The purpose of this chapter is to review the principles of quality monitoring for newborn screening programs at both a hospital and a public health level. The focus is on quality assurance in Early Hearing Detection and Intervention (EHDI). The chapter addresses the need for a systems approach to quality, beginning with the birth screening, moving to follow-up re-screening and on to confirmatory evaluations and life-long intervention and management for the infants identified with permanent hearing loss. Benchmarks are identified to assist those involved in establishing programs or in improving program performance. As the use of electronic information systems in EHDI becomes widespread, the importance of strict computer-based logic rules to assure patient privacy is addressed.

The advent of hearing screening in newborns in many states has led to an increase in the use of genetic testing and related genetic services in the follow-up of infants with hearing loss. A significant proportion of those with congenital hearing loss have genetic etiologies underlying their hearing loss. To ensure that those identified with congenital hearing loss receive the genetic services appropriate to their conditions, the Maternal and Child Health Bureau of the Health Resources and Services Administration funded the American College of Medical Genetics to convene an expert panel to develop guidelines for the genetic evaluation of congential hearing loss. After a brief overview of the current knowledge of hearing loss, newborn screening, and newborn hearing screening, we provide an overview of genetic services and a guideline that describes how best to ensure that patients receive appropriate genetic services. The significant contribution of genetic factors to these conditions combined with the rapid evolution of knowledge about the genetics of these conditions overlaid with the inherently multidisciplinary nature of genetic services provides an example of a condition for which a well-integrated multidisciplinary approach to care is clearly needed.

The Joint Committee on Infant Hearing (JCIH) endorses early detection of, and intervention for infants with hearing loss (early hearing detection and intervention, [EHDI]) through integrated, interdisciplinary state and national systems of universal newborn hearing screening (UNHS), evaluation, and family-centered intervention. The goal of EHDI is to maximize linguistic and communicative competence and literacy development for children who are hard of hearing or deaf. Without appropriate opportunities to learn language, children who are hard of hearing or deaf will fall behind their hearing peers in language, cognition, and social-emotional development. Such delays may result in lower educational and employment levels in adulthood (Gallaudet University Center for Assessment and Demographic Study, 1998). Thus, all infants' hearing should be screened using objective, physiologic measures to identify those with congenital or neonatal onset hearing loss. Audiologic evaluation and medical evaluations should be in progress before 3 months of age. Infants with confirmed hearing loss should receive intervention before 6 months of age from health care and education professionals with expertise in hearing loss and deafness in infants and young children. Regardless of prior hearing screening outcomes, all infants who demonstrate risk indicators for delayed onset or progressive hearing loss should receive ongoing audiologic and medical monitoring for 3 years and at appropriate intervals thereafter to ensure prompt identification and intervention (American Speech-Language-Hearing Association [ASHA], 1997). EHDI systems should guarantee seamless transitions for infants and their families through this process. Appropriate early intervention programs are family-centered, interdisciplinary, culturally competent, and build on informed choice for families (Baker-Hawkins and Easterbrooks, 1994). To achieve informed decision-making, families should have access to professional, educational, and consumer organizations; and they should have opportunities to interact with adults and children who are hard of hearing and deaf (Ogden, 1996; Thompson, 1994). Families should have access to general information on …

The advocacy by pediatricians is imperative if early hearing detection and intervention (EHDI) programs are to be effective, efficient, and successful over the long term. Some pediatricians remain unfamiliar with the rationale for universal screening of all newborns prior to hospital discharge. Pediatricians' questions regarding universal screening are data- and quality-driven. Discussion of the componenets of the EHDI program, answers to pediatricians' most frequently asked questions, supported by data, and the quality indicators used to monitor EHDI programs in Texas are provided.

BACKGROUND Early detection of hearing loss coupled with appropriate early intervention is critical to speech, language, and cognitive development. These competencies serve as the foundation for later academic skills. For these reasons, many states are undertaking aggressive efforts to screen all newborns before hospital discharge. Universal detection of hearing loss in newborns is a three-stage process composed of 1) the birth admission screen, 2) follow-up and diagnosis, and 3) intervention services. Breakdown at any stage jeopardizes the entire effort. The goals of this research are to examine the birth admission screen by reviewing outcome measurements for 54 228 Texas newborns and to evaluate factors that impact outcomes positively or negatively. METHODOLOGY All newborns were screened for hearing loss using a physiologic test of auditory function; either screening auditory brainstem responses or transient evoked otoacoustic emissions. Screening occurred in the newborn and intensive care nurseries, before hospital discharge in 9 sites as part of the nursery protocol. Patients. A total of 54 228 newborns were available for screening. OUTCOME MEASURES Four measures were evaluated and are reported: the number of births screened, the number of newborns who passed the screen before discharge, the number of infants who returned for follow-up, and the number of infants identified with hearing loss. A Birth Screening Performance Index is also calculated. RESULTS Results are reported for calendar years 1994, 1995, 1996, and through June 1997. A total of 54 228 newborns were available for screening; 52 508 were screened before hospital discharge during their birth admission and 50 721 passed this screen. Infants returning for follow-up screen as outpatients numbered 1224. Over this 31/2-year span, 113 infants who failed the birth admission screening had hearing loss that was sensorineural in nature. From these data, the estimated incidence of hearing loss is 3.14/1000 infants. CONCLUSIONS Screening in the nursery with low false-positive rates can be achieved when three elements are present: audiology involvement, hospital support, and automated data and information management. Follow-up measures need improvement. Better tracking methods may help assure that at-risk newborns are connected to services.

To paraphrase Stein (1995), designing, implementing, and maintaining a cost-effective process for UIHD may be the most challenging responsibility we face as audiologists because those activities affect 4 million babies and their families each year. We are confident that we will be successful as audiology implements quality measures, when information management programs are designed to meet our needs, when partnerships are embraced, and when our leadership and management skills are refined. Each baby that passes through our programs will be screened; infants with hearing impairment will be detected and connected to service. The motto of the Sounds of Texas Project is “Detect and connect—one baby at a time.”

No AccessAmerican Journal of AudiologyResearch Article1 Nov 1997The State of the Information Terese Finitzo andPhD Allan O. Diefendorf Terese Finitzo University of Texas, Callier Center, 1899 Inwood Road, Dallas, TX 75235 Google Scholar More articles by this author and Allan O. Diefendorf Indiana University School of Medicine, Indianapolis Google Scholar More articles by this author https://doi.org/10.1044/1059-0889.0603.91 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationTrack Citations ShareFacebookTwitterLinked In References American Speech-Language-Hearing Association. (1994). Joint Committee on Infant Hearing, 1994 Position Statement.Asha, 36, 38–41. Google Scholar Pool, K. D. (1997, April). Sounds of Texas Team Meeting, Dallas, TX. Google Scholar Wheeler, D. J., & Chambers, D. S., (1986). Understanding statistical process control. Knoxville, TN: SPC Press. Google Scholar Additional Resources FiguresReferencesRelatedDetails Volume 6Issue 3November 1997Pages: 91-94 Get Permissions Add to your Mendeley library History Published in issue: Nov 1, 1997 Metrics Topicsasha-topicsasha-article-typesKeywordsdata managementuniversal newborn hearing screeninginformation managementCopyright & PermissionsCopyright © 1997 American Speech-Language-Hearing AssociationPDF DownloadLoading ...

This 1994 Position Statement was developed by the Joint Committee on Infant Hearing. Joint committee member organizations that approved this statement and their respective representatives who prepared this statement include the American Speech-Language-Hearing Association (Allan O. Diefendorf, PhD, Chair; Deborah Hayes, PhD; and Evelyn Cherow, MA, ex officio); the American Academy of Otolaryngology—Head and Neck Surgery (Patrick E. Brookhouser, MD, and Stephen Epstein, MD); the American Academy of Audiology (Terese Finitzo, PhD, and Jerry Northern, PhD); the American Academy of Pediatrics (Allen Erenberg, MD, and Nancy Roizen, MD); and the Directors of Speech and Hearing Programs in State Health and Welfare Agencies (Thomas Mahoney, PhD, and Kathie J. Mense, MS). The Joint Committee on Infant Hearing endorses the goal of universal detection of infants with hearing loss as early as possible. All infants with hearing loss should be identified before 3 months of age, and receive intervention by 6 months of age. I. BACKGROUND In 1982, the Joint Committee on Infant Hearing recommended identification of infants at risk for hearing loss in terms of specific risk factors and suggested a follow-up audiologic evaluation until an accurate assessment of hearing could be made (Joint Committee on Infant Hearing, 1982; American Academy of Pediatrics, 1982). In 1990, the Position Statement was modified to expand the list of risk factors and recommend a specific hearing screening protocol. In concert with the national initiative Healthy People 2000 (US Department of Health and Human Services, Public Health Service, 1990), which promotes early identification of children with hearing loss, this 1994 Position Statement addresses the need to identify all infants with hearing loss.

In a series of studies regarding CNS dysfunction in stuttering, we have examined linguistic and motoric performance in the context of measures of brain function. Previous studies of adults with developmental stuttering identified alterations in brain function (metabolic and electrophysiologic) in cortical regions implicated in models of speech motor control and language processing. We also identified a sub-group of these subjects who exhibited linguistic performance deficits related to speech performance deficits. The present study examined the hypothesis that adults who stutter and who show linguistic performance deficits will also show metabolic alterations in cortical regions classically related to language processing, whereas adults who stutter but who do not show linguistic performance deficits will not show these cortical metabolic alterations. Significant relative blood flow asymmetry (left < right) was observed in middle temporal and inferior frontal cortical regions only for adults who both stuttered and showed linguistic performance deficits. Results support models that explicitly recognize that efficient integration of linguistic, motoric, and cognitive processes is critical to the production of oral/verbal fluency and to understanding sources of fluency failure.

Pool et al 1 recently reported on regional cerebral blood flow (rCBF) data from single-photon emission computed tomography conducted with 20 adult stutterers and 78 age-matched control subjects. From their analyses of rCBF values in 20 regions of interest (ROIs), using one tomographic section per subject, these researchers concluded that they had obtained preliminary evidence for cortical dysfunction in stutterers related to reduced and asymmetric left frontal and temporal perfusion. These are very provocative findings with respect to the diagnosis and, perhaps, treatment of this disorder; they have already prompted one derivative investigation 2 and an inquiry about the researchers' methodology. 3 We also have some inquiries; they concern interpretation and validity of the findings of Pool and colleagues. We believe that their response might also help clarify the importance of their findings. By far, the most significant finding of the study by Pool et al is that brain blood

Neuroscience Center Methodist Medical Center Dallas, Texas (Finitzo); Division of Speech and Hearing Sciences, University of North Carolina, Chapel Hill, North Carolina (Roush)

Tympanometry and pneumatic otoscopy were compared to findings at myringotomy in 86 children (163 ears). Seventy percent of the ears (115) had effusion, as revealed by myringotomy. Sensitivity and specificity for tympanometry were 90% and 86%, respectively. Sensitivity and specificity for pneumatic otoscopy were 93% and 58%, respectively. A chi-square was performed to compare the sensitivity and specificity of tympanometry to otoscopy, revealing tympanometry significantly better at determining non-effusion states. Additionally, a combined otoscopy and tympanometry sensitivity and specificity were calculated for those otoscopy and tympanometry determinations in agreement, revealing both sensitivity and specificity above 90%. A Fisher's exact probability test revealed no significant differences for the accuracy of tympanometry over otoscopy when the determinations of each were not in agreement. Implications of these results are discussed.

In Reply. —We appreciate the interest in our article1shown by Viswanath and his colleagues and welcome the opportunity to respond to their comments. Their first concern is based on comparison of findings for normal control subjects in our study1with findings reported by Devous et al.2Viswanath et al suggest that interhemispheric flow differences for the normal subjects in these studies are opposite for superior and middle temporal regions of interest (ROIs). We are confused by this statement. Within-group interhemispheric flow differences for normal control subjects were not reported by Pool et al.1However, examination of flow values summarized in Table 1 of that article shows that the pattern of interhemispheric difference for normal control subjects is, in fact, similar to the pattern of differences reported by Devous et al2in their Table 4. Questions concerning the validity of the measure and differences in

The 1980s witnessed renewed interest in the relation between developmental stuttering and central nervous system (CNS) abnormalities. We have reported differences between nonstutterers and developmental stutterers on electrophysiologic (QTE) and metabolic (rCBF) measures of brain function. A critical step in the interpretation of results of functional brain imaging studies is to determine the relation, if any, of identified CNS abnormalities to speech motor control in persons who stutter. In this study we addressed the interpretation of rCBF findings by asking whether we could identify patterns of impaired acoustic laryngeal reaction time (LRT) as a function of response complexity parallel to rCBF findings. Stutterer subgroups determined by clinical severity ratings were not differentiated by LRT values as a function of response complexity. Stutterers with relative blood flow asymmetry below the normal median value involving both left superior and middle temporal regions of interest (ROIs) showed significantly longer LRT for the complex response than did normal speakers and stutterers with above-normal median relative flow values to at least one of these temporal ROIs. Stutterer subgroups based on reduced cingulate flow alone were not differentiated by LRT values. Findings are consistent with Goldberg's (1985) model of CNS premotor processing. Findings also suggest that stutterer subgroups might be distinguished by the presence, loci, and relative magnitude of cortical and/or subcortical rCBF abnormality in regions that subserve a fluency-generating system.

Converging evidence suggests that stuttering is associated with deficits in the planning and execution of speech. Evidence also suggests that the onset, development, and loci of stuttering are related to demands language places on speech motor planning and execution. We combined linguistic and vocal motor assessments to address two questions: 1) Can we identify a subgroup of adult stutterers who demonstrate linguistic deficits? and 2) Do linguistically normal and impaired stutterers show different patterns of laryngeal reaction time (LRT) as a function of response complexity? Linguistic performance was evaluated using tasks that assess relatively high-level production and comprehension processes. Responses used to record LRT differed in linguistic and motoric complexity. Only linguistically impaired stutterers showed significant increases in LRT for complex responses. Findings suggest that linguistic and motor processes affect the efficiency and fluency of speech motor control and that both processes be made explicit in models of stuttering.

Spasmodic dysphonia is a disturbance of phonation with laryngeal spasms. We report voice and neurologic examination findings in 45 subjects. Neurologic abnormalities were found in 32 subjects (71.1%). Rapid alternating movement abnormalities, weakness, and tremor were common. Incoordination and spasticity were rare. Lower extremity findings were frequent. Abnormalities were bilateral. Spasmodic dysphonia severity was related to age. Type, severity, and duration of vocal symptoms were not different for subjects with or without neurologic abnormalities. Vocal tremor was more frequent in neurologically abnormal subjects. Involvement of a pallidothalamic-supplementary motor area system could account for neurologic findings, brain imaging findings, and clinical heterogeneity. The view emerging is that spasmodic dysphonia is a manifestation of disordered motor control involving systems of neurons rather than single anatomical sites.