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!

Using a hearing aid presents a number of advantages to a hearing impaired person. First and foremost, you will hear a lot better. Hearing aids do not restore your hearing to normal but they improve it significantly. It becomes easier to hear what other people say. Sounds you have not heard for a long time such as birds singing, door bell ringing, the howling of the wind and water running may become audible to you.

Hearing aid usually improves the user s social, psychological and physical sense of well being.

Study as well as experience shows that hearing aid generally improve the quality of life and will help you:

Get a better relationship with your family
Feel better about yourself
Get better mental health
Improve your physical well being
To concentrate better
Feel more independent and secure
Feel less tired or exhausted
Be more able to participate in social gathering
Be able to increase your social contact
Be able to do better in your job  
Hearing aid will help you to reduce tinnitus
Hearing aid will help you to avoid dementia by helping you to hear and improve cognitive functioning
As you start hearing through hearing aid, you will start socializing yourself and indirectly improve your quality of life.
Why you need to wear two hearing Aids?

Better understanding of speech
Better understanding in group and even in noisy situation
Better ability to tell the direction of the sound (Localization)
Better sound quality
Greater listening comfort
Hearing is less tiring and more pleasant
Keeping both ears active preserves speech understanding in both ears
Effective tinnitus masking
Better understanding of listening to radio and television
Speech will be clear

Advantages Of A Hearing Aid!

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 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.

Dealing With Hearing Loss

Here are 6 signs to watch out for that could indicate 'You need to get your Ears tested':

1. Your TV is blaring: Do you often find people around you shouting above the TV sound complaining that it's too loud? When you find it hard to hear the TV at the average volume and find yourself turning up the sound, this could signify a hearing loss. Doctors are seeing a growing number of patients visiting them after prolonged exposure to loud music and sounds.

2. You find it hard to hear phone conversations: Do you find yourself asking the person on the other end of the phone line to 'repeat' themselves, or find yourself pressing the phone instrument right into your ear? If you are trying so hard to hear, you might find yourself missing out on bits of the conversation as focussing to clearly hear the conversation is exhausting work. Getting a hearing test might be a good option if this sounds like you.

3. You have trouble hearing in noisy environments: When you are out dining with friends or family at a busy restaurant or accompanying a friend shopping in a busy street, all that background noise makes it difficult to hear what the people are saying. People with hearing loss often have problems masking out background noise.

4. Family members telling 'you're going deaf'?: Trust the views of the people closest to you, they never lie about your health. Family members often are the first to sense signs of hearing loss as they find themselves repeating things to you or calling out louder to get your 'attention'.

5. You find yourself staying away from social occasions, family gatherings or avoid being the first to start conversations

6. You find yourself leaning closer to people to follow the conversation or staring at their lips trying to lip-read what's being said.

6 Signs you Need a Hearing Test

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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