A Case of Neurofibromatosis Type II / 대한이비인후과학회지

ABSTRACT

Neurofibromatosis affects primarily cell growth of neural tissues and can cause tumors to grow on nerves at any time and any location. it is a heritable disease that is transmitted as an autosomal-dominant trait. Neurofibromatosis type II is characterized by bilateral acoustic neuromas with high incidence of other tumors. Hearing loss is the most common symptom in patients with neurofibromatosis type II. The other symptoms may be tinnitus, facial weakness, and dizziness. Audiometric test and MRI are essential to diagnose neurofibromatosis type II. Most cases of neurofibromatosis type II may need for surgical procedures. We experienced a case of neurofibromatosis type II having cervical spinal cord neuroma, multiple intracranial meningiomas, and bilateral acoustic neuromas. There was positive family history. Her two brothers also had neurofibromatosis type II, confined by MRI. Her acoustic neuroma on left side was removed successfully via translabyrinthine approach and cervical spinal cord neuroma by cervical laminectomy was removed.