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Diseases » Hearing Impairment » Glossary

Glossary for Hearing Impairment

ABCD syndrome: A rare inherited condition characterized by albinism, deafness, a black lock of hair and intestinal abnormalities.
ADHD: Attention Deficit Hyperactivity Disorder (ADHD) is a mental and behavioral disorder characterized by behavioral problems such as hyperactivity, inattention, concentration difficulty, and other mental symptoms. Typically, ADHD and associated hyperactivity is known as a childhood disorder, although ADD/ADHD in adults is known to be under-diagnosed. It is distinguished from Attention Deficit Disorder (ADD) which has a reduced focus on hyperactivity type symptoms.
ARTS syndrome: A rare lethal syndrome characterized by deafness, optic atrophy and ataxia.
Acoustic neuroma: A benign tumor of the 8th cranial nerve which lies in the tube connecting the inner ear to the brain.
Acrofacial dysostosis, Nager type: A rare genetic disorder characterized by underdeveloped thumbs, forearm and cheekbones as well as ear defects.
Adult ADHD: Adult ADHD, (attention deficit hyperactivity disorder) is a common neurobehavioral developmental disorder with an onset in childhood that continues into adulthood. Children do not simply grow out of ADHD, as is often believed. Just the opposite is commonly true - the symptoms of ADHD often get worse as a child grows into adulthood. The predominant behaviors of adult ADHD are the same as in children and include:
- Inattentiveness
- Hyperactivity
- Impulsivity
These behaviors result in difficulties with:
- Concentration
- Remaining focused on a task or activity
- Controlling behavior
- Hyperactivity or over-activity
The symptoms of adult ADHD can be treated, but there currently is no cure for the disorder. Most people with ADHD can be successfully treated and lead normal, productive lives at home, work, school and with friends and family. The cause or causes of ADHD are not yet known, although researchers believe that genes may be one factor in the development of the disease. It is most likely that the disorder is the result of a combination of elements, including environmental factors, traumatic head injuries, nutrition, and social influences.
Arnold-Chiari malformation type 4: Arnold-Chiari malformation is a rare malformation where the base of the brain enters into the upper spinal canal. Type 4 actually involves a lack of development of a portion of the base of the brain (cerebellum). The prognosis is very poor with death often occurring during infancy.
Arthrogryposis multiplex with deafness, inguinal hernias, and early death: A rare syndrome characterized multiple joint contractures throughout the body, deafness, inguinal hernias and death usually within months of birth.
Ataxia, Hereditary, Autosomal Dominant: A group of rare, dominantly inherited neuromuscular disorder involving degeneration of the brain and spinal cord. The range, progression and severity of symptoms can vary quite considerably depending on the genetic defect involved.
Athabaskan brainstem dysgenesis: A rare neurological disorder caused by abnormal brainstem development and function.
Auditory Processing Disorder: Failure of the brain to correctly process sound.
Basilar artery migraine: Basilar migraine (BM), also known as Bickerstaff syndrome, consists of headache accompanied by dizziness, ataxia, tinnitus, decreased hearing, nausea and vomiting, dysarthria, diplopia, loss of balance, bilateral paresthesias or paresis, altered consciousness, syncope, and sometimes loss of consciousness.
Biotinidase deficiency: A metabolic disorder where the body lacks the enzyme biotinidase needed to process the vitamin called biotin (vitamin H) into carboxylase enzymes.
Borud Syndrome: A very rare syndrome characterized by numerous features including hearing and vision problems, heart muscle disease, ataxia and peripheral neuropathy.
Branchiootic syndrome: A rare inherited disorder characterized by branchial and ear abnormalities. The hearing loss is variable with respect to severity and age of onset.
Branchiootic syndrome 1: A rare inherited disorder characterized by branchial and ear abnormalities. The hearing loss is variable with respect to severity and age of onset. Type 1 is caused by a defect on the EYA1 gene on chromosome 8q13. It is similar to the branchio-oto-renal syndrome except it doesn't involve any kidney abnormalities.
Camurati Engelmann disease, type 2: A rare disorder (described in two patients) which has similar symptoms to the genetic condition called Camurati Engelmann disease but the genetic defect responsible for type 1 is not present in type 2. Type 2 has additional bone abnormalities which were noted on radiographs. Patients tend to suffer flare-ups of their condition which is accompanied by severe pain which may leave the patient incapacitated. Flare-ups can be triggered or made worse by stress, exhaustion, exercise, growth spurts, standing too long, walking too long, infection, illness, injury, surgery, cold weather and sudden changes in air pressure.
Cerebellar hypoplasia: A rare brain disorder where a part of the brain (cerebellum) fails to develop fully. The cerebellum is the part of the brain that controls balance and movement.
Cerebral Palsy: Any brain disorder causing movement disability
Chemical poisoning - Butyl Alcohol: Butyl alcohol is a chemical used mainly in solvents and in pharmaceutical manufacturing processes. Ingestion and other exposures to the chemical can cause various symptoms. The type and severity of symptoms varies depending on the amount of chemical involved and the nature of the exposure.
Chemical poisoning - Lead-containing Paint: Lead pain contains lead as well as other harmful chemicals. The lead in the pain is toxic (especially to young children) and ingesting fresh or old paint can cause serious symptoms. The type and severity of symptoms varies depending on the amount of chemical involved and the nature of the exposure.
Chromosome 22 trisomy mosaic: A rare chromosomal disorder where three copies of chromosome 22 are present in some of the body's cells instead of the normal two. Severity of symptoms is determined by how many cells have the extra chromosomal material.
Chromosome 22, microdeletion 22q11: A rare chromosomal disorder where a portion of the long arm of chromosome 22 is deleted.
Cleft palate: Birth defect of lip and mouth.
Coffin-Lowry syndrome: A rare genetic disorder characterized by down slanting space between eyelids, bulbous nose, soft hands and tapering fingers.
Congenital Disorders of Glycosylation Type Ia: A very rare inherited metabolic disorder where defective carbohydrate compounds are attached to glycoproteins and thus impairing glycoprotein function. Type 1A involves a phosphomannomutase enzyme defect and affects most body systems especially the nervous system and liver function.
Congenital disorder of glycosylation type 1A: A very rare inherited metabolic disorder where defective carbohydrate compounds are attached to glycoproteins and thus impairing glycoprotein function. Type 1A involves a phosphomannomutase enzyme defect and affects most body systems especially the nervous system and liver function.
Deafness: Inability to hear sounds.
Deafness, Y-linked 1: Hearing impairment inherited in a Y-linked manner - male-to-male transmission. There are no other abnormalities associated. The condition was observed in a number of generations of a Chinese family.
Deafness, autosomal dominant nonsyndromic sensorineural 10: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 10 involves a defect on chromosome 6q23.
Deafness, autosomal dominant nonsyndromic sensorineural 11: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 11 involves a defect of myosin VIIA gene on chromosome 11q13.5.
Deafness, autosomal dominant nonsyndromic sensorineural 12: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 12 involves a defect of alpha-tectorin gene on chromosome 11q22-q24.
Deafness, autosomal dominant nonsyndromic sensorineural 13: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 13 involves a defect of COL11A2 gene on chromosome 6p21.3.
Deafness, autosomal dominant nonsyndromic sensorineural 18: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 18 involves a defect on chromosome 3q22.
Deafness, autosomal dominant nonsyndromic sensorineural 2: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 2 involves a defect on chromosome 1p34,1p35.1.
Deafness, autosomal dominant nonsyndromic sensorineural 21: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 21 involves a defect on chromosome 6p21.3.
Deafness, autosomal dominant nonsyndromic sensorineural 25: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 25 involves a defect on chromosome 12q21-q24.
Deafness, autosomal dominant nonsyndromic sensorineural 30: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 30 involves a defect on chromosome 15q25-q26.
Deafness, autosomal dominant nonsyndromic sensorineural 36: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 36 involves a defect of transmembrane cochlear-expressed gene 1 on chromosome 9q13-q21.
Deafness, autosomal dominant nonsyndromic sensorineural 4: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 4 involves a defect on chromosome 19q13.33.
Deafness, autosomal dominant nonsyndromic sensorineural 41: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 41 involves a defect on chromosome 12q24.32-qter.
Deafness, autosomal dominant nonsyndromic sensorineural 43: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 43 involves a defect on chromosome 2p12.
Deafness, autosomal dominant nonsyndromic sensorineural 44: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 44 involves a defect of CCDC50 gene on chromosome 3q28.
Deafness, autosomal dominant nonsyndromic sensorineural 47: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 47 involves a defect on chromosome 9p22-p21.
Deafness, autosomal dominant nonsyndromic sensorineural 48: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 48 involves a defect on chromosome 12q13-q15. The hearing impairment was usually moderate to severe but rarely profound.
Deafness, autosomal dominant nonsyndromic sensorineural 49: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 49 involves a defect on chromosome 1q21-q23.
Deafness, autosomal dominant nonsyndromic sensorineural 6: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 6 involves a defect on chromosome 4p16.1.
Deafness, autosomal dominant nonsyndromic sensorineural 7: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 7 involves a defect on chromosome 1q21-q23.
Deafness, autosomal dominant nonsyndromic sensorineural 8: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 8 involves a defect of alpha-tectorin gene on chromosome 11q22-q24.
Deafness, autosomal dominant nonsyndromic sensorineural 9: Dominantly inherited hearing loss that occurs without any other symptoms - i.e. is not associated with any other condition. Type 11 involves a defect of the cochlin gene on chromosome 14q12-q13.
Deletion 11q: A very rare genetic disorder characterized by the deletion of genetic material from the long arm (q) of chromosome 11 - the genetic material occurs only once in body cells instead of the normal two. The type and severity of symptoms may vary considerably depending on the exact location and size of the deleted genetic material.
Dementia: Various mental impairment conditions.
Depression: Various syndromes with excessive anxiety, phobias, or fear.
Dizziness: Feelings of lightheadedness or giddiness.
Down Syndrome: A chromosome syndrome causing physical effects and mental retardation.
Drug-resistant Streptococcus Pneumoniae Disease: Streptococcal respiratory infection resistant to antibiotics
Ear conditions: Any condition that affects the ear
Facioscapulohumeral muscular dystrophy 1a: An inherited muscle disease involving slowly progressive muscle weakness. The condition is characterized by the early involvement of facial and shoulder muscles. Muscle weakness then spreads to the pelvis and legs. The onset, severity and rate of progression is variable.
Familial Granulomatosis, Blau type: A rare chronic inflammatory condition characterized by arthritis, dermatitis and uveitis. The condition may have a similar presentation to a condition called early-onset sarcoidosis and genetic testing may be needed to distinguish the conditions.
Familial, Systemic, Juvenile Granulomatosis: A rare chronic inflammatory condition characterized by arthritis, dermatitis and uveitis. The condition may have a similar presentation to a condition called early-onset sarcoidosis and genetic testing may be needed to distinguish the conditions.
Fatigue: Excessive tiredness or weakness.
Fetal alcohol syndrome: A pattern of mental and physical birth defects caused by excessive alcohol use during pregnancy. The range and severity of the symptoms may vary greatly.
Glue ear: Fluid accumulation in the middle ear
Head injury: An injury to the head
Hearing Impairment: Reduced ability to hear sounds.
Hearing loss: A very rare condition described in two sisters. It is characterized by vision and hearing problems and incoordination.
Hereditary hearing disorder: Genetic disorders that affects hearing and is passed from parents to offspring.
I cell disease: A rare inherited biochemical disorder characterized by the harmful accumulation of chemicals (glycoproteins and glycoplipids) due to the deficiency of an enzyme (UDP-N-acetylglucosamine-I-phosphotransferase).
Ichthyosiform Erythroderma, Corneal Involvement and Deafness Syndrome: A rare genetic ectodermal disorder characterized by keratitis, skin lesions and sensorineural deafness. The deafness and skin redness are usually present at birth and the corneas of the eye are gradually destroyed by keratitis which can result in loss of vision.
Infantile Refsum Disease: Genetic disease affecting nerve and muscle control.
Johanson-Blizzard Syndrome: A rare genetic disorder involving a range of abnormalities including a characteristic beak-like small nose, hypothyroidism and deafness.
Keratitis Ichthyosis Deafness Syndrome: A rare genetic ectodermal disorder characterized by keratitis, skin lesions and sensorineural deafness. The deafness and skin redness are usually present at birth and the corneas of the eye are gradually destroyed by keratitis which can result in loss of vision.
Klippel Feil Syndrome: A rare congenital disorder characterized by abnormal fusion of two or more vertebrae in the neck. The disorder is often associated with other abnormalities but their incidence is highly variable.
Labrynthitis: Inner ear condition affecting various ear structures
Landau-Kleffner Syndrome: A neurological disorder which results in aphasia, epileptic seizures and inability to recognize sounds.
Meier-Rotschild syndrome: A rare disorder characterized mainly by short stature, small ears and absent kneecaps.
Mental retardation, X-linked, Wittwer type: A rare disorder characterized by severe mental retardation, retarded growth, seizures and vision and hearing problems. The disorder is inherited in a X-linked manner which means that only males display the full range of symptoms whereas female carriers may have mild or no symptoms.
Middle ear infection: Infection of middle ear also called otitis media.
Mitochondrial encephalomyopathy - aminoacidopathy: A very rare syndrome characterized mainly by muscle and brain disease and an amino acid disorder.
Myxedema: Skin and tissue disorder usually due to hypothyroidism
N syndrome: A rare genetic disorder characterized by mental and physical retardation, eye abnormalities, retarded growth, hearing impairment and a high risk of developing cancers, particularly leukemia. It is an extremely rare condition originally described in two brothers.
Neonatal Jaundice: Common skin yellowing jaundice in newborn babies.
Neuroaxonal dystrophy - renal tubular acidosis: A very rare syndrome characterized mainly by muscle and kidney abnormalities.
Neurofibromatosis-2: Genetic disorder often leading to tumors on nerves.
Noise-Induced Hearing Loss: Hearing loss from loud noise exposure.
Oculofaciocardiodental syndrome: A very rare syndrome characterized mainly by eye, face, tooth and heart abnormalities.
Osteogenesis imperfecta Type I: A rare genetic connective tissue disorder characterized by fragile bones, blue sclerae and hyperextensible joints.
Osteogenesis imperfecta, type 1A: A rare genetic connective tissue disorder characterized by fragile bones and hyperextensible joints - a type of osteogenesis imperfecta I where the teeth are opalescent and blue sclerae may be absent.
Osteogenesis imperfecta, type 1B: A rare genetic connective tissue disorder characterized by fragile bones and hyperextensible joints - a milder form of osteogenesis imperfecta I where the teeth are normal and blue sclerae may be absent.
Osteogenesis imperfecta, type 4: A rare genetic connective tissue disorder characterized by fragile bones and blue sclerae - a form of OI involving moderate osteoporosis and no joint hyperextensibility.
Otospondylomegaepiphyseal dysplasia: A rare genetic disorder where abnormal bone growth causes hearing loss, skeletal abnormalities and characteristic facial features.
Pinealoma: A slow-growing type of brain tumor that occurs in the pineal gland. The pineal gland produces a hormone called melatonin which is involved in regulating sleep patterns.
Pineoblastoma, adult: A rare type of highly malignant brain tumor that usually occurs in children. The tumor develops in the pineal region of the brain.
Presbycusis: Hearing loss that tends to occur with age.
Progeria short stature pigmented nevi: A very rare inherited disorder characterized by premature aging, short stature, and immune system deficiency. The type and severity of symptoms is variable.
Rett-like syndrome: A very rare syndrome characterized mainly by
Schindler disease: Schindler disease is a rare congenital metabolic disorder involving a deficiency of a particular enzyme (alpha-N-acetylgalactosaminidase) which results in a buildup of glycoproteins in the body. The condition originates from a genetic mutation and is an inherited disorder. There are three main types of this condition, each with varying severity. The most severe form usually results in death within a few years of birth whereas the mild form doesn't cause symptoms until after the age of 30. The type and severity of symptoms varies depending on which form of the disease is involved.
Schindler disease, type 1: Schindler disease is a rare congenital metabolic disorder involving a deficiency of a particular enzyme (alpha-N-acetylgalactosaminidase) which results in a buildup of glycoproteins in the body. The condition originates from a genetic mutation and is an inherited disorder. There are three main types of this condition, each with varying severity. Type 1 is an infantile form and is the most severe form. It usually results in death within a few years of birth.
Sensorineural hearing loss, early greying, and essential tremor: A rare syndrome characterized by hearing impairment, premature graying of hair and tremors which start in adulthood.
Senter syndrome: A rare genetic ectodermal disorder characterized by ichthyosiform erythroderma and sensorineural deafness.
Syndromic Dystelephalangy: A rare syndrome characterized by bowed end bones of digits, facial anomalies and various other symptoms.
Synovitis granulomatous with uveitis and cranial neuropathies: A rare chronic inflammatory condition characterized by arthritis, dermatitis and uveitis. The condition may have a similar presentation to a condition called early-onset sarcoidosis and genetic testing may be needed to distinguish the conditions.
Tinnitus: Hearing noises in the ears: ringing, roaring, clicks, whistling, or hissing.
Trichinosis: Worm infection usually caught from pigs
Trichorhinophalangeal syndrome type 2: A rare genetic disorder characterized by bony growths, bulbous nose, distinctive facial features and loose excess skin during infancy.
Usher Syndrome: A rare inherited disorder characterized by sensorineural deafness and progressive vision loss.
Velocardiofacial syndrome: A genetic disorder which can present with a wide range of phenotypic manifestations which has lead to a number of different names being assigned to the various presentations e.g. DiGeorge Syndrome and Cayler Anomaly Face Syndrome. There are nearly 200 different symptoms that can occur and the severity of the condition is also highly variable depending on the nature and severity of the symptoms that are present.
Waardenburg syndrome: A rare genetic disorder with a large variation in range and severity of symptoms but generally involves hearing loss, characteristic facial abnormalities and changes in skin, hair and eye pigmentation.
Wegener's granulomatosis: A rare disease involving blood vessel inflammation which can affect the blood flow to various tissues and organs and hence cause damage. The respiratory system and the kidneys are the main systems affected.
Wildervanck syndrome: A rare birth disorder involving hearing loss, fusion of vertebrae and eye movement abnormalities/
X chromosome, monosomy Xp22 pter: A condition that is characterised by the occurrence of only one X chromosome in the genotype of an individual
X chromosome, monosomy Xq28: A condition that is characterised by the occurrence of only one X chromosome in the genotype of an individual
Young Simpson syndrome: A condition that is characterised by mainly mental retardation and physical signs of hypothyroidism
Zadik Barak Levin syndrome: A condition that affects the endocrine system as well as some facial characteristics