MedGen

A bilateral vestibular schwannoma (acoustic neurinoma). [from HPO]

A WHO grade I meningioma characterized by the presence of prominent chronic inflammatory infiltrates that predominate over the meningioma cells. [from NCI]

A grade I, slowly growing meningioma. Only a minority of tumors recur following complete resection. [from NCI]

A benign or malignant neoplasm affecting the olfactory or first cranial nerve. Clinical features may include facial pain and impairments of taste or smell. [from NCI]

A hereditary syndrome affecting the central nervous system that is associated with lesions of the skin and retina. Representative examples include neurofibromatosis, tuberous sclerosis, von Hippel-Lindau syndrome, and Sturge-Weber syndrome. [from NCI]

An autosomal dominant hereditary neoplastic syndrome. Two distinct clinicopathological entities are recognized: neurofibromatosis type 1 and neurofibromatosis type 2. Neurofibromatosis type 1 is associated with the presence of cafe-au-lait cutaneous lesions, multiple neurofibromas, malignant peripheral nerve sheath tumors, optic nerve gliomas, and bone lesions. Neurofibromatosis type 2 is associated with the presence of schwannomas, meningiomas, and gliomas. [from NCI]

A primary or metastatic malignant neoplasm that affects a cranial nerve. [from NCI]

A vestibular schwannoma (also known as acoustic neuroma, acoustic neurinoma, or acoustic neurilemoma) is a benign, usually slow-growing tumor that develops from the VIIIth cranial nerve supplying the inner ear. [from HPO]

A benign peripheral nerve sheath tumor that generally appears as a soft, skin-colored papule or small subcutaneous nodule. Individuals with neurofibromatosis can have numerous neurofibromas. [from HPO]

A benign nerve sheath tumor composed of Schwann cells. [from HPO]

Neurofibromatosis 2 (NF2) is characterized by bilateral vestibular schwannomas with associated symptoms of tinnitus, hearing loss, and balance dysfunction. The average age of onset is 18 to 24 years. Almost all affected individuals develop bilateral vestibular schwannomas by age 30 years. Affected individuals may also develop schwannomas of other cranial and peripheral nerves, meningiomas, ependymomas, and, very rarely, astrocytomas. Because NF2 is considered an adult-onset disease, it may be underrecognized in children, in whom skin tumors and ocular findings (retinal hamartoma, thickened optic nerves, cortical wedge cataracts, third cranial nerve palsy) may be the first manifestations. Mononeuropathy that occurs in childhood is an increasingly recognized finding; it frequently presents as a persistent facial palsy or hand/foot drop. [from GeneReviews]

A tumor made up of nerve cells and nerve fibers. [from HPO]

An inherited genetic condition in which members within a family are at an increased risk for the development of benign and/or malignant neoplasms. [from NCI]

Pathological processes involving the vestibulocochlear nerve; brainstem; or central nervous system. When hearing loss is due to retrocochlear pathology, it is called retrocochlear hearing loss. [from MONDO]

A non-neoplastic or neoplastic disorder that affects the ear. Representative examples include infections, hearing disorders, benign neoplasms, and carcinomas. [from NCI]

Abnormal growth of the cells that comprise the cranial nerves. [from NCI]

A non-neoplastic or neoplastic disorder affecting the acoustic nerve. [from NCI]

A neoplastic or non-neoplastic disorder that affects one of the cranial nerves. [from NCI]