Neurofibromatosis type 2
A hereditary disorder called neurofibromatosis type 2 (NF2) makes tumors grow along your nerves. Although the tumors are often non-cancerous, you may experience various symptoms.
Neurofibromatosis type 2 symptoms
The hearing and balance-related nerves frequently develop tumors in people with NF2.
Typically, these result in symptoms like:
- Hearing loss that worsens gradually over time
- The sensation of ringing or buzzing in the ears
- Balance issues, especially when walking on uneven terrain or in the dark
Other tumors can develop inside the brain, spinal cord, or along the nerves that supply the limbs and legs, resulting in symptoms including muscle weakness in the arms and legs and ongoing headaches.
Causes of type 2 neurofibromatosis
A malfunctioning gene is the root cause of neurofibromatosis type 2 (NF2). Neurological systems can generate uncontrolled growth (tumors) if the NF2 gene is defective.
A parent passes on a defective gene to their child in 50% of cases of NF2. For a child to be at risk of having the disorder, only one of their parents needed to have the defective gene.
Every child has a one in two risks of developing NF2 if the mother or father carries the defective gene.
In some situations, the defective gene seems to arise on its own. The cause of this is unknown.
Identifying type 2 neurofibromatosis
Adults and older children can typically have NF2 diagnosed quickly by looking for common symptoms.
Babies who exhibit NF2 symptoms from birth may be diagnosed with it. Additional testing, such as scans, hearing, sight, or blood tests, may be advised if NF2 is suspected.
Before starting a family, couples with a history of NF2 may want to consider alternatives.
That could involve:
- Conceiving a child using donor sperm or eggs
- Adopting a child
- Undergoing a prenatal test to determine whether the baby will have NF2—either chorionic villus sampling or amniocentesis
- Genetic testing before implantation
Treatment for type 2 neurofibromatosis
NF2 is currently incurable. The course of treatment entails ongoing observation and immediate problem-solving.
Surgery can remove the majority of tumors, but there is a chance that it could result in issues like total deafness or facial paralysis.
A hearing device or learning to lip read can often be helpful for those with NF2, who eventually experience considerable hearing loss. However, most of those with NF2 eventually lose their hearing, and some need a wheelchair or mobility aid.
Reviewed by – Dr. Priyanka, MBBS MD Microbiology
Page last reviewed: 04 October 2022