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International Symbol for Deafness

Deafness is a physical condition characterized by lack of sensitivity to sound.

Merikartano's school for deaf people in Oulu

The word "deaf"

The word deaf is used differently in different contexts, and there is some controversy over its meaning and implications.

Scientific usage

In scientific and medical terms, deafness generally refers to a physical condition characterized by lack of sensitivity to sound. Notated as deaf with a lowercase d, this refers to the audiological experience of someone who is partially or wholly lacking hearing.[1] In legal terms, deafness is defined by degree of hearing loss. These degrees include profound or total deafness (90 dB - 120 dB or more of hearing loss), severe (60 dB - 90 dB), moderate (30 dB - 60 dB), and mild deafness (10 dB - 30 dB of hearing loss). Both severe and moderate deafness can be referred to as partial deafness or as hard of hearing, while mild deafness is usually called hard of hearing.

Cultural usage

Within the Deaf community, the term Deaf is often capitalized when written, and it refers to a tight-knit cultural group of people whose primary language is signed, and who practice social and cultural norms which are distinct from those of the surrounding hearing community. This community does not automatically include all those who are clinically or legally deaf, nor does it exclude every hearing person. According to Baker and Padden, it includes any person or persons who "identifies him/herself as a member of the Deaf community, and other members accept that person as a part of the community."[2]

Demographics

The global deaf population is roughly estimated to be 0.1% of the total population (1 in 1000).[3] The figure is likely to be higher in developing countries than developed countries due to restricted access to health care, and, in some cultures, due to the high rate of intrafamilial marriages. The great majority of people with less than average hearing are elderly or developed hearing loss after leaving school.[4] According to the U.S. National Center for Health statistics, approximately three quarters of deaf and hard-of-hearing Americans experienced the onset of hearing loss after age 18.[4]

Most deaf people, at least in developed countries, have some knowledge of the dominant language of their country. This may include the ability to lip read, to speak, or to read and write. Having some knowledge of both the dominant language and sign language is called bimodal bilingualism.

Categories of deafness

Deafness can be classfied according to type, severity, and age of onset.[5]

Type

Severity

  • Partial – limited hearing loss
  • Profound – complete or near-complete inability to hear

Age of onset

  • Congenital - deafness at birth
  • Pre-lingual – deafness beginning before language is learned
  • Peri-lingual – deafness beginning while in the midst of learning a first language
  • Post-lingual – beginning after a language has been learned

Other classifications

  • Progressive – hearing loss which increases over time
  • Tone deaf – inability to distinguish between relative pitch (in music)
  • Tinnitus – hearing damage characterized by a high pitched ringing in the ears which drowns out other sounds
  • Unilateral – loss of hearing in one ear only

Causes of deafness

Genetic disorders

Genetic disorders are responsible for at least half of the cases of congenital deafness, with the other half caused by environmental factors.[6] Approximately 70% of genetic deafness is nonsyndromic deafness, meaning that the hearing loss is isolated and not associated with other physical features. The remaining 30% of genetic deafness is caused by a genetic syndrome.[7]

Non-syndromic

The majority (~80%) of nonsyndromic deafness is autosomal recessive, approximately 20% is autosomal dominant, about 1% is X-linked, and less than 1% is mitochondrial.[6][7]

  • GJB2 - Changes in the GJB2 gene are the most common genetic cause of hearing loss, accounting for approximately one third of all genetic hearing loss.[8] This gene encodes the Connexin 26 protein. Changes in GJB2 typically cause congenital, mild to profound hearing loss which is inherited in an autosomal recessive manner.[9]
  • GJB6 - This gene encodes the Connexin 30 protein. Individuals with a change in one of their GJB6 genes and one of their GJB2 genes will have hearing loss.[9]

Syndromic deafness

Deafness may be associated with any of the following genetic syndromes:

Environmental causes

Congenital infections

Congenital infections that can cause deafness include:[10][11][12]

Postnatal infections

Postnatal infections that can cause deafness include:

Physical trauma

Types of physical trauma that can cause deafness include:

Toxic

Exposure to many toxic substances can cause deafness, such as:

Occupational

Occupational hazards can cause deafness. Any occupation with exposure to loud noises on a continuous day-to-day basis can result in hearing loss due to nerve end damage. Increased attention to conditions in the work environment has markedly decreased the likelihood of work-related hearing loss. See Noise-induced hearing loss.

Other causes

Other causes of deafness include

Temporary hearing loss

Temporary hearing loss can be caused by:

  • The build-up of wax in the ear canal
  • Foreign body lodged in the ear canal
  • Injury to the head
  • Allergy
  • Blocked Eustachian tubes
  • Scarred or perforated eardrum
  • Ear infections (chronic otitis externa, chronic otitis media, malignant otitis externa)
  • Reaction to medication such as aminoglycosides, chloroquine, quinidine

Deaf identity and culture

Within deaf culture, it is asserted that the label is one of identity, not audiological status. It is seen by them as akin to an ethnic division. It describes shared experiences in the world, not only those directly related to sight and sound (the increased awareness of one over the other) but also the cultural experiences that often inevitably follow from that. The term deaf then, used by many of those who are within the category, has little to do with an ability or inability to hear. Because of all this, and many other sociological forces, you will find some who identify themselves as deaf with much more ability to hear than many who self-identify as hearing or hard of hearing. In print, you can sometimes ascertain that the word is being used to reference the cultural identification because many people now capitalize the word when using it as a cultural label.

People who are part of Deaf culture typically use a sign language (such as American Sign Language) as their primary language and often emphatically see themselves as not disabled, but rather as members of a cultural or language minority.[1] Members of this group use Deaf as a label of cultural identity much more than as an expression of hearing status. Hearing or hard of hearing people may also be considered culturally Deaf if they participate in Deaf culture and share Deaf cultural values; this is sometimes referred as 'attitudinal deafness'.[2]

There are differing opinions over which terms should be used to describe deaf people. Many deaf people find the term hearing-impaired to be outdated and highly offensive, and express frustration that hearing people believe the term to be polite. Many deaf people often prefer to be called either deaf or hard of hearing.[15]

Children of deaf adults

Children of deaf adults (CODAs) with normal hearing ability may consider themselves, and be considered, culturally Deaf or as members of the Deaf community. In some cases they may need speech therapy due to limited exposure to spoken language. An organization, also called CODA, was established in 1983 and now holds annual conferences. There are also support groups for Deaf parents who may be concerned about raising their hearing children, as well as support groups for adult CODAs.

There are also several camps established for CODAs, such as the one at Camp Mark Seven which hosts two separate 2-week programs for CODAs, one from age 9 to 12 and one for CODAs from age 13 to 16 and it usually occurs during the summer, from the last week of June to mid-August.

Notable children of deaf adults

  • Alexander Graham Bell; both his mother and his wife, Mabel Hubbard, were deaf.
  • Edward Miner Gallaudet, founder of Gallaudet University, the world's only university for deaf and hard of hearing students. He is the son of Sophia Fowler Gallaudet and Thomas Hopkins Gallaudet, founder of the American School for the Deaf, the first school for the deaf in the U.S.
  • Homer Thornberry, a United States Representative from the 10th congressional district of Texas from 1948 to 1963.
  • Keith Wann, with two other Deaf actors, perform regularly in a troupe called Iceworm, to showcase the cultural and linguistic barrier faced between the deaf and hearing worlds in a comedic fashion.
  • Lon Chaney, Sr., American actor raised by deaf parents, whose upbringing allowed him to better communicate in silent film.
  • Louise Fletcher, American, Academy Award, Best Actress for One Flew Over the Cuckoo's Nest. In 1975, when Fletcher won the Academy Award for Best Actress, she spoke and signed her acceptance speech for the benefit of her deaf parents.
  • Richard Griffiths, English actor.
  • Stefan LeFors, Canadian football quarterback for the Edmonton Eskimos.

Terminology

Deaf vs. hard of hearing vs. hearing-impaired

Deaf generally implies a profound loss of hearing; someone with a partial loss of hearing is more likely to be referred to as hard of hearing or the qualified partially legally deaf. People with varying degrees of hearing loss have also been referred to as hearing-impaired.

The term hard of hearing may be used to describe all degrees of hearing loss up to and including total deafness. It is more likely to be used by individuals who have lost their hearing as an adult, than by those who are born deaf or lose in as an infant. In the case of profound deafness this may be political correctness, a euphemism for the simpler and accurate "deaf." Interestingly, this is seen as a euphemism only from the side of the mainstream. The Deaf community does not generally aspire to be hearing and sees the hard of hearing label as an indication of a mindset that views them pathologically.

Total deafness is quite rare. Most deaf people can hear a little.[16] However, since hearing loss is generally frequency-based rather than amplitude-based, a deaf person's hearing may not be usable, if the normal frequencies of speech lie in the impaired range.

People with a moderate hearing loss, of about 36–50 dB,[17] generally describe themselves as "partially deaf." Others who were born hearing, but who have partially lost their hearing through illness or injury are "deafened." Those with a slight hearing loss (eg. about 16–35 dB hearing loss),[17] or have lost some of their hearing in old age may prefer an informal term such as "hard of hearing" or "hearing-impaired".

Those with some functional hearing generally do not take part in the Deaf community, and typically work and socialize with hearing people to the best of their ability. People with all degrees of hearing impairment may encounter discrimination when looking for work, while at their jobs, or when socializing with hearing people.

Other meanings of 'deaf'

Deaf is also used as a colloquialism to refer to a recalcitrant individual or someone unwilling to listen, obey or acknowledge an authority or partner. The third line of Shakespeare's Sonnet 29 provides an example:

When in disgrace with fortune and men's eyes

I all alone beweep my outcast state,

And trouble deaf heaven with my bootless cries,

See also

References

  1. ^ a b Ladd, Paddy (2003). Understanding Deaf Culture: In Search of Deafhood.
  2. ^ a b Baker, Charlotte (1978). American Sign Language: A Look at Its Story, Structure and Community. {{cite book}}: Unknown parameter |coauthors= ignored (|author= suggested) (help)
  3. ^ Harrington, Tom (2004-07-01). "Deaf Statistics: Other Countries". Frequently Asked Questions: Deaf Statistics. Retrieved 2006-10-13. {{cite web}}: Check date values in: |date= (help)
  4. ^ a b Holt, Judith (1994). "DEMOGRAPHIC ASPECTS OF HEARING IMPAIRMENT: QUESTIONS AND ANSWERS". DEMOGRAPHIC ASPECTS OF HEARING IMPAIRMENT. Retrieved 2006-10-13. {{cite web}}: line feed character in |title= at position 43 (help)
  5. ^ "Type, Degree, and Configuration of Hearing Loss". American Speech-Language-Hearing Association. Retrieved December 18, 2007.
  6. ^ a b American College of Medical Genetics (May/June 2002). "Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss". Genetics in Medicine. 4 (3): 162–171. {{cite journal}}: Check date values in: |date= (help)
  7. ^ a b Smith, Richard JH; Van Camp, Guy (Jan 30, 2007), "Deafness and Hereditary Hearing Loss Overview", Gene Reviews
  8. ^ "Causes of Hearing Loss, Basic Genetics". National Institute on Deafness and Other Communication Disorders. Retrieved December 18, 2007.
  9. ^ a b Smith, Richard JH; Van Camp, Guy (Dec 21, 2005), "Nonsyndromic Hearing Loss and Deafness, DFNB1", Gene Reviews
  10. ^ Frangulov, Anna (2004). Common Causes of Hearing Loss (PDF). Harvard Medical School Center for Hereditary Deafness. {{cite book}}: Unknown parameter |coauthors= ignored (|author= suggested) (help)
  11. ^ "Causes of Hearing Loss, Nongenetic Hearing Loss". National Institute on Deafness and Other Communication Disorders. Retrieved December 17, 2007.
  12. ^ "Hearing Impairment". March of Dimes. August, 2007. {{cite web}}: Check date values in: |date= (help)
  13. ^ Berke, Jamie (Dec 2007). "Cause of Hearing Loss - Meningitis \ publisher = About.com". {{cite web}}: Missing pipe in: |title= (help)
  14. ^ "Ear Infections: Facts for Parents About Otitis Media". National Institute on Deafness and Other Communication Disorders. Feb 2002.
  15. ^ Deaflinx, "Identity"
  16. ^ Gallaudet University: Demographics of Deafness
  17. ^ a b Description of Degree of Hearing Loss Versus Potential Effects, HandsAndVoices.org

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