Compassionate Use Arm – ABI541 ABI for 10 NF2 Patients

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Brief Title

Compassionate Use Arm - ABI541 ABI for 10 NF2 Patients

Official Title

Compassionate Use Arm - ABI541 Auditory Brainstem Implant for Neurofibromatosis Type 2 Patients With Deafness

Brief Summary

      The Nucleus 24 Auditory Brainstem Implant (ABI) is the only FDA approved device for
      restoration of meaningful hearing in Neurofibromatosis Type 2 (NF2) patients. This device has
      been discontinued, meaning that there is no commercially approved device currently available.
      The replacement model, the ABI541 (an unapproved device), is being investigated in ongoing
      clinical trials. A compassionate use arm of a clinical trial allows patients with NF2 to be
      implanted with this new ABI.
    

Detailed Description

      Currently, the Nucleus 24 (ABI24M) Auditory Brainstem Implant (ABI) is approved for use in
      individuals 12 years of age or older who have been diagnosed with Neurofibromatosis Type 2
      (NF2). Implantation may occur during first or second side tumor removal or in patients with
      previously removed vestibular schwannomas bilaterally. As the ABI24M is now obsolete, there
      is currently no commercially approved device available to restore useful hearing in patients.
      While the manufacturer of the device is pursuing commercial approval of the ABI541, it is
      anticipated that it may be several months or years before final approval. However, it is
      preferable to place the device at the time of tumor removal while the patient has an open
      craniotomy site, thereby avoiding a second surgery and its associated risks solely for the
      purpose device implantation. In addition, placement of an ABI at the time of tumor removal
      optimizes auditory rehabilitation, providing critical auditory sensations that may more
      effectively maintain auditory pathways. The FDA approved a compassionate use arm for an
      ongoing clinical trial to permit implantation of the ABI541 in up to 10 NF2 patients.

      Patients will undergo pre-operative evaluation for ABI surgery. Those who are appropriate for
      surgery will undergo a procedure for implantation of the ABI541. As per manufacturer
      protocol, the parameters of the ABI541 including adjustment of electrode sensitivities and
      activation of specific electrodes will be completed at each follow-up visit post-operatively.
      This process involves adjustment of device parameters by a trained audiologist who
      subsequently administers audiologic tests to confirm optimal activation of the ABI541.

      All audiologic testing and assessments represent the standard of care. These post-operative
      follow-up appointments will occur at 4-12 weeks, three, six, and twelve months, and
      biannually thereafter. Timing of these appointments may vary by as much as four weeks prior
      to or after the planned follow-up date (e.g. between 2-4 months for the 3 month follow-up).
      Unscheduled visits will be taken in the Otology clinic as needed to address any concerns
      patients may have regarding the use of their Nucleus ABI541.
    


Study Type

Expanded Access




Condition

Neurofibromatosis Type 2

Intervention

Nucleus Profile ABI541 Auditory Brainstem Implant


Publications

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information


Recruitment Status

Device





Eligibility Criteria

        Inclusion Criteria:

          -  Diagnosis of Neurofibromatosis Type 2;

          -  Age of 12 years or older

          -  Will be anticipated to be completely deaf as a result of bilateral neurofibromas of
             the auditory nerve, and/or their surgical removal.

        Exclusion Criteria:

          -  Contraindications to surgery or general anesthesia

          -  Intractable seizures or progressive, deteriorating neurological disorder

          -  Unable to participate in behavioral testing and mapping with ABI

          -  Unrealistic expectations on the part of the subject/family regarding possible
             benefits, risks, and limitations that are inherent to the procedure and prosthetic
             device
      

Gender

All

Ages

12 Years - 80 Years


Contacts

, , 

Location Countries

United States

Location Countries

United States

Administrative Informations


NCT ID

NCT02589912

Organization ID

15-113H


Responsible Party

Principal Investigator

Study Sponsor

Massachusetts Eye and Ear Infirmary


Study Sponsor

, , 


Verification Date

December 2019