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Author: admin, 07.09.2015
What Does Neurofibromatosis Type II Have to do With Hearing Loss?Home / Technology / What Does Neurofibromatosis Type II Have to do With Hearing Loss? Most types of hearing loss are due to something happening between the outer ear and cochlea.
An Auditory Brainstem Implant is a type of hearing implant used to help treat hearing loss due to NF II.
NF II is a genetic condition that usually causes non-cancerous tumors to appear on the auditory nerve. An auditory brainstem implant, or ABI, is a type of hearing implant used by individuals with NF II. Each of the twelve silver circles is a single electrode contact and each contact is responsible for stimulating one channel of sound, similar to each of the twelve channels from a CI.
An ABI’s electrode array is attached to the auditory nerve during the same surgery in which the tumors on the auditory nerve are removed. During surgery the surgeon can use tools to determine when he or she has placed the electrode array in the right position.
About this blog:This is the official blog of MED-EL, a leader in implantable hearing loss solutions.


Sensorineural hearing loss, conductive hearing loss, and mixed hearing loss are all examples of a hearing loss that is due to something happening in or before the cochlea. It’s the auditory nerve that helps carry electrical signals from the cochlea towards the brain, where they are interpreted as sound, and it just happens that the auditory nerve is what’s often affected by Neurofibromatosis Type II.
It affects approximately 1 in 33,000 people (0.003%) depending on the part of the world REF. Some may be born with numerous schwannomas already on the brainstem, while others may develop them later in life. A common treatment involves surgically removing the tumors on the auditory nerve, and this where the auditory brainstem implant comes in. It’s sort of like a cochlear implant, but instead of an electrode array inserted into the cochlea it has an electrode array that is attached to the auditory nerve. Because it’s more complex than just inserting an electrode array into the cochlea, the surgery takes approximately 12 hours depending on the number of tumors. This is important because the auditory brainstem doesn’t follow the tonotopic principle in the same way that the cochlea does, and therefore the surgeon needs to check to see what frequency of sound each electrode stimulates. It is a place where you can connect with MED-EL and learn all about hearing and hearing implants.


However, there are also types of hearing loss due to something happening between the cochlea and the brain. Because these tumors, sometimes called schwannomas or acoustic neuromas, appear on the auditory nerve they can have a significant influence on hearing.
Regardless of when it appears, NF II typically causes tinnitus, hearing loss, and balance problems that can develop over time. The auditory nerve is a part of the brainstem and is located between the auditory nerve and the brain, and therefore an ABI completely bypasses the cochlea.
These are called retrocochlear hearing losses, and one of the most common is associated with a genetic condition called Neurofibromatosis Type II, or NFII.



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