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!

Proper and sound hearing is essential for a healthy living. If you are suffering from lack or loss of hearing, chances are that you are missing out on life itself. Therefore, it is imperative that you undergo ayurvedic treatments in case you are suffering from any kind of hearing ailment.

Common Cause of Loss of Hearing
There are various reasons that contribute to the loss of hearing. Some of the common causes are:

Age
Increasing noise
Genetically inherited
Over the years various surgical and non-surgical methods have surfaced that can help repair loss of hearing and damage. Among the other branches of medicines, some home remedies, and ayurvedic treatment for hearing loss and deafness.

Ayurvedic Remedies Fo Hear Loss
Ayurveda is the most old and ancient method. It uses natural herbs in order to correct and treat hearing loss. This has proved to be extremely beneficial and potent and it cures hearing ailments with the help of a number of natural home methods. Some of them are:

Lukewarm mustard oil or sesame oil: If you are suffering from ache in ears or loss in hearing, try putting few drops of warm mustard oil and sesame oil in your ear and hold it with index fingers. At the same time store air in your mouth and let your cheeks contract and bulge for a minute or so. This ayurvedic tip considerably improves hearing and reduces ear pain.
Radish juice and sesame oil: If you are suffering from sores and hearing problems, a mixture of the two can be beneficial. In this ayurvedic method, a small quantity of Radish juice should be boiled with a small quantity of sesame oil, till the juice absorbs the oil. This mixture should be administered twice a day in order to experience the results.
Applewood oil: A variety of ingredients are required in order to create the resultant oil. Initially, cow's urine should be boiled and the applewood powder must be added to it. This mixture should then be filtered and further added with sesame and goat's milk. This concoction is then boiled and the resultant oil should be stored and applied with cotton daily. This improves hearing a great deal.
A mixture of neem and turmeric: In this natural home remedy, turmeric and neem leaves are boiled together. The steam should be taken as well as the water must be applied to the ears and surrounding areas. This has proved to be an extremely effective remedy in treating pus in the ears and consequent hearing loss.
A mixture of Radish juice, turmeric, sesame oil, and ajwain seeds: All these ingredients must be taken in selective quantities and boiled to get the resultant oil. This oil must be stored and used to treat loss of hearing and other ear diseases.

5 Ayurvedic Tips To Improve Hearing

You should consider undertaking a cochlear implant in case you are experiencing hearing loss or are turning deaf. This is a small device that gets planted in your ear through a surgical procedure. It sends impulses to your auditory nerves directly, as a result of which sound signals get carried to your brain. Cochlear implants may not make you hear normally again, but help you with different sounds, and in the recognition of the different sounds around you like alarms, telephones, and door bells.

How it works?

A cochlear implant is fit for children and adults who suffer from the sensorineural hearing loss. This condition damages the tiny hair cells in your inner ear, which is called the cochlea. Hearing becomes impaired when these get damaged.
The device consists of two specific parts. The first part or the receiver-stimulator is placed under your skin via a surgical procedure. The other part of the device is the speech processor. It has to be worn around your neck in the form of a hearing aid.
The external device is, however, larger in size than a usual hearing aid. The receiver gets placed under your skin, behind the ear via a small incision. It is connected to electrodes.
This is inserted into the cochlea of your inner ear. The surgery requires a few hours to be undertaken successfully.
The speech processor has to be placed two weeks after the surgery is undertaken. The processor can be connected to the microphone, and this can be worn around the ear.
You can wear it on any other part of your body, based on your lifestyle and age.
Benefits of cochlear implant
The major benefits of a cochlear implant are as follows:

You might be able to hear speech almost normally.
You will be able to understand speech better, and will not have to depend on lip reading.
It will be much easier and simpler for you to listen to the television and talk over the phone.
You will be able to listen to music better.
You may also be able to pick up various sounds around you that include soft, medium, and loud sounds.
You will be able to control your own voice better. This makes other people understand you better.
If you are suffering from severe hearing loss, a cochlear implant may be able to change your life for the better. However, the results are not the same for all, and vary among people. It is possible for some people to benefit more from such an implant than others. 

Cochlear Implants - 6 Benefits You Must Know!

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? 

BAHA Implant
Bone Anchored Hearing Aid (BAHA) is a device surgically implanted to help people suffering from a loss of hearing. Most conventional hearing aids transmit sound through air conduction. The BAHA device is suitable for conductive or mild-moderate mixed hearing loss.

Problems with hearing in the middle or outer ears can be helped with the BAHA device. The sound waves received by the BAHA device bypass the middle ear and get delivered straight to the functioning cochlea in both ears.

A BAHA device comprises of two parts:
a) The processor 
b) A fixture which is surgically implanted behind the ear in the bone.

The implant is embedded in the bone. The processor, which remains outside, is attached to the implant. There is a microphone on the processor that picks up sounds. The sounds are passed on to the implant. The implant vibrates which triggers the Cochlear. You can consider wearing a BAHA implant if other conventional hearing aids do not work for you. BAHA implants are also given to people with collapsed or irritated ear canals.

Cochlear Implant Surgery

A complex, small electronic device that helps hearing in people with severe hearing loss or deafness is used in the Cochlear Implant Surgery. The cochlear implant comprises of an external piece behind the ear and another piece that is surgically implanted under the skin. A cochlear implant comprises of four parts:
1. A microphone which picks up sounds from the surroundings
2. A speech processor which chooses and arranges the sounds the microphone picks up
3. A receiver/stimulator and transmitter that picks up signals from the speech processor and changes them into electric impulses
4. An electrode ray, a bunch of electrodes, receives impulses the stimulator sends and transmits them to different areas of the auditory nerves.
The implant does not cure deafness; it only gives you a useful portrayal of sounds in the environment and considerable aid in understanding speech.

The surgery is done in the following way:
i. The area around the site of the implant is shaved or cleaned
ii. The patient's vital signs are monitored through cables, patches and monitors attached to him or her
iii. General anaesthesia is administered through IV or through a face mask
iv. After the device is implanted, the patient is taken to the recovery room for the anaesthesia effect to lessen.

BAHA Implant and Cochlear Implant Surgery

What causes hearing loss? + How to ensure you can help it as you age

The partial or total inability to hear is known as hearing impairment or hearing loss. It can be present at birth, or develop later in life.

There are a number of factors, which may cause hearing loss

1. Age - Age is the biggest factor when it comes to the loss of hearing, and you may lose the ability to hear as you age. This condition is known as presbycusis. It becomes difficult to understand high-frequency sounds like that of a child or a woman when you get old.

2. Noise - When you are exposed to loud noises for a prolonged period of time, it damages your ears. This leads to loss of hearing. 5% of the total population of the world is affected by noise (the degree of suffering varies). It may be a result of continuous exposure to loud music or a sudden exposure to a loud noise like an explosion.

3. Hereditary disorders - Hearing disorder may be inherited by the dominant or recessive genes of parents in the child. 70-80% of these cases inherit from the recessive genes, whereas 20-25% inherit hearing loss from the dominant genes.

4. Trauma - Serious injuries of the head/ears may cause loss of hearing, which may be either temporary or permanent. When damage is caused to the brain, the brain fails to process the message conveyed by the ears. So even if the ears are totally functional, a person may face the problem in hearing.

5. Perinatal problems - The ototoxic effects on the fetus due to excess intake of alcohol during pregnancy lead to hearing the loss in about 64% of the infants born to alcoholic mothers.

Also, premature birth can be associated with hearing loss due to high risk of being exposed to noise in neonatal units.

Knowing about the causes of hearing loss can lead you a step closer to preventing this disorder as you age.

Here are some useful tips that can help prevent hearing loss:

1. Be more aware - You should be diligent and aware of the situations, which may risk your hearing ability and should try to avoid such situations as much as possible. Limit your exposure to sources of hazardous noises like firearms, firecrackers, concerts and clubs.

2. Take precautionary measures - If your occupation calls for working at an environment of loud noises, use earplugs or earmuffs to block out the excessive noise. Also, make sure that you work in a place where employers take all the necessary measures of noise control under the federal or state regulations.

3. Monitor your use of gadgets - Monitor and control the use of hearing devices, and try to reduce the use of headphones/ earphones as much as possible.



What causes hearing loss? + How to ensure you can help it as you age

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