Hereditary DeafnessNovember 29th 2013By: Briana Fougere

What is hereditary deafness?

Hereditary deafness is a hearing impairment, partial hearing loss or total inability to hear.

Genetic deafness is caused by one or more changed genes. Close to 100 genes are needed to make a normal, properly functioning ear.

Any change or mutations in one of these genes may cause deafness.

Hereditary deafness can be autosomal dominant or recessive.

Medical Information

How does a person inherit it? Is it dominant or recessive?

It is inherited by a person's parent, or parents, based on their genes.

It is passed in the chromosomes

Syndromic deafness is linked to certain hearing traits as well as hearing impairment. It occurs when the trait is dominant.

Nonsyndromic deafness is having hearing loss as its only impairment. It occurs when the trait is recessive.

What are the genotypes of the parents? Is it an abnormality?

Autosomal Dominant:

Dd, DD  Autosomal Recessive:

dd Neither parent may not

have the gene for deafness at all, the child could still be born with a gene that was mutated.

Hereditary deafness is an abnormality.

It is associated with a problem in the gene formation that deals with hearing and will affect the children for a lifetime.

Karyotype

How prevalent is the disease in the population?

Hereditary deafness is very common.

Most people expect this disease to affect older people because age can come into consideration with this, but hereditary deafness affects every age.

Statistics

Adults ages 65+ 12.3 % of men and14% of women are affected by tinnitus. Tinnitus is ringing or buzzing in your ears.

4,000+ infants are born deaf each year, more than half have a hereditary disorder. Not all hereditary hearing loss is present at birth, some infants may inherit the trait leading to hearing loss later in life.

17%(36 million) of adults report some form of hearing loss.

18% of adults 45-64 years old, 30% of adults aged 65-74, and 47% of adults 75+ have a hearing impairment.

About 2-3 out of every 1,000 children are born deaf or hard-of-hearing. 9/10 children who are born deaf are born to parents who can hear

What are the chances of a person with this disease passing the disease to their offspring?

If a person is diagnosed with hereditary deafness, then there is a good chance their child will be as well, but you can't always predict if a child will have this. 

Some examples of this include:

1. A child is born with two parents who can hear very well, but the child is born deaf. The parents had no clue this would happen and wonder how it did.

2. A newborn can be born as deaf or hard-of-hearing, even if the hereditary deafness is far back into the each of the parent's family history or not prevalent at all.

How is the disease diagnosed?

Otology, audiology, and physical examination, family history, ancillary testing, and molecular genetic testing are used to diagnose hereditary deafness.

Molecular genetic tests are available for many types of syndromic and nonsyndromic deafness.

What are the physical symptoms of the disease?

Abnormalities to the external ear and damage of the eighth cranial nerve, auditory brain stem can occur.

Cerebral cortex are physical symptoms to hereditary deafness or just hearing loss.

What is the life expectancy of someone with the disease?•A person with hereditary deafness can live a normal, healthy life. The only difference will be that they won't be able to hear as well as others, but they are definitely capable of doing anything anyone else can.

How can deafness be treated?

• Deafness can be treated with hearing aids, prescription medications, surgery, and some lifestyle changes.

Personal Information

What is everyday life like? What is the quality of life?

Everyday life for a person with hereditary deafness can be the same as a person who can hear, though sign language would help and play a big role during it. A deaf person can experience different things though activities rather than hearing.

What limitations does the person have?

A person diagnosed with deafness is only limited by their hearing.

Communication would be difficult with a deafened person, because to respond, the other person would have to understand sign language to make out what they're saying.

A person would also need some type of assistant to help them, especially if the deaf person has a medical emergency.

What are organizations can help a family cope with their child having hereditary deafness?

Association of Late-Deafened Adults:http://www.alda.org/ -About people who became deaf after they developed speech and language. The site gives information about the needs of deafened adults.

The Hearing, Speech-Language and Learning Center at Beth Israel Medical Center:http://www.wehealnewyork.org/services/hearing/services/ -Describes the services of The Hearing Center, The Cochlear Implant Center, The Speech-Language Center and The Learning Center at Beth Israel Medical Center, New York City.

Alexander Graham Bell Association: www.agbell.org - Provides general hearing loss information, book recommendations, financial aid and scholarships, and action alerts on legislative developments.

American Society for Deaf Children (ASDC):www.deafchildren.org-Provides support, encouragement, and information to families raising children who are deaf or hard of hearing. Gives the highest quality programs and services for parents.

How possible is it that a cure will be found?

There is no cure for hereditary deafness right now.

The treatments they have are, hearing aids, prescription medications, etc.

Scientists are currently working on finding some type of cure for hereditary deafness or to make hearing a bit better.