Last Updated: 01/6/21
- Cochlear Devices
Nerve Damage and Hearing Loss
- Cochlear Implant (CI)
- Auditory Brainstem Implant (ABI)
- Differences between an ABI and a Typical CI
- Important CI and ABI Information
Bilateral (left and right) not just unilateral (either left or right) vestibular schwannoma (VS) tumors, acoustic neuromas (AN)
is the hallmark tumor growth for individuals with the condition neurofibromatosis type 2 (NF2).
These tumors affect ninety-five percent (95%) of individuals with germline NF2. While tumors may grow elsewhere before VS, these
lesions are often the first noticed for NF2 diagnosis even if other issues occur first. [Ardern-Holmes, 2017] The growth rate varies from person
to person, but tumor growth rate has been proven dependent on an individual's NF2 mutation type; other factors may also apply. [Hexter, 2015]
Hearing loss that is the result of the condition VS is a result of damage to the cochlear nerve (acoustic nerve), the connection of the nerve between the Cochlea
and the brainstem. Traditional hearing aids that amplify sound might offer help while the nerve starts to become damaged. As more damage to the nerve
occurs, a Hearing Aid, which only amplifies sound, will become ineffective. The next step is a cochlear implant (CI) and thanks to medical advancements,
many people with NF2 do well with hearing from a CI for many years. If the cochlear nerve breaks completely; something that would be confirmed or rejected as a result of auditory brainstem response
(ABR) test, sometimes also called brainstem evoked response audiometry (BERA), only an auditory brainstem implant (ABI) will offer any sound.
1. Cochlear Devices
CI - Cochlear Implant
A Cochlear implant (CI) will work if; CN8 (the vestibulocochlear nerve), the vestibular nerves,
the cochlear nerve and the cochlea itself are functioning.
Drug-based tumor treatments like bevacizumab (avastin™)
have begun to allow for an increased period of effectiveness with a CI.
ABI - Auditory Brainstem Implant
When most people suffer from hearing loss, sound amplification is all that is needed to regain the missing sound. The
Cochlear Implant was designed to direct sound to the Cochlea when an eardrum is ruptured, or a bone in the ear or
other internal parts of the Middle Ear is broken. But if damage occurs to the or the cochlear nerve or other locations on the
vestibulocochlear nerve before it splits to become the vestibular nerve (balance nerve) and
cochlear Nerves, an auditory brainstem implant (ABI) is the only option to regain hearing. ABIs direct sound
straight to the brainstem, bypassing the ear components completely.
The brain will need time to adjust and adapt or relearn the new form of hearing that an ABI provides. Sound quality improves
over the course of the first year but can change after that as a result of other tumor growth.
The ABI's quality of sound is different from natural hearing. But with an ABI speech reading (lip-reading) is
often easier. The effectiveness of an ABI combined with lip-reading varies based on an individual's ability to
lip-read and working electrodes. With an ABI, it often becomes easier to understand speech over time.
Processor - Nucleus 6 (N6)
The Current Sound Processor model for cochlear implants (CI) and auditory brainstem implants (ABI) is the
Nucleus 6 (N6). When cochlear releases a new processor, it does not always
work for individuals with an ABI. The N6 is the newest processor for both.
Differences of ABI from Typical CI
Assuming an understanding of a basic typical CI, these are the differences between the ABI systems:
- Internal receiver: The Internal Receiver components are the same as a CI, except for the shape and
function of the sensor, and the location the sensors are placed in the head. The ABI sensor is then placed
at the brainstem where CN8 is meant to leave the brainstem, and with a CI the sensor is placed
inside the Cochlear.
- Receiver magnet removal: During surgery for the implant, individuals who have health issues like NF2 and
require MRIs have the internal receiver magnet removed for safety to
allow for MRI scans. Removal of the internal magnet results in the requirement of a retainer disc to
hold the ABI processor in a fixed location on the head.
A surgeon may not know this when implanting these devices, but it is possible to remove the
magnet later. Requirements of MRI brain scans after implantation is only common for NF2. Radiology departments
for MRIs will not allow an individual to have a scan unless they see documentation from a surgeon confirming
the magnet was removed during implant.
- Magnet: People with CI's can have the magnet removed it is standard to have it inside the head. It is why facilities
assume to refuse an MRI to patients with hearing implants.
- Side Effects: Possible Side Effects of some Electrodes: Each ABI electrode tested can result in stimulating parts of the
brain that could be dangerous.
- Poor Pitch Reception: The quality of final sound of an ABI is not at the same level as CI's.
- Competing Tinnitus: During tests, sounds might compete with Subjective/Objective/GET Tinnitus.
- Switching on and Tune Ups: An ABI tuning results in longer session times with an Audiologist than a typical
Things to know about Switching on and Mapping ABI's:
- Activation is possible six weeks after an ABI is implanted and takes two days.
- Twenty-One Electrodes = Twenty-One Channels of Sound
- Channel Deactivation: Due to the brain's response to a new method of sound side effects of some electrodes not
all of the electrodes are likely to work and which that do may change over time.
- ECG: A heart monitor for safety due to potential problematic electrodes is worn during switch on.
- Individual electrode test
- Tones of working electrodes are set into a range of high to low pitch.
- The range for each electrode - The volume level of each electrode is gradually increased or decreased
- Pitch comparison
- During live test:
- Side Effects: Side effects to electrodes can show up. (twitching/spasms)
- Volume: Overall or individual electrode volume levels might need to be reduced or raised.
- Speech Readers/Lip Reading: Good speech readers might think the volume is good, but it is too low.
CI and ABI Important Information
Medical Treatment Warnings
In their information packets, Cochlear issued a warning certain medical treatments done too close to the implant can break the
implant. These treatments include:
- Electrosurgery Radiosurgery - Gamma Knife, CyberKnife, or Proton Therapy
- Electroconvulsive Therapy
Additional warnings on damage include the following:
- Electrostatic Discharge (ESD) - Static Electricity [Journal of Educational
ESD is the sudden discharge of static electricity. Electronic devices, including cochlear implants (both
the internal and external devices), are susceptible to damage from ESD. No sound from implant could be
from; a programming issue, processor damage, or even receiver failure. [Journal of Educational Audiology,
- Causes of ESD [Cochlear, 2017] [Journal of Educational Audiology, 2002]
- Low Humidity - Hot or cold dry conditions
- Removing clothes
- Playing on plastic slides
- Walking across a carpet
- Handling polyethylene bags
- Pouring polyurethane foam into a box
- Latex balloon coming in contact with hair
- CRT's - Computer monitors and TVs
- Radio Frequency Interference - There is interference with Cochlear Implants and RF (Radio Frequency)
Technology [Cochlear, 2017] [Journal of Educational Audiology, 2002]
- Mobile phones
- Electronic Article Surveillance (EAS) Systems
- Metal detection systems (Such as security checks at airports.)
A Baha (bone-anchored hearing aid), is a solution for people who have a unilateral hearing loss single sided deafness (SSD). It allows
for hearing introduced on both sides of the head to be heard, by transmitting the incoming sound of a deaf ear to the hearing ear.
On the side of the deaf ear, a small hole is drilled into the skull that the processor can be attached to. When attached the sound picked up
by the processor is echoed through the skull bone to the hearing ear. A Baha might be only a temporary hearing solution for a better
hearing for a few years depending on changes in tumors.
A Baha is a titanium piece surgically attached to the skull. The processor is a removable piece that can be attached or disconnected as needed.
Baha piece in skull - MRI Safe
Titanium is not a metal that is an issue or dangerous in MRI scans. Removal of the outer device, the processor, is necessary
before an MRI as well as any parts containing a magnet.
The Vestibulocochlear Nerve affects both the Vestibular Nerve and the Cochlear Nerve. The vestibular nerve affects balance, while the cochlear
nerve affects hearing. When this happens, a CI will not replace hearing loss but leaves ABI as an
option for sound. Learn more about the vestibulocochlear nerve damage.
- NextGen ABI Receivers: Hearing Implant: Cochlear Implant - Auditory Brainstem Implant.
- Davis, Neil L., Jamie M. Rappaport, and James C. MacDougall. "Cochlear and auditory brainstem implants in the management of
acoustic neuroma and bilateral acoustic neurofibromatosis." McGill Journal of Medicine 3 (1997): 115-120.
http://citeseerx.ist.psu.edu/viewdoc/download?doi=10.1.1.557.3776&rep=rep1&type=pdf | DOI: 10.1.1.557.3776
- Bosch, Martina M., et al. "Ophthalmologic findings and long-term course in patients with neurofibromatosis type 2."
American Journal of Ophthalmology 141.6 (2006): 1068-1077.
http://www.ajo.com/article/S0002-9394(06)00015-8/fulltext | DOI: 10.1016/j.ajo.2005.12.042
- Samii, Madjid, and Venelin Gerganov. "Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors."
Surgery of Cerebellopontine Lesions. Springer Berlin Heidelberg, 2013. 315-374.
http://link.springer.com/chapter/10.1007%2F978-3-642-35422-9_6 | DOI: 10.1007/978-3-642-35422-9_6
- Vincent, C. "Auditory Brainstem Implants: How Do They Work?." The Anatomical Record. 295.11 (2012): 1981-1986.
Source: http://onlinelibrary.wiley.com/doi/10.1002/ar.22588/full | DOI: 10.1002/ar.22588
- Cochlear. "Cochlear Nucleus 6 Manual" (2017) Source: http://www.cochlear.com
- Cochlear. "Cochlear Freedom Manual" (2010) Source: http://www.cochlear.com
- Journal of Educational Audiology. "An Electrostatic Discharge (ESD) Control Program for Children with Cochlear Implants." (2002)
- Hexter A, Jones A, Joe H, et al. "Clinical and molecular predictors of mortality in neurofibromatosis 2: a UK national analysis of 1192 patients"
Journal of Medical Genetics Published Online First. (August 2015)
http://jmg.bmj.com/content/early/2015/08/14/jmedgenet-2015-103290.short | DOI: 10.1136/jmedgenet-2015-103290
- Ardern-Holmes, Simone, Gemma Fisher, and Kathryn North. "Neurofibromatosis Type 2: Presentation, Major Complications, and Management, With a Focus
on the Pediatric Age Group." Journal of Child Neurology. 32.1 (2016): 9-22.
http://journals.sagepub.com/doi/abs/10.1177/0883073816666736 | DOI: 10.1177/0883073816666736
- Schwartz, Marc S., et al. "Auditory brainstem implants." Neurotherapeutics. 5.1 (2008): 128-136.
http://www.sciencedirect.com/science/article/pii/S1933721307002565 | DOI: 10.1016/j.nurt.2007.10.068
University of Kansas. Researchposters. "ABI and American Med-El Trial in the United States" (June 2016)
- Unknown "Cochlear Implant Mapping."
Mayo Clinic. "Auditory Brainstem Implants: Recent Progress and Future Perspectives" Neurosci., 29 (January 2019)