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Third Workshop of the NIDCD Working Group on the Early Identification of Hearing Impairment

September 19, 2000
Rockville, Maryland

The Third Workshop of the NIDCD Working Group on Early Identification of Hearing Impairment was held on September 19, 2000, in Rockville, Maryland. The purpose of the workshop was to identify critical research needs in the area of early identification of hearing impairment. The workshop was designed to provide advice to the NIDCD for identifying research to be supported through the Federal Government grant and contract processes. Representatives from the Centers for Disease Control and Prevention, one of NIDCD’s Federal agency partners supporting the early identification of hearing impairment, were present. Members of the Working Group are listed in the Attachment. Amy Donahue, Ph.D., Division of Extramural Research, NIDCD, and Judith Gravel, Ph.D., Albert Einstein College of Medicine, co-chaired the workshop.

The workshop convened at 8:30 a.m. James Battey, Jr., M.D., Ph.D., Director, NIDCD, welcomed the attendees. Dr. Donahue then provided the charge to the workshop. She reminded the Working Group that the process of screening was the focus of the first workshop of the Working Group, and the focus of the second workshop was on the issues following neonatal hearing screening, specifically the diagnostic and intervention strategies. This workshop was intended to review the entire scope of issues related to early identification of hearing impairment and to delineate research priorities.

Each member of the Working Group spoke briefly about what they considered to be the most pressing research questions. There was considerable overlap and agreement among the members of the Working Group on the most urgent issues, needs, and opportunities. The Working Group, under the direction of Drs. Donahue and Gravel, subsequently formulated recommendations to the NIDCD regarding the most pressing research questions in the early identification of hearing impairment. The workshop was adjourned at 2:30 p.m.

Primary Research Opportunities

  1. Characterization of auditory/auditory perceptual system capabilities of infants with hearing loss with the goal of determining appropriate/optimal habilitative strategies (both technological and behavioral) leading to the development of optimal communication skills.
    • Develop techniques for delineating auditory (sensory) thresholds and methods for examining auditory perception (speech and supra-threshold auditory abilities) and multi-modal perceptual integration.
    • Devise methods to determine auditory/linguistic development in infants with hearing loss (including infants with multiple disabilities) for comparison to infants with similar experiences/developmental profiles, as well as to those who hear normally.
    • Examine ways to determine the appropriateness of various types of technologies and approaches to habilitation, the need for a change in technology or intervention strategy, and/or candidacy for cochlear implantation for the individual infant.
  2. Genetics of hearing loss.
    • Determine the genotype and characterize the phenotype of genes associated with hearing loss in infants.
    • Examine the correlation and interaction of genetic information with audiologic/auditory information.
    • Study gene-environment interactions for the purpose of treatment and prevention of hearing loss.
    • Evaluate the use of genetic and other screening methods (e.g., CMV) in early identification programs aimed at newborns and infants.
    • Determine the role of genetic testing in optimum models of service delivery (i.e., screening and assessment protocols) and the impact of genetic screening on diagnosis and follow-up, including social-emotional effects on families. Ethical, legal, and social implications of genetic testing should be considered in the context of unique cultural issues associated with hearing impairment/deafness.
  3. Consequences of over-referral.
    • Examine over-referral (false-positive outcomes) relative to (a) the impact on families resulting from anxiety and changes in family dynamics; (b) costs and risks of unnecessary diagnostics and treatment; (c) overall medical, health care and societal costs; (d) family, social-emotional, and cultural effects; (e) perceived credibility of universal newborn hearing screening (UNHS) programs; and (f) compounding problems of follow-up.
    • Determine methods to reduce over-referral.
    • Consider the consequences of false-positives versus the consequences of missing a hearing loss; that is, compare stresses and costs of late identification (missed, late onset, progressive) of hearing loss with those incurred in over-referral.
  4. Determine the appropriateness of the degree of hearing loss currently targeted in newborn hearing screening programs; specifically bilateral hearing loss of mild (30-40 dB HL) degree and unilateral hearing loss (JCIH, 2000; AAP, 1999).
    • Determine the effects of including mild (including high frequency and unusual configurations) and unilateral hearing loss on the costs and benefits of UNHS programs.
    • Determine the effects of mild (including high frequency and unusual configurations) and unilateral hearing loss on communication development and learning.
    • Study the stability of hearing loss over time; determine the prevalence and manner in which hearing loss (bilateral and unilateral) progresses and/or fluctuates and which children are at highest risk for such outcomes.
    • Examine the impact of otitis media with effusion (OME) on infants with permanent hearing loss. Determine whether hearing losses presently targeted for screening in the U.S. need to be revised.
  5. Impact of early intervention on outcome.
    • Determine how various interventions influence outcome, and how outcome efficacy should be measured.
    • Develop measures to determine whether or not intervention is "successful".
    • Characterize factors that influence intervention outcome (e.g., type of intervention, family interaction, family-clinician interaction, socio-economic status).
    • Examine the effects of early use of sign language on later auditory/oral abilities and psycho-social abilities.
    • Determine how early interventions impact the family and infant’s environment for communication and psycho-social development (including pre-verbal behaviors).
    • With the goal of evaluating and optimizing early intervention programs for young infants with hearing loss, examine the role of pre-verbal behaviors in the assessment and development of communication. Study the effects of early intervention on infants with multiple disabilities.
    • Study the impact of intervention using quality of life measures (determined from the individual, parent, and the healthcare provider).
    • Determine the impact and efficacy of technology (amplification and cochlear implantation) and communication approach on outcome.
    • Examine the ultimate outcomes (e.g., long-term effects, educational cost savings) of early intervention.
  6. Diagnostic assessment.
    • Examine the efficiency of techniques, particularly for the diagnosis of infants who are hard-of-hearing. Determine whether refinements in assessment protocols (measurement techniques) by age (< 6 months; 6-12 months) would result in more timely and accurate diagnosis.
    • Study the impact of protracted diagnosis on the problem of follow-up.
    • Develop (refine) techniques that can be used accurately by practitioners with varying levels of skill.
    • Develop electrophysiologic, middle ear measurement and behavioral test techniques that will improve the accuracy and rapidity of the audiologic assessment.
    • Determine appropriate medical, otologic, developmental and genetic assessment strategies.
    • Evaluate the cost effectiveness of diagnostic techniques and assessment protocols.
  7. Examine health care delivery and system issues with the goal of determining methods to resolve the problem of infants who fail to return for follow-up evaluations (those considered "lost to follow-up").
    • Delineate both the barriers to follow-up and the factors that contribute to successful follow-up.
    • Examine the success of follow-up for hearing loss screening programs in comparison to existing screening initiatives for other health conditions in newborns.
    • Examine issues such as the perceived urgency of a hearing screening failure and perspectives of parents and physicians about hearing screening and follow-up.

Other Considerations

  1. Documentation of the natural history of hearing loss, especially for progressive, delayed onset or fluctuating hearing loss, as well as for unusual configurations of hearing loss, with the goal of incorporating new information into future hearing screening guidelines (e.g., risk indicators, frequency of follow-up).
  2. Determination of sensitive periods/sensitive sequences for various aspects of communication development and factors that influence these periods/sequences.
  3. Identification of skills needed by practitioners for the provision of optimal assessment and management services to infants with hearing loss and delineation of factors that facilitate and restrict access to such services.
  4. Assessment of the impact of screening on the nursery environment including infection control, baby-handling, and disruption of nursery routine.