Neurofibromatosis Type 2

UT Southwestern Medical Center’s Neurofibromatosis Type 2 (NF2) program brings together multiple specialists to provide comprehensive care for those living with this rare and complicated genetic disorder. NF2 is a gene mutation that affects 1 in 25,000 people worldwide. It causes non-cancerous tumors to form on brain and spinal cord nerves that are important for hearing, vision, balance, and swallowing. 

While there is no cure for NF2, our multidisciplinary team can help improve a patient’s quality of life by lessening the debilitating consequences of the disease. Some of the treatments we provide are microsurgery, radiosurgery, chemotherapy, and hearing rehabilitation, including cochlear and auditory brainstem implants. UT Southwestern is the only center in the southern United States that offers auditory brainstem implants (ABI) to treat hearing loss in NF2 patients.

Multidisciplinary Team Specializing in NF2 Treatment

Patients with NF2 usually develop symptoms in their teens and early adulthood. The hallmark of NF2 is bilateral acoustic tumors. These non-cancerous tumors can lead to multiple medical problems such as hearing loss, vision problems, facial paralysis, and swallowing disorders. The numerous and diverse conditions caused by NF2 require a team experienced in treating these unique problems.

Microsurgery

In some cases, microsurgery is required for growing tumors when deafness occurs or the tumors become large enough to cause symptoms. Our neurotologists and neurosurgeons remove the intracranial tumors and offer three microsurgical approaches for the management of the vestibular schwannomas: translabyrinthine, middle fossa, and retrosigmoid craniotomies. These approaches may provide hearing preservation in some cases. 

We have specialists in the following areas who come together to manage the care of our NF2 patients:

• Neurotology
  
• Skull base neurosurgery
  
• Spine surgery
  
• Facial plastic surgery
  
• Oculoplastic surgery
  
• Neuro-ophthalmology
  
• Laryngology
  
• Audiology
  
• Oncology
  
• Speech therapy
  
• Physical therapy
  

Treatments

Chemotherapeutics, such as bevacizumab, have been shown to control tumor growth and preserve hearing in some patients. At UT Southwestern, we have used bevacizumab with good results in several of our patients.

Most of the treatments for NF2 are aimed at symptom management.