590 likes | 1.61k Vues
Diagnostic Pediatric Audiology from Birth to Intervention. Karen M. Ditty, M.S. NCHAM Antonia Brancia Maxon, Ph.D . NECHEAR NCHAM. Timely and Appropriate Diagnosis of Hearing Loss. Newborns screened by 1 month Infants with hearing loss diagnosed by 3 months
E N D
Diagnostic Pediatric Audiology from Birth to Intervention Karen M. Ditty, M.S. NCHAM Antonia Brancia Maxon, Ph.D. NECHEAR NCHAM
Timely and Appropriate Diagnosis of Hearing Loss • Newborns screened by 1 month • Infants with hearing loss diagnosed by 3 months • Amplification use begins within 1 month of diagnosis Benchmarks (JCIH, 2000)
Timely and Appropriate Diagnosis of Hearing Loss • Infants enrolled in family-centered early intervention by 6 months • Ongoing audiological management - not to exceed 3 month intervals • Professionals working with these infants are knowledgeable about all aspects Benchmarks (JCIH, 2000)
Newborns screened by 1 month • Approximately 90% 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 .
Infants with hearing loss diagnosed by 3 months • Progress has been made however it is affected by • Testing site may influence age of diagnosis • Goal is often met in hospital clinics • Less likely in non-hospital centers • Geographic access to services may influence age of diagnosis • Rural communities are less likely to meet the goal
Impediments to Lowering Diagnostic Age • Audiologists lack experience with very young infants • uncomfortable making the final diagnosis. • Defer to and refer for second opinion • Facilities do not have the equipment needed to assess very young infants. • Frequency specific ABR • AC and BC ABR • High frequency tympanometry
Impediments to Lowering Diagnostic Age • Audiologists are not familiar with clinical protocols necessary for making accurate diagnosis with very young infants. • Do not have “norms” • Cannot “read” ABR for this population • Inadequate number of audiologists with pediatric expertise • No academic training to work with very young infants • No clinical training to work with very young infants
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
Aids to Lowering the Age of Diagnosis • Audiologists can get training through continuing education provided by national associations • NCHAM audiology training • Pediatric Diagnostics • Covers the initial diagnostic procedure • Pediatric Amplification Fitting • Covers behavioral assessment, hearing aid selection, fitting and validation and cochlear implants
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
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
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. • Is willing to continue to explain results at follow-up evaluations
Pediatric Diagnostic Test Battery • Comprehensive Case History • Frequency-Specific Auditory Brainstem Response • High Frequency Probe Tone Tympanometry • Transient and/or Distortion Product Otoacoustic Emissions • Hearing aid Fitting with Real Ear Measurements • Behavioral Audiometry • Referrals
Frequency Specific Auditory Brainstem Response • Air Conduction Clicks • Abrupt or rapid onset of a broad frequency bandwidth . • Greatest agreement in the 2000-4000Hz frequency range. • Not enough information across the frequency range • Low frequencies absent
Frequency Specific Auditory Brainstem Response • Tonebursts • Provides information for narrower frequency regions • Better relates to pure tone audiogram • Bone-Conducted Clicks • Should get when either the click or 500-Hz tonebursts responses are not present at expected normal levels.
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)
Frequency Specific Auditory Brainstem Response • Auditory Steady State Response (ASSR) • An electrophysiologic response, similar to ABR • Generated by rapid modulation of “carrier” pure tone amplitude or frequency. • Signal intensity can be as high as 120 dB
Frequency Specific Auditory Brainstem Response • Auditory Steady State Response (ASSR) • Done in conjunction with ABR Clicks, or on a separate occasions • Major advantage is it estimates severe-to-profound HL • Best used in conjunction with ABR and tone burst testing.
ABR (Click and Tone Burst) versus ASSR: Clinical Application • Advantages • Estimates normal hearing • thresholds • Ear-specific BC findings • Diagnosis of AN • Estimates severe to • profound HL • Disadvantages • Can’t estimate profound HL • Skilled analysis required • Limited BC intensity levels • No ear-specific BC findings • Requires sleep or sedation ABR ASSR R. Ruth, 2003
Pediatric Sedation for ABR • 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)
Pediatric Sedation for ABR • Negative outcomes associated with • overdoses, drug interactions • non-trained personnel • injuries on the way to facility (administered at home) • drugs with long half-lives (chloral hydrate, pentobarbital) (J. Hall, 2001)
Pediatric ABR summary • Air conduction measures should be done with insert earphones • Headphones can affect latency of waveform • Bone conduction measures are needed to rule out conductive loss or find conductive component. • Use B-70 bone vibrator • Use mastoid placement
Pediatric ABR summary • Use earlobe inverting electrodes • Use alternating tone burst to minimize artifact • A slower rate (e.g., 11.1/sec) enhances Wave I • Begin testing near maximum intensity (50 dB nHL) • Allows good waveform to be seen • Identify Wave I in ipsilateral ear to verify test ear • Plot I-L function of Wave V
Pediatric ABR summary • Air conduction measures should include frequency specific tone bursts and/or ASSR as part of a battery of electrophysiological tests. • Of the audiological test battery, only an ABR can help determine an auditory neuropathy case; therefore, ASSR should not be performed alone, but as part of a battery of electrophysiological tests.
High Frequency Probe Tone Tympanometry • Tympanometry provides information about middle ear status • add information to BC results • May be affected by conditions in very young infant’s ears • Ear canal and eardrum are very compliant • Use of high frequency probe tone (800 Hz or greater) increases reliability and accuracy in young infants.
Transient & Distortion Product Otoacoustic Emissions • Infants and young children with normal hearing have robust • transient evoked otoacoustic emissions (TEOAE) • distortion product otoacoustic emissions (DPOAE) • TEOAEs and DPOAEs are easily measured in infants and children.
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
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 outer hair cells • the most sensitive and vulnerable part of the • hearing mechanism tested by OAEs. • OAEs provide meaningful information when retrocochlear lesions and/or auditory neuropathy are a concern.
Amplification Assessment and Fitting • Initiate amplification process immediately after diagnosis. • Includes medical clearance • Federal regulation - ENT • Includes earmolds • overnight mailing to get within 1 week • continue to remake to avoid fitting problems
Pediatric amplification fitting • Does not require exhaustive audiological data • Target audiogram • Individual ear information • Ability to conduct real-ear measures • Scheduling flexibility and immediacy • Experience with functional measures of benefit
Real Ear to Coupler Difference Procedure (RECD) • The infant 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
Real ear measurement • The insert phone is coupled to the earmold • The probe microphone is placed into the ear canal • The earmold is inserted into the ear • Test stimulus is presented • Total test time 5-10 minutes per ear
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
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
Basic Audiological Information Used to Fit Amplification • Cochlear status • ABR intensity-latency function • OAEs • Behavioral Responses • target audiogram • speech awareness
Behavioral Response Audiometry • Provides information about how an infant or young child uses hearing • Behavioral observation techniques can be used to give functional information • Sometimes only suprathreshold information is obtained • will get better responses to speech than tones • Can look at amplification benefit
Behavioral Response Audiometry • Look at amplification benefit • Need to provide speech at greater than detection level • Cannot learn language with threshold-only information • All of normal conversational level speech needs to reach child through amplification
Speech Sounds • Range from softest to loudest speech sound = 30 dB • “th” – “ah” • Low frequencies carry suprasegmental, vowel, and voicing information. • High frequencies carry consonant, perceptual, and syntactic cues.
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
Enrollment in Early Intervention • Develop Individualized Family Service Plan (IFSP) • All services • speech and language development • auditory development • assistive technology • Goals and objectives • Timelines
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
Status of EHDI Programs Early Intervention • Many of the programs in the current system designed to serve infants with bilateral severe-profound losses • BUT, majority of those identified have mild, moderate, and unilateral losses • Programs and professionals not appropriate for children and families • Therefore, Part C of IDEA is severely under utilized
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
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
Pediatric Audiology • Pediatric Audiology with newborns and young infants can be challenging!
Pediatric Audiology • But also rewarding!
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