GLOSSARY

ABI

Abbreviation for Auditory Brainstem Implant

ABR

Abbreviation for Auditory Brainstem Response

Acoustic Neuroma

A benign tumor developing on the hearing and balance nerves near the inner ear. A.K.A. AN, Acoustic Neurinoma, or Vestibular Schwannoma

AN

Abbreviation for acoustic neuroma

Artificial Tears

A tumor that begins in the brain or spinal cord in small, star-shaped cells called astrocytes

Astrocytoma

A tumor that begins in the brain or spinal cord in small, star-shaped cells called astrocytes

Atrophy

Decrease in size or wasting away of a body part or tissue

Auditory Brainstem Implant

A small complex electronic device that is surgically placed (implanted) against the brain stem that can restore some level of hearing when the cochlear nerve has been damaged or severed during an acoustic neuroma removal, commonly A.K.A. ABI

Auditory Brainstem Response

A physiological measure of the brainstem's response to sound. It tests the integrity of the hearing system from the ear to the brainstem by measuring the length of time for signals from sound to be detected by the brain

Auditory Nerve

Any of the eighth pair of cranial nerves connecting the inner ear with the brain and transmitting impulses concerned with hearing and balance

Autosomal Dominant Inheritance

A single, abnormal gene on one of the autosomal (non-sex determining) chromosomes from either parent can cause certain diseases. One of the parents will usually have the disease (since it is dominant) in this mode of inheritance. Only one parent must have an abnormal gene in order for the child to inherit the disease. For an autosomal dominant disorder: If one parent has an abnormal gene and the other parent a normal gene, there is a 50% chance each child will inherit the abnormal gene, and therefore the dominant trait. Neurofibromatosis is an autosomal dominant trait

Benign

Not malignant or not cancerous

Bilateral

Affecting or simply "on" both sides

BT

Abbreviation for brain tumor

Cafe au lait

Brown oval spots on the skin the color of coffee with milk

Cataract

Clouding of the lens of the eye or of its surrounding transparent membrane that obstructs the passage of light

Cavernous Sinus

A large channel of venous blood creating a "sinus" cavity bordered by the sphenoid bone and the temporal bone of the skull. The cavernous sinus is an important structure because of its location and its contents which include the third cranial (oculomotor) nerve, the fourth cranial (trochlear) nerve, parts 1 (the ophthalmic nerve) and 2 (the maxillary nerve) of the fifth cranial (trigeminal) nerve, and the sixth cranial (abducens) nerve

Celluvisc

An over-the-counter eye drops for dry eye. They are thicker than artificial tears, so they stay in the eye longer without running

Chemotherapy

The use of chemical agents in the treatment or control of disease

Chromosome 22

The chromosome containing the faulty gene which causes NF2

CI

Abbreviation for Cochlear Implant

CINE MRI's

Cine MRI (as in cinema) is taken the same way a traditional MRI is, with the addition of either a wristband or EKG leads on the patient's chest to measure the heart rate. See http://www.asap4sm.com/cine.html

Cochlear implant

A small complex electronic device that is surgically placed (implanted) within the inner ear to help persons with certain types of deafness to hear. The hearing nerve must be intact to be considered for a cochlear implant

Computerized Tomography
(CT) of the brain

(Also known as CAT or EMT scans). An automatic electronic machine which provides x-ray scans of tissue planes at a given thickness. Scans show the internal structures of the brain. Tumors, brain injury or other abnormalities can be shown. It is also useful to evaluate orbital pathology

Crocodile Tears

Tears produced while eating

Diploid Neoplasm

A tumor (neoplasm) with a diploid number of chromosomes -- that is, with a karyotype that is equivalent to that of a normal cell, with 23 chromosome pairs

Dominant

Being the one of a pair of bodily structures that is the more effective or predominant in action

Dysplasia

Abnormal development of a part of the body

Eighth Cranial Nerve

Responsible for the sense of hearing. It is also pertinent to balance and body position sense

Electromyogram

A test used to detect abnormal muscle electrical activity that can occur in many diseases and conditions, most often performed when patients have unexplained muscle weakness. This test involves using electrodes to send electric signals through nerves

EMG

Abbreviation for Electromyogram

Encephalomalacia

A non-specific term that literally means "softening of the brain". It may be caused by either some pathological neurological process or compression of the brain, either by a tumor or secondary to surgery. For example, it is difficult to avoid encephalomalacia in the cerebellar region when a posterior fossa approach is used in AN surgery<

ENT

Abbreviation for Ear, Nose, and Throat Doctor

Ependymoma

A type of brain tumor derived from the cells that line the cavities within the ventricles of the brain and the central canal of the spinal cord

Fibromyalgia

A syndrome characterized by chronic pain, stiffness, and tenderness of muscles, tendons, and joints without detectable inflammation

Fifth Cranial Nerve

Functions both as the chief nerve of sensation for the face and the motor nerve controlling the muscles of mastication (chewing), also known as the trigeminal nerve

FSR

Abbreviation for Fractionalized Stereotactic Radiosurgery

Gamma knife

A type of radiosurgery machine that acts by focusing low-dosage gamma radiation from many sources on a precise target. Areas adjacent to the target receive only slight doses of radiation, while the target gets the full intensity

Gene

The basic unit of heredity. Thousands of genes, arranged in specific linear order, form a chromosome. Genes come in pairs, each pair is located on one chromosome, with the matching gene on the other chromosome of that pair

Genetic

The basic unit of heredity. Thousands of genes, arranged in specific linear order, form a chromosome. Genes come in pairs, each pair is located on one chromosome, with the matching gene on the other chromosome of that pair

Genetic

Inherited or basic, relating to information contained on genes

Glioblastoma

Malignant brain tumor (also called astrocYtoma Grade fil, IV)

Glioma

A type of brain tumor

HEI

House Ear Institute in Los Angeles, California

Infrared Full Body Scanning

See www.healthscanonline.com

Lacrilube

An over-the-counter ointment for dry eye treatment. A.K.A. "Night Goop"

Learning Disability

A disorder that affects people's ability to either interpret what they see and hear or to link information from different parts of the brain. These limitations can show up in many ways: as specific difficulties with spoken and written language, coordination, self-control or attention

Lisch Nodule

Small masses of pigment on the iris of the eye that do not affect vision. They are often seen in the eyes of individuals with Neurofibromatosis type I (NF-I), and used as a diagnostic criteria for NF-l

Meckel's Cave

A pocket of dura matter (the outermost layer of the meningeal membranes) located near the medial (innermost) end of the petrous portion of the temporal bone-- the bony housing at the base of the cranium that contains the inner ear. Meckel's Cave contains the trigeminal ganglion-- the nerve root of the fifth cranial nerve

Meningioma

A mole is a measure of units, not weight. One mole = 6.023 x 10^23 (10 to the 23rd power) units - also known as Avogadro's number. It is used in chemistry to establish atomic or molecular weight. For example, 1 mole of carbon = 12 grams. If you look at a periodic table, you'll see carbon has an atomic weight of 12. A mole of water would be 18 grams (2 grams hydrogen + 16 grams oxygen). A micromole is one millionth of a mole

Middle Fossa Approach

A surgical approach for removing Acoustic Neuromas

Montgomery® Thyroplasty Implant System

A procedure designed to treat unilateral vocal cord paralysis

MRI
Magnetic Resonance Imaging

A special radiology technique designed to image internal structures of the body using magnetism, radio waves, and a computer to produce the images of body structures

Mutation

A permanent change in the genetic material, usually in a single gene

Myelin

The lipid substance forming an "insulating" sheath around many nerve fiber

Neurofibroma

A benign tumor caused by proliferation of Schwann cells and fibroblasts

Neurofibromatosis (NF)

A genetic disorder of the nervous system that causes multiple, soft tumors to grow anywhere on the body. The disorder is found in both sexes and all races. The inheritance pattern is autosomal dominant and, to date, there is no known cure. Two major genetically distinct forms ofNF have been identified, NF-l and NF-2

Neurofibromatosis Type 1 (NF-l)

The condition is characterized by multiple brown spots on the skin, neurofibromas of varying sizes on or under the skin, Lisch nodules on the iris of the eyes, freckling in the underarm or groin area, learning disabilities, and optic glioma. The gene for NF-I is located on chromosome 17

Neurofibromatosis Type 2 (NF-2)

The condition is characterized by bilateral vestibular schwannomas that cause balance problems, hearing loss, deafness, other tumors of the central and peripheral nervous systems, and cataracts occurring at an early age. The gene for NF-2 is located on chromosome 22

Neurofibromin

A complex protein substance produced by the NF-l gene

Neurofibrosarcoma

Also known as peripheral nerve sheath tumor; a malignant tumor that develops in the cells surrounding these peripheral nerves

Neuropathy

Any and all disease or malfunction of the nerves

Neurosurgeon

A physician trained in surgery of the nervous system and who specializes in surgery on the brain and other parts of the nervous system. Sometimes called a "brain surgeon."

NF1

Abbreviation for neurofibromatosis type 2

NF2

Abbreviation for neurofibromatosis type 2

Nonsense Mutation

A truncated mutation....the protein is truncated by mutation, and so dysfunctional or non-existent depending on where the mutation occurs

NS

Abbreviation for neurosurgeon

Optic Chiasm

The area where the 2 optic nerves cross

Optic Glioma

Tumor affecting the optic nerve

Orbit

The bony cavity of the skull in which the eyeball is located

Otolaryngologist

A specialist in disorders of the ears, nose, throat, head and neck. Sometimes called an "ENT" - ear, nose and throat doctor

Peripheral

Situated away from the center or central nervous system.

Pheochromocytoma

A tumor of the adrenal gland that causes severe high blood pressure

Plexiform Neurofibroma

A diffuse mass of tissue that is vascular, affecting bundles of nerves

Pseudarthrosis

A "false joint" within a long bone, which is a rare but serious complication of NF-l children. Other bone-related abnormalities seen in NF -I include bowing of the long bones of the leg and fractures of long bones that do not heal

PT

Physical Therapy

Puralube

An over-the-counter ointment for dry eye treatment. This is similar to Lacrilube but it's much cheaper

Radiation Therapy

The use of high-energy rays or particles to treat disease

Sarcoma

Malignant soft tissue tumor

Schwann Cell

The cell in which myelin is composed

Schwannoma

A benign tumor cause the proliferation of Schwann cells

Scoliosis

Curvature of the Spine

Segmental Neurofibromatosis

A variant of neurofibromatosis in which manifestations are limited to a single segment of the body, thought to be due to somatic mosaicism

Slit lamp

Device used by ophthalmologists to examine the eyes for Lisch Nodules in individuals with NF-l

Spontaneous Mutation

A change in a gene occurring with no identifiable cause

Strabismus

Crossed Eyes

Tinnitus

Ringing noise in the ear.

Trigeminal Nerve

See Fifth Cranial Nerve

Tumor

An abnormal mass of tissue that results from excessive cell division

Vestibular Schwannoma
(Acoustic Neuroma)

Benign tumor of the eighth cranial nerve (balance nerve) that can cause hearing impairment, balance problems, and deafness. In NF-2, vestibular schwannomas form on both acoustic nerves; therefore, it is bilateral. Formerly known as acoustic neuroma

Vestibulocochlear Nerve

See Eighth Cranial Nerve

Von Recklinghausen Disease

Former term for Neurofibromatosis Type 1