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

Hearing loss is not something limited to elderly men or elderly women only. This is something that can affect any adult at any stage of life. The good news is that it can be managed. Today, there are a number of hearing aids that can help improve hearing ability. These aids allow an individual who has lost part of his or her hearing to live a normal life. For best results, hearing loss should be arrested in its early stages.

Here are a few signs you should look out for.

Muffled Speech: When you hear people talk, their speech should be clearly identifiable. If you hear muffled noises that you have to interpret as words, it could be a sign of hearing loss.
Difficulty understanding words: The human ear has the ability to separate conversational sounds from ambient noise. However, in some cases where the inner ear is damaged, a person might find it difficult to separate the two. In such cases, the person might not be able to understand words clearly as they get confused with background noise. This is a sign that must never be taken lightly.
Feeling the need to ask people to repeat themselves: Once in a while if you find yourself asking another person to repeat what he or she said, there is nothing to worry. However, if this becomes a regular occurrence and happens with many people, then the fault lies in your hearing. Thus, if you notice yourself needing to ask people to repeat themselves or talk slowly, consult a hearing specialist.
Increasing the volume of the radio or television: As children, we are taught that playing music or watching TV at a very high volume can damage the ears. The radio, television and other such appliances should be played at a volume where the information being relayed is clearly audible and people can have a conversation alongside without needing to shout. If you find yourself having to increase the volume constantly, it could be a sign of hearing loss.
Withdrawing from conversations: As hearing loss is aggravated, people begin to feel conscious of having to ask others around them to repeat themselves or talk slowly. This can make them withdraw from a conversation and other social situations. In many cases, the simple reason for avoiding a party could be the discomfort of not being able to hear the other person clearly in a conversation.
 All hearing problems can be better handled by proper investigation, different test, sign and symptoms of the patient, which are carried by a specialist of hearing, called as AUDIOLOGIST, who can look your hearing problems, plan out treatment plan, rehabilitation pre and post etc. Audiologist will definitely help you hear again. 

Common Signs Of Hearing Problems In Adults!

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

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

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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