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!

A deaf child is the one who suffers from hearing loss. Children with deafness may also fail to develop speech. Such children might have no defect in their speech but they cannot speak anything. The main reason is deafness. They do not hear a speech, therefore, cannot develop it. Deafness may not be hereditary, but it occurs due to several other causes. 

The period of 5 years is crucial in a child s life. During this period, they develop their speech and language. Therefore, parents need to take the utmost care for the early identification of hearing loss and treat the child accordingly. 

Causes of Deafness in Children

There are several causes of deafness in children.

Otitis Media: In young children, if the eustachian tube is not fully formed, it forms fluid behind the eardrum. This fluid can get infected and affect the hearing. The fluid may not cause any pain or infection but still, it can harm and cause hearing loss even if it stays for a short time. 

Problems by Birth: Some children have hearing problems by birth. Sometimes, the problem lies in the genes. But, other times during pregnancy or prenatal care, the problem arises. If a mother, while pregnancy, has diabetes or preeclampsia, she is likely to deliver a deaf child. A prematurely born baby also has a high risk of deafness.

Illness or Injury: Some young children suffer hearing loss due to some injury in the ear or some other illness. Illness includes meningitis, measles, chickenpox, and flu. Head injury or listening to loud music for a long time can also be a reason.

Symptoms of a Deaf Child

A deaf child shows no reaction to the loud noises.

He/she doesn t respond to the voices.

The sound of the child is simple that taper off.

The child may pull or rub the ear several times, which is noticeable.

The child is continuously cranky for no particular reason.

He/she does not pay any attention when anyone is talking.

He/she has very little energy to do anything. 

He/she fails to understand the direction.

Often asks to raise the volume of TV or radio.

Have fever and ear pain.

Treatment of the Problem

Hearing loss affects a child s learning ability. So, the problem must be diagnosed and treated immediately. Here are some treatment approaches: 

Treatment for Otitis Media: The very first thing parents and doctors can do is watchful waiting because sometimes the problem cures itself. Some pediatricians might prescribe some antibiotics. If the problems continue, then the doctor will suggest getting an ear tube to the child that will allow the fluid to drain. For this, the child will have to visit an ENT doctor also called Otolaryngologist. 

Treatment for other causes: The child may use a hearing aid. Some can get cochlear implants. 

Conclusion
It is necessary for parents to accept the child with deformity and stay by his/her side. They must get themselves educated and communicate the problem with others to get help. It is essential to stay in touch with the child and take care of him.

Deaf Child - What Should You Know?

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

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!

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

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!

Don't wait any longer, manage your Audiometry Test online with a Lybrate doctor today

Consult for Audiometry Test - Online and Easy!

FilterClear All

COMMON SPECIALITIES

COMMON ISSUES