirmary
- Sponsor type
- University, hospital or other
- Responsible party
- Principal investigator, Dr. Daniel Lee, Massachusetts Eye and Ear Infirmary
- Investigators
- Daniel J Lee, MD FACS, Principal investigator, MEEI/MGH
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT01864291
- Sponsor's protocol ID
- 13-028H
- Acronym
- ABI
- Status last verified
- May 2020
- First submitted
- 22 May 2013
- Last updated
- 2 Jun 2020
- Results posted
- Yes, on ClinicalTrials.gov
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- Yes
- Individual data sharing
- No
Publications
Colletti V, Shannon RV, Carner M, Veronese S, Colletti L. Complications in auditory brainstem implant surgery in adults and children. Otol Neurotol. 2010 Jun;31(4):558-64. doi: 10.1097/MAO.0b013e3181db7055.
PubMed 20393378Sennaroglu L, Ziyal I, Atas A, Sennaroglu G, Yucel E, Sevinc S, Ekin MC, Sarac S, Atay G, Ozgen B, Ozcan OE, Belgin E, Colletti V, Turan E. 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. Otol Neurotol. 2009 Sep;30(6):708-15. doi: 10.1097/MAO.0b013e3181b07d41.
PubMed 19704357Choi JY, Song MH, Jeon JH, Lee WS, Chang JW. Early surgical results of auditory brainstem implantation in nontumor patients. Laryngoscope. 2011 Dec;121(12):2610-8. doi: 10.1002/lary.22137.
PubMed 22109761Colletti V, Shannon R, Carner M, Veronese S, Colletti L. Outcomes in nontumor adults fitted with the auditory brainstem implant: 10 years' experience. Otol Neurotol. 2009 Aug;30(5):614-8. doi: 10.1097/MAO.0b013e3181a864f2.
PubMed 19546832Colletti V, Carner M, Miorelli V, Guida M, Colletti L, Fiorino F. Auditory brainstem implant (ABI): new frontiers in adults and children. Otolaryngol Head Neck Surg. 2005 Jul;133(1):126-38. doi: 10.1016/j.otohns.2005.03.022.
PubMed 16025066Colletti V. Auditory outcomes in tumor vs. nontumor patients fitted with auditory brainstem implants. Adv Otorhinolaryngol. 2006;64:167-185. doi: 10.1159/000094651.
PubMed 16891842Colletti L, Zoccante L. Nonverbal cognitive abilities and auditory performance in children fitted with auditory brainstem implants: preliminary report. Laryngoscope. 2008 Aug;118(8):1443-8. doi: 10.1097/MLG.0b013e318173a011.
PubMed 18496153
Study at a glance
From the registry record, last updated by the sponsor on 2 Jun 2020. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Study of the Nucleus 24 and ABI541 Auditory Brainstem Implants in Pediatric Non-Neurofibromatosis Type 2
- ClinicalTrials.gov ID
- NCT01864291
- Acronym / sponsor ID
- ABI / 13-028H
- Keywords
- Auditory Brainstem Implant, ABI, Nucleus 24, Deafness, Hearing loss, Non-tumor, Non-NF2, Pediatric
- Study type
- Interventional
- Recruitment status
- TerminatedReason stopped: Closed by IRB
- Enrolment
- 9 participants (actual)
- Lead sponsor
- Massachusetts Eye and Ear InfirmaryUniversity, hospital or other
- Study start
- May 2013 (actual)
- Primary completion
- 25 Apr 2019 (actual)
- Study completion
- 25 Apr 2019 (actual)
- First posted
- 29 May 2013 (estimated)
- Results
- Posted 19 May 2020
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Detailed description
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.
Study design
- Primary purpose
- Treatment
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Non-NF2 ABI surgery | Experimental | Device: Nucleus 24 (discontinued) and ABI541 Auditory Brainstem Implants (ABI)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. |
Who may qualify
- Ages
- 18 months to 17 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child
Key inclusion criteria (26 listed)
- Pre-linguistic hearing loss (birth-5 yrs.; age at ABI implantation 18 months-5yrs) with both:
- Post-linguistic hearing loss (<18 yrs. of age) with both:
- No medical contraindications
- Willing to receive the appropriate meningitis vaccinations
- No or limited cognitive/developmental delays which would be expected to interfere with the child's ability to cooperate in testing and/or programming of the device, in developing speech and oral language, or which would make an implant and subsequent emphasis on aural/oral communication not in the child's best interest
Key exclusion criteria (14 listed)
- Pre- or post-linguistic child currently making significant progress with CI - This will be considered if a child is progressing along the expected speech reception hierarchy (SRI-Q) as detailed by Wang et al (50). 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 have reached ceiling effects on the IT-MAIS and have pattern perception beyond chance using the ESP (50). Moreover, there will evidence of improvement in these metrics over time.
- MRI evidence of one of the following:
- 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
- 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.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Nucleus 24 (discontinued) and ABI541 Auditory Brainstem Implants (ABI)Also: ABI, Nucleus 24, ABI541, Nucleus Profile, Cochlear AmericasNucleus ABI541 Auditory Brainstem Implant (ABI) surgery followed by device activation, testing, and clinical assessment for five years following surgery. | Device | Non-NF2 ABI surgery |
Primary outcome
Number of Participants With Major or Minor ABI Complications
Time frame: 1 year from date of surgery
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.
2 secondary outcomes
Change in Pure Tone Threshold (dB HL) Using the ABI
Time frame: 1-4 years from date of surgery
Number of Participants With Similar eABR Morphology on the Day of ABI Activation Compared to Intraoperative Responses
Time frame: 4 to 8 weeks after surgery (day of activation under anesthesia)
Results and safety
Loading the posted results from ClinicalTrials.gov…
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 2 Jun 2020.
- Posted resultsParticipant flow, baseline, all outcome measures and adverse events.
- Background: PubMed 20393378Colletti V, Shannon RV, Carner M, Veronese S, Colletti L. Complications in auditory brainstem implant surgery in adults and children. Otol Neurotol. 2010 Jun;31(4):558-64. doi: 10.1097/MAO.0b013e3181db7055.
- Background: PubMed 19704357Sennaroglu L, Ziyal I, Atas A, Sennaroglu G, Yucel E, Sevinc S, Ekin MC, Sarac S, Atay G, Ozgen B, Ozcan OE, Belgin E, Colletti V, Turan E. 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. Otol Neurotol. 2009 Sep;30(6):708-15. doi: 10.1097/MAO.0b013e3181b07d41.
- Background: PubMed 22109761Choi JY, Song MH, Jeon JH, Lee WS, Chang JW. Early surgical results of auditory brainstem implantation in nontumor patients. Laryngoscope. 2011 Dec;121(12):2610-8. doi: 10.1002/lary.22137.
- Background: PubMed 19546832Colletti V, Shannon R, Carner M, Veronese S, Colletti L. Outcomes in nontumor adults fitted with the auditory brainstem implant: 10 years' experience. Otol Neurotol. 2009 Aug;30(5):614-8. doi: 10.1097/MAO.0b013e3181a864f2.
- Background: PubMed 16025066Colletti V, Carner M, Miorelli V, Guida M, Colletti L, Fiorino F. Auditory brainstem implant (ABI): new frontiers in adults and children. Otolaryngol Head Neck Surg. 2005 Jul;133(1):126-38. doi: 10.1016/j.otohns.2005.03.022.
- Background: PubMed 16891842Colletti V. Auditory outcomes in tumor vs. nontumor patients fitted with auditory brainstem implants. Adv Otorhinolaryngol. 2006;64:167-185. doi: 10.1159/000094651.
- Background: PubMed 18496153Colletti L, Zoccante L. Nonverbal cognitive abilities and auditory performance in children fitted with auditory brainstem implants: preliminary report. Laryngoscope. 2008 Aug;118(8):1443-8. doi: 10.1097/MLG.0b013e318173a011.
- Individual participant dataSharing planned: No
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Daniel J Lee, MD FACS principal investigator
MEEI/MGH
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A study testing Nucleus 24 (discontinued) and ABI541 Auditory Brainstem Implants (ABI) in people with Bilateral Hearing Loss for Causes Other Than Tumors, run by Massachusetts Eye and Ear Infirmary at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 26 inclusion and 14 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: stopped early, as of the sponsor's last update on 2 Jun 2020. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
Yes. Results are posted in the registry (first posted 19 May 2020). A summary is in the For doctors view, and the full results are on ClinicalTrials.gov.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Number of Participants With Major or Minor ABI Complications (time frame: 1 year from date of surgery). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT01864291, which also lists 7 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 2 Jun 2020.