Usher syndrome is a genetic disorder that is characterized by complications in vision and hearing; the most common of them being partial/complete loss of hearing and retinitis pigmentosa. Retinitis pigmentosa is characterized by progressive deterioration of the retina, resulting in deprivation of peripheral vision and subsequent night blindness.

The symptoms and its rate of progression usually vary among people. Usher syndrome is classified into three types:

Type 1: In this type, children are born with balance issues and hearing loss problems. The symptoms of night blindness and loss of peripheral vision associated with  Retinitis Pigmentosa  only appear in the early stages of adolescence.
Type 2: In type 2, children are born with moderate to mild hearing loss problems. Retinitis Pigmentosa develops soon after the child reaches adolescence.
Type 3: In type 3 Usher Syndrome, children are usually born with normal hearing skills, however, loss of hearing and Retinitis pigmentosa occur soon after puberty.
Causes
Hearing loss in Usher syndrome occurs when the nerve cells that are present in the cochlear (inner ear s spiral cavity) are affected by genetic mutation. A similar problem occurs in the cells of the retina leading to loss of vision. These cells allow conversion of light into electrical signals for the brain to interpret them. Both the parents need to pass the mutated gene to the child for it to be affected. If the child has only one gene, then he/she rarely develops the symptoms.

Treatment
This disorder does not have any prescribed course of treatments. The vision loss that occurs in this disorder can be slowed down by nutritional therapy. This therapy involves providing the body with essential amounts of vitamin A which can help in reducing vision loss.

Usher Syndrome - Things You Must Know!

Cochlear Implant is an electronic device replaced with the inner ear (cochlea) of the patients who are unable to send sound waves to the brain.

The surgery is performed on the patients who have the problem of hearing loss due to the damaged hair cells in the inner ears. The implant helps the patient to hear which are not responding to other hearing aids.

Conditions for Cochlear Implant:

Cochlear implants help people to hear with clarity. They are not like hearing aids, which make the sound louder, but it helps send sound waves to the brain and replaces the damaged hair cells in the inner ear. Following are cases where it is beneficial:

People who find it difficult to hear clearly with hearing aids.

Those with moderate hearing loss in both ears.

Patients with 50% or fewer score in the sentence recognition test.

Patients with 60% or less score in sentence recognition test with hearing aids on.

It is suggested to opt for cochlear implants in both ears. The reason is to give the patient a better perspective of sound direction and clarity in understanding the words.

How does the Cochlear Implant work?

Cochlear Implant is an electronic device with an embedded sound processor that has to be worn outside ears or on the body. Following steps are involved in cochlear implant:

The sound captured by the processor is converted digitally by the processor and sent to the coil inside the ears of the patient.

The implant converts digitally coded sound waves to electrical impulses.

The electric impulse simulates the hearing nerve of the patient.

Then sends these impulses to the brain.

Benefits of Cochlear Implants:

The cochlear implant is a blessing for people who have been suffering from hearing loss for a very long time. It provides aid to people who have not benefited from hearing devices. The benefits of the cochlear implant are:

Hear better.

Studies have shown that people with cochlear implants have better sentence recognition scores instead of hearing aids.

The patient can concentrate better in the crowd and can hear conversations across the table.

Reconnect with the missed sounds.

Enjoy music on the phone.

Cochlear Implant improves the quality of life of people with hearing loss. It gives them an aid to understand and engage themselves in a conversation. However, it takes time to learn the interpretation of the sound signals that are received from cochlear implants. But, the patient can learn and lead a normal life with the implant. The factors which affect the benefits of Cochlear implants are:

The extent of hearing loss

The duration of the hearing loss and the condition before implantation

Damage to inner hair cells

Other health issues

The practice and rehabilitation time have been done in practice after implantation

The cochlear implant is performed in the patients after taking extensive tests determining the condition of the patients. It is activated after 2-6 weeks of surgery to give the surgery site a time to heal. Most people are discharged from the hospital after surgery and are prepared for the use of an implant. There are training sessions and classes for patients who have just gone for cochlear implants.

Cochlear Implant - How Do They Help?

Hearing loss is a common sensory problem that generally develops with increasing age or due to prolonged or persistent exposure to loud noises. It is one of the most typical problems that occurs throughout the world. If you are suffering from partial hearing loss, you may find ways of keeping in touch with your family and friends. If you are not suffering from such the following may help you keep your hearing intact for years to come.

Types of hearing loss
There are two main types of hearing loss: 
1. Conductive hearing loss where the problem lies in the middle ear, eardrum or ossicles. A conductive hearing loss affects the passage of sound between the eardrum and the inner ear. Sound passes down the ear canal to the ear drum and through the middle ear, where the sound is transmitted across the middle ear by the three bones called the ossicles to the inner ear.

Causes of conductive hearing loss:
1. Malformation of outer ear, ear canal, or middle ear structures
2. Fluid in the middle ear from colds
3. Ear infection (otitis media - an infection of the middle ear in which an accumulation of fluid may interfere with the movement of the eardrum and ossicles
4. Allergies
5. Poor eustachian tube function
6. Perforated eardrum
7. Benign tumors
8. Impacted earwax
9. Infection in the ear canal
10. Foreign body in the ear
11. Otosclerosis

2. Sensorineural hearing loss where there is damage in the inner ear, cochlea or hearing nerve. Sensorineural hearing loss (snhl) occurs when there is damage to the inner ear (cochlea), or to the nerve pathways from the inner ear to the brain. Most of the time, snhl cannot be medically or surgically corrected. This is the most common type of permanent hearing loss.

Causes of sensorineural hearing loss:
1. Exposure to loud noise
2. Head trauma
3. Virus or disease
4. Autoimmune inner ear disease
5. Hearing loss that runs in the family
6. Aging (presbycusis)
7. Malformation of the inner ear
8. Meniere's disease
9. Otosclerosis 
10. Tumors

Coping with hearing loss
Antibiotics and eardrops are often prescribed to treat hearing loss and its symptoms. In many cases, people suffering from severe hearing loss use hearing aids. Hearing aids are tiny instruments that people use in their ears to make sounds louder. People who suffer from hearing loss cope with their problems through increased concentration and focus. The usual way involves reading the moving lips of the person while they talk. Prevention is better than cure and is the best solution to avoid long term hearing loss. Avoiding continuous loud noises, to the possible extent helps prevent hearing loss to a great extent.

All About Hearing Loss

Your hearing is one of the most important function your body carries out and sometimes you realize that you are losing your sense of hearing, when it is too late to get it back. There are two main reasons for this. One of the reason is age  and the other is when the inner ear's hair cells break down and do not pick up sound well. Here are 7 ways to safeguard your hearing.

1. Try to avoid loud places
It is not advisable to go to places where you have to shout to be heard, such as in a street, a concert or a construction site.

2. Buy low noise rating equipment
The equipments in your house will make sound that you hear the most. Try to avoid these equipments by buying appliances with a low noise rating.

3. Wear hearing protection at loud places
However, it is true that it is not always possible to avoid loud noises. This is when you need to get hearing protection. Earplugs and earmuffs are two of the best ways to make sure that even when you are in a loud place, your hearing does not get affected. Earplugs and earmuffs generally reduce sound by 15 to 30 decibels, which may be crucial to make sure that later in your life, you do not lose your hearing.

4. Avoid smoke
Smoking raises your chance of hearing loss. Second hand smoke does the same thing. Therefore, try to avoid, both smoking and secondhand smoking. 

5. Remove earwax properly
Earwax cannot be removed properly using a cotton swab. Instead, you should use an irrigation kit. Remember this as otherwise; the earwax could muffle your hearing.

6. Avoid medications which reduce hearing
Certain medications increase hearing loss. Therefore, double check with your doctor to make sure your medicines will not make you lose your hearing.

7. Get your hearing tested
Finally, get your hearing tested as identifying the problem early on can help stop worsen the situation.

7 Tips to Safeguard Your Hearing

Hearing has a deep impact on your life. It determines the kind of life you are going to lead. It has been found by various studies and surveys that hearing aids drastically improve the quality of life you lead. It is the much needed relief to a life, burdened by the stress and strains of living with hearing impairment. The hearing aids are not just instruments. This is a way to live a life of dignity. According to the Evaluation of the Social and Economic Costs of Hearing Impairment report, hearing loss leads to socio and economic stress in ones  life in major continents like Europe, Australia and the United States.

A research conducted by the National Council on Aging found that over 2,000 people living with hearing impairment or loss experienced positive changes in their respective lives after using hearing aids. They experienced improvements in social, emotional, psychological and physical spheres of life. Furthermore, a US study claims that nine in ten people using hearing aid say that their life has changed for the better after using the aids. It can also be said that hearing aid users lead a better life than non-users of these aids.

How these aids better your life?
Hearing aids gift you a life of expressions and dignity. They can connect you to the rest of the world and empower you to maintain your individualism. You are empowered to make your point in front of others and understand their perspectives too. Here s what a hearing aid can do for you:

Users of hearing aids have a better life as they have improved cognitive functioning. The family members claim that the users of these aids become more interactive. They are lesser of an introvert than the non-users.

It improves the social life of user. He or she can participate in group activities and their family and other ties or relationships go smooth. There is no communication gap.
Hearing aids build up your confidence. You regain your lost dignity and faith on your abilities. Hearing loss kicks you to a life of loneliness, alienation and leaves you with low confidence levels.
It helps you in having better psychological functioning. It keeps you away from psychological problems like depression, personality disorders and over-all absurd behavioural changes.
It improves the understanding in closer or personal relationships. The warmth in a relationship is regained or bettered with effective communication.

Hearing Aids - How Can They Improve Quality of Life?

It is that time of the year when insects are all around. Suddenly one feels a buzzing sound or an itchy feel in the ear. To your horror, one feels a live insect is in your ear, which can be extremely annoying and is like a nightmare. Insects often get stuck when they enter our ears as they cannot fly or crawl out. They try to find their way out and their movements inside our ears can be very uncomfortable, painful and can be itchy as well. Moreover, they can easily produce infection.

In this scenario, do not try to remove an insect with cotton swab, tweezers or hair clip as it will make the situation worse and can lodge the insect deeper into the ear canal, which can possibly damag the eardrum, leading to permanent hearing loss. If an individual is not certain about the potential harm taht can be caused by the insect in the ear, one should seek medical care immediately. Insects in the ear are common reasons for visits to doctor's clinic, especially in children.

If one suspects an insect in ear, one may experience pain, swelling, blood or crackling. One may even feel biting, stinging, hearing loss or dizziness. It is best to stay calm in this situation as being active may lodge the bug further in ear or cause it to move further back or cause serious damage to the sensitive eardrum

One way to try to remove a bug in ear is by tilting the ear toward the ground and attempt to wiggle the ear. Grasp the earlobe and give it a wiggle. If the bug is not too far into ear canal, it may fall out on its own. If the bug is still alive and is not too far inside of the ear canal, it may simply come out on its own. If one stays calm and keep objects including fingers away from ears, it is likely that the bug will find its way back out of ear.

One can also try to flush the ear with warm water with a dropper or a bulb syringe. This can be done by holding head upright and stretching the ear canal by pulling the outer ear and then putting a steady stream of warm water into ear. Tilt head to the side to drain out the ear. Do not try this if one suspects that ear drum has been ruptured to prevent additional damage.

To avoid stinging or eardrum rupture from scratching or biting , one may use a drop or two of mineral, baby, or olive oil inside your ear canal to kill the insect. Finally, visit an ENT specialist doctor as they can remove the insect by special suction devices. Post insect removal, one must look out for signs of infection as swelling, dizziness, hearing loss, fever, and pain. Finally follow up with ENT specialist or Otolaryngologist for the final opinion.

How to Remove Insect from Ear at Home? 

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