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!

You may find out that your child is suffering from hearing loss when he is born or he may be diagnosed with the condition later in life. Hearing loss in children is commonly caused by otitis media, birth problems and on the account of certain injuries or illnesses.

Signs and symptoms

The early-stage symptoms of hearing loss in children are as follows:

No reaction to extremely loud noises

No response or reaction to your voice

The child makes some sounds, which taper off

The other symptoms which follow include:

He may pull and rub his ears.

He may act cranky constantly without any proper reason.

He might stop paying attention.

Low energy levels are indicated.

He may find it hard to follow directions.

He may ask you to increase the volume of the radio or TV.

Fever and ear pain are also likely symptoms.

Treatment

Early hearing loss can affect a child s language learning skills. If the problem is diagnosed and treated soon, the problem with language can be avoided. The method of treatment depends on the cause of hearing loss in your child and the severity of hearing loss.

There are certain medicines for hearing---prescribed by your ENT Surgeon. Please take the point watchful waiting. Even, if you have the slightest doubt of hearing loss contact ENT immediately.

The primary treatment methods of hearing loss in children include the following:

Watchful waiting: Sometimes, the condition may resolve on its own and the treatment involves simple monitoring of your child for positive changes.

Medicines: There are certain medicines for hearing loss in children, which may be prescribed by your pediatrician.

Ear tubes: Ear tubes may be recommended if medicines and monitoring do not work. The tubes allow fluid to drain and help in preventing infections. A minor surgery has to be undertaken on your child for getting the ear tubes placed. This will solve hearing loss and other related issues.

Hearing aids: Hearing aids may be used to allow your child to hear better. It is safe for children to use ear tubes after the age of one month. For the right hearing aid for your child, you need to consult a hearing specialist.

Implants: Cochlear implants are used to treat hearing loss in many children. These are electronic devices that are put in the inner ear to benefit hearing. These should be used only if hearing aids did not succeed and these are meant to be used only in the case of children with serious hearing problems.

There are several other devices, which can help your child with hearing loss. For suggestions and recommendations, you must take your child to a hearing specialist.

Hearing Loss In Children - Signs And Treatment Of It!

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?

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 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

Hearing is one of the five senses that you or anyone else relies on for communication and a whole host of other things. Among the five senses, touch, smell, taste, hearing and sight, it is often hearing, which is the most easily affected. You don't have to be a senior citizen to experience hearing loss as it is quite common among younger people as well. This problem is also getting widespread with each passing day.

Before coping with hearing loss, the first job is to detect it
If you have a hunch that you or any of your loved ones is suffering from hearing loss, it is important that you get it diagnosed. Some of the common symptoms of hearing loss will be:

Hearing difficulties while talking to someone over the phone
Straining to hear conversations with family members or friends in household situations 
People usually need to repeat what they talk to you with a louder voice
You often hear complaints that you keep the TV too loud.
In crowded situations, such as family gatherings or a restaurant, you have problems listening to people.
Although this condition tends to affect senior citizens, an increasing trend shows younger people i.e. people mostly in their 40 and 50s and even younger than that suffer from this condition.

How to deal with hearing loss?
Some of the options to deal with hearing loss are mentioned below.

Once diagnosed with hearing problems, change your ways of communication: If you or your loved ones have been diagnosed with hearing problems, it is important that both adjust your ways to deal with the problem. Rather than getting angry at the other for not being able to communicate effectively, develop and strategize ways to make communication less problematic and erratic.
Cochlear implants: This is a newer technology, which has gained popularity in the past decade or so. These are directly implanted into the inner ear and are designed to bypass the damaged areas of the ear and directly stimulate the auditory nerve. These have become very popular, although, are not a replacement of natural hearing. It is only prescribed for people who have severe hearing loss or are deaf.
Hearing aids: Although selecting a hearing aid is difficult as the patient needs to be comfortable, once selected it is one of the best solutions for the hearing impaired. The functionality of these devices can be extended with the hearing assistive technology or HAT devices that can focus the audio waves depending on the situations and give a more tailored approach for every individual's needs.

Coping with Hearing Loss

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