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!

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

Did you know your earphones are making you deaf? Here's how.

Fact: Roughly 1.1 billion people worldwide within the age group of 12-35 have been found to be at an increased risk of developing hearing problems.

Listening to loud music on handheld devices using earphones has become a common trend among youngsters these days. You might find it very relaxing to plug in your earphones and escape into your own world of music, but you need to know that this can cause serious damage to your ears. The worst part: you won't realise your ears are being damaged until it's too late.

So, exactly how is loud music damaging your ears?

Continuous exposure to loud music from earphones or other sources results in a medical condition known as Noise-Induced Hearing Loss (NIHL), which can be associated with irreversible damage to the ears resulting in deafness.

When you hear loud music for a considerable amount of time every day it affects your hair cells (nerve cells responsible for sending sound signals to the brain) negatively, so that their ability to respond to sound decreases. If this keeps on happening for many months, eventually the hair cells are damaged beyond repair. These cells cannot be regenerated, making you permanently deaf.

How loud is too loud?

If your ears are exposed to sounds at 95, 100, 105, 110 and 115 dB (decibel, the unit used for measuring sound) for 4 hours, 2 hours, 1 hour, 30 minutes and 15 minutes each day respectively, your ears are at risk of getting severely damaged. Also, playing music at 120 dB or above can damage your ears instantly. You can have a realistic idea about the relation between decibels and sounds you commonly hear by referring to this list:

30 dB: soft whisper
75 dB: busy traffic
90 dB: noise of a motorcycle at 25 feet
100 dB: noise of a farm tractor
140 dB: jet plane taking off
Moreover, if you experience the following symptoms regularly, there's a high chance that you need to get your ears treated soon:

A ringing sound in your ears when you are at a quiet place, which vanishes after a few minutes
You need to raise the volume of TV or music to the fullest to hear it properly
You have difficulty in hearing people talking at a distance of just 3 feet
Tips For Safer Listening

Use earplugs: The louder the noise and the longer you're exposed to it, the greater the chance of damaging your hearing. Protect your ears with ear protectors   earplugs or earmuffs   and get away from the noise as quickly or as often as you can. 
Turn down the music: Don't listen to your personal music player at very high volumes and never to drown out background noise. If the music is uncomfortable for you to listen to, or you can t hear external sounds when you ve got your headphones on, then it's too loud. It's also too loud if the person next to you can hear the music from your headphones.
Use the 60:60 rule: To enjoy music from your MP3 player safely, listen to your music at 60% of the maximum volume for no more than 60 minutes a day. 
Wear headphones: When listening to your personal music player, choose noise-cancelling headphones, or go retro with older muff-type headphones. Ear-bud style headphones and in-the-ear headphones are less effective at drowning out background noise. 
Turn down the dial: Turn down the volume on your TV, radio or hi-fi a notch. Even a small reduction in volume can make a big difference to the risk of damage to your hearing. 
Use earplugs when you re listening to live music: They can reduce average sound levels by between 15 and 35 decibels. They re widely available at many live music venues and shouldn t spoil your enjoyment of the music.
Don't put up with work noise: If you re experiencing noise at work, talk to your human resources (HR) department or your manager and ask for advice on reducing the noise and getting hearing protection.
Wear ear protectors: Wear ear protectors (earplugs or earmuffs) if you are using noisy equipment such as power drills, saws, sanders or lawn mowers.
Be careful in the car: Listening to music in a confined space increases the risk of hearing damage. Don t listen to music too loud for too long.
Have a hearing detox: Give your ears time to recover after they ve been exposed to loud noise. According to Action on Hearing Loss, you need at least 16 hours of rest for your ears to recover after spending around two hours in 100dB sound, for example in a club. Reducing this recovery time increases the risk of permanent deafness.

Your Headphones Can Turn You Deaf!! 

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

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?

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!

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