Bilateral Acoustic Neurofibromatoses (BANF) is a rare genetic disorder characterized by the development of tumors, known as acoustic neuromas or vestibular schwannomas, on the eighth cranial nerves. These nerves are responsible for transmitting sound and balance information from the inner ear to the brain. Individuals with BANF develop bilateral tumors, meaning that both ears are affected.
The tumors in BANF are typically non-malignant, but they can lead to various symptoms and complications. Common signs of BANF include gradual hearing loss, tinnitus (ringing in the ears), dizziness, imbalance, and facial numbness or weakness. Other less common symptoms may include headache, difficulty swallowing, and problems with coordination.
BANF is a hereditary condition caused by mutations in the NF2 gene, which plays a role in tumor suppression. It is inherited in an autosomal dominant pattern, meaning that an affected individual has a 50% chance of passing the mutated gene to their children. However, about 50% of BANF cases occur spontaneously without a family history.
Diagnosis of BANF often involves a combination of clinical evaluation, audiometric testing, magnetic resonance imaging (MRI), and genetic testing to confirm the presence of the NF2 gene mutation.
Management of BANF involves a multidisciplinary approach, including regular monitoring with MRI scans to assess tumor growth, surgery to remove or reduce tumor size, radiation therapy, and hearing aids or cochlear implants to address hearing loss. Regular follow-up and support from healthcare professionals, including neurologists, otolaryngologists, and genetic counselors, are crucial in the long-term management of individuals with BANF.
