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 loss is not something limited to elderly men or elderly women only. This is something that can affect any adult at any stage of life. The good news is that it can be managed. Today, there are a number of hearing aids that can help improve hearing ability. These aids allow an individual who has lost part of his or her hearing to live a normal life. For best results, hearing loss should be arrested in its early stages.

Here are a few signs you should look out for.

Muffled Speech: When you hear people talk, their speech should be clearly identifiable. If you hear muffled noises that you have to interpret as words, it could be a sign of hearing loss.
Difficulty understanding words: The human ear has the ability to separate conversational sounds from ambient noise. However, in some cases where the inner ear is damaged, a person might find it difficult to separate the two. In such cases, the person might not be able to understand words clearly as they get confused with background noise. This is a sign that must never be taken lightly.
Feeling the need to ask people to repeat themselves: Once in a while if you find yourself asking another person to repeat what he or she said, there is nothing to worry. However, if this becomes a regular occurrence and happens with many people, then the fault lies in your hearing. Thus, if you notice yourself needing to ask people to repeat themselves or talk slowly, consult a hearing specialist.
Increasing the volume of the radio or television: As children, we are taught that playing music or watching TV at a very high volume can damage the ears. The radio, television and other such appliances should be played at a volume where the information being relayed is clearly audible and people can have a conversation alongside without needing to shout. If you find yourself having to increase the volume constantly, it could be a sign of hearing loss.
Withdrawing from conversations: As hearing loss is aggravated, people begin to feel conscious of having to ask others around them to repeat themselves or talk slowly. This can make them withdraw from a conversation and other social situations. In many cases, the simple reason for avoiding a party could be the discomfort of not being able to hear the other person clearly in a conversation.
 All hearing problems can be better handled by proper investigation, different test, sign and symptoms of the patient, which are carried by a specialist of hearing, called as AUDIOLOGIST, who can look your hearing problems, plan out treatment plan, rehabilitation pre and post etc. Audiologist will definitely help you hear again. 

Common Signs Of Hearing Problems In Adults!

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!! 

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 

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

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

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