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Causes of Deafness

Populations of people living near airports or freeways are exposed to levels of noise typically in the 65 to 75 dB(A) range. If lifestyles include significant outdoor or open window conditions, these exposures over time can degrade hearing.

The U.S. EPA and various states have set noise standards to protect people from these adverse health risks. 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 and other disruptive effects from noise, such as sleep disturbance, stress-related problems, learning detriment, etc.

Noise-Induced Hearing Loss (NIHL) typically is centered at 3000, 4000, or 6000 Hz. As noise damage progresses, damage starts affecting lower and higher frequencies.

On an audiogram, the resulting configuration has a distinctive notch, sometimes referred to as a “noise notch.” As aging and other effects contribute to higher frequency loss (6-8 kHz on an audiogram), this notch may be obscured and entirely disappear.

Louder sounds cause damage in a shorter period of time. Estimation of a “safe” duration of exposure is possible using an exchange rate of 3 dB. As 3 dB represents a doubling of intensity of sound, duration of exposure must be cut in half to maintain the same energy dose. For example, the “safe” daily exposure amount at 85 dB A, known as an exposure action value, is 8 hours, while the “safe” exposure at 91 dB(A) is only 2 hours (National Institute for Occupational Safety and Health, 1998).

Note that for some people, sound may be damaging at even lower levels than 85 dB A. Exposures to other ototoxins (such as pesticides, some medications including chemotherapy, solvents, etc.) can lead to greater susceptibility to noise damage, as well as causing their own damage. This is called asynergistic interaction.

Most people are unaware of the presence of environmental sound at damaging levels, or of the level at which sound becomes harmful. Common sources of damaging noise levels include car stereos, children’s toys, transportation, crowds, lawn and maintenance equipment, power tools, gun use, and even hair dryers.

Noise damage is cumulative; all sources of damage must be considered to assess risk. If one is exposed to loud sound (including music) at high levels or for extended durations (85 dB A or greater), then hearing impairment will occur. Sound levels increase with proximity; as the source is brought closer to the ear, the sound level increases.

Genetic Deafness

Hearing loss can be inherited. Both dominant genes and recessive genes exist which can cause mild to profound impairment. 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.

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. Dominant and recessive hearing impairment can be syndromic or non-syndromic.

Disease or illness

  • Measles may result in auditory nerve damage
  • Meningitis may damage the auditory nerve or the cochlea
  • Autoimmune disease has only recently been recognized as a potential cause for cochlear damage. Although probably rare, it is possible for autoimmune processes to target the cochlea specifically, without symptoms affecting other organs.Wegener’s granulomatosis is one of the autoimmune conditions that may precipitate hearing loss.
  • Mumps (Epidemic parotitis) may result in profound sensorineural hearing loss(90 Decibel|dB or more), unilateral (one ear) or bilateral (both ears).
  • Presbycusis is a progressive hearing impairment accompanying age, typically affecting sensitivity to higher frequencies (above about 2 kHz).
  • 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.
  • AIDS and ARC patients frequently experience auditory system anomalies.
  • HIV (and subsequent opportunistic infections) may directly affect the cochlea and central auditory system.
  • Chlamydia may cause hearing loss in newborns to whom the disease has been passed at birth.
  • 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.
  • Premature birth results in sensorineural hearing loss approximately 5% of the time.
  • 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.
  • Superior canal dehiscence, a gap in the bone cover above the inner ear, can lead to low-frequency conductive hearing loss, autophony and vertigo

Medications

Some medications cause irreversible damage to the ear, and are limited in their use for this reason. The most important group is the aminoglycosides (main member gentamicin).
Various other medications may reversibly affect hearing. This includes some diuretics, aspirin and NSAIDs, and macrolide antibiotics.

Extremely heavy hydrocodone (Vicodin or Lorcet) abuse is known to cause hearing impairment. Commentators have speculated that radio talk show host Rush Limbaugh’s hearing loss was at least in part caused by his admitted addiction to narcotic pain killers, in particular Vicodin and OxyContin

Exposure to Ototoxic Chemicals

In addition to medications, hearing loss can also result from specific drugs; metals, such as lead; solvents, such as toluene; and asphyxiants. These are mostly industrial chemicals, uncommon in residencies. Combined with noise, these ototoxic chemicals have an additive effect on a person’s hearing loss.

Hearing loss due to chemicals starts in the high frequency range and is irreversible. It damages the cochlea with lesions and degrades central portions of the auditory system.

For some ototoxic chemical exposures, particularly styrene,the risk of hearing loss can be higher than being exposed to noise alone. Controlling noise and using hearing protectors are insufficient for preventing hearing loss from these chemicals. However, taking antioxidants helps prevent ototoxic hearing loss, at least to a degree

Physical trauma

  • 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. Exposure to a single event of extremely loud noise (such as explosions) can also cause temporary or permanent hearing loss. A typical source of acoustic trauma is an excessively loud music concert.

Categories of hearing impairment

Hearing loss is categorized by its severity and by the age of onset. Two persons with the same severity of hearing loss will experience it quite differently if it occurs early or late in life. Furthermore, a loss can occur on only one side (unilateral) or on both (bilateral).

Types

As discussed above, there are three major types of hearing loss: neural/sensorineural, conductive, or a combination of both. Treatment depends upon the type of hearing loss that is present.

Quantification of hearing loss

An audiologist conducting an audiometric hearing test in a sound-proof testing booth.

The severity of hearing loss is measured by the degree of loudness, as measured in decibels, a sound must attain before being detected by an individual.

Hearing loss may be ranked as mild, moderate, severe or profound. It is quite common for someone to have more than one degree of hearing loss (i.e. mild sloping to severe).

The following list shows the rankings and their corresponding decibel ranges:

  • Mild:
    • for adults: between 25 and 40 dB
    • for children: between 20 and 40 dB
  • Moderate: between 41 and 55 dB
  • Moderately severe: between 56 and 70 dB
  • Severe: between 71 and 90 dB
  • Profound: 90 dB or greater

The quietest sound one can hear at different frequencies is plotted on an audiogram to reflect one’s ability to hear at different frequencies. The range of normal human hearing (from the softest audible sound to the loudest comfortable sound) is so great that the audiogram must be plotted using a logarithmic scale.

This large normal range, and the different amounts of hearing loss at different frequencies, make it virtually impossible to accurately describe the amount of hearing loss in simple terms such as percentages or the rankings above.

Measuring hearing loss in terms of a percentage is debatable in terms of effectiveness, and has been compared to measuring weight in inches. Though in specific legal situations, where decibels of loss are converted via a recognized legal formula, one can infer a standardized “percentage of hearing loss” which is suitable for legal purposes only.

Another method for determining hearing loss, is the Hearing in Noise Test (HINT). HINT technology was developed by the House Ear Institute, and is intended to measure an ability to understand speech in quiet and noisy environments.

Unlike pure-tone tests, where only one ear is tested at a time, HINT evaluates hearing using both ears simultaneously (binaural), as binaural hearing is essential for communication in noisy environments, and for sound localization.

Age of onset of deafness

The age at which the deafness develops is crucial to spoken language acquisition. Post-lingual deafness are far more common than pre-lingual deafness.

If the hearing loss occurs at a young age, interference with the acquisition of spoken language and social skills may occur. Hearing aids, which amplify the incoming sound, may alleviate some of the problems caused by hearing impairment, but are often insufficient. Cochlear implants artificially stimulate the VIIIth Nerve by providing an electric impulse substitution for the firing of hair cells.

Cochlear implants are not only expensive, but require sophisticated programming in conjunction with patient training for effectiveness. People who have hearing impairments, especially those who develop a hearing problem in childhood or old age, require support and technical adaptations as part of the rehabilitation process.

Pre-lingual deafness

Prelingual deafness is hearing impairment that is sustained prior to the acquisition of language, which can occur as a result of a congenital condition or through hearing loss in early infancy.

Prelingual deafness impairs an individual’s ability to acquire a spoken language, but children born into signing families rarely have delays in language development. Most pre-lingual hearing impairment is acquired via either disease or trauma rather than genetically inherited, so families with deaf children nearly always lack previous experience with sign language.

Post-lingual deafness

Post-lingual deafness where hearing loss is adventitious after the acquisition of speech and language, usually after the age of six. It may develop due to disease, trauma, or as a side-effect of a medicine. Typically, hearing loss is gradual and often detected by family and friends of the people so affected long before the patients themselves will acknowledge the disability. Common treatments include hearing aids and learning lip reading.

Hard-of-hearing

People who are hard of hearing have varying amounts of hearing loss but usually not enough to be considered deaf. How people classify themselves relative to hearing loss or deafness is a very personal decision and reflects much more than just their ability to hear.

The phrase hard of hearing, normally used as an adjective, can also be used as a noun, referring to people with hearing impairment as the hard of hearing. People who consider themselves culturally Deaf prefer the term “hard of hearing” or “deaf”, and perceive “hearing impaired” as an insult.

For many people, some of whom are associated with IFHOH, IFHOHYP organizations, the difference between hard of hearing and deaf lies mostly in one’s preference to use oral or sign language. Hard of hearing people prefer oral language alone or sign language derived from (or used together with) oral language. They see deaf people/communities as preferring to use sign language as primary means of communication.

There is, however, often no clear distinction and terminology quite varies between countries or communities, see alternative opinion on “Hard of Hearing”. Additionally also “deafened”, “late-deafened”, “persons with hearing loss/hearing disability” are used.

Hearing impaired persons with partial loss of hearing may find that the quality of their hearing varies from day to day, from one situation to another, or not at all. They may also, to a greater or lesser extent, depend on both hearing aids and lip-reading. They may perhaps not always be aware of it, but they do admit it is important to see the speaker’s face in conversation.

Many people with hearing loss have better hearing in the lower frequency ranges (low tones), and cannot hear as well or at all in the higher frequencies. Some people may merely find it difficult to differentiate between words that begin with consonantal sounds such as the fricatives or sibilants, z, or th, or the plosives d, t, b, or p. They may be unable to hear thin, high-pitched or metallic noises, such as birds chirping or singing, clocks ticking, etc. Often, they are able to hear and understand men’s voices better than women’s.

Others will find their condition much worse if circumstances in their immediate environment affect the way they are able to use their hearing aids, or prevent them from employing their speech reading skills. A room with a high ceiling and a lot of reverberation will affect the sound of a speaker’s voice adversely.

The position of the listener, too, can make a difference; for example, if only the listener’s ineffectual ear is turned towards the speaker, then the listener will have trouble hearing. Difficulties can also arise for the listener trying to lip-read, if the speaker is sitting with his back against the light-source and is in this way obscuring his face. A rule of thumb is that bright lighting is to the hearing-impaired what noise is to the hearing: a source of distraction.

The speaker’s accent; a topic with many potentially unfamiliar words; the softness of his/her voice; a speech impediment; a habit of holding a hand in front of his/her mouth or turning his/her face away at times: all these tendencies cause problems for the hard-of-hearing, especially when they have to rely on lip-reading. Rustling papers and turning pages are the noises that hearing aids pick up first.

Noisy situations are especially difficult, because hearing loss affects not only the ability to hear sounds, but also the ability to localize and filter out background noise.

Unilateral hearing loss

People with unilateral hearing loss (single sided deafness/SSD) can hear typically (or better) in one ear, but have trouble hearing in the other ear. Problems with this type of deficit is inability to localize sounds (ie. unable to tell where traffic is coming from) and inability to process out background noise in a noisy environment, such as in a restaurant.

Social impact

Pre-lingual impairment

In children, hearing loss can lead to social isolation for several reasons. First, the child experiences delayed social development that is in large part tied to delayed language acquisition. It is also directly tied to their inability to pick up auditory social cues. This can result in a deaf person becoming generally irritable.

A child who uses sign language, or identifies with the Deaf sub-culture does not generally experience this isolation, particularly if he/she attends a school for the deaf, but may conversely experience isolation from his parents if they do not know sign language.

A child who is exclusively or predominantly oral (using speech for communication) can experience social isolation from his or her hearing peers, particularly if no one takes the time to explicitly teach her social skills that other children acquire independently by virtue of having normal hearing.

Finally, a child who has a severe impairment and uses some sign language may be rejected by Deaf peers, because of an understandable hesitation in abandoning the use of existent verbal and speech-reading skills. Some in the Deaf community can view this as a rejection of their own culture and its mores, and therefore will reject the individual preemptively.

Post-lingual impairment

Those who lose their hearing later in life, such as in late adolescence or adulthood, face their own challenges, living with the adaptations that make it possible for them to live independently.

They may have to adapt to using hearing aids or a cochlear implant, develop speech-reading skills, and/or learn sign language. The affected person may need to use a TTY (teletype), interpreter, or relay service to communicate over the telephone.

Loneliness and depression can arise as a result of isolation (from the inability to communicate with friends and loved ones) and difficulty in accepting their disability. The challenge is made greater by the need for those around them to adapt to the person’s hearing loss.

Many relationships can suffer because of emotional conflicts that occur when there are general miscommunications between family members. Generally, it’s not only the person with a hearing disability that feels isolated, but others around them who feel they are not being “heard” or paid attention to, especially when the hearing loss has been gradual.

Many people opt not to choose hearing aids for fear of looking old, since hearing loss is usually associated with old age, which equals ineffectiveness in some societies. Family members then feel as if their hearing loss partner doesn’t care about them enough to make changes to reduce their disability and make it easier to communicate.

Hearing loss in children

12% of children aged 6 – 19 years have permanent hearing damage from excessive noise exposure. The American Academy of Pediatrics advises that children should have their hearing tested several times throughout their schooling:

  • When they enter school
  • At ages 6, 8, and 10,
  • At least once during middle school
  • At least once during high school

Besides screening children for hearing loss, schools can also educate them on the perils of hazardous noise exposure. Research has shown that people who are educated about noise-induced hearing loss and prevention are more likely to use hearing protectors at work or in their private live.

Medical treatments

Approaches

In addition to hearing aids there exist cochlear implants of increasing complexity and effectiveness. These are useful in treating the mild to profound hearing impairment when the onset follows the acquisitions of language and in some cases in children whose hearing loss came before language was acquired.

Recent research shows variations in efficacy but some studies,show that if implanted at a very young age, some profoundly impaired children can acquire effective hearing and speech, particularly if supported by appropriate rehabilitation such as auditory-verbal therapy

Views of treatments

There has been and will continue to be considerable controversy within the culturally Deaf community over cochlear implants. For the most part, there is little objection to those who lost their hearing later in life or culturally Deaf adults (voluntarily) choosing to be fitted with a cochlear implant.

Many in the culturally Deaf community strongly object to a deaf child being fitted with a cochlear implant (often on the advice of an audiologist; new parents may not have sufficient information on raising deaf children and placed in an oral-only program that emphasizes the ability to speak and listen over other forms of communication such as sign language.

Another issue is the fact that the implanted deaf child has to avoid team or full-contact sports to minimize the chances of a head injury, which carries a greater risk where the implant is involved.

Gene therapy

A 2005 study achieved successful regrowth of cochlea cells in guinea pigs. It is important to note, however, that the regrowth of cochlear hair cells does not imply the restoration of hearing sensitivity as the sensory cells may or may not make connections with neurons that carry the signals from hair cells to the brain.

A 2008 study has shown that gene therapy targeting Atoh1 can cause hair cell growth and attract neuronal processes in embryonic mice. It is hoped that a similar treatment will one day ameliorate hearing loss in humans.

Adaptations to hearing impairment

Many hearing impaired individuals use assistive devices in their daily lives:

  • Individuals can communicate by telephone using Telecommunications Device for the Deaf (TDD). These devices look like a typewriters or word processors and transmit typed text over regular telephone lines. Other names in common use are textphone and minicom.
  • There are several new Telecommunications Relay Service technologies including IP Relay and captioned telephone technologies.
  • Mobile textphone devices came onto the market as of 2004, allowing simultaneous two way text communication.
  • Videophones and similar video technologies can be used for distance communication using sign language. Video conferencing technologies permit signed conversations as well as permitting an sign language-English interpreter to voice and sign conversations between a hearing impaired person and that person’s hearing party, negating the use of a TTY device or computer keyboard.
  • In the U.S., the UK, the Netherlands and many other western countries there are Telecommunications Relay Services so that a hearing impaired person can communicate over the phone with a hearing person via a human translator. Wireless, internet and mobile phone/SMS text messaging are beginning to take over the role of the TDD.
  • Phone captioning is a service in which a hearing person’s speech is captioned by a third party, enabling a hearing impaired person to conduct a conversation with a hearing person over the phone.
  • Hearing dogs are a specific type of assistance dog specifically selected and trained to assist the deaf and hearing impaired by alerting their handler to important sounds, such as doorbells, smoke alarms, ringing telephones, or alarm clocks.
  • Other assistive devices include those that use flashing lights to signal events such as a ringing telephone, a doorbell, or a fire alarm.

Many different assistive technologies, such as hearing aids, are available to those who are hearing impaired. People with cochlear implants, hearing aids, or neither of these devices can also use additional communication devices to reduce the interference of background sounds, or to mediate the problems of distance from sound and poor sound quality caused by reverberation and poor acoustic materials of walls, floors and hard furniture.

Three types of wireless, one-way wireless exist along with hard-wired devices. A wireless device used by people who use their residual hearing has two main components. One component sends the sound out to the listener, but is not directly connected to the listener with the hearing loss. The second component of the wireless system, the receiver, detects the sound and sends the sound to the ear of the person with the hearing loss. The three types of wireless devices are the FM system, the audio induction loop and the infra red system. Each system has advantages and benefits for particular uses.

The FM system can easily operate in many environments with battery power. It is thus mobile and does not usually require a sound expert for it to work properly. The listener with the hearing loss carries a receiver and an earpiece. Another wireless system is the audio induction loop which permits the listener with hearing loss to be free of wearing a receiver provided that the listener has a hearing aid or cochlear implant processor with an accessory called a “telecoil”.

If the listener doesn’t have a t-coil or telecoil, then she must carry a receiver with an earpiece. The third kind of wireless device for people with hearing loss is the infra red (IR) device which also requires a receiver to be worn by the listener. Usually the emitter for the IR device, that is, the component that sends out the signal, uses an AC adaptor.

The advantage of the IR wireless system, is that people in adjoining rooms cannot listen in on conversations, and thus it is confidential and necessary for situations where privacy and confidentiality are required or chosen. Another way to achieve confidentiality is to use a hardwired amplifier which sends out no signal beyond the earpiece that is plugged directly into the amplifier. That amplifier of the hardwired device also has a microphone inside of it or plugged into it.

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