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!

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?

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!

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 

While most noise is just in the background for us, loud noises, either in small spurts or prolonged exposure, can cause hearing loss. This has been happening more frequently as the levels of noise in an industrialized society go further up.

Noise induced hearing loss explained
Loud noises may damage the sensitive structure of your ear, which process the sound waves into information. They eventually become nonfunctional, resulting in a loss of hearing in that range. If exposure to loud noises continues, then this might extend to the entire range of hearing and may cause total hearing loss.

What may cause noise induced hearing loss?
Some of the scenarios which may cause noise induced hearing loss are as follows:
1. Listening to music on high volume: If you constantly listen to music or watch movies at your home on very loud levels, then it may result in noise induced hearing loss. In fact, listening to music or watching movies with headphones in your ears is one of the leading causes for hearing loss. Headphones have deceptively loud levels and may result in damaged hearing overtime.

2. Noise of household tools: Household tools such as drills, mixers, grinders, mowers, and saws can expose you to loud levels of noise. Even a quick exposure for a few seconds could temporarily damage your hearing. 

3. Occupational problems: Occupations such as construction, factory work and military that require the use of power tools on a constant basis can also cause hearing loss. The sound from the resulting friction might damage your hearing very quickly. Explosions and impacts are also causes of hearing loss in the military and law enforcement departments.

Prevention and treatment
Treatment: It is difficult to treat hearing loss as in most cases; it may be permanent, except sudden shocks, when your hearing might come back to normal. The only treatments for hearing loss are hearing aids or cochlear implants which can restore hearing partially.

Prevention: Prevention is the best method to avoid hearing loss and in most cases, you should wear ear protection such as mufflers or sound level reduction devices to protect your hearing. Also, practice listening to music, watching TV or movies at lower levels, especially if you are on the headphones. 

Induced Hearing Loss - Facts You Should Be Careful about It!

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

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