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Newborn screening (NBS) is a public health program of screening in infants shortly after birth for conditions that are treatable, but not clinically evident in the newborn period. The goal is to identify infants at risk for these conditions early enough to confirm the diagnosis and provide intervention that will alter the clinical course of the disease and prevent or ameliorate the clinical manifestations. NBS started with the discovery that the amino acid disorder phenylketonuria (PKU) could be treated by dietary adjustment, and that early intervention was required for the best outcome. Infants with PKU appear normal at birth, but are unable to metabolize the essential amino acid phenylalanine, resulting in irreversible intellectual disability. In the 1960s, Robert Guthrie developed a simple method using a bacterial inhibition assay that could detect high levels of phenylalanine in blood shortly after a baby was born. Guthrie also pioneered the collection of blood on filter paper which could be easily transported, recognizing the need for a simple system if the screening was going to be done on a large scale. Newborn screening around the world is still done using similar filter paper. NBS was first introduced as a public health program in the United States in the early 1960s, and has expanded to countries around the world.
Audiology is a branch of science that studies hearing, balance, and related disorders. Audiologists treat those with hearing loss and proactively prevent related damage. By employing various testing strategies, audiologists aim to determine whether someone has normal sensitivity to sounds. If hearing loss is identified, audiologists determine which portions of hearing are affected, to what degree, and where the lesion causing the hearing loss is found. If an audiologist determines that a hearing loss or vestibular abnormality is present, they will provide recommendations for interventions or rehabilitation.
An otoacoustic emission (OAE) is a sound that is generated from within the inner ear. Having been predicted by Austrian astrophysicist Thomas Gold in 1948, its existence was first demonstrated experimentally by British physicist David Kemp in 1978, and otoacoustic emissions have since been shown to arise through a number of different cellular and mechanical causes within the inner ear. Studies have shown that OAEs disappear after the inner ear has been damaged, so OAEs are often used in the laboratory and the clinic as a measure of inner ear health.
Audiometry is a branch of audiology and the science of measuring hearing acuity for variations in sound intensity and pitch and for tonal purity, involving thresholds and differing frequencies. Typically, audiometric tests determine a subject's hearing levels with the help of an audiometer, but may also measure ability to discriminate between different sound intensities, recognize pitch, or distinguish speech from background noise. Acoustic reflex and otoacoustic emissions may also be measured. Results of audiometric tests are used to diagnose hearing loss or diseases of the ear, and often make use of an audiogram.
Auditory neuropathy (AN) is a hearing disorder in which the outer hair cells of the cochlea are present and functional, but sound information is not transmitted sufficiently by the auditory nerve to the brain. Hearing loss with AN can range from normal hearing sensitivity to profound hearing loss.
Early childhood intervention (ECI) is a support and educational system for very young children who have been victims of, or who are at high risk for child abuse and/or neglect as well as children who have developmental delays or disabilities. Some states and regions have chosen to focus these services on children with developmental disabilities or delays, but Early Childhood Intervention is not limited to children with these disabilities.
The auditory brainstem response (ABR), also called brainstem evoked response audiometry (BERA), is an auditory evoked potential extracted from ongoing electrical activity in the brain and recorded via electrodes placed on the scalp. The measured recording is a series of six to seven vertex positive waves of which I through V are evaluated. These waves, labeled with Roman numerals in Jewett and Williston convention, occur in the first 10 milliseconds after onset of an auditory stimulus. The ABR is considered an exogenous response because it is dependent upon external factors.
Auditory neuropathy spectrum disorder (ANSD) is a specific form of hearing loss defined by the presence of normal or near-normal otoacoustic emissions (OAEs) but the absence of normal middle ear reflexes and severely abnormal or completely absent auditory brainstem response (ABRs).
Universal neonatal hearing screening (UNHS), which is part of early hearing detection and intervention (EHDI) programmes, refer to those services aimed at screening hearing of all newborns, regardless of the presence of a risk factor for hearing loss. UNHS is the first step in the EHDI program which indicates whether a newborn requires further audiological assessment to determine the presence or absence of permanent hearing loss. Newborn hearing screening uses objective testing methods to screen the hearing of all newborns in a particular target region, regardless of the presence or absence of risk factors. Even among developed countries, until the 1990s, it could take years for hearing-impaired child to be diagnosed and to benefit from a health intervention and amplification. This delay still can happen in developing countries. If children are not exposed to sounds and language during their first years of life because of a hearing loss, they will have difficulty in developing spoken or signed language; cognitive development and social skills could also be affected. This screening separates children into two groups—those with a high index of suspicion and those with a low index of suspicion. Those in the first group are referred for diagnostic testing.
Hands & Voices is a non-profit organization based in Colorado, with chapters worldwide, which offers support and advocacy for the families of children who are deaf or hard of hearing. Services include peer support, resource guides, and advocacy for Early Hearing Detection and Intervention (EHDI).
The Congressional Hearing Health Caucus (Caucus), a caucus of the United States Congress, was created in 2001 in cooperation with the National Campaign for Hearing Health, a public education and advocacy project run by the Deafness Research Foundation The focus of the Congressional Hearing Health Caucus includes several aims that promote hearing health and encourage universal newborn hearing health screenings for all Americans. Those most at risk for hearing-related concerns are newborns, infants, and the elderly, particularly if such issues are left undetected. Therefore, a primary goal of the Congressional Hearing Health Caucus is to see that auditory abilities of all Americans are screened, including universal hearing screening for newborns. One of the co-founders and co-chairmen of the Caucus was former Congress Member James T. Walsh (R-NY). The Director of the National Campaign for Hearing Health at the time of launch of the CHHC, was Elizabeth Thorp, who had herself been born with unilateral deafness not discovered until she was eight years old.
Beth S. Benedict is a professor in the Department of Communication Studies at Gallaudet University, advocate for the deaf, and a mentor for families with deaf children. Her research focuses on early intervention, early language acquisition, and family involvement. Benedict is also an advocate for the use of bilingualism in education of the deaf - incorporating the value of American Sign Language in deaf children. Benedict advocates for deaf-hearing partnerships, avoiding audism, the importance of bilingual education, deaf culture and the use of sign language while also working as a family mentor for families with deaf children. Recently, she was a keynote speaker for an International Deaf Studies conference and the featured speaker for the deaf education summit. Benedict takes what she researches about deafness and education and shares it broadly by way of talks and application - for example, she has helped the Georgia School for the Deaf work on developing bilingual education in their programs. In 2015 Benedict was the featured speaker at the Deaf education summit in Louisiana - a conference that brought together practitioners, educators, and parents to discuss local issues surrounding education of deaf children.
An audiologist, according to the American Academy of Audiology, "is a person who, by virtue of academic degree, clinical training, and license to practice and/or professional credential, is uniquely qualified to provide a comprehensive array of professional services related to the prevention of hearing loss and the audiologic identification, assessment, diagnosis, and treatment of persons with impairment of auditory and vestibular function, and to the prevention of impairments associated with them."
Despite having the largest economy in South America or Central America, Brazil is still considered a developing country due to its low gross domestic product, or GDP, per capita, low living standards, high infant mortality rate and other factors. "With regard to hearing health, the Brazilian government established the national policy for giving attention to hearing health in 2004, in which the Ministry of Health, considering the social magnitude of hearing impairment in the Brazilian population and its consequences, presented the proposal to structure a network of services set up by regions and in hierarchy that aims to be implemented in all federative units of Brazil, with integrated actions to promote ear health, hearing impairment prevention, treatment and rehabilitation organized and managed by the National Health System, Sistema Único de Saúde (SUS), in Portuguese," writes Bevilacqua et al. (2010)
Audiology and hearing health professionals in India is made up of Indian healthcare professional that focus on audiological and hearing problems.
Canada is the largest country located in North America and is home to around 357,000 deaf and 3.21 million hard-of-hearing people. The country can be split into Francophone and Anglophone regions, and has both French and English as official languages. The majority of Canada is considered Anglophone and the province of Quebec along with small parts of New Brunswick, Ontario, and Manitoba are primarily French-speaking. The presence of these two main languages and cultures also brings forth different deaf cultures between the two regions. In Francophone regions, the official language used by deaf and hard-of-hearing people is Quebec Sign Language, or Langue des Signes Quebecoise (LSQ).
According to The Deaf Unit Cairo, there are approximately 1.2 million deaf and hard of hearing individuals in Egypt aged five and older. Deafness can be detected in certain cases at birth or throughout childhood in terms of communication delays and detecting language deprivation. The primary language used amongst the deaf population in Egypt is Egyptian Sign Language (ESL) and is widely used throughout the community in many environments such as schools, deaf organizations, etc. Ths article focuses on the many different aspects of Egyptian life and the impacts it has on the deaf community.
South Korea's Deaf population began to come to prominence in recorded history in the late 19th century with the implementation of special education. Since then, they have gained government recognition and legal rights.
Italy is a country located in the south of Europe. The capital city is Rome. There are about 59 million people in Italy, and about 3.5 million Italians have some form of hearing loss. Among them, around 70,000 people are severely deaf. The European Union for the Deaf reports that the majority of the deaf people in Italy use Italian Sign language (LIS). LIS has been an official sign language in Italy since 2021. Italy, among other countries, ratified the Convention on the Rights of Persons with Disabilities (CRPD) and is slowly improving conditions for deaf humans in Italy. Many major organizations in Italy fight for deaf rights and spread awareness to the Italian National Agency for the protection and assistance of the Deaf and Associated Italian Families for the Defense of the Rights of Deaf and Hard of Hearing Individuals (FIADDA). Newborns in Italy also receive universal hearing screenings. Education in Italy is directed towards oralism although sign language is also used. LIS is a stable language and used by 10,000 to 1,000,000,000 users in Italy.
Judy R. Dubno is an American scientist and researcher in the field of audiology. She is a distinguished university professor and director of research in the department of otolaryngology at the Medical University of South Carolina in Charleston. She is recognized for her scientific contributions to the understanding of presbycusis, a condition of hearing loss that occurs gradually for many aging adults. She has been involved in the development and implementation of several new methods for assessing hearing loss, including the Hearing in Noise Test (HINT) and Speech Intelligibility Index (SII). She has won numerous awards for her work, including the Jerger Career Award for Research in Audiology in 2011. She served as President of the Acoustical Society of America from 2014–2015.
References
- ↑ White KR, & Maxon, AB (1995). Universal screening for infant hearing impairment: Simple, beneficial, and presently justified. International Journal of Pediatric Otorhinolaryngology, 32, 201-211.
- ↑ Maxon AB, White KR, Behrens TR, & Vohr BR (1995). Referral rates and cost efficiency in a universal newborn hearing screening program using transient evoked otoacoustic emissions (TEOAE). Journal of American Academy of Audiology, 6, 271-277.
- ↑ White KR, Behrens TR, & Strickland B (1995). Practicality, validity, and cost-efficiency of universal newborn hearing screening using transient evoked otoacoustic emissions. Journal for Children with Communication Disorders, 17(1), 9-14.
- ↑ White, K. R., Vohr, B. R., Maxon, A. B., Behrens, T. R., McPherson, M. G., & Mauk, G. W. (1994). Screening all newborns for hearing loss using transient evoked otoacoustic emissions. International Journal of Pediatric Otorhinolaryngology, 29, 203-217.
- ↑ Maxon, A. B., White, K. R., Vohr, B. R., & Behrens, T. R. (1993). Using transient evoked otoacoustic emissions for neonatal hearing screening. British Journal of Audiology, 27, 149-153.
- ↑ White, K. R., Vohr, B. R., & Behrens, T. R. (1993). Universal newborn hearing screening using transient evoked otoacoustic emissions: Results of the Rhode Island Hearing Assessment Project. Seminars in Hearing, 14(1), 18-29.
- ↑ Johnson, M. J., Maxon, A. B., White, K. R., & Vohr, B. R. (1993). Operating a hospital-based universal newborn hearing screening program using transient evoked otoacoustic emissions. Seminars in Hearing, 14(1), 46-56.
- ↑ Early Identification of Hearing Impairment in Infants and Young Children. NIH Consens Statement Online 1993 Mar 1-3 [2010/11/17];11(1):1-24. http://consensus.nih.gov/1993/1993HearingInfantsChildren092html.htm
- ↑ White KR (1997). Realities, myths, and challenges of newborn hearing screening in the United States. American Journal of Audiology, 4, 90-94.
- ↑ Johnson JL, Kuntz NL, Sia CC J, & White KR (1997). Newborn Hearing Screening in Hawaii. Hawaii Medical Journal, 56, 352-355.
- ↑ White KR, Weirather Y, Behrens TR, & Strickland B (1997). Realities, myths, and challenges of newborn hearing screening in the United States. Central and East European Journal of Otorhinolaryngology and Head and Neck Surgery, 2(2), 74-84.
- ↑ White KR (2003). The current status of EHDI programs in the United States. Mental Retardation and Developmental Disabilities Research Reviews, 9(2), 79-88.
- ↑ White KR, Forsman I, Eichwald J, Munoz K (2010). The evolution of early hearing detection and intervention programs in the United States. Semin Perinatol. 34(2):170-9.
- ↑ White KR (2007) Early Intervention for children with permanent hearing loss: Finishing the EHDI revolution. The Volta Review. 106(3), 237-258.
- ↑ Smith RJ, Bale JF and White KR. (2005). Sensorineural hearing loss in children. Lancet, 365(9462), 879-890.
- ↑ White KR (2004). Early hearing detection and intervention programs: Opportunities for genetic services. American Journal of Medical Genetics, 130A, 29-36.