The Clinical Application of the HearLAB Cortical Evoked Response System For Determining Aided Benefit And Adjustment of Hearing Aids in Children. Vanessa Salisbury, Clinical Scientist, Brighton and Sussex University Hospital Trust Dr Laura Booth, Clinical Scientist, Royal Berkshire Hospital NHS trust The Auditory Cortex The end of the road NAL: Dillon, Van Dun, Carter, Gardner-Berry EUHA 2010, Hannover The auditory cortex Auditory cortex orientation NAL: Dillon, Van Dun, Carter, Gardner-Berry EUHA 2010, Hannover The Cortical Auditory Evoked Potential (CAEP) • Also known as the P1, N1, P2 complex • Latency • P1- 60ms, N1- 100ms, P2- 180 ms • Infant response • N1 absent in young infants • P1, N1, P2 mature at different rates (Kushnerenko et al., 2002) • Maturation complete by 19 years old (Ponton et al., 2000) EUHA 2010, Hannover Adult CAEP Adult P2 5.0 N1 2.5 µV P1 0.0 -2.5 0 100 200 300 400 500 600 NAL: Dillon, Van Dun, Carter, Gardner-Berry EUHA 2010, Hannover Paediatric CAEP • Infant CAEP Infants P 10 µV 5 N 0 -5 -100 0 100 200 300 ms 400 500 600 NAL: Dillon, Van Dun, Carter, Gardner-Berry EUHA 2010, Hannover Clinical uses of the CAEP • Assessment of adult thresholds (Richards and De Vidi, 1995; Tsu et al., 2002) • Children who are unable to provide reliable behavioural thresholds • Renewed interest in the verification of hearing aid amplification in infants (Purdy, 2005) • Universal newborn hearing screening • Confirming hearing loss at younger age • Fitting hearing aids at younger age - at least 60 % of children fitted before 6 months (Wood et al., 2006) EUHA 2010, Hannover Hearing aid fitting in young infants • Current U.K. best practice • Auditory Brainstem Response (ABR) threshold or Auditory Steady State Response (ASSR) threshold • Real Ear to Coupler Difference (RECD) • DSL5 • Parental and professional observations • Provides valuable feedback regarding general audibility of environmental sounds and speech • How do we measure speech audibility in young infants? EUHA 2010, Hannover Speech Evoked CAEPs in Infants • Clinical need for objective assessment of speech audibility • Speech stimulus can be used for CAEP assessment (Ostroff et al., 1998) • Speech is useful when testing aided function • Longer duration stimulus subject to hearing aid processing • Speech audibility - aim of hearing aid fitting. EUHA 2010, Hannover Speech Evoked CAEPs in Infants • Correlates well to behavioural thresholds (Purdy 2005; Tsu et al., 2002, Carter et al., 2010) • Significant correlation to functional measures of hearing aid performance in children (Golding et al., 2007) • Additional information for children with ANSD (Rance 2002) EUHA 2010, Hannover Speech evoked CAEPs in infants • Not widely used in a clinical environment. • Variable waveform morphology and latency particularly associated with infants (Ponton et al., 2000; Kushnerenko et al., 2002; Kurtzberg, 1984) • Maturation, • Mental alertness, • Movement, • Experienced eye needed to interpret waveforms (Ponton et al., 2000) EUHA 2010, Hannover Current Speech evoked CAEP assessment methods • Existing ERA equipment • Stimulus presented via soundfield • Aided and unaided assessment • WAV speech files – Various speech sounds – Various intensity levels • Experienced clinician interprets the results – Repetition of waveforms – Latency and morphology characteristics EUHA 2010, Hannover Current Speech Evoked CAEP assessment methods • HearLAB • Developed at NAL laboratories, Sydney, Australia • Speech stimulus sampled from running speech – /m/ (0.25 – 0.5 kHz), – /g/ (0.8 – 1.6 kHz), – /t/ (2 – 8kHz), • 3 intensity levels – Soft speech (55dB), – conversational speech (65dB), – loud speech (75dB) • Aided and unaided speech EUHA 2010, Hannover Hearlab Speech Stimulus 70.0 1/3 octave SPL 60.0 50.0 40.0 30.0 20.0 10.0 0.0 -10.0 -20.0 100 GAE TAE MAE ILTASS @ 65 1000 Frequency EUHA 2010, Hannover 10000 Automatic CAEP waveform detection • Automatic waveform detection • Statistical analysis Hotellings T2 (p-value) • ‘As good as if not better than the human eye’ • Normally hearing infants (Carter et al., 2010) – 30 dB SL = 85% correctly identified as present when speech was audible – 5% false alarm rate • Children with SNHL (Van Dun et al., submitted for publication) – 10 dB SL = 75% correctly identified as present when speech was audible – 5 % false alarm rate EUHA 2010, Hannover The HearLAB NAL: Dillon, Van Dun, Carter, Gardner-Berry EUHA 2010, Hannover CAEP infant assessment • Straightforward for an experienced paediatric audiologist • Infant awake and facing speaker • Fed, watered, nappy changed! • Appropriate play • Maintain alert state • Minimise myogenic noise • 3 electrode placement (vertex, mastoid, forehead (common) EUHA 2010, Hannover CAEP assessment EUHA 2010, Hannover HearLAB assessment screen NAL: Dillon, Van Dun, Carter, Gardner-Berry EUHA 2010, Hannover Child 1- verifying speech audibility with a softband BAHA • Child 1 has a right sided cleft lip and palate and developmental delay. • 8 weeks old – bilateral asymmetrical moderate to severe conductive hearing loss, confirmed using ABR. • 9 weeks – fitted with softband BAHA • 9 months old – unable to obtain reliable behavioural data due to developmental delay • Child 1 recently discovered BAHA as a new toy! Parents finding BAHA difficult to manage. • Unable to verify benefit of BAHA EUHA 2010, Hannover Child 1 EUHA 2010, Hannover Child 1 – unaided CAEP results EUHA 2010, Hannover Child 1 aided CAEP results EUHA 2010, Hannover Child 1 - outcome • Without BAHA • CAEPs present for /t/ at 65 dB • CAEP was not present for /g/ at 65 dB but present at 75 dB. • With BAHA • CAEP present for /g/ at 65 dB • Conclusion • The BAHA provides significant benefit by making a wider range of speech sounds audible at average conversational level. • Parents were reassured and encouraged by results -both unaided and aided. • Subsequent VRA behavioural assessment confirmed a bilateral moderate upward sloping conductive hearing loss EUHA 2010, Hannover Child 2 - verifying speech audibility with hearing aids • 7 weeks old- bilateral moderate to severe sloping sensorineural hearing loss confirmed using ABR • 9 weeks – fitted with Nios Micro hearing aids using sound recover • 8 months – reliable behavioural assessment confirmed ABR levels were accurate and stable • Required confirmation that full range of speech sounds were audible EUHA 2010, Hannover Child 2 EUHA 2010, Hannover Child 2 – Aided CAEP results EUHA 2010, Hannover Child 2 – outcome • CAEP was present for /g/ and /t/ at 65 and 55 dB with hearing aids in place. • Conclusion – Hearing aids are maintaining the audibility of speech at soft and louder levels in both mid and high frequencies. • Child 2’s parents – ‘we’re pleased to know his hearing aids are doing their job. It’s reassuring to see that he can hear speech’. EUHA 2010, Hannover Child 3- verifying high frequency speech audibility with hearing aids • • • • • 6 weeks old – confirmed bilateral mixed profound hearing loss 8 weeks old – fitted with bilateral hearing aids 2 years old- Bilateral grommets inserted Behavioural results confirmed bilateral severe hearing loss 2yrs 10 months - recently became very difficult to test reliably although results obtained indicate hearing levels are stable. • Parents are concerned that Child 3’s speech sounds ‘flat’ • Clinical need to confirm hearing aids are providing access to speech – particularly high frequencies. EUHA 2010, Hannover Child 3 – aided CAEP results EUHA 2010, Hannover Child 3 outcome • CAEP present for /t/ at 65dB. /m/ • CAEP absent for /m/ at 65 dB. • Child 3 is able to hear high frequency speech at least at conversational level • Parents and clinicians reassured re. hearing aid fitting. • 55 dB speech would have been useful but Child 3 became too distressed. To be completed at next appointment. EUHA 2010, Hannover Child 4 – verifying hearing aid prescription • Child 4 has downs syndrome • 8 months - diagnosed with bilateral moderate mixed hearing loss using ABR and fitted with bilateral hearing aids • 21 months - unable to obtain any reliable behavioural information. • Child 1 recently removing aids. • No up to date behavioural information to verify hearing aid prescription EUHA 2010, Hannover Child 4 – unaided CAEP results EUHA 2010, Hannover Child 4 – aided CAEP results (1) EUHA 2010, Hannover Child 4 - outcome • Unaided • CAEP absent using /t/ and /m/ 65 dB • Aided 1 • CAEP absent for /m/ at 65 dB and 75 dB, • Present for /t/ at 65dB • Response seen only to high frequency stimulus with current hearing aid prescription. • Changed hearing aid prescription to increase gain in low frequencies. • Aided 2 • CAEP present for /t/ at 65dB • CAEP absent for /m/ at 65dB • ABR repeated and showed a deterioration in hearing thresholds particularly in low frequencies. Hearing aid prescription altered and CAEP now present for both low and high freq speech at quiet and conversation speech sounds. EUHA 2010, Hannover Child 4 – Aided results (2) EUHA 2010, Hannover Child 5- confirm speech audibility with hearing aids. • 11 days old – whooping cough, ECMO treatment • Developmental delay • 13 months – bilateral severe to profound sensorineural hearing loss confirmed using behavioural and ABR data, hearing aid fitted. • 3 years old – speech and language therapist concerned that he is able to hear speech • Need to confirm speech audibility. EUHA 2010, Hannover Child 5 – Aided CAEP results EUHA 2010, Hannover Child 5 outcome • CAEP present for /t/ and /m/ at 65 dB with hearing aids • A range of speech is audible at average conversational level. • There is cortical activation to speech stimulus within the auditory cortex • Informs speech and language therapy EUHA 2010, Hannover Child 6- Is speech audible? • 3 years 9 months • Developmental delay including delayed speech production • Recent MRI confirmed brain damage • Behavioural assessments inconsistent but indicate an overall high frequency severe sensorineural hearing loss. • DPOAEs present bilaterally. • Can she hear high frequency speech? • Does she need a hearing aid? EUHA 2010, Hannover Child 6 – Unaided CAEP results EUHA 2010, Hannover Child 6 – conclusion • CAEP present to /t/ and /m/ at 65 and /t/, /g/ and /m/ at 55 dB • High frequency speech is audible at average and quiet conversational levels. • Parents reassured • Hearing aid not indicated • ? Implications for neurologist re. cortical activation. EUHA 2010, Hannover Child 7 – Auditory neuropathy spectrum disorder (ANSD) • Child 3 born 3 months premature • 5 months gestational age (g.a.) - diagnosed with Auditory neuropathy spectrum disorder (ANSD) using ABR and CMs. • 10 months g.a.- reliable behavioural assessment shows an asymmetrical moderate to severe hearing loss. • 10 months g.a. – Bilateral hearing aids fitted • Ongoing delayed speech development and attention difficulties. He uses total communication. • Parents report fluctuating understanding of speech • ABR shows fluctuating response • 6 years old – A referral for consideration of cochlear implantation has been made • Assessment of speech evoked CAEPs EUHA 2010, Hannover Child 7 – Aided CAEP results EUHA 2010, Hannover Child 7 - outcome • CAEP present for /t/ and /g/ at 65dB • Speech is audible for low and high frequency speech sounds at average conversational levels ……today. • Indicates potential for speech development….today. EUHA 2010, Hannover Are Speech evoked CAEPs useful in clinical practice? • Methodology straightforward and fun for an experienced paediatric audiologist and the parents. • Allow ~ 45 minute appointment • Children are awake! • ‘Real world’ stimulus. • They can complement current audiology good practice • Infants too young for behavioural assessment • ANSD • Children for whom speech and or behavioural testing is not reliable. EUHA 2010, Hannover Are Speech evoked CAEPs useful in clinical practice? • Inform parents and clinician re. child’s access to speech • Verify changes in hearing aid prescription. • Indicate further assessment or onward referral • Cochlear implant, • ABR under general anaesthetic/sedation/ evening home visit • Inform other health professionals e.g. speech and language therapy, neurology. • Indicate maturity of auditory system • Auditory training EUHA 2010, Hannover Is the Hearlab a useful clinical tool? • Yes! • Automatic waveform detection makes speech evoked CAEP assessment more accessible to audiologists whatever their experience. • Analysis is consistent across clinicians. • Speech sounds cover relevant speech range both for frequency and intensity. • Instant results! EUHA 2010, Hannover Is the Hearlab a useful clinical tool? • However…. • Automatic waveform detection could restrict new applications of infant CAEPs • Software upgrades available to reflect up to date research • Clinicians may want a wider range of speech sounds and intensity levels. • Software updates may be available should the clinical need arise • Portability. • Overall, It has become an invaluable addition to our clinical toolkit. EUHA 2010, Hannover Thank you! • Dr Laura Booth, Clinical Scientist, Audiology Department, Reading, U.K. • Dr Bram Van Dun, NAL laboratories, Sydney, Australia • Dr Rob Low, Clinical Scientist, Audiology department, Brighton. EUHA 2010, Hannover