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

NCT ID: NCT01736267

Last Updated: 2020-05-06

Basic Information

• Recruitment Status: TERMINATED
• Clinical Phase: NA
• Total Enrollment: 3 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2012-11-30
• Study Completion Date: 2019-04-25

Brief Summary

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.

Detailed Description

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.

Conditions

Bilateral Hearing Loss for Causes Other Than Tumors

Study Design

• Allocation Method: NA
• Intervention Model: SINGLE_GROUP
• Primary Study Purpose: TREATMENT
• Blinding Strategy: NONE

Study Groups

Non-NF2 ABI surgery

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.

Group Type: EXPERIMENTAL

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

Intervention Type: DEVICE

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

Interventions

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

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

Intervention Type: DEVICE

Other Intervention Names

ABI, Nucleus 24, Cochlear Americas, ABI541, Nucleus Profile

Eligibility Criteria

Inclusion Criteria

• 18 years of age or older
• English as the primary language
• Medically and psychologically suitable
• Willing to receive/have received meningitis / pneumococcal vaccinations
• Able to comply with study requirements, including travel to the investigational site
• Cochlear or retrocochlear anomaly/pathology that interferes with transmission of auditory information from the cochleae to the brainstem, resulting in severe to profound bilateral deafness (thresholds of 90 dB or worse in both ears on pure tone audiometry ranging from 250 to 2,000 Hz and speech recognition scores ≤ 30% in both ears). All subjects will undergo bone conduction audiometry and tympanometry to confirm sensorineural hearing loss and rule out potential middle ear disorders.

• Conditions that cannot be otherwise treated, with conventional hearing aids or cochlear implants. If CI were previously used, subjects will have had a failed response, defined as ≤ 30% speech recognition and patient perception of inadequate benefit to continue using the device.
• Expected subjects include those with these diagnoses:

• Bilaterally severe/completely ossified cochleae
• Bilateral cochlear malformations leading to poor CI outcomes
• Bilateral temporal bone fractures, where the VIIIth cranial nerves have been disrupted
• Bilateral cochlear nerve agenesis
• Not a CI candidate based on above listed pathology, intolerable adverse effects with CI (e.g. stimulation of the facial nerve), or Evoked Auditory Potential testing predictive of a poor response

Exclusion Criteria

• Anomalies/pathology involving the brainstem or cortex
• Retrocochlear pathology due to NF2 or other types of cranial nerve or brainstem neoplasm
• Co-existing medical conditions that require irradiation of the brainstem or auditory cortex
• Medical or psychological conditions that serve as contraindication to surgery
• Additional handicaps that would prevent or limit participation in evaluations
• Unrealistic patient or family expectations regarding the benefits, risks, and limitations inherent to the procedure and the prosthetic device
• Pregnant women

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Massachusetts Eye and Ear Infirmary

OTHER

Sponsor Role: lead

Responsible Party

Dr. Daniel Lee

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

Responsibility Role: PRINCIPAL_INVESTIGATOR

Principal Investigators

Daniel J Lee, MD, FACS

Role: PRINCIPAL_INVESTIGATOR

MEEI, HMS, MGH

Locations

Massachusetts Eye and Ear Infirmary

Boston, Massachusetts, United States

Countries

United States

References

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.

Reference Type: BACKGROUND

PMID: 20393378 (View on PubMed)

Sennaroglu 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.

Reference Type: BACKGROUND

PMID: 19704357 (View on PubMed)

Choi 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.

Reference Type: BACKGROUND

PMID: 22109761 (View on PubMed)

Colletti 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.

Reference Type: BACKGROUND

PMID: 19546832 (View on PubMed)

Provided Documents

Document Type: Study Protocol and Statistical Analysis Plan

View Document

Other Identifiers

MEEI HSC 12-061

Identifier Type: -

Identifier Source: org_study_id