Audiological Assessment and Support Linda K. Wright, AuD Doctor of Audiology

Joint Committee on Infant Hearing American Academy of Pediatrics – Joint Committee on Infant Hearing -- then type in the search area Joint Committee on Infant Hearing Updated document in October 2007 Clarifications made in February 2008

Definitions for familiarity JCIH – Joint Committee on Infant Hearing EHDI – Early Hearing Detection and Intervention (re-authorization of act) OAE – Otoacoustic Emissions ABR – Auditory Brainstem Response AN/AD – Auditory Neuropathy or Auditory Dyssynchrony

JCHI looking for… Definition of hearing losses they are looking to identify include: Congenital permanent bilateral, unilateral sensory, or permanent conductive hearing loss, neural hearing loss

Screening and Re-screening protocols Separate protocols for NICU and well-baby nursery NICU for more than 5 day admission have to undergo automated auditory brainstem response screening (cannot be screened and passed by OAEs alone), looking for all types of hearing loss – including neural NICU regardless of length of stay – any infant with hyperbilirubinemia requiring exchange transfusion must undergo screening by automated auditory brainstem response NICU Infants that do not pass ABR screening, referral direct to Audiologist for re-screen of both ears (even if only one ear failed) and when indicated a comprehensive diagnostic eval including ABR

Screening and Re-screening protocols, continued… For infants re-admitted in the first month of life (NICU or well baby) where the readmission is associated with a potential risk for HL – repeat screening before they are discharged All infants should receive a physiologic hearing screening by 1 month of age (NICUs, well-baby, discharged before screening, transferred to another hospital, born outside a hospital, equipment failure) Most re-screenings performed at the hospital of birth or place that physician makes a referral to

Audiology Diagnostics Auds with pediatric experience Infants who fail re-screen should receive diagnostic testing by 3 months of age, and have at least 1 ABR (children under 3) to confirm permanent loss ABR should use frequency specific tone burst stimuli by air and bone (when indicated) to determine degree and configuration of HL ABR should include click stimuli using both condensation and rarefaction polarity to help identify neural HL (AN/AD) should use 1000 Hz probe tone tympanometry (younger infants), DP or TE OAEs, case history, observations of the child

Audiology Diagnostics, continued… Re-evals needed for those that pass the re-screen are to be determined on an individualized basis taking into consideration things such as risks of delayed on-set environmental exposures any infant with even one risk factor should receive diagnostic hearing test by 24 – 30 months of age – or more frequent as determined by family, physician, etc… case history observations and behaviors of the child Re-evals for those that pass re-screen, but it is determined they need more additional testing…could/should include: Tympanometry OAEs reflexs behavioral pure tone testing could also include ABR if behavioral testing is unreliable or if ABR has never been performed in the past

Audiology and Educational follow-up Infants diagnosed with permanent HL (and the family is pursing amplification) should be fit within one month of the diagnosis Any child with any degree of HL (one or two ears) should be considered eligible for early intervention services and receive services as soon as possible (or by 6 months of age) Intervention services should be provided by professions with experience in HL (educations for deaf/HH, Auds, SLPs) Both home based and center-based options should be available for educational follow-up

Medical Home = Primary Care Physician for the child Regular monitoring of developmental milestones, parent concerns, middle ear status, etc… Children who do not pass speech/language milestone assessments should be referred by medical home to SLP and Auds Every infant with confirmed HL needs to be referred for medical evaluation, preferably to an ENT – cultures of blood/urine, CT scans, MRIs, physical examination, referral for genetics testing (may be referred by diagnostic Aud)

Communication of screening results, diagnostic results, referrals, etc… Birth hospital with EHDI coordinator should tell parents and medical home the infant hearing screening results Provide parents with follow-up info, resources Communicate in culturally sensitive means All screenings, re-screening and diagnostic follow-ups communicated to medical home and EHDI coordinator

Communication of screening results, diagnostic results, referrals, etc… State EHDI programs have to report to federal entities. State EHDI programs rely on birthing hospital to provide inpatient screening results (as with other tests that are run on newborns), rely on Auds to indicate re-screen results, rely on Auds to indicate diagnostic results, rely on early interventionists to indicate enrollment, etc…

Support of the Family and Child Families need to be aware of all communication options for their child, in a non-biased manner All states should have a way to track the results Support groups for parents - Hands and Voices National, MI H&V, AG Bell, Hearing Loss Association of America with state and local chapters

State of MI EHDI MI EHDI releases quarterly data on all births in MI, results of hearing screenings, follow-up reports on newborns re-screened and that receive diagnostic evals MI has 100% of its hospitals screening newborns for hearing (voluntarily) Not all MI hospitals have the same screening equipment (OAEs vs. ABRs) Initial screenings are performed by nurses trained in the screening procedure Re-screens may or may not be performed by an Audiologist

State of MI EHDI Currently the state of MI does NOT reimburse hospitals for performing this screening, they do it voluntarily. This is why we, and other states, need to abide by guidelines, report/track results, show improvement, show enrollment in early intervention services… Consider supporting the re-authorization of the EHDI act (see info from Karl White provided on wikispaces under this weeks topic)

Stats At the time of the 2000 JCIH statement: 38% of infants screened annually in the US. In 2007 – 95% of US infants are screened annually! Several studies indicate variance in the prevalence of newborns with congenital hearing loss in the United States. The overall estimates are between 1 to 6 per 1,000 newborns. Most children with congenital hearing loss have hearing impairment at birth and are potentially identifiable by newborn and infant hearing screening. However, some congenital hearing loss may not become evident until later in childhood. Kemper, A.R., & Downs, S.M. (2000, May). A cost-effectiveness analysis of newborn hearing screening strategies. Archives of Pediatric and Adolescent Medicine, 154(5): Cunningham, M., & Cox, E.O. (2003, February). Hearing assessment in infants and children: Recommendations beyond neonatal screening. Pediatrics, 111(2): Task Force on Newborn and Infant Hearing. (1999, February). Newborn and infant hearing loss: Detection and intervention. Pediatrics, 103(2):

Stats Profound, early-onset deafness is present in 4-11 per 10,000 children, and is attributable to genetic causes in at least 50% of cases. Marazita, M.L., et. al. (1993, June 15). Genetic epidemiological studies of early-onset deafness in the U.S. school-age population. American Journal of Medical Genetics, 46(5):

Unpublished Doctoral Project Work CMU student in Doctor of Audiology Program Doctoral Project on Newborn Hearing Follow-up Testing Surveyed 38 sites that were indicated by the EHDI program as approved sites for follow-up diagnostic hearing testing States – 6 - MI, 20 - OH, and 12 –IL A few bits of data could not be counted – so #s may not all add up, but just want to show some overall positives and negatives

Positives from the Doc project survey Majority of sites tested both ears – even if only one ear failed Majority of sites use DPOAEs as part of their diagnostic test battery Majority of sites that perform tympanometry as part of the test battery use the 1000 Hz probe tone (others use 226 or 660 Hz) Majority of sites also distribute pediatric hearing aids (if needed for the family/child) Majority of sites fit amplification by 6 months of age (remember newer JCIH recommends amplification fit (if desired by parent) by 1 month after diagnosis Majority of sites use a frequency of re-evals every 3 months Majority of sites indicate they discuss and offer other assistive listening devices Majority of sites indicate they contact the physician with results Majority of sites indicate they notify state EHDI of the results

Negatives from the Doc project survey Fifteen sites do NOT perform tympanograms as part of their battery A few sites indicated they do NOT have ABR equipment – yet they were listed by the state EHDI as being an approved site for diagnostic evaluations Most sites do NOT perform the frequency specific tone bursts (500 – 4000 Hz) as recommended in the 2007 statement – Most sites just perform click stimuli Twelve sites indicate they do NOT perform the switch in signal polarity to evaluate for AN/AD Not all sites reported they notify their state EHDI or the infants physician About half of sites do NOT mention possibility of genetics testing About half of sites have fit children within one month of the diagnosed hearing loss

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