Causes of Hearing Loss

Which common factors affect hearing?

Of the five senses, hearing is overlooked. For something so seemingly simple,
so much can change how the ear hears. Hearing is important, and there are many
things that can contribute to hearing loss. The biological factors, chemical influence,
and the living conditions. That begins the questions of, in what way do genes affect
hearing? In what way do chemical products such as medication alter hearing? How
might someones living conditions affect their hearing? Of all the questions, the
biggest question about hearing loss: Which common factors affect hearing?
In what way do genes affect hearing?
The ear is a complex organ of the body. It is very small, and is hidden well
within the head. It comprises the outer ear, middle ear and inner ear. The outer ear
is what is visible, including the ear canal until the ear drum. The middle ear is on
the other side of the ear drum and contains three tiny bones (ossicles) called the
malleus, incus, and the stapes. They all vibrate to the sound waves so as to amplify
them for the ear drum. The middle ear stops at the Eustachian tube, which is
responsible for equalizing pressure to protect the eardrum and prevent fluid buildup.
The inner ear, also known as the Osseous Labyrinth (Labyrinth for short) it is
composed of the cochlea, vestibule, semicircular canals, vestibular and the auditory
nerve.
Genes are a huge part of how the body develops. If there is a mutation in any
part of the genetic code, it can spell disaster for the bodys function. From genes,
many things can affect the way a person hears. There are three kinds of hearing
loss, all with varying severities. Conductive hearing loss is when there is a problem
with part of the outer or middle ear so that sound cant reach the cochlea. (Carol,
p.8) Sensory and Nerve hearing loss can be defined with the term Sensorineural
hearing loss, meaning that the hearing loss is caused by damage to the inner ear.
This can include the sensorial hairs in the cochlea, or the auditory vestibular nerve.
A mix of both Sensory and Conductive hearing loss is called Mixed Hearing loss.
Hearing loss can be acquired at any stage in ones life. Certain flawed genes
do not activate until 20-30 years of age, others cause an infant to be born with
mild to profound hearing loss. In fact, a study from 1969-1999 performed by
Gallaudet University found that, hereditary factors (genes) are responsible for more
than 50% of deafness that occurs in children. (Arnos et al., 2011). There are over
400 Genetic and Hereditary disorders that can cause Deafness. From syndromic and
non-syndromic disorders, to malformations and tumors, it comes down to the
integrity of the genetic code. It is entirely possible for a child to be born deaf if
there is no record of their parents experiencing hearing difficulties. In some cases,
a person can inherit a gene mutation, but they do not experience hearing loss. This
person is called a carrier. A carrier of a gene mutation can pass it along to future
offspring who might experience hearing loss. (Hearing loss that runs in families,
2014). Undesirable genes can be inherited from parents, or be the first mutation in
the genetic line. Mutations can be defined as either familial or sporadic. Familial
mutations occur when more than one family member has the specific mutation.
Sporadic occurs when it is the only mutation of its kind in the family genetic line.

Within genetic disorders, the hearing loss that is caused can be categorized
by their characteristics. Syndromic hearing loss is when the child shows other
symptoms as well as hearing loss. Non-syndromic, on the other hand, is when the
child does not present any other symptoms besides hearing loss. It is far more
common for genetic disorders to affect only the ear as, [only] 30% of inherited
hearing loss is associated with a syndrome. (Hearing loss that runs in families,
2014) Therefore, the other 70% of cases are where the gene affected has significant
influence only on the ability to hear.
In the case of syndromic hearing loss, the damage to the ear can vary,
although the loss of hearing is not the only problem caused by the genetic
mutation. For instance, in the case of Trisomy 13, the damage to the ear is not the
top priority for parents. Trisomy 13 is usually sporadic, and occurs when there is
three copies of the chromosome 13 in the DNA. Trisomy 13 can be brutal to the
patient, as infants are born with a multitude of birth defects, Neurological defects,
and/or congenital heart disease. Complications begin almost immediately, causing
90% of infants to die within a year. (Chad Haldeman-Englert, 2015). In this case,
hearing loss was associated among a group of symptoms of a serious genetic
disorder, however, not all syndromes that result in hearing loss are this serious.
On the other hand, non-syndromic hearing loss has many variables. They can
be sporadic or familial, they can range in severity from mild to profound and they
can occur for a multitude of reasons. Research has narrowed down some genes that
are responsible for a majority of cases, but not all. Some gene mutations are more
commonly linked to hearing loss than others. Researchers have identified more
than 90 GJB2 gene mutations that cause a form of non-syndromic Deafness.
(Genetics Home Reference, 2012) This places the GJB2 mutation high on the list for
causes of hearing loss. There are many mutated genes besides this gene that
causes hearing loss, but they could stay dormant until an older age, or until a
chemical trigger is introduced.
GJB2, also known as Gap Junction protein Beta 2, is a gene found on the 11 th
and 12th position of the long arm of chromosome 13.This gene contains the
instructions for a protein called Connexin 26. Connexin proteins form channels
called gap junctions that permit the transport of nutrients, charged atoms (ions),
and signaling molecules between neighboring cells that are in contact with each
other. (Genetics Home Reference, 2012) The Connexin 2 cells found in the inner
ear are critical to functional hearing. The Connexin 26 (CX26) is believed to be
crucial for maintaining the level of potassium ions in the cochlea. If the proper level
of ions is not maintained, then the sensory hairs found in the spiral shape of the
cochlea cannot convert sound waves into electrical impulses. (Genetics Home
Reference, 2012) The sound waves need to be transferred into electrical impulses in
order for the auditory nerve to send the message to the brain. Therefore, if the
CX26 is not functioning as it should, or is disfigured, causing an abnormality of ion
levels, then hearing is interfered with. The congenital sensorineural hearing loss can
vary in severity, dependant on the level of mutation of the GJB2 gene.
A mutation in the GJB2 gene causes congenital deafness, meaning they are
born deaf. Although, some genes will cause what is known as late-onset progressive
non-syndromic hearing loss. The gene identified is referred to as DFNA10, the most
common type of neurological dysfunction in the elderly. It can be either acquired or
inherited. (O'Neill et al., 1996) The gene lies in chromosome 6, between sections
22 and 23. This gene begins to take effect with age, starting with mild hearing loss

greater than 25 Db, to severe greater than 75Db. It is one of many genes that cause
late-onset hearing loss.
Hearing can be distorted if the ear itself is malformed. The shape of the outer
ear is important for the travel of sound waves to the ear drum. Even the size of the
eardrum could affect how sound is heard. In the middle and inner ear, defects get
complicated and have more severe consequences. The small bones in the middle
ear are crucial for the soundwaves to be caught, if they are fused together in any
way that prevents vibration (Ostosclerosis), hearing is unable to function. If the
Eustachian tube fails to equalize pressure, then the tympanic membrane (ear
drum), can sag or tear. This would prevent the ear drum from collecting the sound
waves amplifies from the Ossicles in the tympanic cavity. The inner ear develops
problems in the form of infections, fluid build-up, or genetic mutations that cause
malfunction. (Henderson, 2015)
Genetic mutations can be something as simple as a tumor. A tumor, also
known as a neoplasm, is an abnormal mass of tissue which may be solid or fluidfilled. A tumor does not mean cancer - tumors can be benign (not cancerous), premalignant (pre-cancerous), or malignant (cancerous). (Nordqvist, 2015) Benign
tumors can be harmless just as much as harmful. They can be something simple like
skin tags or moles, which cannot grow or spread. Although, benign tumors can pose
just as much as a threat to life as pre-malignant and malignant tumors. If the tumor
presses against any nerves or organs, it can impede functionality or increase the
actions of some glands that could endanger health.
In the case in Vestibular Schwannoma (Acoustic Neuroma), [It] is a benign,
usually slow-growing tumor that develops from the balance and hearing nerves
supplying the inner ear. The tumor comes from an overproduction of Schwann cells-the cells that normally wrap around nerve fibers like onion skin to help support and
insulate nerves. (Nidcd.nih.gov, 2010) This tumor presses on the nerves, causing
loss of balance, and can inhibit hearing completely. It is difficult to remove the
tumor from the brain as it lies so close to the nerves, that there is a high risk of
damaging them. It is believed that the chromosome 22 contains the gene
responsible for this tumor. The gene is responsible for the growth of Schwann cells
and control over other tumors. It is thought that when the gene malfunctions or is
mutated, the Schwann cells grow at an increased rate, causing the tumor to develop
until it becomes a problem. Hearing loss could be permanently damaged if the
nerve is damaged from surgery or prolonged development of the tumor.
Infections are a simple way to lose hearing. The infection can cause
temporary hearing loss or damage the ear to permanently change its function.
Infection usually causes conductive hearing loss, Painful (but treatable) as fluid or
infection in the middle ear (Otitis media), prevents the eardrum from vibrating.
(Pope, p.20, 1997) Infections can cause hearing loss at any age. One of the most
common forms of infection that causes hearing loss is Meningitis. Meningitis can be
bacterial, fungal, parasitic or viral. Both the infection itself and the medication
prescribed to cure it can damage the hearing. Bacterial Meningitis is the most
common form to affect hearing, especially in youth. Gallaudet University performed
a study in 2006 and discovered that bacterial meningitis is the cause of hearing loss
in 3.2% of American youth. This places bacterial meningitis as the second most
common cause of hearing loss. (Beltone.com, 2015)

Meningitis can cause hearing loss through fluid build-up, nerve damage,
sensory damage and/or physical damage to the cochlea. Meningitis is when there is
a fluid build-up in the brain and spinal column, and the pressure can become a
problem. If there is sufficient pressure placed on the nerves they can stop sending
or receiving signals. This results in hearing loss. However, pressure from fluids are
not restricted to the brain. If there is inflammation in the inner ear, such as in the
cochlea (Labrinthitis), then the sensory hairs are viable to damage. The hairs could
be suffocated of the nutrition they need by fluids, the cochlea could be inflamed,
leading to sensory hair death. The hair cells are important for the cochlea to receive
the vibrations from the surrounding bones in the ear in order to hear properly.
Sensory hearing loss ensues, but is not always severe. It can be simply a
dampening of volume or muffling of voices. On the other hand, it is possible to
recover from infections such as meningitis or rubella without permanent damage to
the ears. If caught early enough, the damage to the cochlea or middle ear can be
reversed and treated. (Beltone.com, 2015)
If the infection is contracted by an expectant mother, the same thing cannot
be said about the state of the infants hearing when born. If the mother catches an
infection or disease such as rubella, they can deter development of the ear of the
infant, causing permanent profound hearing loss. Infection can pass on from the
mother to the fetus creating birth defects or damage to the auditory nerve. The
infection can manipulate the DNA in order to replicate itself, therefor causing
mutations in the still developing fetus. This kind of cognitive hearing loss is
irreversible and can cause many complications after the childs birth. The hearing
loss may not immediately present itself, after birth. Until the brain, nerves or ears
have fully developed, the damage can stay undetected.
Any damage to the ear increases the risks for loss of hearing. Although, the
damage does not necessarily have to be caused directly by disease, infection or
hereditary factors. It could simply be caused by head trauma or trauma directly
placed on the ear. This can be known as Post-Traumatic Conductive Hearing loss.
This involves disruption of the ossicular chain (bones of the middle ear), perforation
of the eardrum (tympanic membrane) or hemotympanum. There are minor cases
and major cases that can be fixed with surgery and implants.
A disruption of the ossicular chain can qualify as the malleus, incus and
stapes being out of alignment, fractures to any of the ossicles, or fusion of the
stapes that prevents proper vibration. (Gerstenmaier, 2015) Perforation of the
eardrum is when a small hole or tear occurs in the tympanic membrane. This can
alter or disable the ability to hear properly. However, most eardrum holes tend to
heal on their own in a few short weeks if taken care of properly. The severity of the
hearing loss is determined usually by the size of the perforation. The more
soundwaves that can pass through without making contact with the tympanic
membrane, the more the hearing is affected negatively. The causes of an eardrum
hole are usually from injury, infection, or chronic Eustachian tube disorders. A
perforated eardrum from trauma can occur if the ear is struck directly, if they have
a skull fracture, after a sudden explosion or if an object (such as a bobby pin, Q-tip,
or stick) is pushed too far into the ear canal. (American Academy of
Otolaryngology-Head and Neck Surgery, 2014) Hemotympanum is the presence of

blood behind the ear drum, inside the middle ear. This fills the cavity and disrupts
the travel of sound waves, thus inhibiting hearing. Hemotympanum can be caused
by head trauma and an indicator of brain trauma as well, or of genetic malfunctions
in the Eustachian tube. (ENT Clinic Sydney)
In what way do chemical products such as medication alter hearing?
Chemicals have a significant effect on the ability to hear. There are
medications, chemical products and environmental toxins that can all cause
damage to the ear. These chemicals, medications in particular, are not what is
expected by the public to cause hearing loss. Ototoxicity is, quite simply, ear
poisoning, which results from exposure to drugs or chemicals that damage the inner
ear or the vestibulo-cochlear nerve (the nerve sending balance and hearing
information from the inner ear to the brain). (Haybach, 2001) There are over 200
known ototoxic medications on the market in the form of prescription or over the
counter medication. The chemicals affect different parts of the ear, and the hearing
loss can be temporary, or permanent dependant on the individual case.
Each chemical has its own way of affecting the ear; most symptoms are
temporary and will recover once the drug is stopped. Ototoxic chemicals can be
eaten, travel through the skin, or breathed into the body. Once in the blood, they
can go to the ear and be soaked up by the hearing nerve. This may damage the
nerve and cause hearing loss. This can also cause hearing loss by damaging the
inner ear hair cells, like the hearing loss caused by noise.(Kgwefane, 2015) Since
these chemicals are poison to the ear, it is important to take proper care of what
chemicals are put into the body.
Ototoxic chemicals are not easy to identify, however, there are types of
medications and elements that have been linked to ototoxicity. Such as
Aminoglycoside as antibiotics (Gentamicin, Kenomycin, Neomycin), Salicylates as
aspirin, Quinines, Loop diuretics, Anti-neoplastics as anti-cancer drugs (Cisplantin),
Others, non-steroidal anti-inflammatory, antibiotics, antihistamines, cardiovascular,
anti-convulsant, anti-depressant and anti-psychotic drugs. (Cook, 2015) Besides
these medications, environmental chemicals or natural elements can have a serious
effect on not just the ears but the internal organs as well. They are comprised as
gas, heavy metals and organic solvents such as Arsenic, Carbon monoxide, Helium,
Lead, and Mercury. The chances of recovering hearing after prolonged contact with
any of these are slim, as they are very damaging to nerves and sensitive organs in
the body.
Besides ototoxic chemicals, noise significantly increases the chances of
hearing loss. The higher the volume and frequency of the noise, the more damage
occurs. When hearing loss occurs, the higher frequencies will fade away first. Noise
and chemicals will damage hearing by increasing blood flow and level of penetration
to the cochlea, also by upsetting the intracellular energy production. Existing noise
induced hearing loss will speed damage. (Cook, 2015) Therefore, workplaces that
involve loud noises and exposure to ototoxic chemicals can increase the chances of
hearing loss. Caution is to be used when working in jobs such as heavy industry
factories, painting, boat building, construction, firefighting, fueling vehicles and
aircraft, printing and military involvement. These jobs involve situations in where
ototoxic chemicals and noise are combined and can affect hearing in a negative
manner. (Ccohs.ca)

How might someones living conditions affect their hearing?

Where someone lives decides what stimulants they come into contact with.
These stimulants are either positive such as aromas and sounds, or negative, such
as chemicals and loud noise. Living in an area with stimulants that contribute to
hearing loss increase the chances of becoming Deaf or Hard of Hearing. Stimulants
that can cause hearing loss can include, but not limited to, genetic line, noise
exposure, exposure to infection and living in close contact with ototoxic chemicals.
In small, tight-knit towns, genetics can be restricted to a small gene pool.
This increases the risk of genetic mutations in the DNA. The founder effect can be
associated with genetic mutations in hearing loss. The founder effect is the loss of
genetic variation that occurs when a new population is established by a very small
number of individuals from a larger population. (Raina et al., 2012) The founder
effect strengthens the odds of any mutations in genes such as GJB2 that are linked
to hearing loss.
Working in jobs that have a high level of contact with noise is damaging to
the ear. It increases the stress on the ear and causes noise induced hearing loss.
This affects jobs from construction, stage crew, factories or even the houses located
nearby. 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.
For unprotected ears, the allowed exposure time decreases by one half for each 5
dB increase in the average noise level.(American-hearing.org, 2012) Therefore, the
higher the noise exposure, the higher the risks and damage done.
Living in an area prone to infections such as meningitis and rubella are huge
risks. Not only can the infections themselves cause hearing loss, but as stated
earlier, the medications used to treat them are considered ototoxic. In the case of a
pregnancy, it is not just the mother who could have their hearing affected. It is the
child that could develop a mutation and pass it on to future generations, or could
have congenital hearing loss. Infection is a huge risk, and living is close
communities with little hygienic resources further increase the spread of infection.
Living in close contact with ototoxic chemicals and environmental toxins
poses just as much of a threat as infection. The chemicals have countless effects on
the ear. Chemical exposure is rather easy to come by. Chemicals or environmental
toxins can enter the body, [through] inhalation (breathing), skin contact, digestive
system (ingestion or eating) or injection. (Ccohs.ca, 2009) Anything in the vicinity
can pose a threat. Such as living in an apartment above a boiler room risks carbon
monoxide poisoning, or working in a factory can lead to mercury or lead poisoning.
The ways to come into contact with ototoxic chemicals and environmental toxins in
the workplace and living environment are endless.

When the topic of hearing loss is brought up, the question always asked is:
which common factors affect hearing? In what way do genes affect hearing? In what
way do chemical products such as medication alter hearing? How might someones
living conditions affect their hearing? It is found that genes have a significant effect
on hearing loss. Hearing loss is classified as syndromic or non-syndromic, based on
if other symptoms are present alongside it. Hearing is changed from head trauma or
mutations in the genetic code, causing birth defects, hair cell death, and
displacement of the inner parts or the wrong protein to be made. Chemicals that

affected the ear were labelled as ototoxic, and there are a vast number or both
medical and environmental chemicals that encourage hearing loss. The
environment in which they live changes influence these factors have on the ear. The
amount of noise exposure changes how the loss progresses over time, the
communities affect the genetic pool, increasing the biological causes, and increase
chemical exposure as well, often times without them knowing of the danger until
hearing is lost. Hearing is affected from genetics, chemical exposure, and living
conditions. Not all hearing loss caused by them is temporary, as once the hair cells
are dead, they are dead. However, is there a way to revive them? Is there a way to
overcome all the factors to recover hearing?

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