The House Institute Foundation’s Center for Acoustic Neuroma and Neurofibromatosis (NF) Research is actively engaged in translational research that includes laboratory-based analysis of tumor tissue and the evaluation of innovative surgical techniques. The House Clinic researchers primarily work with patients living with NF type 2 who struggle from acoustic neuroma tumors that impact balance, the ability to hear, swallow, and speak, as well as eye and facial movement.
About Neurofibromatosis and Acoustic Neuromas
Neurofibromatosis (NF) is a genetic disorder that causes tumors to form on nerve tissue. There are two types of neurofibromatosis. Patients with NF type 1 often develop tumors on or close to the skin around the spine or in the brain. The House Clinic researchers primarily work with patients living with NF type 2, which often involves tumors in both ears and multiple simultaneous brain and spinal cord tumors. The most common tumor associated with NF type 2 is the acoustic neuroma.
Acoustic Neuromas, also known as vestibular schwannomas, are slow-growing tumors of the balance and hearing nerves. As they grow, they can compress nerves for facial movement, swallowing, speaking, eye movement, hearing, and balance.
HIF faculty in the Center for Acoustic Neuroma and NF Research are actively engaged in translational research that includes laboratory-based analysis of tumor tissue and the evaluation of innovative surgical techniques. The research is guided by HIF’s commitment to improve the quality of life for patients living with acoustic neuromas and neurofibromatosis.
Areas of Research
The House Institute Foundation’s (HIF) Center for Acoustic Neuroma and NF Research includes many accomplishments and studies that demonstrate the Center’s commitment to improving care for patients with acoustic neuromas or NF. The Center’s ongoing Natural History Study of NF2, initially funded by the Department of Defense, investigates growth patterns of NF2 tumors to allow for a better understanding of tumor progression and treatment timeline. As a major contribution to NF research, HIF developed the NF in vitro cell lines that are now used around the world to test drug therapies for NF. Finally, current research focuses on surgical techniques for acoustic neuromas. These include the trans-labyrinthine approach and the middle fossa approach, which can be used as a hearing preservation strategy or for decompression.
Natural History Study
The aim of this ongoing, longitudinal study is to understand the natural history of tumors and symptoms in patients with NF2. Due to the rarity of the disease and diverse presentation of symptoms in patients, little is known about the progression of NF2, and there are limited treatments. The Center intends to use clinical data, obtained over the course of the study, to discover common patterns and develop personalized treatments for NF2. The Natural History study will be key in fully understanding the cause, progression, and treatment of NF2, as well as aid concurrent studies of NF2 sequencing, and revolutionize treatment for NF2.
Clinical Tumor Bank
The Center has established a tumor bank that holds various tumor and nervous system tissues from patients with and without NF2. The clinical tumor bank allows for the identification of targeted treatment options for different NF2 tumor profiles. At the time of surgery, tumor tissue, ear tissues, serum and plasma, and cerebrospinal fluid samples are collected for further exploration. The clinical tumor bank is used for exploration of chemotherapies. Collected tumor tissue will be used to test against specific chemotherapeutic agents to discover the most effective agent for tumor growth arrest and shrinkage.
Proteomics and Genomics of NF2
The Center is conducting proteomic and genomic analysis of NF tumor samples. Sequencing tumors will help to identify drivers of phenotype for NF2, and why tumors may respond more to some treatments than to others. Sequencing may also illuminate genetic factors that contribute to those outcomes. The Center will use these analyses, with a patient-driven approach, to create genetic and protein profiles for individual NF2 patients. Tumor sequencing may reveal secondary pathways and mutations that will respond to specific agents and aid treatment outcomes, driving clinical and research practices.
Research Goals
An ongoing initiative in the Center is mapping patients’ histories with NF2 and identifying patterns, specific NF2 mutations and pathways, and unique profiles to develop more personalized medicine for children and adults with NF2. Ultimately, the goal is to use the current Natural History data alongside tissue bank data to personalize medicine for NF2. The Center’s genomics and proteomics findings will work in conjunction with Natural History variables to better understand NF2 mutations and pathways that may have implications for groundbreaking clinical care.
Natural History Study of NF2, initially funded by Department of Defense
This study established the growth patterns of vestibular schwannomas in patients with NF2. This allows us to better understand how and when these tumors progress and when treatment is indicated.
Development of House Ear Institute NF Cell Lines
These in vitro cell lines are used around the world to test drug therapies for potential efficacy in NF.
Pioneered the Auditory Brainstem Implant (ABI)
This is the first central neural prosthesis for hearing restoration. ABI technology is critical when the cochlear nerve is damaged by tumors in NF2.
Surgical techniques for vestibular schwannomas (acoustic neuromas)
Physicians at the House Institute have developed surgical techniques for treating vestibular schwannomas in NF2 that are used throughout the world. These include the trans-labyrinthine approach and the middle fossa approach, which can be used as a hearing preservation strategy or for decompression.
