Familial Acoustic Neuromas, also known as hereditary acoustic neuromas or familial vestibular schwannomas, are a rare genetic condition characterized by the presence of multiple acoustic neuromas, which are non-cancerous tumors that grow on the nerve responsible for hearing and balance.
Acoustic neuromas typically develop on the eighth cranial nerve, also known as the vestibulocochlear nerve, which connects the inner ear to the brain and plays a crucial role in transmitting sound and maintaining balance. In Familial Acoustic Neuromas, individuals inherit a gene mutation that predisposes them to the development of these tumors. As a result, affected individuals often develop multiple acoustic neuromas in both ears over their lifetime, and may experience progressive hearing loss, tinnitus (ringing in the ears), dizziness, balance problems, and facial weakness or paralysis.
Familial Acoustic Neuromas are inherited in an autosomal dominant manner, meaning that a person who has the mutated gene has a 50% chance of passing it on to each of their children. Genetic testing can help identify individuals carrying the gene mutation, which can be valuable for early diagnosis and proactive management, such as regular screenings and interventions to manage symptoms and prevent complications.
Treatment options for Familial Acoustic Neuromas may include observation, surgical removal of tumors, or radiation therapy, depending on the size, location, and symptoms associated with the tumors. Regular monitoring and collaboration between healthcare professionals, including neurosurgeons, otolaryngologists, audiologists, and genetic counselors, are crucial aspects of comprehensive care for individuals with Familial Acoustic Neuromas.
