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!

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?

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

A cochlear implant is an electronic device that is used to restore hearing ability in people who suffer from hearing loss due to inner ear damage. Unlike hearing aids which amplify sounds so people with hearing loss are better able to perceive them, cochlear implants directly transmit electronic sound signals to the auditory nerve. They work by compensating for damaged cochlea, the part of inner ear that converts sound waves into neural impulses which are perceived by the brain. 

A child who cannot hear will also never be able to speak. 
Inability to hear and speak puts a person at disadvantage in society. 
Simple tasks like talking on the phone which we take for granted become impossible. 
Conventional methods of learning like attending a classroom lecture become impossible. 
Brain development and intelligence is closely linked to development of speech and language. 
Inability of parents to communicate with child leads to emotional distancing. 
Integrating a hearing disabled person in to society becomes a frustrating experience for all involved. 
Cost of integration per capita in much higher when compared to cost of Cochlear Implant. 
Children with Cochlear Implants are fully integrated in society with normal hearing & speech. 
Children with Cochlear Implants can attend regular schools & colleges 
Early identification of hearing impairment gives children a better chance of developing speech and language skills, and of making the most of social and emotional interaction from an early age. 
Cochlear implants offer a multitude of benefits to people experiencing hearing loss, especially infants and children with severe hearing loss who are just starting to process language and speech. Some of these benefits are: 

Hearing and communication   Cochlear implants improve hearing significantly, especially to a level where normal communication can be restored. It enhances the person s ability to hear speech without relying on visual cues such as lip reading or hand signs. It makes telephonic conversation a lot easier. Cochlear implants also help people regulate their own voice and volume so that it is easier for others to understand them. 
Quality of life - It can help people with hearing loss listen to and enjoy music and also watch television with sounds. It helps people listen to sounds in a noisy environment and differentiate soft sounds from loud ones. 
Safety   Cochlear implants enhance the individual s safety as it allows the person to be able to hear warning sounds like honking cars or ambulance sirens. This will allow people to navigate public spaces in a much safer way. 
Society - For children with hearing loss, cochlear implants can help them adjust a lot better to regular schools instead of requiring special institutions. 
Brain development - It can help infants with cochlear damage develop their speech recognition and speaking skills much faster. 
Cochlear implants 

Only device of its kind that can restore a special sense.
Consists of 2 parts-
One part is surgically implanted in the body 
Second part is worn behind the ear and communicates with internal part using wireless signals 
Persons of any age can get a cochlear implant, from less than one year old children to 90 year old persons have received implants all around the world. 
Cochlear implants can be done for partial hearing loss also, know as Hybrid implant that preserves the residual natural hearing and augments hearing only in certain sound frequencies in which hearing loss is present. 
Are being used for indications other than hearing loss also like Tinnitus. 
Surgery is a day care procedure for most patients. 
Consult a doctor to get a clear assessment of whether you would be an eligible candidate for a cochlear implant and what to expect from the procedure.

Cochlear Implant - The Many Benefits Of It

Loss or impairment of hearing can happen due to a variety of causes and may be temporary or permanent in nature, depending on the type and severity of the condition. One of the most common causes of hearing impairment is age. The degeneration of one's vital organs and senses becomes a reality for many people as the advancement of age happens, and the delicate insides of the ears are no different. Noise pollution, injury to the ear drum or any other part of the ear, as well as the movement of particles inside are only a few of the many reasons for loss of hearing. One of the many ways of dealing with long term hearing impairment is by using a hearing aid. Read on to know how you can regain your hearing with hearing aids.

1. Long Forgotten Sound: A hearing aid will help you reacquaint yourself with long forgotten and subtle sounds that you may not have been able to hear for a long time. Many times, this kind of impairment also makes us forget to notice the fact that we do not hear so many normal sounds in our everyday lives - sounds that we probably did not notice even earlier. This may include the whirring of a fan, the whoosh of the air, the pitter patter of pets' feet and so much more. A hearing aid will help in bringing about an adjustment to these sounds that you will actually end up discovering again.

2. Technicalities: The hearing aid basically works by letting your hearing and ears readjust to the various volumes, intensity and vibrations that one feels when different sounds reach the ears. This helps in hearing better.

3. Amplification: A hearing aid is basically an electroacoustic device that fits in neatly behind your ear where it is tucked away from plain sight. This device helps in amplifying sounds so that you can catch them and hear them more clearly.

4. Modulation: The ENT specialist will usually prescribe a certain kind of hearing aid based on your individual condition in terms of volume, size, power and circulation. These factors help in modulating the sounds in a certain way so that they reach the nerve pathways from the inner ear and travel to the brain in a proper and effective way. This is usually done in a customised way to cater to the specific type and cause of your hearing loss.

Using a hearing aid is a matter of retraining your hearing rather than restoring it completely. These aids will be helpful in regaining a better sense of hearing within the condition you are going through, rather than reversing the condition completely.

Regain Your Hearing With Hearing Aids

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 

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