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About Conductive deafness-malformed external ear syndrome

What is Conductive deafness-malformed external ear syndrome?

Conductive deafness-malformed external ear syndrome is a rare genetic disorder characterized by hearing loss due to malformation of the external ear. It is caused by a mutation in the GJB2 gene, which is responsible for the production of a protein called connexin 26. This protein is essential for the proper functioning of the inner ear and is responsible for the transmission of sound waves to the brain. People with this disorder typically have a malformed external ear, which can lead to hearing loss. Treatment typically involves the use of hearing aids or cochlear implants.

What are the symptoms of Conductive deafness-malformed external ear syndrome?

The symptoms of Conductive deafness-malformed external ear syndrome include:

-Hearing loss
-Abnormalities of the external ear, such as a malformed or absent ear canal, a malformed or absent ear drum, or a malformed or absent middle ear
-Frequent ear infections
-Tinnitus (ringing in the ears)
-Balance problems
-Speech and language delays

What are the causes of Conductive deafness-malformed external ear syndrome?

1. Abnormal development of the outer ear, including malformation of the ear canal, ear drum, or middle ear bones.

2. Infections of the ear, such as otitis media, which can cause inflammation and scarring of the ear canal.

3. Exposure to loud noises, which can damage the delicate structures of the ear.

4. Blockage of the ear canal due to wax buildup, foreign objects, or fluid.

5. Certain medications, such as certain antibiotics, that can cause hearing loss.

6. Genetic factors, such as a family history of hearing loss.

7. Diseases such as meningitis, measles, mumps, or rubella, which can cause hearing loss.

8. Head trauma or injury, which can damage the delicate structures of the ear.

What are the treatments for Conductive deafness-malformed external ear syndrome?

1. Surgery: Surgery is the most common treatment for conductive deafness-malformed external ear syndrome. This may involve reconstructing the external ear, repairing the eardrum, or inserting a prosthetic device to improve hearing.

2. Hearing Aids: Hearing aids can be used to amplify sound and help improve hearing.

3. Cochlear Implants: Cochlear implants are electronic devices that are surgically implanted in the inner ear to help improve hearing.

4. Assistive Listening Devices: Assistive listening devices, such as FM systems, can be used to amplify sound and help improve hearing.

5. Speech Therapy: Speech therapy can help improve communication skills and help the person learn to use their hearing more effectively.

What are the risk factors for Conductive deafness-malformed external ear syndrome?

1. Genetic predisposition
2. Exposure to loud noises
3. Infections of the middle ear
4. Trauma to the ear
5. Certain medications
6. Age-related hearing loss
7. Congenital malformations of the ear
8. Otosclerosis
9. Meniere's disease
10. Acoustic neuroma

Is there a cure/medications for Conductive deafness-malformed external ear syndrome?

There is no cure for Conductive deafness-malformed external ear syndrome, but there are treatments available to help manage the condition. These treatments include hearing aids, cochlear implants, and speech therapy. Medications may also be prescribed to help manage any associated symptoms, such as pain or infection.