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Comment on the Joint Committee Statement
FROM THE JOINT COMMITTEE ON INFANT HEARING
C o m m e n t on the Joint Committee Statement Enclosed in this issue of the BULLETINis the Joint Committee on Infant Hearing 1994 Position Statement just released for publication. The Joint Committee member organizations that have prepared and approved this position statement include the American Academy of Otolaryngology-Head and Neck Surgery, the American Academy of Audiology, the American Academy of Pediatrics, the American Speech-Language Hearing Association and the Directors of Speech and Hearing Programs in State Health and Welfare Agencies. Here is what you need to knowThe 1994 Position Statement: I. Endorses the goal of universal detection of infants with hearing loss and encourages continuing research and development to improve techniques for detection of and intervention for hearing loss as early as possible. 2. Maintains a role for the indicators (formerly called risk factors] described in the 1990 Position Statement. 3. Identifies indicators associated with delayed or late onset hearing loss and recommends procedures to monitor infants with these indicators. 4. Recognizes the adverse effects of fluctuating conductive hearing loss from persistent or recurring otitis media with effusion [OME] and recommends monitoring infants with OME for hearing loss. 5. Endorses the provision of intervention services in accordance with Part H of the Individuals with Disabilities Education Act [IDEA], Public Law 102-I 15.
~llach year in the United States, about 4,000 children are born profoundly deaf and another 37,000 children are born with milder degrees of hearing impairment that can also affect speech, language and • educational development. Therefore, about 6 of every 1,000 children born will have some degree of hearing impairment (Northern & Hayes 1994). Hearing impairment in children is 20 times more prevalent than other birth defects such as phenylketonuria (PKU), sickle cell anemia and hypothyroidism for which screening is routinely done at birth (Downs 1993). Comparatively, it is less expensive to identify an infant with a hearing loss than to identify an infant with PKU, sickle cell anemia or hypothyroidism. Screening all infants would cost about $9,300 to detect one infant with a hearing loss as compared to $40,000 to detect one infant with PKU (Downs 1993).
Reprinted from the American Academy of Otolaryngology-Head and Neck Surgery BULLETIN 1994;13(12):insert. OTOLARYNGOLHEAD NECKSURG 1995;113:188-90.
188
Why the need for universal detection of infants with hearing loss as early as possible?
There is sufficient evidence that early identification of hearing loss in children and early intervention in the management of hearing loss in these children are essential for normal speech, language and educational development. Since hearing during the first six months of life is important for normal speech and oral language development, identification of infants with hearing loss before age 3 months is desirable. It has also been demonstrated that of all the variables examined, the age at which special education commences has been demonstrated to have the largest effect on development of language and communication skills (Levitt et al). "Healthy People 2000'' is an ambitious plan published by the Department of Health and Human Services in 1990 containing a national strategy for significantly improving the health of the nation over the coming decade, One of the objectives is the recommendation to reduce the average age at which children with significant hearing impairment are identified to no more than 12 months of age by the year 2000. Utilizing the risk factors (now called indicators)
Otolaryngology Head and Neck Surgery Volume 113
Number 3
followed by ABR testing in those infants with one or more risk factors, as recommended by the Joint Committee on Infant Hearing 1990 Position Statement, statewide and regional hearing screening programs failed to detect at least 50% of those children born with significant hearing loss and the ages of identification of hearing loss in children in the United states remained close to 36 months. (Mauk et al 1991) Advances in the technology of objective assessment of hearing have augmented the feasibility Of early detection of hearing loss beyond the rate of detection that could be achieved with use of the risk factors (indicators) alone. New methods are readily available to screen all newborns for hearing loss prior to hospital discharge. (Peters, 1986; Kilney, 1987; Bonfels et al, 1988; Stevens et al, 1989). As various states have implemented universal hearing screening programs using ABR and OAE, there is growing evidence that these objective test methods are sensitive, specific and cost effective (White & Behrens, 1993 and Marlowe 1993). Cognizant of poor results utilizing only the risk factor hearing screening protocol and in recognition of the evolution of present technology, the NIH Consensus Conference on Early Identification of Hearing Loss in Infants and Young Children concluded in March 1993 that all infants should be screen ed for hearing impairment preferably prior to hospital discharge. The screening procedure recommended by the expert panel would involve first screening the hearing of all infants with a test that measures otacoustic emissions (OAE). The panel further recommended that infants who fail OAE screening have additional testing of auditory brainstem responses to confirm the validity of the OAE failure. Those infants who fail ABR testing should have comprehensive hearing evaluation before six months of age. The Joint Committee on Infant Hearing 1994 Position Statement endorses universal detection of infants with hearing loss as early as possible. The Joint Coramittee on Infant Hearing is using the phrase universal hearing "detection" rather than universal hearing "screening" since the term "detection" connotates an outcome rather than a protocol. In keeping with this philosophy, the Joint Committee on Infant Hearing 1994 Position Statement discusses ABR and OAE. Rather than recommending a specific protocol, the Joint Committee on Infant Hearing recommends that each team of
COMMENT ON THE JOINT COMMITTEE STATEMENT t 8 9
health care professionals seeking to develop an infant hearing detection program evaluate and select the technique most suitable for the locale and available resources. The Joint Committee on Infant Hearing 1994 Position Statement preserves a meaningful role for the indicators (formerly called risk factors). The 1994 Position Statement lists those indicators for both neonates and infants and mentions the value of parent and/or care giver concern regarding hearing, speech, language and/or developmental delay as an important indicator for possible hearing loss. The indicators also aid in determining a cause of hearing loss as well as helping to identify those syndromes that can include hearing loss. The 1994 Position Statement adds a third list of indicators for infants who require periodic monitoring of hearing because of the possibility of delayed onset hearing loss. These infants should have their hearing monitored every six months until age 3 years and at appropriate intervals thereafter. Conditions that can be responsible for delayed onset hearing loss include family history of hereditary childhood hearing loss, in utero cytomegalovirus infection, and neurodegenerative disorders such as Neurofibromatosis II. This category of indicators also includes recurrent or persistent otitis media with effusion (OME) and conductive hearing loss as well as other disorders of Eustachian tube function that, if persistent, can affect a child's speech and language development much in the same way that sensorineural hearing loss during early life can affect speech and language development. One of the major weaknesses of many infant hearing screening programs is the significant number of cases with lack of follow-up and lack of established protocols for interventional services once a child is diagnosed as having a hearing loss (Welsh and Slater 1993). The 1994 Position Statement devotes a section to early intervention which must be provided in accordance with the Individuals with Disabilities Education Act (IDEA) Public Law 102-119 (formerly PH-99457). The Joint Committee on Infant Hearing recommends that evaluation and assessment of an infant identified with a hearing loss should be accomplished by a team of specialists including a physician with expertise in the management of early childhood otological disorders, an audiologist with expertise in assessment of infants and young children, a speech-language pathologist, a sign language specialist and/or teacher of the deaf
t90
Otolaryngology Head and Neck Surgery September 1995
COMMENT ON THE JOINT COMMITTEE STATEMENT
as well as the parent/care giver. The parent/care giver should be educated and given all options. Choices in regard to the management of their child's hearing loss and early intervention services, based on the family's preference, should begin by six months of age. As an otolaryngologist-head and neck surgeon, you are a vital part of the team concerned with diagnosis and treatment of children with early onset hearing loss (Marlowe & Epstein 1994). First, it is important that you become an advocate for universal hearing detection of infants with hearing loss in your community and in the hospitals in which you are affiliated. It is your responsibility to educate your professional colleagues, especially the primary care physicians involved with the care of infants and children, and the administrators and other professionals associated with your participating hospitals in regard to the merits of universal newborn and infant hearing screening. You should be active in the establishment and proper functioning of newborn and infant hearing screening programs. You should advocate prompt referral of all newborns and infants who fail hearing screening tests and/or who demonstrate the presence of indicators for hearing loss. You should be an advocate for continuous monitoring of the hearing of children who manifest indicators suggesting delayed or late onset hearing loss. And finally, you should be aware of all options and methods of management of hearing loss, taking into consideration family preference. The successful management of early or delayed onset hearing loss in children depends on early intervention. Hearing aids, for example, when indicated, can be provided for an infant as soon as the diagnosis is made, even within the first ~few months of life. The goal of all concerned should be implementation of the guidelines set forth by the Joint Committee on Infant Hearing 1994 Position Statement in order to achieve the objective of Healthy People 2 0 0 0 - to appropriately diagnose at the earliest possible age and manage as effectively as possible children with hearing loss before one year of age by the year 2000.
Kenneth M Grundfast, MD is former co-chairman
and representative of the Joint Committee on Infant Hearing. Stephen Epstein, MD represents the AAO-HNSF on the Joint Committee on Infant Hearing, REFERENCES
Bonfels, P., Uziel, A. and Pujol, R. Screening for auditory dysfunction in infants by evoked otacoustic emissions.Archives of Otolaryngology-Head and Neck Surge~ 114, 887-890 1988 Consensus Development Conference-Early Identification of Hearing Impairment in Infants and Young Children, National Institute of Health, Office of Medical Applications of Research, Bethesda, MD, March 1-3, 1993 Downs, M.P. Benefits of screening at birth: economic, educational and functional factors. NIH Consensus Development Conference Program and Abstracts, Public Health Service, 63-66 1993 Healthy People 2000. Objective 17.16, 464-465. U.S. Department of Health and Human Services, Public Health Service, DHHS Publications No. (PHS) 91-50213, U.S. Government Printing Office, Washington, D.C. 20402-1990 Joint Committee on Infant Hearing 1990 Position Statement, American Academy of Otolaryngology-Head and Neck Surgery BULLETIN, March 1991 Kilney, P. Algo-1 automated infant hearing screener: preliminary results. Seminars in Hearing 8:133-141, 1987 Levitt, H., McGarr, N.S. and Geffner, D. Development of Language and Communications Skills in Hearing Impaired Children. ASHA Monographs Number 26, Rockville, MD: American Speech Language Hearing Association, October 1987 Marlow, J., Epstein, S. Professional hearing care for infants and children: the future. Hearing Instruments 45:10 Suppl. 2 1994 Marlowe, J. Screening all newborns for hearing impairment in a community hospital. American Journal of Audiology 2:22-25 1993 Mauk, O., White, K., Mortensen, L. and Behrens, T. The effectiveness of screening programs based on high risk characteristics in early identification of hearing impairment. Ear and Hearing 12:312-319 1991 Northern, J.L., Hayes, D. Universal screening for infant hearing impairment: necessary, beneficial and justifiable. Bulletin of the American Academy of Audiology 6:3, 10-13 May-June 1994 Peters, J. An automated infant screener using advanced evoked response technology. Hearing Journal 39:25-30, 1986 Stevens, J, Webb, H., Hutchinson, J., Connell, J., Smith, M. and Buffin, J. Click evoked otacoustic emissions in neonatal screening. Ear and Hearing 11:128-133, 1990 Welsh, R., Slater, S. The state of infant hearing impairment identification programs. ASHA 49:52 April 1993 White, K.R. and Behrens, T.R. (Eds) The Rhode Island Hearing Assessment Project: Implications for Universal Newborn Hearing Screening. Seminars in Hearing 14:1,1-122 1993
C o m m e n t on the Joint Committee Statement Enclosed in this issue of the BULLETINis the Joint Committee on Infant Hearing 1994 Position Statement just released for publication. The Joint Committee member organizations that have prepared and approved this position statement include the American Academy of Otolaryngology-Head and Neck Surgery, the American Academy of Audiology, the American Academy of Pediatrics, the American Speech-Language Hearing Association and the Directors of Speech and Hearing Programs in State Health and Welfare Agencies. Here is what you need to knowThe 1994 Position Statement: I. Endorses the goal of universal detection of infants with hearing loss and encourages continuing research and development to improve techniques for detection of and intervention for hearing loss as early as possible. 2. Maintains a role for the indicators (formerly called risk factors] described in the 1990 Position Statement. 3. Identifies indicators associated with delayed or late onset hearing loss and recommends procedures to monitor infants with these indicators. 4. Recognizes the adverse effects of fluctuating conductive hearing loss from persistent or recurring otitis media with effusion [OME] and recommends monitoring infants with OME for hearing loss. 5. Endorses the provision of intervention services in accordance with Part H of the Individuals with Disabilities Education Act [IDEA], Public Law 102-I 15.
~llach year in the United States, about 4,000 children are born profoundly deaf and another 37,000 children are born with milder degrees of hearing impairment that can also affect speech, language and • educational development. Therefore, about 6 of every 1,000 children born will have some degree of hearing impairment (Northern & Hayes 1994). Hearing impairment in children is 20 times more prevalent than other birth defects such as phenylketonuria (PKU), sickle cell anemia and hypothyroidism for which screening is routinely done at birth (Downs 1993). Comparatively, it is less expensive to identify an infant with a hearing loss than to identify an infant with PKU, sickle cell anemia or hypothyroidism. Screening all infants would cost about $9,300 to detect one infant with a hearing loss as compared to $40,000 to detect one infant with PKU (Downs 1993).
Reprinted from the American Academy of Otolaryngology-Head and Neck Surgery BULLETIN 1994;13(12):insert. OTOLARYNGOLHEAD NECKSURG 1995;113:188-90.
188
Why the need for universal detection of infants with hearing loss as early as possible?
There is sufficient evidence that early identification of hearing loss in children and early intervention in the management of hearing loss in these children are essential for normal speech, language and educational development. Since hearing during the first six months of life is important for normal speech and oral language development, identification of infants with hearing loss before age 3 months is desirable. It has also been demonstrated that of all the variables examined, the age at which special education commences has been demonstrated to have the largest effect on development of language and communication skills (Levitt et al). "Healthy People 2000'' is an ambitious plan published by the Department of Health and Human Services in 1990 containing a national strategy for significantly improving the health of the nation over the coming decade, One of the objectives is the recommendation to reduce the average age at which children with significant hearing impairment are identified to no more than 12 months of age by the year 2000. Utilizing the risk factors (now called indicators)
Otolaryngology Head and Neck Surgery Volume 113
Number 3
followed by ABR testing in those infants with one or more risk factors, as recommended by the Joint Committee on Infant Hearing 1990 Position Statement, statewide and regional hearing screening programs failed to detect at least 50% of those children born with significant hearing loss and the ages of identification of hearing loss in children in the United states remained close to 36 months. (Mauk et al 1991) Advances in the technology of objective assessment of hearing have augmented the feasibility Of early detection of hearing loss beyond the rate of detection that could be achieved with use of the risk factors (indicators) alone. New methods are readily available to screen all newborns for hearing loss prior to hospital discharge. (Peters, 1986; Kilney, 1987; Bonfels et al, 1988; Stevens et al, 1989). As various states have implemented universal hearing screening programs using ABR and OAE, there is growing evidence that these objective test methods are sensitive, specific and cost effective (White & Behrens, 1993 and Marlowe 1993). Cognizant of poor results utilizing only the risk factor hearing screening protocol and in recognition of the evolution of present technology, the NIH Consensus Conference on Early Identification of Hearing Loss in Infants and Young Children concluded in March 1993 that all infants should be screen ed for hearing impairment preferably prior to hospital discharge. The screening procedure recommended by the expert panel would involve first screening the hearing of all infants with a test that measures otacoustic emissions (OAE). The panel further recommended that infants who fail OAE screening have additional testing of auditory brainstem responses to confirm the validity of the OAE failure. Those infants who fail ABR testing should have comprehensive hearing evaluation before six months of age. The Joint Committee on Infant Hearing 1994 Position Statement endorses universal detection of infants with hearing loss as early as possible. The Joint Coramittee on Infant Hearing is using the phrase universal hearing "detection" rather than universal hearing "screening" since the term "detection" connotates an outcome rather than a protocol. In keeping with this philosophy, the Joint Committee on Infant Hearing 1994 Position Statement discusses ABR and OAE. Rather than recommending a specific protocol, the Joint Committee on Infant Hearing recommends that each team of
COMMENT ON THE JOINT COMMITTEE STATEMENT t 8 9
health care professionals seeking to develop an infant hearing detection program evaluate and select the technique most suitable for the locale and available resources. The Joint Committee on Infant Hearing 1994 Position Statement preserves a meaningful role for the indicators (formerly called risk factors). The 1994 Position Statement lists those indicators for both neonates and infants and mentions the value of parent and/or care giver concern regarding hearing, speech, language and/or developmental delay as an important indicator for possible hearing loss. The indicators also aid in determining a cause of hearing loss as well as helping to identify those syndromes that can include hearing loss. The 1994 Position Statement adds a third list of indicators for infants who require periodic monitoring of hearing because of the possibility of delayed onset hearing loss. These infants should have their hearing monitored every six months until age 3 years and at appropriate intervals thereafter. Conditions that can be responsible for delayed onset hearing loss include family history of hereditary childhood hearing loss, in utero cytomegalovirus infection, and neurodegenerative disorders such as Neurofibromatosis II. This category of indicators also includes recurrent or persistent otitis media with effusion (OME) and conductive hearing loss as well as other disorders of Eustachian tube function that, if persistent, can affect a child's speech and language development much in the same way that sensorineural hearing loss during early life can affect speech and language development. One of the major weaknesses of many infant hearing screening programs is the significant number of cases with lack of follow-up and lack of established protocols for interventional services once a child is diagnosed as having a hearing loss (Welsh and Slater 1993). The 1994 Position Statement devotes a section to early intervention which must be provided in accordance with the Individuals with Disabilities Education Act (IDEA) Public Law 102-119 (formerly PH-99457). The Joint Committee on Infant Hearing recommends that evaluation and assessment of an infant identified with a hearing loss should be accomplished by a team of specialists including a physician with expertise in the management of early childhood otological disorders, an audiologist with expertise in assessment of infants and young children, a speech-language pathologist, a sign language specialist and/or teacher of the deaf
t90
Otolaryngology Head and Neck Surgery September 1995
COMMENT ON THE JOINT COMMITTEE STATEMENT
as well as the parent/care giver. The parent/care giver should be educated and given all options. Choices in regard to the management of their child's hearing loss and early intervention services, based on the family's preference, should begin by six months of age. As an otolaryngologist-head and neck surgeon, you are a vital part of the team concerned with diagnosis and treatment of children with early onset hearing loss (Marlowe & Epstein 1994). First, it is important that you become an advocate for universal hearing detection of infants with hearing loss in your community and in the hospitals in which you are affiliated. It is your responsibility to educate your professional colleagues, especially the primary care physicians involved with the care of infants and children, and the administrators and other professionals associated with your participating hospitals in regard to the merits of universal newborn and infant hearing screening. You should be active in the establishment and proper functioning of newborn and infant hearing screening programs. You should advocate prompt referral of all newborns and infants who fail hearing screening tests and/or who demonstrate the presence of indicators for hearing loss. You should be an advocate for continuous monitoring of the hearing of children who manifest indicators suggesting delayed or late onset hearing loss. And finally, you should be aware of all options and methods of management of hearing loss, taking into consideration family preference. The successful management of early or delayed onset hearing loss in children depends on early intervention. Hearing aids, for example, when indicated, can be provided for an infant as soon as the diagnosis is made, even within the first ~few months of life. The goal of all concerned should be implementation of the guidelines set forth by the Joint Committee on Infant Hearing 1994 Position Statement in order to achieve the objective of Healthy People 2 0 0 0 - to appropriately diagnose at the earliest possible age and manage as effectively as possible children with hearing loss before one year of age by the year 2000.
Kenneth M Grundfast, MD is former co-chairman
and representative of the Joint Committee on Infant Hearing. Stephen Epstein, MD represents the AAO-HNSF on the Joint Committee on Infant Hearing, REFERENCES
Bonfels, P., Uziel, A. and Pujol, R. Screening for auditory dysfunction in infants by evoked otacoustic emissions.Archives of Otolaryngology-Head and Neck Surge~ 114, 887-890 1988 Consensus Development Conference-Early Identification of Hearing Impairment in Infants and Young Children, National Institute of Health, Office of Medical Applications of Research, Bethesda, MD, March 1-3, 1993 Downs, M.P. Benefits of screening at birth: economic, educational and functional factors. NIH Consensus Development Conference Program and Abstracts, Public Health Service, 63-66 1993 Healthy People 2000. Objective 17.16, 464-465. U.S. Department of Health and Human Services, Public Health Service, DHHS Publications No. (PHS) 91-50213, U.S. Government Printing Office, Washington, D.C. 20402-1990 Joint Committee on Infant Hearing 1990 Position Statement, American Academy of Otolaryngology-Head and Neck Surgery BULLETIN, March 1991 Kilney, P. Algo-1 automated infant hearing screener: preliminary results. Seminars in Hearing 8:133-141, 1987 Levitt, H., McGarr, N.S. and Geffner, D. Development of Language and Communications Skills in Hearing Impaired Children. ASHA Monographs Number 26, Rockville, MD: American Speech Language Hearing Association, October 1987 Marlow, J., Epstein, S. Professional hearing care for infants and children: the future. Hearing Instruments 45:10 Suppl. 2 1994 Marlowe, J. Screening all newborns for hearing impairment in a community hospital. American Journal of Audiology 2:22-25 1993 Mauk, O., White, K., Mortensen, L. and Behrens, T. The effectiveness of screening programs based on high risk characteristics in early identification of hearing impairment. Ear and Hearing 12:312-319 1991 Northern, J.L., Hayes, D. Universal screening for infant hearing impairment: necessary, beneficial and justifiable. Bulletin of the American Academy of Audiology 6:3, 10-13 May-June 1994 Peters, J. An automated infant screener using advanced evoked response technology. Hearing Journal 39:25-30, 1986 Stevens, J, Webb, H., Hutchinson, J., Connell, J., Smith, M. and Buffin, J. Click evoked otacoustic emissions in neonatal screening. Ear and Hearing 11:128-133, 1990 Welsh, R., Slater, S. The state of infant hearing impairment identification programs. ASHA 49:52 April 1993 White, K.R. and Behrens, T.R. (Eds) The Rhode Island Hearing Assessment Project: Implications for Universal Newborn Hearing Screening. Seminars in Hearing 14:1,1-122 1993