Screening, Diagnosis and Early Intervention: The Pediatric Audiologist’s Role

1. Screening, Diagnosis and Early Intervention: The Pediatric Audiologist’s Role Antonia Brancia Maxon, Ph.D. NECHEAR Karen M. Ditty, M.S. Texas ENT Specialists, P.A. NCHAM

2. Timely and Appropriate Diagnosis of Hearing Loss Benchmarks (JCIH, 2000) • Newborns screened by 1 month • Infants with hearing loss identified by 3 months • Amplification use begins within 1 month of diagnosis • Infants enrolled in family-centered early intervention by 6 months • Ongoing Audiological management - not to exceed 3 month intervals • Professionals are knowledgeable

3. Newborns screened by 1 month • Currently approximately 86% of all newborns in the United States have their hearing screened at birth • The number of infants referred for diagnostic audiological evaluations has dramatically increased .

4. Infants with hearing loss diagnosed by 3 months • Progress has been made • Testing site may influence age of diagnosis • Geographic access to services may influence age of diagnosis

5. Impediments to Lowering Diagnostic Age • Audiologists lack experience with very young infants and are uncomfortable making the final diagnosis. • Facilities do not have the equipment needed to assess very young infants. • Audiologists are not familiar with clinical protocols necessary for making accurate diagnosis with very young infants. • Inadequate number of audiologists with pediatric expertise

6. Aids to Lowering the Age of Diagnosis • Although there are no national protocols or standards many states have guidelines for their audiologists. • These guidelines can be obtained via the following link on the NCHAM website: http://www.infanthearing.org/states/table.html • NCHAM audiology training • Pediatric Diagnostics • Pediatric Amplification Fitting

7. Pediatric Audiologist • Have the appropriate audiological equipment and protocols for testing newborns and young infants. • Can evaluate a child’s hearing within a short period of time after being contacted for an appointment. • Specializes in working with infants and young children. • Wants to work with infants and young children. • Has worked with Part C program in their state

8. Pediatric Audiologist • Is familiar with the procedures of the Part C system, including IFSP development and procedures for acquiring hearing aids or assistive technology. • If dispenses hearing aids: • can make earmolds, • has loaner hearing aids available • provides hearing aids on a trial basis • has resources to repair hearing aids quickly

9. Pediatric Audiologist • Is willing to review the test results of the audiological evaluation face to face with the family, respecting the Cultural Differences of family units. • Is willing to provide a comprehensive written report with a copy of the test findings in a timely manner.

10. Pediatric Diagnostic Test Battery • Comprehensive Case History • Frequency-Specific Auditory Brainstem Response • High Frequency Probe Tone Tympanometry • Transient and/or Distortion Product Otoacoustic Emissions • Behavioral Audiometry • Referrals

11. Frequency-Specific ABR Accuracy of pure tone threshold estimates with tone burst ABR • High correlation (>.94) for infants and older children (Stapells, et al, 1995) • 90% of ABR thresholds within 20 dB of PT thresholds with most within 10 dB • audiometric configuration does not affect accuracy of match (Oates and Stapells, 1998)

12. Pediatric ABR • Air conduction measures should be done with insert earphones - can affect latency • Bone conduction measures are needed to rule out conductive loss or find conductive component. • Without BC will extend time until diagnosis

13. Pediatric ABR-Sedation • Who and When • 4 months to 5 years • Options • conscious sedative • mild general anesthesia • Monitoring • administered and managed by nurse • monitor O2, HR and BP • crash cart and suction available (J. Hall, 2001)

14. Pediatric ABR-Sedation • Negative outcomes associated with • overdoses, drug interactions • non-trained personnel • injuries to facility (administered at home) • drugs with long half-lives (chloral hydrate, pentobarbital) (J. Hall, 2001)

15. Pediatric Immittance Measures • Provide information about middle ear status to add to BC information • May be affected by conditions in very young infant’s ears - highly compliant • Use of high frequency probe tone (800 Hz or greater) increases reliability and accuracy in young infants.

16. Pediatric Evoked OAEs • Infants and young children with normal hearing have robust • transient evoked otoacoustic emissions (TEOAE) • distortion product otoacoustic emissions (DPOAE) • TEOAEs and DPOAEs and easily measured in infants and children.

17. Middle Ear Effects on OAEs • Middle ear effusion may • obliterate emission • eliminate low frequency component • Negative middle ear pressure may • reduce amplitude, particularly in high frequencies

18. OAE Summary • OAEs are objective evidence of healthy cochlear function • The vast majority of hearing impairment in the low-risk population is a result of malfunction of the cochlear / outer hair cell system, the most sensitive and vulnerable part of the hearing mechanism tested by OAEs. • OAEs provide meaningful information when Retrocochlear and/or auditory neuropathy are a concern.

19. Behavioral Response Audiometry • Provides information about how an infant or young child uses hearing • Behavioral observation techniques can be used to give functional information • only suprathreshold information is obtained • will get better responses to speech than tones • Can look at amplification benefit

20. Amplification Assessment and Fitting • Initiate amplification process immediately after diagnosis. • Includes medical clearance • Includes earmolds - overnight mailing to get within 1 week • Does not require exhaustive audiological data

21. Pediatric amplification fitting • Ability to conduct real-ear measures • Scheduling flexibility and immediacy • Experience with functional measures of benefit

22. Basic Audiological Information Used to Fit Amplification • Hearing Sensitivity • ABR frequency specific information - low, mid and high frequency • Individual ear measures: insert phones • Middle Ear Status • Tympanometry - high frequency • BC to rule out conductive loss

23. Basic Audiological Information Used to Fit Amplification • Cochlear status • ABR intensity-latency function • OAEs • Behavioral Responses • target audiogram • speech awareness

24. Prescriptive Approach to Hearing Aid Fitting • Prescriptive methods designed to consider earmolds and person’s own ear canal, etc., • Select targets (gain, output) • real ear measures • coupler measures

25. Real Ear to Coupler Difference Procedure (RECD) • The infant’s ear is smaller than an adult ear • More SPL for same input compared to adult • Differences can be as large as 15-20 dB • Many hearing-aid fitting algorithms do not take these differences into account. • RECD affects estimates of: • Threshold • Real-ear gain and output

26. RECD • After the RECD is obtained, all hearing aid testing can be done in the test box • RECD values are entered into the hearing aid fitting program to provide a more accurate estimate of real-ear aided gain and output • The RECD will change as the child grows. A good rule of thumb is to obtain a new RECD when a new earmold is needed

27. Prescriptive Approach to Hearing Aid Fitting • Desired Sensation Level - DSL (Seewald, et al, 1996) • Uses minimal audiometric data • Real ear measures • Adjustments for pediatric ears • Used to determine target gain and output settings

28. DSL Goal • Provide children with amplified speech that is audible, comfortable, and undistorted across broadest relevant frequency range possible. • Infant acquiring language has access to speech of others • Infant acquiring language has access to own speech

29. Speech Sounds • Range from softest to loudest speech sound = 30 dB • Low frequencies carry suprasegmental, vowel, and voicing information. • High frequencies carry consonant, perceptual, and linguistic cues.

30. Referral to and Enrollment in Early Intervention • Know established Part C guidelines in state • Know child eligibility criteria • automatic enrollment - diagnosed condition • significant developmental delay • Know state guidelines for selecting a program

31. Enrollment in Early Intervention • Develop Individualized Family Service Plan (IFSP) • All services • speech and language development • auditory development • assistive technology • Goals and objectives • Timelines

32. Components of IFSP for I/T with Hearing Loss • Amplification provision • parent education • Audiological monitoring • Development of auditory skills • Communication development • listening skills - speech perception • speech production • language development • Monitoring middle ear status

33. Status of EHDI Programs: Early Intervention • State Coordinators estimate: • Only 53% of infants with hearing loss are enrolled in EI programs before 6 months of age • Only 31% of states have adequate range of choices for EI programs

34. Barriers to Early Intervention • 30-40% of children with hearing loss demonstrate additional disabilities that may affect communication and related development. • Families who live in under-served areas may have less accessibility, fewer professional resources, deaf or hard of hearing role models, or sign language interpreters available to assist them. • A growing number of children with hearing loss in the United States are from families that are non-native English Speaking. JCIH 2000

35. Some babies are born listeners.. • If we: • use the elements of an effective EHDI program • use the JCIH 2000 Benchmarks • use appropriate diagnostic protocols and procedures • refer to early intervention • are active participants in early intervention