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!

What Causes Meniere s Disease?
Meniere's Disease is a disorder that basically affects the sense of balance and hearing of the patient. This condition takes place in the inner ear and it may cause problems like vertigo, where patients experience a spinning sensation and a ringing sound as well. This is a chronic disorder, which requires a number of lifestyle changes so that it can be managed and treated without becoming a debilitating problem.

Read on to know more about the symptoms and causes of the condition.

Symptoms: The various signs and symptoms of this disease include a feeling of fullness in the ear, which has been affected by the condition. Also, most patients have a spinning sensation and a ringing sound in the affected ear. Nausea and vomiting may also be caused by this condition, along with sweating and loss of balance. It is also normal to feel uncoordinated when you are suffering from this condition.

Secondary Symptoms: This condition may also cause many other complications, which are known as secondary symptoms. These include anxiety, depression and stress. This usually happens due to the problems in the ear and even the problem of vertigo. This may cause problems for people who climb heights or ladders for their work or even for other recreational causes. The constant sense of dizziness and spinning may cause a person to become anxious and undertake stress when it comes to carrying out various kinds of functions in day to day living.

Causes:  There are various causes of this condition, even though many medical reports suggest that the exact cause has not really been understood so far. As any doctor will tell you, the in the inner ear there is a labyrinth which contains fluids. When there is a problem with the composition and level of these fluids, the patient may develop Meniere s Disease. This condition specifically affects the inner part of the ear. This part is made up of passages and a cavity as well as soft membrane like structures with hair like sensors. These sensors respond to the fluid s movement within the ear. When this fluid does not have the appropriate levels, it can lead to many complications like Meniere s Disease, which affect the nerve impulse that these sensors catch and transmit to the brain.
Stages: This disease progresses over a set of three stages. In the very first stage, the patient will experience much dizziness and nausea, before that progresses to the second stage. In this stage, the patient will have moderate to severe vertigo. This is also known as the middle stage. Finally, this may culminate into the third or late stage, which leads to more frequent episodes of vertigo. The hearing of the patient and the ringing sound in the ears will also get worse in this stage.

Meniere s Disease -  What are the Reasons Behind It 

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?

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

The sense of hearing is a complex one. The ears are divided into three parts: the outer ear, the middle ear and the inner ear. While the outer ear sends the sound to the middle of the ear, the middle ear sends the same to the inner ear through the conductor. The inner ear in turn sends the auditory signal to the brain where the signal is processed and gives rise to a sense of hearing. 

Problems in hearings can arise if there is a problem in any of these parts. However, more often than not it is the middle or inner ear that is affected. However, there may also be situations when there are problems in both the middle and the inner ear. In that case, it is known as a mixed hearing loss. 

What is Mixed Hearing? 

Loss If there is damage to the middle ear, it may precipitate a conductive hearing loss. Now, in addition to that, if there is a sensorineural problem, then it gives rise to mixed hearing. So, in the case of a mixed hearing loss, all the parts of the ear and even the brain may be involved. There may be damage to the outer ear, middle ear, inner ear, as well as the auditory nerve. 

Causes of Hearing Loss 

Since mixed hearing loss may result from damage to any part of the ear, it is necessary that the causes be grouped into different categories. 

  Sensorineural Hearing Loss 

Sensorineural loss may result from drugs, different illnesses, head trauma, malformation of the inner ear etc. Genetic predisposition may also precipitate such problems. 

  Conductive Hearing Loss 

Such type of hearing loss may result from excessive deposition of earwax, ear infections, perforation in eardrums, malformation of the middle or outer ear, as well as problems with the fluid in the middle ear. 

Symptoms of Hearing Loss 

Reduced hearing in either or both ears is considered to be delineating symptoms of mixed hearing loss. 

Treatment 

Since mixed hearing loss can result from sensorineural failure as well as damage to outer or middle ear, treatment needs to be divided into different groups too.   To treat Conductive Hearing Loss, doctors can use surgical or medical options. 

  For sensorineural hearing loss, doctors usually prescribe hearing aids. This indicates that treatment for mixed hearing loss may include treatment for conductive hearing loss as well as that for sensorineural hearing loss. So, it is usually a combination of both medical and surgical options. Physicians may prescribe both medicines and hearing aids or resort to surgical interventions and also prescribe hearing aids. Sometimes, physicians use special hearing aids or implants to treat mixed hearing loss. 

Conclusion 

In case a person is sensing reduced hearing in one or both ears, he/she needs to seek medical advice. It may be a symptom of mixed hearing loss. Getting in touch with an experienced physician is the first step towards that.

Mixed Hearing Loss - How To Treat It?

Hearing loss is experienced by millions of people these days. Ageing is not the only factor that brings hearing impairment. The causes are many. Certain medications, continuous exposure to loud noise, genetic involvement, injury and some medical conditions may cause hearing loss.

There are quite a few myths that people have come to believe about hearing loss over the years. Since it is such a common phenomenon, here we take a look at the common myths surrounding hearing loss.

Myth no. 1: Hearing loss is exclusive to elderly.

Fact: As said before, hearing loss can be an outcome of various causes. Nearly half of the people suffering from the same are below the age of 55 years. No matter what your age is, you must always get your ears checked, especially if you are feeling that are you missing things.

Myth no. 2: Diagnosing hearing loss is easy.

Fact: Most people do not come to know about the condition until it gets worse. Also, your physician never really checks for hearing loss symptoms in a general check-up unless you ask for it specifically.

So, always get a check-up done, like you do for other probable diseases.

Myth no. 3: There's no effective solution for hearing loss.

Fact: Like there have been advancements in the medical field for everything else, there are aids available these days that improve your hearing and have finer adjustments for noise adaptation. Also, there are certain other procedures and surgeries that have proved to improve the condition in many.

So, seek help as soon as possible.

Myth no. 4: The sounds aren't loud enough; my ears are healthy and fine.

Fact: If there is a problem you're experiencing with hearing, you have got to accept that and get it treated. Avoiding a certain condition will only get things worse for you.

Also, hearing aids are no more a stigma. Ear aid devices have designs similar to earphones these days, which are comfortable enough to wear. Ignoring a medical condition or inability to accept the same would only do more harm instead of making things fine.


Common Myths About Hearing Loss

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