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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.
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.
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. If you wish to discuss about any specific problem, you can consult an Ent Specialist.
Why is my voice so hoarse? .I am suffering to some hearing problem.in my ear canal it is a small whole .what should I do?
My mother is a 46 year old. Recently she consulted a ENT doctor because she was hearing a buzzing noise in her ear. The doctor said, there is a hole in the ear and hence needs to be covered with a surgery. Could you please advice if this can be cured 100% by surgery? Also, why would any one get holes in their ears? My mom is a housewife and her daily life is pretty similar to that of an average Indian house-wife's.
My husband says that if he can sing songs and recite dialongs /songs there should not be a problem with the hearing. Can you please explain the science behind it so that I can explain the same.
I am 40 yrs old male suffering from loss of hearing (hearing loss due to crackers noise from oct' 2014) question is which type of medication or medicines I should use?
Daily I have to talk and listen to my clients at my office. I use my voice a lot. But at the end of the day I feel pain in my throat. I hydrate throughout the day. Pls tell me what to do to improve my voice box and not to pain. I don't want to take medicine. Visited Dr. many times but didn't see much improvement. Thanks.
-does not responds to sound by making sounds, or by looking at the speaker.
- isn't using gestures, such as bye-bye & does not stops activity in response to'no.
- does not point to five body parts and has trouble imitating tow word sentences
- does not follow a serious two simple but related commands.
- about three quaters of the child's speech at 3 years and by 4 years old, a child should mostly be understood even by people who don't know the child.
If your answer is" yes, consult a speech language pathologist