DEAFNESS

Presented By:
Shehzadi Noor-E-Sehar
Roll No:19
MS-1

Presented To:
Dr.Mehwish
Medical Biotechnology

LAHORE COLLEGE FOR WOMEN

UNIVERSITY
Contents:

introduction ......................................4
Classification....................................5
Conductive and sensorineural hearing impairments....................................................................5

Causes of conductive hearing loss...............................................................................................6

Causes of sensorineural hearing loss...........................................................................................8

Quantification of hearing loss...........8

Age of onset.......................................9
Pre-lingual deafness.....................................................................................................................9

Post-lingual deafness...................................................................................................................9

Unilateral and bilateral hearing impairment............................................................9

Causes.............................................10
Age.............................................................................................................................................10

Long-term exposure to environmental noise.............................................................................10

Genetic.......................................................................................................................................11

Disease or illness........................................................................................................................12

Medications................................................................................................................................13

Exposure to ototoxic chemicals.................................................................................................14

Physical trauma..........................................................................................................................15

Management....................................15
Approaches................................................................................................................................15

Views of treatments...................................................................................................................16

Gene therapy..............................................................................................................................16

Assistive techniques and devices for hearing impairment.........................................................16

Resources and interventions.....................................................................................................18

Hearing loss in children.................19
Genetic deafness in Pakistani population...............................................................20
Myths regarding people with hearing losses...........................................................21
Social consequences........................22
Inclusion vs. pullout...................................................................................................................22

References.......................................24
DEAFNESS

INTRODUCTION
Deafness is a condition wherein the ability to detect certain frequencies of sound is
completely or partially impaired. When applied to humans, the term hearing impaired is rejected
by the Deaf Culture movement, where the terms deaf and hard-of-hearing are preferred.

Hearing sensitivity is indicated by the quietest sound that a person can detect, called
the hearing threshold. In the case of humans and some animals, this threshold can be accurately
measured by a behavioral audiogram. A record is made of the quietest sound that consistently
prompts a response from the listener. The test is carried out for sounds of different frequencies.
There are also electro-physiological tests that can be performed without requiring a behavioral
response.

Fig: Anatomy of human ear

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 Normal hearing thresholds within any given species are not the same for all frequencies. If
different frequencies of sound are played at the same amplitude, some will be perceived as loud,
and others quiet or even completely inaudible. Generally, if the gain or amplitude is increased, a
sound is more likely to be perceived. Ordinarily, when persons use sound to communicate,
hearing in that type of animal is most sensitive for the frequencies produced by calls, or in the
case of humans, speech. All levels of the auditory system contribute to this sensitivity toward
certain frequencies, from the outer and tracts that convey the nerve impulses of the auditory
portion of the brain. Another aspect to hearing involves the perceived clarity of a sound rather
than its amplitude. In humans, that aspect is usually measured by tests of speech perception.
These tests measure one's ability to understand speech, not to merely detect sound.

CLASSIFICATION
Hearing impairments are categorized by their type (conductive, sensorineural, or both), by
their severity, and by the age of onset. Furthermore, a hearing impairment may exist in only one
ear (unilateral) or in both ears (bilateral).

Fig: difference between conductive and sensorineural deafness.

CONDUCTIVE AND SENSORINEURAL HEARING IMPAIRMENTS

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 A conductive hearing impairment is an impairment resulting from dysfunction in any of the
mechanisms that normally conduct sound waves through the outer ear, the eardrum or the bones
of the middle ear.

CAUSES OF CONDUCTIVE HEARING LOSS

External ear

 Cerumen (earwax)
 Otitis externa (ear infection)
 Foreign body in the external auditory canal (not always)
 Exostoses
 Tumour of the ear canal
 Congenital atresia

Tympanic membrane

 Tympanic membrane perforation

 Membrane tension by different pressures in the external and middle ear. This
can temporarily occur, for example, by the environmental pressure changes as
when shifting altitude, or inside a train going into a tunnel. It is managed by any of
various methods of ear clearing maneuvers to equalize the pressures.

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Middle ear

Fluid accumulation is the most common cause of conductive hearing loss in the middle ear,
especially in children. Major causes are ear infections or conditions that block the eustachian
tube, such as allergies or tumors. Blocking of the eustachian tube leads to increased pressure in
the middle ear relative to the external ear, and this causes decreased motion of both
the ossicles and the tympanic membrane.

Inner ear

Severe Otosclerosis, form of mechanical conductive hearing loss most commonly found in
people who have been subjected to intense noise. Occurs when there is an obstruction in either
the oval window and/or the round window. This type of hearing loss can usually be repaired by
surgical opening of the blockage.

A sensorineural hearing impairment is one resulting from dysfunction in the inner ear,
especially the cochlea where sound vibrations are converted into neural signals, or in any part of
the brain that subsequently processes these signals. The vast majority of human sensorineural
hearing loss is associated with abnormalities in the hair cells of the organ of Corti in the cochlea.
This dysfunction may be present from birth due to genetic or developmental abnormalities, or
arise through trauma or disease during the lifetime of an individual. There are also very unusual
sensorineural hearing impairments that involve the VIIIth cranial nerve, the Vestibulocochlear
nerve or, in rare cases, auditory cortex. Damage to parts of the brain that process auditory signals
can lead to a condition in which sounds may be heard at normal thresholds, but the quality of the
sound perceived is so poor that speech cannot be understood. Sensorineural hearing loss
associated with abnormalities of the auditory system in the brain is called Central Hearing
Impairment.

The great majority of human sensorineural hearing loss is caused by abnormalities in

the hair cells of the organ of Corti in the cochlea. There are also very unusual sensorineural
hearing impairments that involve the eighth cranial nerve (the vestibulocochlear nerve) or the
auditory portions of the brain. In the rarest of these sorts of hearing loss, only the auditory
centers of the brain are affected. In this situation, central hearing loss, sounds may be heard at
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normal thresholds, but the quality of the sound perceived is so poor that speech can not be
understood.

CAUSES OF SENSORINEURAL HEARING LOSS

Sensory hearing loss that results from abnormalities of the central auditory system in the
brain is called central hearing impairment. Since the auditory pathways cross back and forth on
both sides of the brain, deafness from a central cause is unusual. This type of hearing loss can
also be caused by prolonged exposure to very loud noise, for example, by wearing headphones
on full blast for a few hours.

QUANTIFICATION OF HEARING LOSS

The severity of a hearing impairment is ranked according to the additional intensity above a
nominal threshold that a sound must be before being detected by an individual; it is (measured
in decibels of hearing loss, or dB HL). Hearing impairment may be ranked as mild, moderate,
moderately severe, severe or profound are defined.

Mild:

 For adults: between 26 and 40 dB HL

 For children: between 20 and 40 dB HL

 Moderate: between 41 and 55 dB HL

 Moderately severe: between 56 and 70 dB HL

 Severe: between 71 and 90 dB HL

 Profound: 90 dB HL or greater

Hearing sensitivity varies according to the frequency of sounds. To take this into account,
hearing sensitivity can be measured for a range of frequencies and plotted on an audiogram.

For certain legal purposes such as insurance claims, hearing impairments are described in
terms of percentages. Given that hearing impairments can vary by frequency and that audiograms
are plotted with a logarithmic scale, the idea of a percentage of hearing loss is somewhat

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arbitrary, but where decibels of loss are converted via a recognized legal formula, it is possible to
calculate a standardized "percentage of hearing loss" which is suitable for legal purposes only.

AGE OF ONSET
The age at which hearing loss occurs is crucial for the acquisition of a spoken language.

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 is hearing impairment that is sustained after the acquisition of
language, which can occur as a result of disease, trauma, or as a side-effect of a medicine.
Typically, hearing loss is gradual and often detected by family and friends of affected individuals
long before the patients themselves will acknowledge the disability. Common treatments include
hearing aids and learning lip reading. Post-lingual deafness is far more common than pre-lingual
deafness.

UNILATERAL AND BILATERAL HEARING IMPAIRMENT

People with unilateral hearing impairment (single sided deafness/SSD) have an impairment
in only one ear. This can impair a person's ability to localize sounds (e.g., determining where
traffic is coming from) and distinguish sounds from background noise in noisy environments.

A similar effect can result from King-Kopetzky syndrome (also known as Auditory
disability with normal hearing and obscure auditory dysfunction), which is characterized by an
inability to process out background noise in noisy environments despite normal performance on
traditional hearing tests. See also: "cocktail party effect", House Ear Institute's Hearing In Noise
Test.

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CAUSES
The following are some of the major causes of hearing loss.

AGE
Presbycusis, the progressive loss of ability to hear high frequencies with increasing age,
begins in early adulthood, but does not usually interfere with ability to understand conversation
until much later. Although genetically variable it is a normal concomitant of aging and is distinct
from hearing losses caused by noise exposure, toxins or disease agents.

LONG-TERM EXPOSURE TO ENVIRONMENTAL NOISE

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. (EPA, 1974).

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.

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 Some American health and safety agencies (such as OSHA-Occupational Safety and Health
Administration and MSHA-Mine Safety and Health Administration), use an exchange rate of 5
dB. While this exchange rate is simpler to use, it drastically underestimates the damage caused
by very loud noise. For example, at 115 dB, a 3 dB exchange rate would limit exposure to about
half a minute; the 5 dB exchange rate allows 15 minutes.

While OSHA, MSHA, and FRA provide guidelines to limit noise exposure on the job, there
is essentially no regulation or enforcement of sound output for recreational sources and
environments, such as sports arenas, musical venues, bars, etc. This lack of regulation resulted
from the defunding of ONAC, the EPA's Office of Noise Abatement and Control, in the early
1980s. ONAC was established in 1972 by the Noise Control Act and charged with working to
assess and reduce environmental noise. Although the Office still exists, it has not been assigned
new funding.

Many 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
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 nonsyndromic. Recent gene mapping has
identified dozens of nonsyndromic dominant (DFNA#) and recessive (DFNB#) forms of
deafness.

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 The first gene mapped for non-syndromic deafness, DFNA1, involves a splice
site mutation in the formin related homolog diaphanous 1 (DIAPH1). A single base
change in a large Costa Rican family was identified as causative in a rare form of low
frequency onset progressive hearing loss with autosomal dominant inheritance
exhibiting variable age of onset and complete penetrance by age 30.
 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.
 The congenital defect microtia can cause full or partial deafness depending upon
the severity of the deformity and whether or not certain parts of the inner or middle ear
are affected.
 Mutations in PTPRQ Are a Cause of Autosomal-Recessive Nonsyndromic
Hearing Impairment.

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 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).

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 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) and
platinum based chemotherapeutics such as cisplatin.

Various other medications may reversibly affect hearing. This includes

some diuretics, aspirin and NSAIDs, and macrolide antibiotics.

The 1995 Miss America Heather Whitestone lost her hearing after receiving strong
antibiotics for haemophilus influenzae. Extremely heavy hydrocodone (Vicodin or Lorcet) abuse
is known to cause hearing impairment. Commentators have speculated that radio talk show

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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 (foundin crude oil, gasoline and automobile exhaust, for
example); andasphyxiants. 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.[7] For some ototoxic chemical exposures, particularly styrene,[8] 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. The
following list provides an accurate catalogue of ototoxic chemicals:

Drugs

 antimalarial,
 antibiotics,
 anti-inflammatory (non-steroidal),
 antineoplastic, diuretics
• solvents
 toluene, styrene,
 xylene, n-hexane, ethyl benzene,
 white spirits/stoddard,
 carbon disulfide,
 fuels,
 perchloroethylene,
 trichloroethylene,
 p-xylene
 asphyxiants
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  carbon monoxide, hydrogen cyanide

Metals

 lead,
 mercury,

Others

• organotins (trimethyltin)
• Pesticides/Herbicides
• paraquat, organophosphates

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. I King Jordan lost his hearing after suffering a skull fracture as a
result of a motorcycle accident at age 21.

MANAGEMENT

APPROACHES
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

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require sophisticated programming in conjunction with patient training for effectiveness. The
United States Food and Drug Administration reported that cochlear implant recipients may be at
higher risk for meningitis. People who have hearing impairments, especially those who develop a
hearing problem in childhood or old age, may need the support and technical adaptations as part
of the rehabilitation process. 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 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 or total
communication. This may be because not all audiologists are familiar with the benefits of sign
language to the deaf child and family.

There are many arguments against the cochlear implant including the loss of deaf culture
and the fact that the expensive surgery does not always restore hearing.

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.

ASSISTIVE TECHNIQUES AND DEVICES FOR HEARING IMPAIRMENT

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

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 Individuals can communicate by telephone using Telecommunications Device for
the Deaf (TDD). These devices look like 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 a 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.
 Video Relay Service and Video Remote Interpreting services also use a third-
party telecommunication service to allow a deaf or hard-of-hearing person to
communicate quickly and conveniently with a hearing person, through a sign
language interpreter.
 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.
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  The advent of the Internet's World Wide Web and closed captioning has given
the hearing impaired unprecedented access to information. Electronic mail and
online chat have reduced the need for deaf and hard-of-hearing people to use a
third-party Telecommunications Relay Service in order to communicate with the
hearing and other hearing impaired people.

RESOURCES AND INTERVENTIONS

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 devices 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. Another
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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.

Inside the classroom, children with hearing impairments may also benefit from
interventions. These include providing favorable seating for the child. This can be done by
having the student sit as close to the teacher as possible so that they will be able to hear the
teacher, or read the teacher's lips more easily. When lecturing teachers should try to look at the
student as much as possible so that the student has the option of trying to hear exactly what it is
the teacher is saying, or they can read the teacher's lips. Limit unnecessary noise in the
classroom. If a student has a hearing aid, they are likely to hear a lot of unwanted noises. Pairing
hearing impaired students with hearing students is important so that the non-hearing student can
ask the hearing student questions about concepts that they may not have caught. When teaching
students with hearing impairments, try to use overheads as much as possible. Overheads allow
the teacher to write, as well as maintain visual focus on the hearing impaired student. For those
students who are completely deaf, one of the most common interventions is having the child
communicate with others through an interpreter using sign language.

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 lives.

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GENETIC DEAFNESS IN PAKISTANI POPULATION
The consanguineous marriage pattern has significant implication for increased rate of
recessive genetic disorders. High rate of consanguinity in any population along with other factors
such as religion, ethnicity, language and geography, usually lead to create genetically isolated
groups in which typically confined, well-documented, extended and multigenerational pedigrees
with several cases of rare diseases are expected.2 The extended pedigrees were readily used by
geneticists for their linkage studies and for mapping many monogenic autosomal recessive
disorders. In this regard, various isolated populations such as the Pakistani population and certain
other communities have played prominent role in identifying the novel mutations in these
autosomal recessive genetic disorders.

In Pakistan, there is a strong cultural preference for consanguineous marriage and an

associated relatively high prevalence of recessively inherited disorders. There are number of
factors that significantly increase the prevalence of genetic disorders in the Indian subcontinent.
The huge population of Indian sub-continent including Pakistan, India and Bangladesh also
provides an opportunity for studies of genetic disorders like deafness. The study of
consanguineous families has led to the identification of many new genes. But the population of
Pakistan is the goldmine for these studies due to its unique geography and history. In addition, it
is a mixture of diverse ethnicities with unique familial and social characteristics.
Deafness (or hearing impairment) is the loss of ability to hear normally, whether permanent loss
or fluctuating. Deafness is clinically and genetically heterogeneous and can be caused by
environmental as well as genetic factors. It is estimated that the prevalence of profound bilateral
hearing loss is 1.6 per 1000 in Pakistan and 70% of hearing loss arises in consanguineous-
families.

The genetically determined deafness or hearing impairment can be divided into two
categories; syndromic and non-syndromic forms. The autosomal recessive forms are usually
more severe than the other forms and are almost exclusively due to cochlear defects.
Syndromic hearing impairment may account for up to 30% of prelingual deafness, which in most
cases is of conductive and mixed type but its relative contribution to all deafness is much
smaller, reflecting the occurrence and diagnosis of post lingual hearing loss. Over 400 genetic
syndromes that include hearing loss have been described.6 Syndromic hearing loss is categorized
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according to the mode of inheritance. Syndromic hearing loss can have many modes of
transmission, including maternal inheritance due to a mitochondrial mutation.
Non-syndromic deafness is a paradigm of genetic heterogeneity. It is estimated that more than
70% of hereditary hearing loss is non-syndromic. The different gene loci for non-syndromic
deafness are designated DFN (for DeaFNess). Loci for genes inherited in autosomal dominant
forms are referred to as DFNA, and for genes inherited in an autosomal recessive forms as
DFNB, and those for genes inherited in an X-linked forms as DFN.

MYTHS REGARDING PEOPLE WITH HEARING LOSSES

There are many myths regarding people with hearing losses including, but not limited to:

Everyone who is deaf or hard of hearing uses sign language.

 There are a variety of different sign systems used by hearing-

impaired individuals.
 Individuals who experience hearing loss later in life usually do not
know sign language.
 People who are educated in the method
of oralism or mainstream do not always know sign language.

People who cannot hear are not allowed to drive.

 Deaf people may use special devices to alert them to sirens or other
noises, or panoramic mirrors to enable improved visibility.

 Many countries allow deaf people to drive, although at least 26 countries

do not allow deaf citizens to hold a driver's license.

All forms of hearing loss can be solved by hearing aids or Cochlear Implants.

 While many hearing-impaired individuals do use hearing aids, others may

not benefit from the use of a hearing aid.
 For some hearing-impaired individuals who experience distortion of
incoming sounds, a Cochlear Implant may actually worsen the distortion.
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A lack of hearing correlates to a lack of intelligence.

 A person's intelligence level is unrelated to whether or not the person can hear.

All deaf/hard of hearing people are experts in Deaf Culture.

 Deaf people may have a variety of different beliefs, experiences, and

methods of communication.
 This may be influenced by the age at which hearing was lost and the
individual's personal background.

All deaf people want to be hearing.

 While some individuals with hearing loss want to become hearing, this is
not the case for everyone. Some take pride in their deafness or view themselves
as a minority rather than a disabilitygroup.

People who can't hear can't use a phone.

 Teletypewriters, Video phones and cell phone text messages are used by
deaf people to communicate.

Everyone who cannot hear can lip read.

 Only about 30% of spoken English is visible on the lips.

 Lip reading requires not only good lighting, but also a good understanding
of the spoken language in question and may also depend on contextual
knowledge about what is being said.

Most deaf people have deaf parents.

 Less than 5% of deaf children in the United States have a deaf parent.

SOCIAL CONSEQUENCES
INCLUSION VS. PULLOUT

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 Because a hearing impairment is a disability that is prevalent in many children throughout
the United States today, one may be asking themselves what is the best schooling environment
for these students. There have been many mixed opinions on the subject between those who live
in Deaf communities, and those who have deaf family members who do not live in Deaf
communities. Deaf communities are those communities where only sign languages are typically
used.

Many parents who have a child with a hearing impairment prefer their child to be in the
least restrictive environment of their school. This may be because most children with hearing
loss are born to hearing parents. This can also be because of the recent push for inclusion in the
public schools.

It is commonly misunderstood that least restrictive environment means mainstreaming or

inclusion. Sometimes the resources available at the public schools do not match up to the
resources at a residential school for the deaf. Many hearing parents choose to have their deaf
child educated in the general education classroom as much as possible because they are told that
mainstreaming is the least restrictive environment, which is not always the case. However, there
are those parents, some of which who live in Deaf communities feel that the general education
classroom is not the least restrictive environment for their child. These parents feel that placing
their child in a residential school where all children are deaf may be more appropriate for their
child because the staff tend to be more aware of the needs and struggles of deaf children.
Another reason that these parents feel a residential school may be more appropriate is because in
a general education classroom, the student will not be able to communicate with their classmates
due to the language barrier.

In a residential school where all the children use the same language(whether it be a school
using ASL, Total Communication or Oralism), students will be able to interact normally with
other students, without having to worry about being criticized. An argument supporting
inclusion, on the other hand, exposes the student to people who aren't just like them, preparing
them for adult life. Through interacting, children with hearing disabilities can expose themselves
to other cultures which in the future may be beneficial for them when it comes to finding jobs
and living on their own in a society where their disability may put them in the minority. These
are some reasons why a person may or may not want to put their child in an inclusion classroom.

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REFERENCES
• Current Diagnosis & Treatment in Otolaryngology: Head & Neck Surgery, Lalwani, Anil
K. (Ed.) Chapter 44: Audiologic Testing by Robert W. Sweetow, PhD, Jennifer McKee
Bold, AuD, Access Medicine
• Tox Town – Toluene – Toxic chemicals and environmental health risks where you live
and work – Text Version". toxtown.nlm.nih.gov. Retrieved 2010-06-09.
• Thais C. Morata. "Addressing the Risk for Hearing Loss from Industrial
Chemicals".cdc.gov. Retrieved 2008-06-05.
• Johnson, Ann-Christin. "Occupational exposure to chemicals and hearing impairment –
the need for a noise notation" (PDF). Karolinska Institutet: 1–48.
• Free Phone Caption Service for the Deaf, Hard of Hearing, and hearing impared.
PhoneCaption.com. Retrieved on 2010-12-10.
• An Educators Guide to Hearing Disability Issues. (n.d.). Retrieved July 19, 2009 & Facts
About Hearing Loss. (2005). Retrieved July 19, 2009, from Alexander Bell Association for
the Deaf and Hard of Hearing.
• "Mortality and Burden of Disease Estimates for WHO Member States in
2002" (xls). World Health Organization. 2002.
• "Noise-Induced Hearing Loss: Promoting Hearing Health Among Youth" CDC Healthy
Youth!: 1-7-09.
• Myths About Deaf and Hard of Hearing. Tutorworkshop.org. Retrieved on 2010-12-10.
• "The World of the Deaf," The Washington Post, February 26, 1978, p. G1.
• Tim Grieve (October 7, 2003). "Did popping painkillers make Rush lose his
hearing?".Salon.com. Retrieved 2008-09-08.
• Andy Coghlan (2005-02-14). "Gene therapy is first deafness 'cure'". NewScientist.com
News Service.

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