Hearing Loss

One of the main symptoms with NF2 is mild to severe hearing loss in either one or both ears. The hearing loss is caused by the presence of brain tumors known as bilateral acoustic neuromas/vestibular schwannoma.

Due to the nature of the VS (vestibular schwannoma) impacting the vestibulocochlear nerve (Cranial Nerve V111), there is a certain complexity when treating hearing loss for people with NF2. It will depend on the behavior (growth rate) of the VS, and the quality of the cochlear/auditory nerve.

To treat hearing loss, a doctor may suggest one of the below possibilities:

Treating Hearing Loss

  1. Hearing Aid

    If the patient’s hearing loss is mild and the VS unneeded to be treated at the time, a person with NF2 can receive the standard hearing aid to help with their hearing.

  2. CI (Cochlear Implant)

    Generally, a CI is not recommended because of the presence of tumors. In recent years, there have been some cases of those with NF2 who, despite tumors, were able to be implanted and found it beneficial.

    Marie Drew, the former president of AdvocureNF2, corresponded with Dr. Brackmann of House Clinic to gather more information on how it’s possible that a person with NF2 could receive a CI.

    In summary, as Dr. Brackmann had suggested in his letter, ask your doctor for a promontory stimulation test to check whether your cochlear nerve is intact which could make you eligible for a CI

  3. ABI (Auditory Brainstem Implant)

    In the cases where the cochlear nerve has lost its ability due to a VS and/or a VS will need to be surgically removed, an ABI (Auditory Brainstem Implant) can be placed. An ABI appears similar to a cochlear implant, however an ABI does not require a functional cochlear nerve as it “bypasses even the damaged hearing nerves by placing electrodes in the next center up the pathway to the brain, the cochlear nucleus complex in the brainstem.Source: House Clinic

    For more information about the ABI, feel free to email your questions to Dr. Brackmann (dbrackmann@hei.org) at House Clinic.

  4. ASL (American Sign Language) / Lip-Reading

    For those who prefer not to be or cannot be implanted with a hearing device, learning sign language or how to lip read would be of most benefit to aid with communication challenges.

    Look to your local colleges or deaf organizations which would offer courses in ASL or lip-reading.

    There are also helpful websites to learn basic ASL online. A couple are:

  5. CART & Captel

    CART and Captel are assistive devices for those with hearing loss. CART (Communication Access Real Time), provides voice to speech text through a professional typist. Learn more about CART with our article, Optimizing Communication for A Doctor Appointment.  Captel provides captioned phone calls for people who are deaf but have the ability to speak over the phone. Learn more about Captel at: www.captel.com