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What are Audiology Disorders?
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Acoustic Neuroma

Acoustic Neuroma is a nonmalignant tumor on the 8th cranial nerve. Patients with acoustic neuroma may experience hearing loss, imbalance, and facial weakness. Those with extremely large tumors may also have other neurological problems. Many persons with acoustics will eventually need brain surgery to remove the tumor. Those who opt not to have surgery will likely need to have periodic imaging studies to determine if it is still safe to leave the tumor without treatment.

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Atresia and Microtia

Some babies are born without an external ear canal or an abnormal outer ear, which can cause hearing problems. This lack of development of the external ear canal is called congenital atresia. It is a birth defect and is often accompanied by abnormalities of both the middle ear bones as well as the external ear. Another birth defect is microtia, which is an abnormal growth or under-development of the external ear. Microtia can vary from a minor abnormality to a small tag of skin or cartilage being the only indication of the ear being there. Microtia often occurs along with atresia, but atresia can also appear isolated and in this case the external ear looks normal except for lacking the ear canal. In some cases, surgery may be an option. This depends on a range of different aspects and normally the operation does not take place until the child is about four years old. An alternative to surgery is a bone implantable hearing aid, which bypasses the obstruction in the ear canal.

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Autoimmune Inner Ear Disease (AIED)

Autoimmune inner ear disease (AIED) is a syndrome of progressive hearing loss and/or dizziness that is caused by antibodies or immune cells which are attacking the inner ear. In most cases, there is reduction of hearing accompanied by tinnitus (ringing, hissing, roaring) which occurs over a few months. About 50% of patients with AIED have symptoms related to balance (dizziness or unsteadiness).

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Barotrauma refers to injury sustained from failure to equalize the pressure of an air-containing space with that of the surrounding environment. The most common examples of barotrauma occur in air travel and scuba diving. Although the degree of pressure changes are much more dramatic during scuba diving, barotraumatic injury is possible during air travel. Symptoms of barotrauma include “clogging” of the ear, ear pain, hearing loss, dizziness, ringing of the ear (tinnitus), and hemorrhage from the ear.

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Central Auditory Processing Disorder (CAPD)

Central Auditory Processing Disorder (CAPD) is a hearing disability unrelated to hearing loss. To a person with Central Auditory Processing Disorder (CAPD) the sounds of speech are muddled together because the speech processing centers of the brain are unable to distinguish one speech sound from another. Even if their hearing is normal, people with Central Auditory Processing Disorder (CAPD) have difficulty understanding speech. The problem is especially pronounced in situations with background noise. People with Central Auditory Processing Disorder (CAPD) experience no immediate benefit from using a hearing aid. However, assistive devices, such as FM equipment may alleviate some problems. Auditive training programs may also be of benefit.

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Cogan's Syndrome 

Cogan’s syndrome is an autoimmune disease. The inflammation in the eye and ear are due to the patient’s own immune system producing antibodies that attack the inner ear and eye tissue. Onset of the disease is generally a brief episode of inflammatory eye pain. Shortly following these ocular symptoms, patients develop bilateral audiovestibular symptoms, including hearing loss, vertigo (dizziness) and tinnitus (ringing in the ears). Immediate treatment for Cogan’s syndrome is with steroids, which reduce the inflammatory response.

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Congenital Hearing Loss

Congenital hearing loss is the presence of deafness at birth. Genetic factors (hereditary) are thought to cause more than 50% of all hearing loss. Hearing loss from genetic defects can be present at birth or develop later on in life. Most genetic hearing loss can be described as autosomal recessive or autosomal dominant. Other, more rare types of genetic hearing loss include X-linked (related to the sex chromosome) or mitochondrial inheritance patterns. In autosomal recessive hearing loss, both parents carry the recessive gene and pass it along to the child. Parents are often surprised to discover their child has a hearing loss because they are not aware that they are carrying a defective gene. This type of inheritance pattern accounts for about 70% of all genetic hearing loss. An autosomal dominant hearing loss occurs when an abnormal gene from one parent is able to cause hearing loss even though the matching gene from the other parent is normal. The parent who is carrying the dominant gene may also have hearing loss as well as other signs and symptoms that make up a genetic syndrome. The autosomal dominant pattern accounts for 15% of all genetic hearing loss cases. Genetic syndromes have a group of signs and symptoms that together indicate a specific disease. There are many genetic syndromes that include hearing loss as one of the symptoms.

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Glomus Tumors

Paragangliomas are also called “glomus” tumors, a commonly used but inaccurate name. These are usually benign, slow-growing but highly vascular tumors that cause symptoms by their 1) mass effect in small spaces such as the ear, 2) high blood flow, 3) invasion of adjacent structures, and 4) secretion of hormones, which is rare. As they grow, their mass effect can block sound transmission in the middle ear, causing conductive hearing loss, and the high blood flow within them is usually perceived as a pulsating sound (a form of tinnitus). With larger tumors, structures in the temporal bone can be invaded, including the facial nerve, leading to facial paralysis, or the inner ear, causing sensorineural hearing loss or vertigo (the illusion of motion). Rarely, these tumors can produce hormones such as adrenaline, which can cause episodes of sweating, flushing, headache, and a rapid heartbeat. Treatment options for paragnangliomas include 1) observation, or “watchful waiting,” where the patient has CT and/or MRI scans periodically to monitor the growth of the tumor, 2) radiation therapy, or 3) surgical removal. 

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People with hyperacusis have perfectly normal hearing. They are not able to hear "better than anybody else", but what they do have is reduced tolerance to specific sounds and sound levels that are not normally regarded as loud. For some patients, the problem arises at sound levels as low as 25 dB - making them unable to be diagnosed properly by the normal, acoustic testing equipment at the doctor's clinic, since such equipment operates at much higher sound levels - 80 dB or more. But for people with hyperacusis, these everyday sounds can cause pain, frustration, and even terror. Children have been seen running away from specific sounds, that were distressing to them - but which are harmless to everybody else. Doctors are quick to point out that the cure for hyperacusis is neither silence nor earplugs. On the contrary - too little noise can worsen the problem, gradually making the hyperacusic patient unable to lead a normal life. Instead, people suffering from reduced tolerance to sounds - hyperacusis - should be surrounded by low-level, pleasant sounds at all times: Radios at low volume or even a special noise generator can be used for constant stimulation to help the brain readjust to normal everyday sounds.

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Labyrinthitis is an ear disorder characterized by inflammation of the semicircular ear canal in the inner ear, known as the labyrinth. Due to the inflammation of the labyrinth, its function becomes interrupted, including the regulation of balance. This disorder is thought to be caused by a viral or bacterial infection, since it usually occurs following otitis media or an upper respiratory infection. It also occurs due to allergy, cholesteatoma, or ingestion of certain drugs that damage the inner ear. Labyrinthitis usually disappears spontaneously after several weeks. To ease the symptoms, the infection can be treated with antibiotics prescribed by your doctor. Moreover, it is advisable to take certain precautions during the episode of labyrinthitis, such as keeping still and resting, resuming activity gradually, and avoiding reading, sudden position changes and bright lights.

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Misophonia is characterized by having strong reactions to selective sounds. It is not to be confused with hyperacusis where sound is perceived as abnormally loud or physically painful. Hyperacusis and misophonia are both disorders related to “decreased sound tolerance”. However, hyperacusis is a condition in which auditory information is unbearably loud. In misophonia, it is repeating (or patterned sounds) which are intolerable. Individuals with misophonia are set off or “triggered” by very specific patterned sounds, such as chewing, coughing, pencil tapping, sneezing etc. Misophonia is a neurological disorder in which auditory (and sometimes visual) stimuli are misinterpreted within the central nervous system. However, misophonia and what causes misophonia is still something of a mystery. The symptoms of misophonia - strong reactions to selective sounds - often appear in late childhood but its onset can be seen at any age. It usually starts with a single trigger-sound. More sounds are then added to the list of triggers. Currently, there is no cure or treatment for misophonia. An Audiologist can however refer you to different kinds of therapy. Even just to talk about your condition might be helpful.

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Noise Induced Hearing Loss

Noise induced hearing loss is a permanent hearing impairment resulting from prolonged exposure to high levels of noise. One in 10 Americans has a hearing loss that affects his or her ability to understand normal speech. Excessive noise exposure is the most common cause of hearing loss. When noise is too loud, it begins to kill cells in the inner ear. As the exposure time to loud noise increases, more and more hair cells are destroyed. As the number of hair cells decreases, so does your hearing. Currently, there is no way to restore life to dead hair cells; the damage is permanent. Hearing loss usually develops over a period of several years. Since it is painless and gradual, you might not notice it. What you might notice is a ringing or other sound in your ear (tinnitus), which could be the result of long-term exposure to noise that has damaged hearing. If you think you have grown used to a loud noise, it probably has damaged your ears, and there is no treatment – no medicine, no surgery, not even a hearing aid, that truly corrects your hearing once it is damaged by noise. The only thing you can do at this point is to protect what remaining hearing you have. 

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Otosclerosis is caused by an abnormal growth of bone in the ears, which causes hearing loss. In most cases, a surgical operation will restore the hearing. Otosclerosis has to do with the three small bones in the middle ear, more specifically the stapes. A part of the bone will grow abnormally and this bone growth will prevent the stapes from vibrating normally in response to sound. In its early stages, the sufferer will not be affected. Otosclerosis is often inherited, although isolated cases do occur. For instance, some connections to measles and pregnancy have been observed as worsening the case of otosclerosis. Both ears may be affected but it is more common for one ear to be more severely affected than the other. If left untreated, hearing loss typically worsens progressively until late middle age when complete deafness occurs. Hearing aids help people who are suffering from conductive deafness, including otosclerosis. However, a hearing aid will not cure the deafness. Since the deafness is progressive, more powerful hearing aids may be needed as time goes by. In the early stages, hearing aids are a great help for those who do not wish to undergo surgery.

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Tinnitus is described as any abnormal ear noise and can arise in the outer ear, the middle ear, the inner ear or the brain. It is not a disorder in and of itself, but rather a symptom and may be perceived as ringing, buzzing or humming. Some people describe it as a “clicking” or “pulsing” sound. These sounds may come and go, but for most tinnitus sufferers, the symptoms produce a constant, maddening drone. The effects range from slight annoyance to severe disruption of everyday life. The American Tinnitus Association estimates that more than 50 million Americans suffer from tinnitus. Due to the personal and unique nature of each tinnitus condition, proper evaluation and specialized treatment is necessary. Although there isn’t a single cure for tinnitus, audiologists are experienced at providing individual solutions on a case-by-case basis. After completing a hearing test, your professional may refer you to an otolaryngologist for further examination, if medical treatment is necessary.

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