Neurofibromatosis type II(NF2) is clinically characterized by the presence of bilateral vestibular schwannomas. The exclusive goal in management of NF2 patients is hearing preservation. It has been controversial to determine whether to wait and see or to try early surgical management for hearing preservation. The authors experienced a case of vestibular schwannoma in association with NF2, which was partially removed and decompressed via middle fossa approach with hearing preservation. Based on our experience, we recommend a new strategy for hearing preservation on early surgical intervention of vestibular schwannoma. The criterias of the patient include (1) bilateral normal or serviceable hearing: (2) small sized tumorand: (3) young age. We suggest that the early surgical intervention including decompression may be considered for the hearing preservation especially on the favorable side to approach.
Decompression ; Hearing ; Humans ; Neurofibromatoses* ; Neurofibromatosis 2* ; Neuroma, Acoustic

Cochlear Implantation ; Genes, Neurofibromatosis 2 ; Humans ; Neurofibromatosis 2/surgery* ; Neuroma, Acoustic/surgery* ; Sequence Deletion

The authors have experienced 4 cases of neurofibromatosis with C-P angle tumors in 1979. In 3 cases of them, C-P angle tumors were confirmed surgically, and in one another case, bilateral C-P angle tumors with multiple intracranial and extracranial tumors were found in C-T scan. Where suspicion of a bilateral tumor of the 8th nerve arises, it is reasonable always to seek evidence of neurofibromatosis, otherwise described as von Recklinghausen's disease. It is recommended that intracranial tumors of the patient with central neurofibromatosis should be removed for prolongation of life, though the another tumors will grow after that.
Humans ; Life Support Care ; Neurofibromatoses* ; Neurofibromatosis 1 ; Neurofibromatosis 2 ; Neuroma, Acoustic*

Plexiform schwannoma is a rare benign tumor arising from the peripheral nerve sheath and characterized by a multinodular and plexiform growth pattern. This tumor usually arises sporadically. In rare cases, plexiform schwannomas have been associated with neurofibromatosis type 2. Plexiform schwannoma should be differentiated from plexiform neurofibroma, because the latter is pathognomonic tumor of neurofibromatosis type 1 and has a potential of malignant transformation. We report a case of multiple plexiform schwannomas associated with bilateral acoustic neuromas and meningioma.
Meningioma ; Neurilemmoma* ; Neurofibroma, Plexiform ; Neurofibromatoses* ; Neurofibromatosis 1 ; Neurofibromatosis 2* ; Neuroma, Acoustic ; Peripheral Nerves

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.
Dizziness ; Hearing Loss ; Humans ; Incidence ; Laminectomy ; Magnetic Resonance Imaging ; Meningioma ; Neurofibromatoses* ; Neurofibromatosis 2* ; Neuroma ; Neuroma, Acoustic ; Siblings ; Spinal Cord ; Tinnitus

We present two cases of plexiform schwannoma, a benign peripheral nerve sheath tumor, characterized by a plexiform growth pattern. Plexiform schwannoma must be distinguished from plexiform neurofibroma because of the propensity of the latter for malignant degeneration. Although plexiform schwannoma usually arises sporadically, it can be rarely associated with neurofibromatosis type 2. We report two cases of plexiform schwannoma. One was single sporadic lesion on the abdomen of a 28-year-old woman. The other showed multiple plexiform schwannomas associated with bilateral acoustic neuromas.
Abdomen ; Adult ; Female ; Humans ; Neurilemmoma* ; Neurofibroma, Plexiform ; Neurofibromatosis 2 ; Neuroma, Acoustic ; Peripheral Nerves

Bilateral vestibulopathy (BV) is characterized by oscillopsia and imbalance during locomotion. Half of the cases of BV are idiopathic, and BV due to brain tumor has been reported only rarely. We report a case of BV due to bilateral cerebellopontine-angle tumors in a patient with neurofibromatosis type 2. Careful history on oscillopsia and imbalance during locomotion, and regular follow-ups using the head-impulse test and dynamic visual acuity are warranted in patients with neurofibromatosis type 2.
Brain Neoplasms ; Cerebellopontine Angle ; Follow-Up Studies ; Humans ; Locomotion ; Neurofibromatoses ; Neurofibromatosis 2 ; Neuroma, Acoustic ; Visual Acuity

Neurofibromatosis type 2 (NF2) is a dominantly inherited genetic condition. Bilateral vestibular schwannoma, which are benign tumors, composed of neoplastic Schwann cells that arise from the eighth cranial nerve, are the hallmark of NF2. Standard approaches for treatment of growing vestibular schwannoma include observation, surgical removal and radiation therapy. Molecular targeted therapies also present great prosperity in recent years. In this review, we summarize the latest progresses on the treatment of NF2-associated vestibular schwannoma.
Humans ; Molecular Targeted Therapy ; Neurofibromatosis 2 ; radiotherapy ; surgery ; therapy ; Neuroma, Acoustic ; radiotherapy ; surgery ; therapy

Humans ; Neuroma, Acoustic ; surgery

Neurofibromatosis 1(NF-1, Von Recklinghausen disease) and Neurofibromatosis 2(NF-2, bilateral acoustic neurinoma) have been established as distinct disorders by the National Institutes of Health(NIH) Consensus Development Conference in 1988. We recently have encountered a patient of NF-2 who develop multiple intracranial and spinal tumors 12 years after the surgical treatment of Schwannoma of lumbar nerve roots. The tumors were right acoustic neurinoma, left cerebellopontine angle(CPA) meningioma, multiple intracranial meningiomas, intraorbital glioma, multiple ependymomas of the brain stem and cervical spinal cord, and meningiomas of cervical nerve roots. We present this case with a review of reported cases of neurofibromatosis.
Academies and Institutes ; Acoustics ; Brain Stem ; Ependymoma ; Glioma ; Humans ; Meningioma ; Neurilemmoma ; Neurofibromatoses* ; Neurofibromatosis 2* ; Neuroma, Acoustic ; Spinal Cord