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All content on this website, including dictionary, thesaurus, literature, geography, and other reference data is for informational purposes only. Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the vestibulocochlear nerve (cranial nerve VIII), the inner ear, or central processing centers of the brain.
The great majority of human sensorineural hearing loss is caused by abnormalities in the hair cells of the organ of Corti in the cochlea.
Sensory hearing loss that results from abnormalities of the central auditory system in the brain is called central hearing impairment.
This type of hearing loss can also be caused by prolonged exposure to very loud noise, for example, being in a loud workplace without hearing protection, or having headphones set to high volumes for a long period. The Weber test, in which a tuning fork is touched to the midline of the forehead, localizes to the normal ear in people with this condition. Congenital cholesteatoma - squamous epithelium is normally present on either side of the tympanic membrane. Physical trauma - either due to a fracture of the temporal bone affecting the cochlea and middle ear, or a shearing injury affecting cranial nerve VIII. Perilymph fistula, usually due to a rupture of the round or oval windows and the leakage of perilymph. Cerebellopontine angle tumour (junction of the pons and cerebellum) (the cerebellopontine angle is the exit site of both the facial nerve(CN7) and the vestibulocochlear nerve(CN8). Populations living near airports or freeways are exposed to levels of noise typically in the 65 to 75 dbA range. The most common type of congenital hearing impairment in developed countries is DFNB1, also known as Connexin 26 deafness or GJB2-related deafness. The most common dominant syndromic forms of hearing impairment include Stickler syndrome and Waardenburg syndrome. The most common recessive syndromic forms of hearing impairment are Pendred syndrome, large vestibular aqueduct syndrome and Usher syndrome. Autoimmune disease has only recently been recognized as a potential cause for cochlear damage. Mumps (epidemic parotitis) may result in profound sensorineural hearing loss (90 dB or more), unilaterally (one ear) or bilaterally (both ears).
Adenoids that do not disappear by adolescence may continue to grow and may obstruct the Eustachian tube, causing conductive hearing impairment and nasal infections that can spread to the middle ear.
HIV (and subsequent opportunistic infections) may directly affect the cochlea and central auditory system. Fetal alcohol syndrome is reported to cause hearing loss in up to 64% of infants born to alcoholic mothers, from the ototoxic effect on the developing fetus, plus malnutrition during pregnancy from the excess alcohol intake. Syphilis is commonly transmitted from pregnant women to their fetuses, and about a third of the infected children will eventually become deaf.
Otosclerosis is a hardening of the stapes (or stirrup) in the middle ear, and causes conductive hearing loss. Some medications cause irreversible damage to the ear, and are limited in their use for this reason.
There can be damage either to the ear itself or to the brain centers that process the aural information conveyed by the ears. People who sustain head injury are especially vulnerable to hearing loss or tinnitus, either temporary or permanent.
Exposure to very loud noise (90 dB or more, such as jet engines at close range) can cause progressive hearing loss. Sensorineural hearing loss can be treated with hearing aids, which amplify sounds at preset frequencies to overcome a sensorineural hearing loss in that range. Some research suggests idebenone alone or combined with vitamin E may delay the onset of hearing loss or perhaps reverse it.[6] Use of these agents for this purpose is considered experimental now.
Inter-trial coherence as a marker of cortical phase synchrony in children with sensorineural hearing loss and auditory neuropathy spectrum disorder fitted with hearing aids and cochlear implants. OBJECTIVE: Although brainstem dys-synchrony is a hallmark of children with auditory neuropathy spectrum disorder (ANSD), little is known about how the lack of neural synchrony manifests at more central levels. Systemic lupus erythematosus can affect inner ear by disrupting either the hearing or balance system. Acetaminophen and NAPQI are toxic to auditory cells via oxidative and endoplasmic reticulum stress-dependent pathways. Pain relievers containing N-acetyl-para-aminophenol, also called APAP, acetaminophen or paracetamol, in combination with opioid narcotics are top-selling pharmaceuticals in the U.S.
Topical insulin-like growth factor 1 treatment using gelatin hydrogels for glucocorticoid-resistant sudden sensorineural hearing loss: a prospective clinical trial. The great majority of human sensorineural hearing loss is caused by abnormalities in the hair cells of the organ of Corti in the cochlea.
Noise induced hearing loss is a permanent hearing impairment resulting from prolonged exposure to high levels of noise. The damage caused by noise, called sensorineural hearing loss, can be caused by several factors other than noise, but noise-induced hearing loss is different in one important way – it can be reduced or prevented altogether. Human speech, which ranges from 300 to 4,000 Hz, sounds louder to most people than noises at very high or very low frequencies. Loss of high frequency hearing also can distort sound, so that speech is difficult to understand even though it can be heard. In addition, the duration (how long you are exposed to a noise) can affect the extent of noise induced hearing loss. Every gunshot produces a noise that could damage the ears of anyone in close hearing range. Habitual exposure to noise above 85 dB will cause a gradual hearing loss in a significant number of individuals, and louder noises will accelerate this damage. The Occupational Safety and Health Administration, in its Hearing Conservation Amendment of 1983, requires hearing conservation programs in noisy work places. When noise measurements indicate that hearing protectors are needed, the employer must offer at least one type of earplug and one type of earmuff without cost to employees. Non-occupational noises are also regularly encountered during recreational activities and are a source of premature hearing reduction. If you think you have grown used to a loud noise, it probably has damaged your ears, and there is currently no treatment – no medicine, no surgery, not even a hearing aid, that truly corrects your hearing once it is damaged by noise. If you suspect a hearing loss, consult a physician with special training in ear care and hearing disorders (called an otolaryngologist or otologist).
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. While it may seem silly and obvious to point this out, usually the best way to prevent future injury from noise is to avoid exposure to noise!
Earmuffs fit over the entire outer ear to form an air seal so the entire circumference of the ear canal is blocked, and they are held in place by an adjustable band. The mechanism of noise induced damage is proposed to include reactive oxygen species (Henderson, 2006; Le Prell, 2003), which can cause cell death.
Glucorticoids, such as cortisol, may modulate hearing sensitivity (Canlon et al 2007) and also shows some protective effects (Le Prell et al 2003, Oishi & Schacht 2011). Gene therapy and stem cell therapy are also under investigation for the treatment of sensorineural hearing loss(Sun et al 2011).

Studies have shown that one half of the workers wearing hearing protectors receive one half or less of the noise reduction potential of their protectors because these devices are not worn continuously while in noise or because they do not fit properly. A hearing protector that gives an average of 30 dB of noise reduction, if worn continuously during an 8-hour work day, becomes equivalent to only 9 dB of protection if taken off for one hour in the noise. During the hour with unprotected ears, the worker is exposed to 1,000 times more sound energy than if earplugs or muffs had been worn. Hearing protectors reduce hearing and therefore do reduce one’s ability to understand normal conversation. There are a number of strategies that can be used to reduce the change of noise injury from other instrumentalists. Hearing aids can be set to limit noise and thus can be set up so that they do not contribute to additional hearing loss.
The American Hearing Research Foundation is a non-profit foundation that funds research into hearing loss and balance disorders related to the inner ear and is also committed to educating the public about these health issues. Tell a friend about us, add a link to this page, or visit the webmaster's page for free fun content. There are also very unusual sensorineural hearing impairments that involve the eighth cranial nerve (the vestibulocochlear nerve) or the auditory portions of the brain. Since the auditory pathways cross back and forth on both sides of the brain, deafness from a central cause is unusual. Externally, within the external auditory meatus or ear canal and internally within the middle ear. Meniere's disease is characterized by sudden attacks of vertigo, lasting minutes to hours preceded by tinnitus, aural fullness, and fluctuating hearing loss. If lifestyles include significant outdoor or open window conditions, these exposures over time can degrade hearing. More than 40 genes have been identified to cause deafness.[2] There are also 300 syndromes with related hearing loss, and each syndrome may have causative genes. If a family has a dominant gene for deafness, it will persist across generations because it will manifest itself in the offspring even if it is inherited from only one parent. Please discuss this issue on the talk page and conform with Wikipedia's Manual of Style by replacing the section with a link and a summary of the repeated material, or by spinning off the repeated text into an article in its own right. Although probably rare, it is possible for autoimmune processes to target the cochlea specifically, without symptoms affecting other organs. It is considered by some to be a degenerative process, although there has never been a proven link to aging. Exposure to a single event of extremely loud noise (such as explosions) can also cause temporary or permanent hearing loss. In China, the First Hospital of Jiaxing cooperated together with MD Ferdinand Bisselink from the Medical Center Sanit in The Netherlands,for treatment of patients with sudden deafness. One in 10 Americans has a hearing loss that affects his or her ability to understand normal speech. This typically occurs in individuals who are exposed to gunfire or firecrackers, and hear ringing in their ears after the event (tinnitus). The scale runs from the faintest sound the human ear can detect, which is labeled 0 dB, to over 180 dB, the noise at a rocket pad during launch. When hearing impairment begins, the high frequencies are often lost first, which is why people with hearing loss often have difficulty hearing the high-pitched voices of women and children. Hearing impaired people often have difficulty detecting differences between certain words that sound alike, especially words that contain S, F, SH, CH, H, or soft C, sounds, because the sound of these consonant is in a much higher frequency range than vowels and other consonants.
For unprotected ears, the allowed exposure time decreases by one half for each 5 dB increase in the average noise level. This includes a yearly hearing test for the approximately five million workers exposed to an average of 85 dB or more of noise during an 8-hour work day. As an alternative, individual hearing protectors are required when noise averages more than 90 dB during an 8-hour day. If the yearly hearing tests reveal hearing loss of 10 dB or more in higher pitches in either ear, the worker must be informed and must wear hearing protectors when noise averages more than 85 dB for an 8-hour day. Peak noise levels, in dB, are provided in the following table taken from Smith et al, 1999.
Genetic factors may make some individuals more susceptible (Davis et al 2003, Konings et al 2009).
As a general rule, noise may damage your hearing if you have to shout over background noise to make yourself heard, the noise hurts your ears, it makes your ears ring, or you are slightly deaf for several hours after exposure to the noise. Examples of situations where you should wear them are when you are using power tools, noisy yard equipment, or firearms. Earmuffs will not seal around eyeglasses or long hair, and the adjustable headband tension must he sufficient to hold earmuffs firmly around the ear. The better earplugs and muffs are approximately equal in sound reduction, although earplugs are better for low frequency noise and earmuffs for high frequency noise. This is because decibels are measured on a logarithmic scale, and there is a 10-fold increase in noise energy for each 10 dB increase. A normal hearing person wearing hearing protectors should be able to understand a regular conversation.
High frequencies tend to emanate in almost a straight line, while low frequencies are present in nearly all directions. For someone whose life is affected by noise induced hearing loss, a hearing aid may be a very reasonable thing to consider.
At the American Hearing Research Foundation (AHRF), we have funded basic research on noise induced hearing loss in the past, and are interested in funding sound research in the future.
In the rarest of these sorts of hearing loss, only the auditory centers of the brain are affected. There are both external causes of damage, like noise trauma and infection, and intrinsic abnormalities, like deafness genes. Within the middle ear the simple epithelium gradually transitions into ciliated pseudostratified epithelium lining the Eustachian tube now known as the pharyngotympanic tube becoming continuous with the respiratory epithelium in the pharynx. A history of an event that increased intracranial pressure or caused trauma is usually present). For example, the safe daily exposure amount at 85 dB is 8 hours, while the safe exposure at 91 dB(A) is only 2 hours (National Institute for Occupational Safety and Health, 1998). If a family had genetic hearing impairment caused by a recessive gene, it will not always be apparent, as it will have to be passed onto offspring from both parents. Decibels are measured logarithmically, being 20 times the log of the ratio of a particular sound pressure to a reference sound pressure. But even cap guns and firecrackers can damage your hearing if the explosion is close to your ear.
Other things that have been linked with an increased risk of noise induced hearing loss include smoking (Palmer et al 2004, Wild et al 2005), male gender, race, poor diet, diabetes, cardiovascular disease (Daniel 2007) and concomitant exposure to carbon monoxide or hydrogen cyanide (Fechter 2004).
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. They are available in a variety of shapes and sizes to fit individual ear canals and can be custom made.
Simultaneous use of earplugs and muffs usually adds 10 to 15 dB more protection than either used alone. Antioxidants that have been studies include N-acetylcyteine, magnesium, salicylate, vitamin E and ebselen (Coleman et al 2007, Le Prell et al 2007, Lynch & Kil 2005, Sendowski et al 2006, Suckfuell et al 2007).

D-methionine (D-met), salicylate, ebselen, N-acetylcysteine (NAC), ACE Mg, and sodium thiosulfate are all in or near clinical trials.
A person who has impaired hearing already, perhaps due to noise, might be unable to understand coworkers when they are wearing hearing protectors.
A ringing in the ears, called tinnitus, commonly occurs after noise exposure, and it often becomes permanent. Learn more about donating to American Hearing Research Foundation (AHRF) to diagnose and treat noise induced hearing loss. Transcriptional changes in adhesion-related genes are site-specific during noise-induced cochlear pathogenesis.
Pharmacological rescue of noise induced hearing loss using N-acetylcysteine and acetyl-L-carnitine. Therapeutic efficacy of magnesium after acoustic trauma caused by gunshot noise in guinea pigs. Cell-cell junctions: a target of acoustic overstimulation in the sensory epithelium of the cochlea. In this situation, cortical deafness, sounds may be heard at normal thresholds, but the quality of the sound perceived is so poor that speech cannot be understood.
The squamous epithelium hyperplasia within the middle ear behaves like an invasive tumour and destroys middle ear structures if not removed.
EPA and various states have set noise standards to protect people from these adverse health risks. From the treated patients more as 80% get the hearing back after a treatment in a single user HBOT cabine for 40 times, which was done in 5 days per week. The National Institute of Health reports that about 15 percent of Americans aged 20 to 69 have high frequency hearing loss related to occupational or leisure activities. This means that as decibel intensity increases by units of 20, each increase is 10 times the lower figure. A low pitch such as a deep voice or a tuba makes fewer vibrations per second than a high voice or violin. The highest permissible noise exposure for the unprotected ear is 115 dB for 15 minutes per day.
Or, you may have trouble understanding what people say; they may seem to be mumbling, especially when you are in a noisy place such as in a crowd or at a party. It is also generally prudent to avoid things that might contribute to ear damage — try to avoid ototoxic drugs like aspirin, and avoid whenever possible exposing yourself to situations where your ear might be damaged (for example, Scuba diving). Two recent studies have demonstrated a degradation of cell-cell junctions (important for structural and function purposes) and genetic transcriptional changes in animal models. See the work of Kathleen Campbell for a thorough discussion of pharmacologic otoprotective agents .
A maximum allowable while on-the-job followed by exposure to a noisy lawnmower or loud music will definitely exceed the safe daily limit.
Ear monitors are small in-the-ear devices that look like hearing aids that can be used to electronically protect hearing, while allowing the musicians to hear themselves. Humming just prior to, and through a loud noise such as a cymbal crash or rim shot may provide some protection. Some people react to loud noise with anxiety and irritability, an increase in pulse rate and blood pressure, or an increase in stomach acid. The EPA has identified the level of 70 db(A) for 24 hour exposure as the level necessary to protect the public from hearing loss (EPA, 1974). Recent gene mapping has identified dozens of nonsyndromic dominant (DFNA#) and recessive (DFNB#) forms of deafness. Because of occupational risk of noise induced hearing loss, there are government standards regulating allowable noise exposure. Thus, 20 decibels is 10 times the intensity of 0 decibels, and 40 decibels is 100 times as intense as 20 decibels.
Unlike other instruments, the ability to hear the high-frequency harmonics is crucial to these musicians. Note that for such situations, it may be that there is no type of hearing protection that will stop a very loud noise from affecting you. This means that noise-induced damage can modify “genetic maintenance” of the cochlea (Cai et al 2012, Zheng & Hu 2012). Acoustic monitors are stethoscope like devices that block sound from other in the group, but allow the instrumentalist to hear their own instrument. Small protective muscles in the ear contract naturally when we sing or hum, and thus humming may protect from other noises. Very loud noise can reduce efficiency in performing difficult tasks by diverting attention from the job. People working before the mid1960s may have been exposed to higher levels of noise where there were no laws mandating use of devices to protect hearing. If you have any of these symptoms, you may have nothing more serious than impacted wax or an ear infection, which might be simply corrected. Too much ear protection can result in overplaying and not enough protection can result in hearing loss. Persons interested in the actual physical quantification of sound use units of sound pressure level (SPL). In addition, attending live concerts (where noise levels can exceed 120 dB) can damage hearing, as well as listening to loud music through headphones. Evidence suggests that loud rock music along with increased use of portable radios with earphones may be responsible for this phenomenon.
Young children, who generally have the best hearing, can often distinguish sounds from about 20 Hz, such as the lowest note on a large pipe organ, to 20,000 Hz, such as the high shrill of a dog whistle that many people are unable to hear.
On audiograms, however, sound intensity is calibrated in hearing level (HL), meaning that the reference sound is one that that just barely heard by a normal population. Ordinary cotton balls or tissue paper wads stuffed into the ear canals are very poor protectors; they reduce noise only by approximately 7 dB. The sound will be perceived in the affected ear when a unilateral conductive hearing loss is present or in the unaffected ear when there is a unilateral sensorineural hearing loss. Advances in hearing aid technology and cochlear implants are helping to improve communication for people affected by hearing loss.
You will be asked about other medical conditions and about what over-the-counter and prescription medications you take. You may be asked to cover one ear at a time while listening to sounds at different volumes. Tests using a tuning fork can check if there is damage to the parts of the middle ear and eardrum that vibrate. However, the doctor will try to identify the cause of your hearing loss before treatment can begin.

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