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GENETIC– conditions that have a characteristic of deafness/hearing loss which may be passed on from generation to generation; inherited OSTEOGENESIS IMPERFECTA: Also known as Brittle Bone Disease. Significant hearing loss has been reported in approximately 50% of people with osteogenesis imperfecta (OI). incidence of hearing loss is much higher than in the general population. Sometimes visible deformities in the ossicles and inner ear can lead to hearing loss. Sometimes the cause of the loss is not visible. Environmental factors can contribute to hearing loss in a person with OI in the same way as for anyone else. Hearing loss can start at any time but among people who have OI the loss often starts at an earlier age and the likelihood increases with age. OTOSCLEROSIS: An abnormal bone growth in the middle ear This growth prevents the ear from vibrating in response to sound waves -- which must happen in order for you to hear. This lack of vibration leads to hearing loss that continues to get worse with time. the most frequent cause of middle ear hearing loss in young adults. It affects about 10% of the U.S. population. gets worse slowly, usually beginning in early to mid- adulthood more common in women than in men usually affects both ears Risks include pregnancy (which may trigger onset) and a family history of hearing loss. Caucasians are more susceptible than others to otosclerosis. WAARDENBURG SYNDROME: PHYSICAL FEATURES INCLUDE: o Widely spaced, almond shaped eyes o Different color eyes, or one eye 2 colors o Broad nasal bridge o White forelock of hair Sensorineural hearing loss found in 60% of individuals with WS. Autosomal dominant trait CONGENITAL deafness/hearing loss present at birth but not necessarily hereditary; acquired during fetal development Rubella Syndrome: a contagious infection in which there is a rash on the skin caused by a virus that is spread through the air or by close contact vaccination will protect from rubella However, if a mother is infected during early pregnancy, rubella can cause defects in the developing baby. The unborn baby can develop congenital rubella syndrome, which typically has a poor outcome. Defects are rare if the infection occurs after the 20th week of pregnancy. o COMPLICATIONS INCLUDE: Cataracts Congenital heart defects DEAFNESS Ear infections Mental retardation Microcephaly Miscarriage Stillbirth CONGENITAL CYTOMEGALOVIRUS (CMV): If a mother gets a CMV infection for the first time in her life while pregnant, it can be passed to the fetus during birth. CMV presents itself as similar to the common cold or mononucleosis Ability to damage the central nervous system and auditory system At birth, newborn will not have any symptoms As the baby grows, the damage caused by CMV will become more prevalent Preventative measures: washing hands CONGENITAL ATRESIA OF THE AUDITORY CANAL: Abnormal closure of the external auditory canal Can be bone, tissue, or both INFECTIOUS – deafness/hearing loss as a result to infection MENINGITIS: An infection of the meninges, the membranes covering/protecting the brain and spinal cord. Of all the common causes of hearing loss, this one is the most frightening, the most deadly. SYMPTOMS INCLUDE: o Rapid, high fever o Vomiting o Headache o Spots on the body o Painful joins o Extreme weakness o Glazed, faraway eyes How does hearing loss come into the picture?? o Rapid, high fevers can cause singeing of the cilia, cause irreparable damage! MEASLES/MUMPS: Typically begins with a mild to moderate fever. Other symptoms that may accompany: o Dry cough o Runny nose o Inflamed eyes (conjunctivitis) o Sensitivity to light o Tiny white spots with bluish centers found on the inside of the mouth o A skin rash made up of large flat blotches that often flow into one another Fever can spike as high as 104* or 105*. DEAFNESS/HEARING LOSS may occur as a result of high fever and damaged cilia. A vaccine will protect against measles. OTITIS MEDIA: Commonly known as EAR INFECTIONS Among the most common illnesses of early childhood Most children stop having ear infections by age 4 or 5. Middle ear infections occur when germs, viruses or bacteria get into the middle ear. (Can start with sore throughs, colds, respiratory problems, etc.) Rapid onset, short duration Fluid builds up in the middle ear cavity, becomes then becomes infected. Results in bulging eardrum accompanied by pain; eardrum may perforate to drain liquid Chronic=at least one month long; acute=over several weeks. Children are more susceptible to OM because of their eustachian tubes – short, parallel to ground Antibiotics are used to treat Chronic OM may result in hearing loss – damage to tympanic membrane and ossicles OM in general may result in temporary minor hearing loss SCARLET FEVER: Caused by an infection of the streptococcus bacteria SYMPTOMS: o Most obvious- RASH. Rash looks like bad sunburn with tiny bumps; may be itchy. Appears on neck and face and leaves an unaffected area around the mouth. It then spreads to the rest of the body and then eventually fades. o Reddened sore throat o Fever above 101* o Swollen glands in neck o Tonsils have white/yellow spots o Tongue may have white/yellowish coating Otitis media is a complication of Scarlet Fever If fever becomes too high for a prolonged amount of time, cilia in the cochlea may become damaged. TRAUMATICdeafness/hearing loss as a result to trauma in the ear of the head Traumatic perforation of the eardrum Skull Fracture – temporal bone ACOUSTIC TRAUMA: o Injury to the hearing mechanisms in the inner ear due to very loud noise o May be caused by: An explosion near the ear Gunshots Long-term exposure to loud noises o Hearing loss: Usually partial and involving highpitched sounds May slowly get owrse o Can cause Tinnitus (ringing in the ears) BAROTRAUMA: Pressure-related ear pain SYMPTOMS: o Ear discomfort o Hearing loss (slight) o Sensation of fullness or stuffiness in ears o Dizziness May occur from: o Altitude changes o Congested nose o Upper respiratory infection COMPLICATIONS: o Ruptured/perforated eardrum o Acute ear infection o Loss of hearing TOXIC – deafness/hearing loss as a result of chemicals ASPIRIN, ACETAMINOPHEN and NSAIDs: The American Journal of Medicine: researchers determined that regular use of aspirin, acetaminophen, and non-steroidal anti-inflammatory drugs (NSAIDs) increases the risk of hearing loss in men, particularly in younger men, under the age of 60. Aspirin - regular users under 50 and those aged 50-59 years - 33% more likely to have hearing loss than were nonregular users, but there was no association among men aged 60 years and older. NSAIDs - regular users aged under 50 were 61% more likely, those aged 50-59 were 32% more likely, and those aged 60 and older were 16% more likely to develop hearing loss than nonregular users of NSAIDs. Acetaminophen, regular users aged under 50 were 99% more likely, regular users aged 5059 were 38% more likely, and those aged 60 and older were 16% more likely to have hearing loss than nonregular users of acetaminophen. Can cause tinnitus ETHACRYNIC ACID: Diuretic – can be used for high blood pressure, congestive heart failure, liver failure and kidney failure. Ethacrynic acid can cause hearing loss. Sometimes, this hearing loss is permanent. It is more common when high doses of ethacrynic acid are given intravenously or when combined with other medications that can cause hearing loss. Let your healthcare provider know right away if you notice hearing loss or ringing in the ears (tinnitus). AMINOGLYCOSIDE ANTIBIOTICS: Used to treat a special type of bacteria Broken down easily in the stomach, making it standard to administer through injection When injected, the side effects include possible damage to the EARS and kidneys. This drug is considered ototoxic. The hearing loss associated with these antibiotics is PERMANENT. AGE RELATED – gradual hearing loss due to increase in age. PRESBYCUSIS: loss of hearing that gradually occurs in most individuals as they grow older Hearing loss is a common disorder associated with aging About 30-35 percent of adults between the ages of 65 and 75 years have a hearing loss. It is estimated that 40-50 percent of people 75 and older have a hearing loss. The loss associated with presbycusis is usually greater for high-pitched sounds Most commonly it arises from changes in the inner ear of a person as he or she ages, but presbycusis can also result from changes in the middle ear or from complex changes along the nerve pathways leading to the brain. most often occurs in both ears, affecting them equally because the loss is gradual, people suffering from presbycusis may not be aware of their diminishing hearing. OCCUPATIONAL – deafness/hearing loss as a result of an occupation (job) that requires exposure to prolonged loud noises Occupational hearing loss is damage to the inner ear from noise or vibrations due to certain types of jobs or entertainment. Sounds above 90 decibels (dB, a measurement of the loudness or strength of vibration of a sound), particularly if the sound is prolonged, may cause such intense vibration that the inner ear is damaged. 90 dB - ______________________________________________________ ______________________________________________________ 100 dB - ______________________________________________________ 120 dB - ______________________________________________________ 130 dB - ______________________________________________________ A general rule of thumb is that if you need to shout to be heard, the sound is in the range that can damage hearing. Some jobs, such as construction, airline ground maintenance, farming, and jobs involving loud music or machinery, carry high risk for hearing loss. In the U.S., the maximum job noise exposure is regulated by law. Both the length of exposure and the decibel level are considered. If the sound is at or greater than the maximum levels recommended, protective measures are required. _______________________________________________________________________________________ Pump up the volume, pump up the volume, pump up the volume – DON’T! "Audiologists believe tens of thousands of young people are causing serious damage to themselves, and are likely to suffer tinnitus and loss of hearing in later life. The experts say MP3 players should be designed to prevent people playing music above 90db, about two-thirds of the maximum volume of a typical device," the report warns. Apple's device easily exceeds that volume limit - in fact a French law (emulated by Apple across Europe) requires that the computer company cap the iPods volume, but even the capped maximum still exceeds expert recommendations, at 100db. The experts also warn people that they should only listen to their music player for a maximum one hour per day. TEMPORARY CAUSES OF HEARING LOSS: Build-up of wax in the ear canal Foreign body lodged in the ear canal Injury to the head Allergy Blocked eustachian tubes Scarred or perforated eardrums Ear infections – chronic otitis media Reaction to a medication