Symptoms may be mild, moderate, severe, or profound. A patient with mild hearing impairment may have problems understanding speech, especially if there is a lot of noise around, while those with moderate deafness may need a hearing aid. Some people are severely deaf and depend on lip-reading when communicating with others.
People who are profoundly deaf can hear nothing at all. In order to communicate spontaneously and rapidly with people, they are totally reliant on lip-reading and/or sign language. People who are born deaf find lip-reading much harder to learn compared with those who became hearing impaired after they had learned to communicate orally (with sounds).
Some diseases or circumstances that can cause deafness include:
- Chicken pox.
- Sickle cell disease.
- AIDS - offspring of mothers who had AIDS during pregnancy have a much higher risk of being deaf by the age of 16 years.
- Lyme disease.
- Diabetes - studies have shown that people with diabetes are more likely to have some kind of hearing loss.
- Tuberculosis (TB), experts believe that the medication, streptomycin, used to treat TB may be the key risk factor.
- Hypothyroidism - and underactive thyroid gland.
- Some cancers.
- Second-hand smoke exposure can increase hearing loss risk in teenagers.
Many people globally have untreated hearing loss. It is estimated that 23 million Americans live with untreated impaired hearing.
Hearing loss vs. deafness
Hearing aids may be worn by people with hearing impairment or partial deafness.
Hearing loss refers to a diminished ability to hear sounds like other people do
Deafness refers to the inability to understand speech through hearing even when sound is amplified.
Profound deafness means the person cannot hear anything at all; they are unable to detect sound, even at the highest volume possible.
Degree of hearing impairment - a person's severity of hearing impairment is categorized by how much louder than "usual levels" sound volumes need to be set at before they can detect a sound.
Degree of deafness - any degree of deafness means the person cannot understand speech through hearing at any level of amplification. If a person is profoundly deaf, they cannot detect sounds at any volume. Some people define profoundly deaf and totally deaf in the same way, while others say totally deaf is the end of the hearing spectrum.
How do we hear things?
Sound waves enter the ear, go down the ear canal (auditory), and hit the eardrum, which vibrates. The vibrations from the eardrum pass to the three ossicles - bones called the malleus (hammer), incus (anvil), and stapes - in the middle ear.
These ossicles amplify the vibrations, which are then picked up by small hair-like cells in the cochlea; they move as the vibrations hit them, the movement data is sent through the auditory nerve to the brain. The brain processes the data, which we interpret as sound.
There are three different types of hearing loss:
1) Conductive hearing loss
This means that the vibrations are not passing through from the outer ear to the inner ear, specifically the cochlea. It can be due to an excessive build-up of earwax, glue ear, an ear infection with inflammation and fluid buildup, a perforated eardrum, or a malfunction of the ossicles (bones in the middle ear). Also, the eardrum may be defective.
Ear infections can leave scar tissue, which damages the functioning of the ear drum.
The ossicles may be impaired due to infection, trauma, or their fusing together (ankylosis).
2) Sensorineural hearing loss
Hearing loss is caused by dysfunction of the inner ear, the cochlea, auditory nerve, or brain damage. Usually, this kind of hearing loss is due to damage of the hair cells in the cochlea. As humans get older, the hair cells lose some of their function, and our hearing gets worse. In Western Europe and North America, it is estimated that over half of all people over 70 have hearing impairment caused by degenerated hair cells in the cochlea.
Long-term exposure to loud noises, especially high-frequency sounds, is another common reason for hair cell damage. Damaged hair cells cannot be replaced. Currently, research is looking into using stem cells to grow new ones.
Sensorineural total deafness may be due to birth defects, inner ear infections, or head trauma. If the eardrum and middle ear are functioning properly, patients may benefit from a cochlear implant - a thin electrode is inserted into the cochlea, it stimulates electricity through a tiny microprocessor that is placed behind the ear, under the skin.
3) Mixed hearing loss
This is a combination of conductive and sensorineural hearing loss. Long-term ear infections can damage both the eardrum and the ossicles. Sometimes, surgical intervention may restore hearing, but it does not always work.
Deafness and speech
Hearing loss can affect the speech abilities of an individual depending on when it occurs.
Prelingual deafness is when a person is unable to hear properly before learning how to speak.
Prelingual deafness refers to the inability to hear properly, or at all before the patient learned how to utter or understand speech.
In such cases, the individual was born with a congenital condition or lost their hearing very early in life, during infancy.
People with prelingual deafness have never acquired speech with sound.
In the majority of cases, people with prelingual deafness have hearing parents and siblings and were born into families who did not know sign language. Consequently, they also tend to have slow language development. The very few who were born into signing families tend not to have delays in language development.
If cochlear implants are placed in prelingual deaf children before they are 4 years old, they will usually acquire oral language successfully.
Oral language and the ability to use social cues are very closely interrelated. That is why children with hearing loss, especially those with severe symptoms, may not only experience delayed language development, but their social development will take longer too. Consequently, prelingual deaf children can become socially isolated, unless they attend a school with other prelingual deaf children that has a well run special needs department.
Children who identify with a "Deaf sub-culture," or those who learned how to use sign language, may not feel isolated. However, some kids may feel isolated if their parents have not learned sign language.
A prelingual deaf child whose parents and other family members encourage them to the learn social skills acquired and used by hearing children are less likely to experience social isolation.
There are cases of profoundly deaf children who find themselves in no-man's land. They are on the outer fringes of their hearing peers' social circles, while at the same time are not fully accepted by totally deaf peers because they are not fluent in sign language.
Most people with some kind of hearing loss have post-lingual deafness. They had acquired spoken language before their hearing was diminished. Losing their sense of hearing may have been caused by a medication side effect, trauma, infection, or a disease.
In most cases, people lose their hearing gradually; household members, friends, and teachers may have noticed something was wrong before they themselves acknowledged the disability. Depending on the severity of hearing loss, the individual may have had to use hearing aids, had a cochlear implant inserted, or learned how to lip-read.
People who experience hearing loss face different challenges, depending on when it occurs and how long it takes to develop. They have to become familiar with new equipment, perhaps undergo surgery, learn sign language and lip reading, and use various communication devices. A feeling of isolation is a common problem, which can sometimes lead to depression and loneliness; add to that the process of coming to terms with a disability. It is also a challenge for household members, loved ones, and close friends, who have to adapt to the person's hearing loss.
Miscommunication can place a strain on relationships, a strain not only for the person with the hearing impairment, but also people around them. If the hearing loss is gradual and has not yet been diagnosed, family members may mistakenly believe that the patient is becoming more distant.
Unilateral and bilateral deafness
Unilateral deafness (single-sided deafness or SDD) refers to just one ear, while bilateral means a hearing impairment in both.
People with unilateral hearing impairment may find it hard to carry on a conversation if the other person is on their "deaf" side. Pinpointing where a sound is coming from may be more difficult, compared with those who can hear well with both ears. Understanding what others are saying when there is a lot of noise about may be hard.
When there is no background noise, or very little, a person with unilateral deafness has virtually the same aural communicative abilities as somebody with normal hearing in both ears.
Babies born with unilateral deafness tend to have speech developmental delays. They may find it harder to concentrate when they go to school. Social activities may be more challenging than it is for children with no hearing problems.
A symptom is something only the patient can feel and describe to a doctor, nurse or somebody else, such as a pain, dizziness, ringing in the ears, or fatigue. A sign is something that somebody else might detect on the patient, examples include a rash, swelling, bleeding, or bruising.
The symptoms associated with hearing impairment depend on its cause; some people are born without being able to hear, others suddenly become deaf due to an accident or illness. A build-up of earwax can cause sudden hearing loss. We all lose a certain amount of hearing during late-middle and old age. In most cases, deafness symptoms progress gradually over time.
Hearing impairment in babies
The following signs may indicate a hearing problem (but not always):
- Before the age of 4 months, the baby does not turn their head toward a noise.
- By the age of 12 months, the baby still does not utter a single word.
- The baby does not appear to be startled by a loud noise.
- The baby responds to you when they can see you, but much less so (or not at all) when you are out of sight and call out their name.
- The baby seems to be aware of some sounds only.
Hearing impairment in toddlers and children
The following signs may indicate a hearing problem (but not always):
- The child is behind the others of their age in oral communication.
- The child keeps saying "What?" or "Pardon?"
- The child talks in a very loud voice, and tends to produce louder-than-normal noises.
- When the child speaks, their utterances are not clear.
Four levels of deafness
There are four levels of deafness (possibly 5 in some countries), they are:
- Mild deafness or mild hearing impairment - the patient can only detect sounds between 25-29 decibels (dB). They may find it hard to understand everything other people are saying, especially if there is a lot of background noise.
- Moderate deafness or moderate hearing impairment - the patient can only detect sounds between 40-69dB. Following a conversation just from hearing is very difficult without using a hearing aid.
- Severe deafness - the person only hears sounds above 70-89dB. A severely deaf person must either lip-read or use sign language in order to communicate, even if they have a hearing aid.
- Profound deafness - anybody who cannot hear a sound below 90dB is profoundly deaf; some profoundly deaf people cannot hear anything at all, at any level of decibels. Communication is done with sign language and/or lip-reading.
Obviously, if the hearing impaired deaf person can read and write, they may also communicate by reading and writing.
Patients who suspect something is wrong with their hearing will initially go and see their doctor. The doctor will talk to the patient and ask several questions regarding the symptoms, including when they started, whether or not they have got worse, and whether there is any pain.
A physical examination
The doctor will look into the patient's ear using an otoscope (auriscope); an instrument with a light at the end. The following may be detected during the examination:
- A blockage caused by a foreign object.
- A collapsed ear drum.
- An accumulation of earwax.
- An infection in the ear canal.
- An infection in the middle ear (if the ear drum bulges).
- Cholesteatoma - skin growth behind the eardrum, in the middle ear.
- Fluid in the ear canal.
- There is a hole in the ear drum (perforated ear drum).
Doctors will ask questions regarding the patients hearing, these might include:
- Do you often find yourself asking people to repeat what they said?
- Do you find it hard to understand people on the telephone?
- Do you miss the door bell when it rings? If so, does this happen frequently?
- When you chat to people face-to-face, do you have to focus carefully?
- Has anybody ever mentioned to you that you might have a problem with your hearing?
- Do you find more people mumble today than they used to?
- When you hear a sound, do you often find it hard to determine where it is coming from?
- When several people are talking, do you find it hard to understand what one of them is telling you?
- Are you often told that the television, radio, or any sound-producing device is too loud?
- Do you find the speech of men easier to understand than women's or children's?
- Are you in a noisy environment for a good proportion of each day?
- Have you often found yourself misunderstanding what other people say to you?
- Do you hear rushing, hissing, or ringing sounds?
- Do you avoid group conversations?
Anybody who answers "yes" to most of the above questions should see their doctor and have their hearing checked.
General Screening test
An audiometer or hearing test may be used to help diagnose hearing impairments or deafness.
A doctor may ask the patient to cover one ear and describe how well they hear words uttered at different volumes, as well as checking sensitivity to other sounds.
If the doctor suspects the patient has a hearing problem, they will probably be referred to a specialist, either an ear, nose, and throat (ENT) specialist (otolaryngologist) or an audiologist.
Further tests will be carried out, including:
A tuning fork test - also known as the Rinne test. This test may have been done by the doctor. A tuning fork is a metal instrument with two prongs that produces a sound when it is struck. Simple tuning fork tests may help the doctor detect whether there is any hearing loss, and where the problem is.
A tuning fork is vibrated (512 Hz) and placed against the mastoid bone (behind the ear). The patient is asked to indicate when they no longer hear any sound. The fork, which is still vibrating, is then place 1-2 centimeters from the auditory canal; the patient is asked whether they can hear the fork. As air conduction is greater than bone conduction, the patient should say "yes." If they say "no" at this point, it means that their bone conduction is superior to their air conduction, meaning there is a problem with sound waves getting to the cochlea via the ear canal.
Audiometer tests - the patient wears earphones, sounds are directed into one ear at a time. A range of sounds at various tones are presented to the patient who has to signal each time a sound is heard. Each tone is presented at various volumes so that the audiologist can determine at which point the sound at that tone is no longer detected. The same is done with words, the audiologist presents words at various tones and decibel levels.
Bone oscillator test - used to find out how well vibrations are passed through the ossicles, the three bones in the inner ear. A bone oscillator is placed against the mastoid. The aim is to see how well the auditory nerve is working.
Routine screening of children
The American Academy of Pediatrics recommends that children have their hearing tests when they start school, then at 6, 8, and 10 years of age, at least once when they are in middle school and once during high school.
The otoacoustic emissions (OAE) test involves inserting a small probe into the outer ear; it is usually done while the baby is asleep. The probe emits sounds and checks for "echo" sounds bouncing back from the ear (otoacoustic emission).
If there is no echo, the baby might not necessarily have a hearing problem, but doctors will need to carry out further tests to make sure and to find out why
There is help for patients with all types of hearing loss. Treatment depends on why the deafness exists and how severe it is.
Sensorineural hearing loss is incurable. When the hair cells in the cochlea are damaged, they cannot be repaired. However, various treatments and strategies can help improve the patient's quality of life.
These are devices that help in hearing. There are several types of hearing aids; they come in a range of sizes, circuitries, and levels of power. They do not cure deafness, but amplify the sound that enters the ear so that the listener can hear things more clearly.
Hearing aids consist of a battery, loudspeaker, amplifier, and microphone. Today, they are very small, discreet, and can be fitted inside the ear. Many of the modern versions can distinguish background noise from foreground sounds, such as speech.
For a person with profound deafness, a hearing aid is not suitable.
The audiologist takes an impression of the patient's ear to make sure the device fits well. It will be adjusted to the patient's auditory requirements.
Examples of hearing aids:
Behind-the-ear (BTE) hearing aids - these consist of a dome (earmold) and a case, with a connection from one to the other. The case is behind the pinna (outer ear, the part that sticks out); the connection to the dome comes down the front of the ear. The sound from the device is routed to the ear either electrically or acoustically.
BTE hearing aids tend to last longer than other devices because the electrical components are located outside the ear (less moisture and earwax damage). These devices are more popular with children who need a sturdy and easy-to-use device.
In-the-canal (ITC) hearing aids - these fill the outer part of the ear canal and can be seen, but only just. Soft ear inserts, usually made of silicone, are used to position the loudspeaker inside the ear. These devices fit most patients straight away, and have better sound quality.
Completely-in-the canal (CIC) hearing aids - these are tiny devices, but are not recommended for people with severe hearing loss.
Bone conduction hearing aids - for patients with conductive hearing loss, as well as those unable to wear conventional type hearing aids. The vibrating part of the device is held against the mastoid with a headband. The vibrations go through the mastoid bone, to the cochlea. These devices can be painful or uncomfortable if worn for too long.
Inserted to help patients whose hearing impairment is caused by hair cell damage in the cochlea. The implants usually help most people understand speech better. The latest cochlear implants have new technology which helps patients enjoy music, understand speech better even with background noise, and use their processors while they are swimming.
According to the National Institutes of Health, as of December 2012, there were about 58,000 adults and 38,000 children with cochlear implants in America. The World Health Organization says approximately 219,000 people globally are recipients, most of them in industrial countries.
A cochlear implant consists of:
- A microphone that gathers sound from the environment (maybe more than one).
- A speech processor that prioritizes sounds that matter more to the patient, such as speech. The electrical sound signals are split into channels and sent through a very thin wire to the transmitter.
- A transmitter. This is a coil that is secured with a magnet. It is located behind the outer ear. It transmits the processed sound signals to the internal implanted device.
- A surgeon secures a receiver and stimulator in the bone beneath the skin. The signals are converted into electric impulses and sent through internal wires to the electrodes.
- Up to 22 electrodes are wound through the cochlea. The impulses are sent to the nerves in the scala tympani (the lower passages of the cochlea), and then directly to the brain. The number of electrodes depends on which company makes the implant.
Children will usually have cochlear implants in both ears, while adults tend to have one in just one ear.
Sign language and lip-reading
Some people with hearing impairment may have speech problems, as well as difficulties in understanding what other people say. A high percentage of people with hearing impairment can learn other ways of communicating. Lip reading and sign language can replace or complement oral communication.
Also known as speechreading, lip reading is a method for understanding spoken language by watching the speaker's lip, facial and tongue movements, as well as extrapolating from the data provided by the context and any residual hearing the patient might have.
People who became hearing impaired after they learned to speak can pick up lip reading rapidly; this is not the case for those who are born hearing-impaired.
This is a language that uses signs made with the hands, facial expressions, and body postures, but no sounds - it is used mainly by those who are deaf. There are several different types of sign languages. British Sign Language (BSL) is very different from American Sign Language (ASL). For instance, BSL uses a two-handed alphabet, whereas American sign language uses a one-handed alphabet.
Some countries use the sign language introduced by missionaries from faraway - Norwegian sign language is used in Madagascar.
Sign language is completely different from the spoken form, word order (syntax) and grammar in BSL is not the same as it is in spoken English. ASL is more grammatically similar to spoken Japanese than spoken English.
There is nothing we can do to prevent congenital deafness (when you are born with the condition), or hearing impairments due to illnesses or accidents. However, some measures can be taken to reduce the risk of losing some of your sense of hearing.
The structures in our ears can be damaged in several different ways. Long term exposure to very loud noise - above 85dB can eventually cause hearing loss. A typical lawn mower emits about 85dB.
The following measures may help protect your hearing:
- TV, radio, music players, and toys - do not set the volume too high. Children especially are very sensitive to the damaging effects of loud music. Researchers found that noisy toys put children's hearing at risk.
- Headphones - focus on isolating what you want to hear; block out all outside noise as much as possible, instead of drowning it out with high volume.
- The workplace - if you work in a noisy environment, wear earplugs or ear muffs. Even in discos, nightclubs, and pubs - earplugs are discreet and hardly noticeable.
- Leisure venues - if you go to pop concerts, motor racing, drag racing, and other noisy events, wear earplugs.
- Cotton swabs - do not prod them into your or your children's ears. The same applies for cotton or tissues.