Wednesday, February 18, 2009

Sources

"Hereditary Hearing Loss Test." Asper Biotech. 4 Aug. 2008. Asper Ophthalmics. 18 Feb. 2009 .

"Quick Statistics." NIDCD. 4 Aug. 2008. USA.gov. 18 Feb. 2009 .

Smith, Richard J, and Guy V. Camp. "Deafness and Hereditary Hearing Loss Overview." Gene Reviews. 2 Dec. 2008. 18 Feb. 2009 .

Lippincott Williams & Wilkins. "Hearing Loss – Congenital." WD. 2008. 18 Feb. 2009 .

"Organizations and Associations." Patient & Family Resource Guide. 19 Feb. 2009 .

How possible is it that cure will be found?

There is no cure for hereditary deafness right now; there are only treatments, such as hearing aids, perscription medications, etc. Scientists are currently working on finding some type of cure for hearing loss or to make hearing a bit better.

What are some organizations that can help a family cope with a child's disorder?

Some organizations that can help families with deafened members include:

Association of Late-Deafened Adults: http://www.alda.org/
This association is comprised of individuals who became deaf after the time of development of speech and language. The web site provides resources, information and promotes advocacy and awareness of the needs of deafened adults. It offers include a list of regional chapters and self-help support groups, an email board and discussion lists, volunteer opportunities, conference information and more.

The Hearing, Speech-Language and Learning Center at Beth Israel Medical Center: http://www.wehealnewyork.org/services/hearing/services/
The Beth Israel Medical Center web site 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
Alexander Graham Bell is an international membership organization of parents of children who are deaf and hard of hearing, adults with hearing loss and professionals. This web site 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
ASDC is a national, non-profit membership organization providing support, encouragement, and information to families raising children who are deaf or hard of hearing. ASDC's primary mission is to advocate for the highest quality programs and services for parents so they can make informed choices about their children’s educational, communication, personal and social needs.


What limitations does the person have?

A person diagnosed with deafness is only limited by their hearing. Their other senses will react to things as others' can. 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, whether it's just a walk in the park or if the deafened person has a medical emergency.

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 deafened person can experience different things along the lines of doing rather than hearing.

How can the disease be treated?

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

What is the life expectancy of someone with the disease?

A person can live a normal, healthy life while having hereditary deafness. They just won't be able to hear as well as others, but are definitely capable of doing anything anyone else can.

What are the physical symptoms of the disease?

Abnormalities to the external ear and damage of the eighth cranial nerve, auditory brain stem, or cerebral cortex are physical symptoms to hereditary deafness or just hearing loss.

How is the disease diagnosed?

Otologic, audiologic, and physical examination, family history, ancillary testing, and molecular genetic testing are used as well. Molecular genetic tests are available for many types of syndromic and nonsyndromic deafness. Testing for deafness-causing mutations plays a role in diagnosis and genetic conseling.

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.

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.

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 prevalent is the disease in the population?

Hereditary deafness is very common throughout the country. Most people would expect this disease to usually affect older people because age can come into consideration with this, but hereditary deafness affects every age.

  • Of adults ages 65 and older in the United States, 12.3 percent of men and nearly 14 percent of women are affected by tinnitus.
  • More than 4,000 infants born deaf each year, more than half have a hereditary disorder. However, not all hereditary hearing loss is present at birth. Some infants may inherit the tendency to acquire hearing loss later in life.
  • Approximately 17 percent (36 million) of American adults report some degree of hearing loss.
  • There is a strong relationship between age and reported hearing loss: 18 percent of American adults 45-64 years old, 30 percent of adults 65-74 years old, and 47 percent of adults 75 years old or older have a hearing impairment.
  • About 2 to 3 out of every 1,000 children in the United States are born deaf or hard-of-hearing. Nine out of every 10 children who are born deaf are born to parents who can hear.

If this disease is genetic--what are the possible genotypes of the parents? If the disease is a chromosomal abnormality, describe the abnormality.

Thousands of infants are born deaf each year, over half having a hereditary disorder. Neither parent may not have the gene for deafness at all and the child could still be born with a mutated gene for hearing. If one parent did have the faulty gene, you could look back at their family history of hereditary deafness and be able to determine their genotype, as well as with the other parent. This abnormality in a person of any age is associated with the malformation of the certain gene that deals with hearing and will affect them for a lifetime.

How does a person inherit this genetic disorder? Is it dominant or recessive? Is it genetic or chromosomal?

Hereditary deafness is inherited by a person's parent, or parents, based on their genes. In the chromosomes, just as a person's hair or eye color is identified, a person's hearing balance can be passed on, dominant or recessive, syndromic or nonsyndromic. Syndromic being associated with certain hearing traits as well as hearing impairment. Nonsyndromic being hearing loss as its only impairment. Dominant transmission of deafness needs only one faulty gene, from either parent to cause the hearing loss, however recessive transmission of deafness requires a faulty gene from both the mother and father.

What is a Genetic Counselor?

A genetic counselor studies different types of diseases and how they are inherited in families. A genetic counselor can help and inform family members about diseases, or a specific disease, and can identify who can be at risk in the future based on a family's history.