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HEI FACT SHEET |
ACOUSTIC NEUROMAS (Vestibular Schwannomas, scientific term) |
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DESCRIPTION: |
A vestibular schwannoma (VS), commonly known as an acoustic neuroma, refers to a tumor occurring on the balance (vestibular) nerve. Vestibular schwannomas result from a genetic mutation in the NF2 gene, but not all individuals who develop vestibular schwannomas have the disease known as NF2. When a vestibular schwannoma occurs in people who do not have the NF2 disease, the tumor is unilateral. In other words, it occurs on either the left or the right vestibular nerve, but not on both sides. Unlike people with NF2 disease, people with a unilateral vestibular schwannoma do not have any other tumors and they are almost always the only person in a family with a VS. A unilateral vestibular schwannoma is not a hereditary condition, and is referred to as a "unilateral sporadic vestibular schwannoma (USVS).
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TREATMENT: |
A cochlear implant cannot be used to treat an individual with NF2 because the auditory nerve is not able to carry signals from the cochlea to the cochlear nucleus in the brainstem. Instead, an NF2 patient may benefit from an auditory brainstem implant (ABI), which is designed to stimulate the auditory portion of the cochlear nucleus in the brainstem and send sound signals directly to the brain.
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CURRENT |
In the past decade, great strides have been made in terms of radiographic diagnosis, surgical approaches to vestibular schwannomas and understanding of the molecular biology of NF2. Unfortunately, similar advances in understanding of natural history of vestibular schwannomas, fundamental to the advancement of treatments, have not been made. HEI has undertaken a clinical study to define the growth rate and clinical course of vestibular schwannomas in NF2-affected individuals. Results of the study have led to a better understanding of vestibular schwannomas in NF2 and improved the framework of clinical centers, data management and scientific expertise for future studies and treatment trials. |
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FUTURE DEVELOPMENT: |
Researchers in HEI's Department of Auditory Prosthesis and Perception (DAIP) are currently working to improve the technology of the existing auditory brainstem implant (ABI) through the development of a newer prosthetic device, the penetrating auditory brainstem implant (PABI). The current ABI provides most recipients benefit through increased sound awareness, which aids in lipreading. Because few ABI recipients are able to understand speech without lipreading, the level of performance achieved with the ABI is poorer than that obtained by people with a cochlear implant (CI). The newer PABI, and the pitch-selective microstimulation provided by its penetrating electrode array, may provide speech comprehension closer to that experienced by multi-channel cochlear implant users. |
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CONTACT: |
Christa Spieth Nuber |