Neurofibromatosis, type 2

This code identifies Neurofibromatosis type 2 (NF2), a rare genetic disorder characterized by the development of benign tumors, primarily schwannomas, along nerves in the brain and spinal cord. These tumors most commonly affect the vestibular nerves, leading to bilateral vestibular schwannomas (acoustic neuromas), which can cause hearing loss, balance issues, and other neurological deficits. NF2 is distinct from Neurofibromatosis type 1 (NF1) in its genetic basis and clinical manifestations.

Use this code when documentation clearly indicates a diagnosis of Neurofibromatosis type 2. This diagnosis is typically confirmed through genetic testing, clinical presentation of bilateral vestibular schwannomas, or other characteristic tumors such as meningiomas, ependymomas, or cataracts. Documentation should specify "type 2" to differentiate it from other forms of neurofibromatosis.

• Do not confuse with Neurofibromatosis type 1 (NF1); ensure the documentation explicitly states "type 2."
• Code any associated manifestations, such as hearing loss or specific tumor types, as secondary diagnoses.
• Review genetic testing results or pathology reports for definitive confirmation of NF2.