Update on Auditory Electrophysiology James W. Hall III, PhD Adjunct Professor Nova Southeastern University Salus University University of Florida Extraordinary

Update on Auditory Electrophysiology

James W. Hall III, PhDJames W. Hall III, PhD

Adjunct ProfessorAdjunct Professor

Nova Southeastern UniversityNova Southeastern University

Salus UniversitySalus University

University of FloridaUniversity of Florida

Extraordinary ProfessorExtraordinary Professor

University of Pretoria South AfricaUniversity of Pretoria South Africa

[email protected] www.audiologyworld.net

James W. Hall III Audiology Consulting, LLCJames W. Hall III Audiology Consulting, LLC

Introduction to auditory electrophysiology … 85-years of Introduction to auditory electrophysiology … 85-years of research and clinical applicationresearch and clinical application

Electrocochleography (ECochG) … It’s for much more than Electrocochleography (ECochG) … It’s for much more than just the diagnosis of Meniere’s Diseasejust the diagnosis of Meniere’s Disease

ABR … Over 4000 peer-reviewed publications in the past ABR … Over 4000 peer-reviewed publications in the past decade alonedecade alone

ASSR … Filling an important niche in auditory assessment of ASSR … Filling an important niche in auditory assessment of children and adultschildren and adults

Cortical auditory evoked responses … Now we’re really Cortical auditory evoked responses … Now we’re really assessing hearingassessing hearing

Summary, Questions & AnswersSummary, Questions & Answers


Cortical auditory evoked Cortical auditory evoked responsesresponsesP300 response and other P300 response and other

cognitive evoked responsescognitive evoked responsesAuditory late response (ALR)Auditory late response (ALR)Auditory middle latency Auditory middle latency

response (AMLR)response (AMLR) Auditory steady state response Auditory steady state response

(ASSR)(ASSR) Auditory brainstem response Auditory brainstem response

(ABR)(ABR) Electrocochleography (ECochG)Electrocochleography (ECochG)

Update on Auditory Electrophysiology:Objective Assessment from Cochlea to Cortex

Update on Auditory Electrophysiology: Behavioral Audiometry Isn’t Always Enough

Auditory electrophysiology is feasible in patients who Auditory electrophysiology is feasible in patients who cannot be assessed validly with behavioral techniquescannot be assessed validly with behavioral techniques Newborn and older infantsNewborn and older infants Difficult-to-test childrenDifficult-to-test children Sick patientsSick patients False and exaggerated hearing loss, e.g.,False and exaggerated hearing loss, e.g.,

PseudohypacusisPseudohypacusis Functional hearing lossFunctional hearing loss Non-organic hearing lossNon-organic hearing loss ““CrocksCrocks””

Auditory electrophysiology is feasible in sleeping and Auditory electrophysiology is feasible in sleeping and unconscious patientsunconscious patientsNaturally sleeping children or adultsNaturally sleeping children or adultsChildren who are sedated or anesthetized for auditory Children who are sedated or anesthetized for auditory

assessmentassessmentAnesthetized patients undergoing surgery who require Anesthetized patients undergoing surgery who require

intra-operative neurophysiologic monitoring intra-operative neurophysiologic monitoring Comatose head-injured patients who require intensive Comatose head-injured patients who require intensive

care unit monitoringcare unit monitoring Automated auditory electrophysiology procedures can be Automated auditory electrophysiology procedures can be

performed by non-audiologic personnel for early newborn performed by non-audiologic personnel for early newborn hearing screening (e.g., automated ABR)hearing screening (e.g., automated ABR)

Update on Auditory Electrophysiology: Behavioral Audiometry Isn’t Always Enough

Auditory electrophysiology has greater Auditory electrophysiology has greater sensitivitysensitivity to to auditory function than behavioral audiometry (e.g., ABR in auditory function than behavioral audiometry (e.g., ABR in early identification of retrocochlear dysfunction)early identification of retrocochlear dysfunction)

Auditory electrophysiology has greater Auditory electrophysiology has greater site specificity site specificity to to auditory function than behavioral audiometry, e.g., auditory function than behavioral audiometry, e.g., ECochG differentiates sites of dysfunction in auditory ECochG differentiates sites of dysfunction in auditory

neuropathy spectrum disorder (ANSD)neuropathy spectrum disorder (ANSD)ABR differentiates among cochlear, eighth nerve, and ABR differentiates among cochlear, eighth nerve, and

brainstem sites of dysfunctionbrainstem sites of dysfunctionCortical auditory evoked responses specify site of Cortical auditory evoked responses specify site of

dysfunction with central auditory nervous systemdysfunction with central auditory nervous system

Update on Auditory Electrophysiology: Behavioral Audiometry Isn’t Always Enough (2)



ABR … Over 4000 peer-reviewed publications in the past ABR … Over 4000 peer-reviewed publications in the past decade alonedecade alone

ASSR … Filling an important niche in children and adultsASSR … Filling an important niche in children and adults Cortical auditory evoked responses … Now we’re really Cortical auditory evoked responses … Now we’re really

assessing hearingassessing hearing Summary, Questions & AnswersSummary, Questions & Answers


Wever EG and Bray CW. Wever EG and Bray CW. 1930.1930. Action currents in the Action currents in the auditory nerve in response auditory nerve in response to acoustic stimulation. to acoustic stimulation. Proceedings of the National Proceedings of the National Acad of Science (USA) 16: Acad of Science (USA) 16: 344-350.344-350.

Wever EG and Bray CW. 1930.Wever EG and Bray CW. 1930. Auditory nerve impulses. Auditory nerve impulses. Science 71: 215.Science 71: 215.

Original Description of Electrocochleography (ECochG)

E. Glen Weaver, Ph.D.(October 16, 1902 — September 4, 1991)

ELECTROCOCHLEOGRAPHY:85 Years Old and Still Clinically Important!

Time in Years

1930

Wever & Bray(CM in cat)

Fromm et al(CM in human)

1935

Davis(SP)

1950 1954

Tasaki(AP in human)

Ruben(CM & AP clinically)

1960

Yoshie, Portmann

(TT CM & AP)

Coats(EAC)

1967 1974

Coats, Eggermont,

Gibson(Dx of MD)

1990

Hall(I/O)

1996

Various(ANSD, CI)

Electrocochleography (ECochG):Generators

Cochlear microphonic (CM)Cochlear microphonic (CM) Outer hair cellsOuter hair cells Receptor potentialsReceptor potentials

Summating potential (SP) Summating potential (SP) Inner hair cells (> 50%)Inner hair cells (> 50%) Outer hair cellsOuter hair cells Organ of CortiOrgan of Corti

Action potential (AP)Action potential (AP) Afferent fibers in distal 8th cranial nerveAfferent fibers in distal 8th cranial nerve Spiral ganglionSpiral ganglion

SP CMAP

Electrocochleography (ECochG):Stimulus Polarity is Important

ECochG Electrode Options: The Closer to the Cochlea, the Better

TipTrode

TM Electrode

Trans-Tympanic Promontory Electrode

TIPtrode: Part Transducer and Part Electrode

Sub-Dermal Needle Electrode for Trans-Tympanic Promontory ECochG Recording

ELECTROCOCHLEOGRAPHY (ECochG):“Traditional” Clinical Applications

Estimation of auditory sensitivity in young and difficult-to-Estimation of auditory sensitivity in young and difficult-to-test childrentest children

Enhancement of wave I in ABR measurementEnhancement of wave I in ABR measurement Enhance inter-wave latency analysis in neuro-Enhance inter-wave latency analysis in neuro-

diagnosisdiagnosis Confirm ear-specific test findings for AC and BC ABR Confirm ear-specific test findings for AC and BC ABR

(wave I is a biological marker for ipsilateral 8(wave I is a biological marker for ipsilateral 8thth nerve) nerve) Minimize the need for masking of non-test earMinimize the need for masking of non-test ear

Intra-operative neurophysiological monitoringIntra-operative neurophysiological monitoring Prompt documentation of cochlear statusPrompt documentation of cochlear status Enhance inter-wave latency analysisEnhance inter-wave latency analysis

Diagnosis of MeniereDiagnosis of Meniere’’s diseases disease

“Traditional” Clinical Applications of ECochG:Enhanced Wave I and I/O Monitoring

ECochG in Diagnosis of Meniere’s Disease: Abnormally Large SP/AP Ratio

Coats AC (1981). The summating potential in Meniere’s and non-Meniere’s ears. Archives of Otolaryngology, 107, 199-208

Pou, Hirsch, Durrant et al. (1996). Efficacy of tympanic ECochG in the Pou, Hirsch, Durrant et al. (1996). Efficacy of tympanic ECochG in the diagnosis of endolymphatic hydrops. diagnosis of endolymphatic hydrops. AJO, 17AJO, 17

Levine, Margolis, Daly. (1998). Use of ECochG in the diagnosis of Levine, Margolis, Daly. (1998). Use of ECochG in the diagnosis of MeniereMeniere’’s Disease. s Disease. Laryngoscope,108Laryngoscope,108

Orchik, Shea, Ge. (1998). Summating potential and action potential Orchik, Shea, Ge. (1998). Summating potential and action potential ratio in Meniereratio in Meniere’’s Disease before and after treatment. s Disease before and after treatment. AJO, 19AJO, 19

Zack-Williams et al (2012). A comparison of ECochG and high-pass Zack-Williams et al (2012). A comparison of ECochG and high-pass noise masking of ABR for diagnosis of Meniere’s disease. noise masking of ABR for diagnosis of Meniere’s disease. Int J Int J Audiol, 51, Audiol, 51, 783-787783-787

Claes et al (2013). Does “cochlear Meniere’s disease exist? An Claes et al (2013). Does “cochlear Meniere’s disease exist? An ECochG and audiometric studyECochG and audiometric study. Audiol Neurotol, 18. Audiol Neurotol, 18, 63-70, 63-70

Oh et al (2014). Can we use ECochG as a clinical tool in the diagnosis Oh et al (2014). Can we use ECochG as a clinical tool in the diagnosis of Meniere’s disease during the early symptomatic period? of Meniere’s disease during the early symptomatic period? Acta Acta Otolaryngol, 134, Otolaryngol, 134, 771-775771-775

ELECTROCOCHLEOGRAPHY: Selected Papers(PubMed Search on 9/9/14 at www.nlm.nih.gov with key words “Meniere’s

Disease” and “electrocochleography” showed > 400 publications)

ELECTROCOCHLEOGRAPHY (ECochG):More Recent Clinical Applications

Auditory dysfunction in tinnitusAuditory dysfunction in tinnitus Cochlear and round window (Soundbridge) implants Cochlear and round window (Soundbridge) implants

Round window and intra-cochlear recordingsRound window and intra-cochlear recordingsIntra-operative verification of cochlear implant functionIntra-operative verification of cochlear implant functionIntra-operative documentation of low frequency cochlear Intra-operative documentation of low frequency cochlear

function during electrode insertionfunction during electrode insertionPrediction of speech perception outcomesPrediction of speech perception outcomes

Diagnosis of auditory neuropathy spectrum disorder (ANSD)Diagnosis of auditory neuropathy spectrum disorder (ANSD)Differentiation of pre- versus post-synaptic auditory Differentiation of pre- versus post-synaptic auditory

dysfunctiondysfunction

ELECTROCOCHLEOGRAPHY (ECochG):Cochlear Implant Applications

Harris et al (2011). Preliminary results and technique for Harris et al (2011). Preliminary results and technique for electrophysiological intra-operative monitoring of residual hearing electrophysiological intra-operative monitoring of residual hearing during cochlear implantation. during cochlear implantation. Cochlear Implants Int, 12, Cochlear Implants Int, 12, 209-215209-215

Mandala et al (2012). Electrocochleography during cochlear Mandala et al (2012). Electrocochleography during cochlear implantation for hearing preservation. implantation for hearing preservation. Otolaryngol Head Neck Surg, Otolaryngol Head Neck Surg, 146, 774-781146, 774-781

Calloway et al (2014). Intracochlear electrocochleography during Calloway et al (2014). Intracochlear electrocochleography during cochlear implantation. cochlear implantation. Otol Neurotol, 35Otol Neurotol, 35, 1451-7, 1451-7

McClellan et al (2014). Round window electrocochleography and McClellan et al (2014). Round window electrocochleography and speech perception outcomes in adult cochlear implant subjects: speech perception outcomes in adult cochlear implant subjects: Comparison with audiometric and biographical information. Comparison with audiometric and biographical information. Otol Otol Neurotol, EpubNeurotol, Epub

Role of ECochG in the Diagnosis and Management of Auditory Neuropathy Spectrum Disorder (ANSD)

Cerebello-pontine angle (CPA)

Internal Auditory Canal(Auditory Nerve)

Spiral ganglion cells

IHC - 8th CN Synapse (glutamate)

Inner hair cells

Outer hair cells

Guidelines for the Identification and Guidelines for the Identification and Management of Infants and Young Management of Infants and Young Children with Auditory Neuropathy (2010).Children with Auditory Neuropathy (2010).

Panel of Experts: Panel of Experts: Yvonne Sininger, Ph.D.Yvonne Sininger, Ph.D.Arnold Starr, M.D.Arnold Starr, M.D.Christine Petit, M.D., Ph.D.Christine Petit, M.D., Ph.D.Gary Rance, Ph.D.Gary Rance, Ph.D.Barbara Cone, Ph.D.Barbara Cone, Ph.D.Kai Uus, M.D., Ph.D.Kai Uus, M.D., Ph.D.Patricia Roush, Au.D.Patricia Roush, Au.D.Jon Shallop, Ph.D.Jon Shallop, Ph.D.Charles Berlin, Ph.D.Charles Berlin, Ph.D.

Electrococheography (ECochG): Diagnosis of Auditory Neuropathy Spectrum Disorder

Identification and Diagnosis of Auditory Neuropathy Spectrum Disorder (ANSD):

Minimal Test Battery (2010 ANSD Guidelines)

Tests of cochlear hair cell functionTests of cochlear hair cell functionOtoacoustic emissions (OAEs)Otoacoustic emissions (OAEs)Cochlear microphonic (ECochG Cochlear microphonic (ECochG and ABR)and ABR)

CM may be present when OAEs are absent (e.g., with middle ear CM may be present when OAEs are absent (e.g., with middle ear dysfunction)dysfunction)

Tests of auditory nerve functionTests of auditory nerve functionABR for high intensity click stimulation (e.g., 80 to 90 dB nHL) with ABR for high intensity click stimulation (e.g., 80 to 90 dB nHL) with

separate averages for:separate averages for: Rarefaction stimulus polarityRarefaction stimulus polarity Condensation stimulus polarityCondensation stimulus polarity

Additional tests Additional tests Acoustic reflex measurement (generally acoustic reflexes are absent in Acoustic reflex measurement (generally acoustic reflexes are absent in

ANSD)ANSD)Suppression of otoacoustic emissions (abnormal, e.g, no suppression Suppression of otoacoustic emissions (abnormal, e.g, no suppression

in ANSD)in ANSD)

Essential Role of Electrocochleography (ECochG) in the Diagnosis and Management of Auditory Neuropathy Spectrum Disorder (ANSD): Medical diagnoses (1)

Perinatal DiseasesPerinatal DiseasesHyperbilirubinemiaHyperbilirubinemiaHypoxic insultsHypoxic insultsIschemic insultsIschemic insultsPrematurity Prematurity

Neurological DisordersNeurological DisordersDemyelinating diseasesDemyelinating diseasesHydrocephalusHydrocephalusImmune disorders, e.g., Guillain-Barre sydromeImmune disorders, e.g., Guillain-Barre sydromeInflammatory neuropathiesInflammatory neuropathiesSevere developmental delaySevere developmental delay

Neuro-metabolic diseasesNeuro-metabolic diseases Genetic and Hereditary EtiologiesGenetic and Hereditary Etiologies

Family historyFamily historyConnexin mutations, e.g., GJB3 (D66del)Connexin mutations, e.g., GJB3 (D66del)Otoferlin (OTOF) geneOtoferlin (OTOF) geneNon-syndromic recessive auditory neuropathyNon-syndromic recessive auditory neuropathyHereditary motor sensory neuropathies (HMSN), e.g., Hereditary motor sensory neuropathies (HMSN), e.g.,

Charcot-Marie-Tooth syndromeCharcot-Marie-Tooth syndromeLeberLeber’’s hereditary optic neuropathys hereditary optic neuropathyWaardenburgWaardenburg’’s syndromes syndromeNeurogenerative diseases, e.g., FriedreichNeurogenerative diseases, e.g., Friedreich’’s ataxias ataxia

Mitochondrial disorders, e.g., mitochondrial enzymatic Mitochondrial disorders, e.g., mitochondrial enzymatic defectdefect

Essential Role of Electrocochleography (ECochG) in the Diagnosis and Management of Auditory Neuropathy Spectrum Disorder (ANSD): Medical diagnoses (2)

Other Procedures Important in the Diagnosis and Management of Auditory Neuropathy Spectrum Disorder (2008 Guidelines)

Components of assessmentComponents of assessmentPediatric and developmental historyPediatric and developmental historyOtologic evaluation, plusOtologic evaluation, plus

Imaging of cochlea with CT Imaging of cochlea with CT Imaging auditory nerve with MRIImaging auditory nerve with MRI

Medical genetics evaluationMedical genetics evaluationOphthalmologic evaluationOphthalmologic evaluationNeurological evaluation to assess:Neurological evaluation to assess:

Peripheral nerve functionPeripheral nerve functionCranial nerve functionCranial nerve function

Communication assessmentCommunication assessment

Comprehensive Assessment of Auditory Neuropathy Spectrum Disorder (ANSD):

MRI of Auditory Nerve

Brainstem and inner ear abnormalities in children with auditory Brainstem and inner ear abnormalities in children with auditory neuropathy spectrum disorder and cochlear nerve deficiency. Huang neuropathy spectrum disorder and cochlear nerve deficiency. Huang et al. (UNC). American J Radiol, 31, 2010et al. (UNC). American J Radiol, 31, 2010• CND was identified in 33.0% of children and 26.9% of ears with CND was identified in 33.0% of children and 26.9% of ears with

ANSD ANSD • Significantly more patients with bilateral CND had intracranial Significantly more patients with bilateral CND had intracranial

abnormalities than those with unilateral CND (60.0% versus 15.8%). abnormalities than those with unilateral CND (60.0% versus 15.8%). Unilateral auditory neuropathy caused by cochlear nerve deficiency. Unilateral auditory neuropathy caused by cochlear nerve deficiency.

Liu et al (China)Liu et al (China). Int J Otolaryngol. Int J Otolaryngol, 2012., 2012.• Cochlear nerve deficiency can be seen by electrophysiological Cochlear nerve deficiency can be seen by electrophysiological

evidence and may be a significant cause of unilateral AN. evidence and may be a significant cause of unilateral AN. • Inclined sagittal MRI of the internal auditory canal is recommended Inclined sagittal MRI of the internal auditory canal is recommended

for the diagnosis of this disorder. for the diagnosis of this disorder.

Auditory Neuropathy Spectrum Disorder ANSD): Defining Site of Dysfunction is the Key to Accurate Diagnosis

and Effective Management

Summating potential (SP) Summating potential (SP)

Cochlear microphonic (CM) Cochlear microphonic (CM)

Action potential (AP) Action potential (AP)

Santarelli, Starr, Michalewski & Arlsan (2008). Neural and receptor Santarelli, Starr, Michalewski & Arlsan (2008). Neural and receptor cochlear potentials obtained by transtympanic cochlear potentials obtained by transtympanic electrocochleography in auditory neuropathy. Clinical electrocochleography in auditory neuropathy. Clinical Neurophysiology, 119, 1028-1041.Neurophysiology, 119, 1028-1041.8 subjects (with AN versus 16 normal subjects (5-48 years)8 subjects (with AN versus 16 normal subjects (5-48 years)Diagnosis: Presence of DPOAEs and absence of ABRDiagnosis: Presence of DPOAEs and absence of ABREnlarged CM in AN patients Enlarged CM in AN patients Etiology Etiology

Hereditary (3)Hereditary (3) Immunolological (3)Immunolological (3)Degenerative (1)Degenerative (1) Congenital (1)Congenital (1)

ECochG measures includedECochG measures includedCMCMSPSPAPAP

ECochG in ANSD: Refining Diagnosis of Site of Dysfunction

McMahon, Patuzzi, Gibson & Sanli. (2008) Frequency-specific McMahon, Patuzzi, Gibson & Sanli. (2008) Frequency-specific electrocochleography indicates that presynaptic and electrocochleography indicates that presynaptic and postsynaptic mechanisms of auditory neuropathy exist. Ear & postsynaptic mechanisms of auditory neuropathy exist. Ear & Hearing, 29, 314-325.Hearing, 29, 314-325.SubjectsSubjects

14 subjects (7 male and 7 female) with AN versus 2 14 subjects (7 male and 7 female) with AN versus 2 normal subjects( Dx at 3-24 months)normal subjects( Dx at 3-24 months)

Diagnosis: large CM potentials and absent ABR Diagnosis: large CM potentials and absent ABR Genetic etiology for 6 subjectsGenetic etiology for 6 subjectsSevere to profound audiometric thresholdsSevere to profound audiometric thresholdsAll subjects received cochlear implantsAll subjects received cochlear implants

ECochG recorded with non-inverting “golf club” electrode ECochG recorded with non-inverting “golf club” electrode near round window electrode via myringotomynear round window electrode via myringotomy

ECochG in ANSD: Refining Diagnosis of Site of Dysfunction (1)

ECochG in ANSD: Examples of ECochG Components(McMahon et al, 2008)

Analysis Time Analysis Time 10 ms 10 ms

CondensationCondensation

Rarefaction Rarefaction

CM CM

SP SP

N1 N1

N2 N2

Alternating Alternating

DP (dentritic potential)DP (dentritic potential)

McMahon, Patuzzi, Gibson & Sanli. (2008) Frequency-McMahon, Patuzzi, Gibson & Sanli. (2008) Frequency-specific electrocochleography indicates that presynaptic specific electrocochleography indicates that presynaptic and postsynaptic mechanisms of auditory neuropathy and postsynaptic mechanisms of auditory neuropathy exist. Ear & Hearing, 29, 314-325.exist. Ear & Hearing, 29, 314-325.Purpose of study was to better define physiology Purpose of study was to better define physiology

mechanisms of AN to guide management including mechanisms of AN to guide management including cochlear implantationcochlear implantation

ECochG in AN consistent with: ECochG in AN consistent with: Pre-synaptic mechanism (abnormal SP) = good EABR Pre-synaptic mechanism (abnormal SP) = good EABR

and CI benefitand CI benefitPost-synaptic mechanism (normal SP + dentritic Post-synaptic mechanism (normal SP + dentritic

potential) but no AP = poor or absent EABR and poor potential) but no AP = poor or absent EABR and poor CI benefitCI benefit

ECochG in ANSD: Refining Diagnosis of Site of Dysfunction (2)



ABR … Over 11,000 peer-reviewed publications with > 4000 ABR … Over 11,000 peer-reviewed publications with > 4000 within the past decade alone!within the past decade alone!

ASSR … Filling an important niche in children and adultsASSR … Filling an important niche in children and adults Cortical auditory evoked responses … Now we’re really Cortical auditory evoked responses … Now we’re really

assessing hearingassessing hearing Summary, Questions & AnswersSummary, Questions & Answers


The First Comprehensive Description of the Auditory Brainstem Response (ABR) in Humans

Jewett D and Williston J. Jewett D and Williston J.

Auditory evoked far fields Auditory evoked far fields averaged from the scalp of averaged from the scalp of humans. humans.

Brain 4: 681-696, 1971.Brain 4: 681-696, 1971.

Don Jewett Don Jewett

Jewett & Williston, 1971Normal Subject Waveform Consistency …

the Ideal Clinical Tool

Auditory Brainstem Response (ABR):An Evidence-Based Test Protocol

StimulusClicks

Tone burstsSpeech

ElectrodesAuditory Evoked

Response System Filters

AmplifiersSignal Averager

I III V

8 ms

0.5 uV

Generators of ABR Waves:A Far-Field Response from Sub-Cortical Auditory Pathways

Generator sitesGenerator sites I = Distal auditory nerveI = Distal auditory nerve III = Caudal brainstemIII = Caudal brainstem V = Lateral lemniscusV = Lateral lemniscus

Relation between anatomy and Relation between anatomy and responseresponse Multiple structures contribute to Multiple structures contribute to

single waves single waves Waves reflect axonal activityWaves reflect axonal activity

LateralityLaterality Wave I = ipsilateral to stimulusWave I = ipsilateral to stimulus Waves III and V = contralateral to Waves III and V = contralateral to

stimulusstimulus

Robert Galambos, MD, PhDDirector of the Laboratory (Yale University) Where the ABR was

“Discovered” and Pioneer of Early Pediatric Applications of ABR

19741974: Hecox KE & Galambos R. Brain : Hecox KE & Galambos R. Brain stem auditory evoked responses in stem auditory evoked responses in human infants and adults. human infants and adults. Archives Archives of Otolaryngology 99.of Otolaryngology 99.

19751975: Schulman-Galambos C. & : Schulman-Galambos C. & Galambos R. Brain stem evoked Galambos R. Brain stem evoked responses in premature infants. responses in premature infants. JSHR 18JSHR 18..

19791979: Schulman-Galambos C. & : Schulman-Galambos C. & Galambos R. Brain stem evoked Galambos R. Brain stem evoked response audiometry in newborn response audiometry in newborn hearing screening. hearing screening. Archives of Archives of Otolaryngology 105Otolaryngology 105::

James Jerger, Ph.D.“Father of Diagnostic Audiology”

Baylor College of Medicine and Methodist Hospital, Houston TX

Larry Mauldin Circa 1975Helped Build

Early AER System

The Cross-Check Principle in Pediatric Audiology(Jerger J & Hayes D. Arch Otolaryngol 102: 1976)

The Cross-Check Principle Pediatric Audiology(Jerger J & Hayes D. Arch Otolaryngol 102: 1976)

““We have found than simply observing the auditory behavior of children We have found than simply observing the auditory behavior of children does not always yield an accurate description of hearing lossdoes not always yield an accurate description of hearing loss””……

““The basic operation of this principle is that no result be accepted until it The basic operation of this principle is that no result be accepted until it is confirmed by an independent measure.is confirmed by an independent measure.””

Test Battery:Test Battery:• Behavioral audiometry Behavioral audiometry • Immittance (impedance) measurementsImmittance (impedance) measurements

TympanometryTympanometryAcoustic reflexes (contralateral only with SPAR)Acoustic reflexes (contralateral only with SPAR)

• Brainstem-evoked response audiometry (BSER now ABR)Brainstem-evoked response audiometry (BSER now ABR)Click stimulus air conduction Click stimulus air conduction Click stimulus bone conductionClick stimulus bone conduction

1948 Vintage Audiologist Using 1980 Model Nicolet CA-1000 with Infamous “Aural Dome” TDH Earphones

40 Years of Evidence-Based ABR Applications (> 11K Pubs) Since Hecox & Galambos (1974)

Newborn hearing screeningNewborn hearing screening Diagnosis of hearing loss in infants and young childrenDiagnosis of hearing loss in infants and young children

Frequency-specific estimation of auditory sensitivityFrequency-specific estimation of auditory sensitivityDifferentiation of conductive vs. sensorineural hearing lossDifferentiation of conductive vs. sensorineural hearing loss

Neuro-diagnosis of retrocochlear and brainstem disorders in Neuro-diagnosis of retrocochlear and brainstem disorders in children and adultschildren and adults

Neurophysiological monitoringNeurophysiological monitoringIntra-operative during surgical proceduresIntra-operative during surgical proceduresNeuro-intensive care unit in head injured patientsNeuro-intensive care unit in head injured patients

Description of neural representation of auditory processing in Description of neural representation of auditory processing in brainstem with speech evoked ABRbrainstem with speech evoked ABR

Auditory Brainstem Response: The Research Continues Unabated

(> 350 Publications Each Year)

Descriptions of ABR applications in new clinical entities and Descriptions of ABR applications in new clinical entities and populationspopulations

Replications of traditional ABR studies in countries with Replications of traditional ABR studies in countries with emerging or rapidly growing audiology communities, e.g., emerging or rapidly growing audiology communities, e.g., India, China, Brazil, Brazil, IranIndia, China, Brazil, Brazil, Iran

Studies of auditory processing with speech evoked ABRStudies of auditory processing with speech evoked ABR Investigations of new technology for ABR measurementInvestigations of new technology for ABR measurement

Auditory Brainstem Response:Applications in New Clinical Entities and Populations (Plus new technology and studies in emerging audiology countries)

Elkabariti et al (2014). Speech evoked auditory brainstem Elkabariti et al (2014). Speech evoked auditory brainstem response findings in children with epilepsy. response findings in children with epilepsy. Int J Pedi ORL, 78, Int J Pedi ORL, 78, 1277-80 (Egypt)1277-80 (Egypt)

Talebi & Rezazadeh (2012). The role of ABR in diagnosing Talebi & Rezazadeh (2012). The role of ABR in diagnosing auditory impairments of Dejerine-Sottasauditory impairments of Dejerine-Sottas. Int J Pedi ORL, 76, . Int J Pedi ORL, 76, 915-917 (Iran)915-917 (Iran)

Jecmenica & Opancina (2014). Characteristics of brain stem Jecmenica & Opancina (2014). Characteristics of brain stem auditory evoked potentials in children with hearing impairment auditory evoked potentials in children with hearing impairment due to infectious diseases. due to infectious diseases. J Child NeurolJ Child Neurol, Epub (Serbia), Epub (Serbia)

Auditory Brainstem Response:Applications in New Clinical Entities and Populations

Allen & Allan (2014). Auditory processing disorders: Allen & Allan (2014). Auditory processing disorders: Relationship to cognitive processes and underlying neural Relationship to cognitive processes and underlying neural integrity. integrity. Int J Pedi ORL, 78Int J Pedi ORL, 78, 198-208 (click ABR and ARs), 198-208 (click ABR and ARs)

Hasani & Jafari (2013). Effect of infant prematurity on ABR at Hasani & Jafari (2013). Effect of infant prematurity on ABR at preschool age. preschool age. Iran J ORL, 25Iran J ORL, 25, 107-114 (Iran), 107-114 (Iran)

Kouni et al (2013). Brainstem auditory evoked potentials for Kouni et al (2013). Brainstem auditory evoked potentials for acoustic clicks and complex verbal sounds in young adults with acoustic clicks and complex verbal sounds in young adults with learning disabilitieslearning disabilities. Am J Otolaryngol, 34. Am J Otolaryngol, 34, 646-651 (Greece), 646-651 (Greece)

Quevedo et al (2012). Auditory brainstem response in gas Quevedo et al (2012). Auditory brainstem response in gas station attendants. station attendants. Brazilian J ORL, 78Brazilian J ORL, 78, 63-68, 63-68

Weich et al (2012). Brain stem evoked response audiometry in Weich et al (2012). Brain stem evoked response audiometry in former drug users. former drug users. Brazilian J ORL, 78Brazilian J ORL, 78, 90-96, 90-96

Auditory Brainstem Response:Revisiting Old Research Topics

Stevens et al (2013). ABR in newborns: Effects of electrode Stevens et al (2013). ABR in newborns: Effects of electrode configuration, stimulus rate, and EEG rejection levels on test configuration, stimulus rate, and EEG rejection levels on test efficiency. efficiency. Int J Audiol, 52, Int J Audiol, 52, 706-712706-712

Liu & Liu (2013). Hearing screening and diagnosis in a large Liu & Liu (2013). Hearing screening and diagnosis in a large sample of infants in central China. sample of infants in central China. J Med Screen, 20J Med Screen, 20, 21-26 (N = , 21-26 (N = 11,894)11,894)

Vinodh et al (2014) Reversibility of brainstem evoked response Vinodh et al (2014) Reversibility of brainstem evoked response audiometry abnormalities at 3 months in term newborns with audiometry abnormalities at 3 months in term newborns with hyperbilirubinemia. hyperbilirubinemia. Indian Pediatr, 51Indian Pediatr, 51, 134-135 (India), 134-135 (India)

Jiang & Wilkinson (2014). Impaired function of the ABR in term Jiang & Wilkinson (2014). Impaired function of the ABR in term neonates with hyperbilirubinemia. neonates with hyperbilirubinemia. Brain Dev, 36Brain Dev, 36, 212-218 , 212-218 (China)(China)

Auditory Brainstem Response:Revisiting Old Research Topics

Mann et al (2012). Brainstem auditory evoked responses in Mann et al (2012). Brainstem auditory evoked responses in different phases of menstrual cycle. different phases of menstrual cycle. J Clin Diagn Res, 6, J Clin Diagn Res, 6, 1640-1640-1643 1643 [Do you remember? [Do you remember? Doty RL, Hall JW III, Flickinger GL, Sondheimer SJ. Cyclical changes in olfactory and auditory sensitivity during the menstrual cycle: No attenuation by oral contraception medication. In Olfaction and Endocrine Regulation. Breipohl H (ed). London: I.R.L. Press, 1982]

Rosa et al (2014). ABR: Reference-values for age. Rosa et al (2014). ABR: Reference-values for age. Codas, 26, Codas, 26, 117-121 (Brazil) Naves et al (2012). Assessment of inter-117-121 (Brazil) Naves et al (2012). Assessment of inter-examiner agreement and variability in the manual classification examiner agreement and variability in the manual classification of ABR. of ABR. Biomed Eng Onine, 22Biomed Eng Onine, 22, 11-16, 11-16

Koors, Thacker & Coelho (2013). ABR in the diagnosis of Koors, Thacker & Coelho (2013). ABR in the diagnosis of vestibular schwannomas: A meta-analysis. Am J Otolaryngol, vestibular schwannomas: A meta-analysis. Am J Otolaryngol, 34, 34, 195-204195-204

Auditory Brainstem Response:Innovative New Applications

Ramkumar, Hall et al (2013). Tele-ABR using a satellite connection Ramkumar, Hall et al (2013). Tele-ABR using a satellite connection in a mobile van for newborn hearing testingin a mobile van for newborn hearing testing. J Telemed Telecare, . J Telemed Telecare, 19, 233-23719, 233-237

Auditory Brainstem Response:Speech Evoked ABR Assessment of Auditory Processing

Rance et al (2012). Auditory processing deficits in individuals Rance et al (2012). Auditory processing deficits in individuals with open-angle glaucomawith open-angle glaucoma. Int J Audiol, 51. Int J Audiol, 51, 10-15, 10-15

Fujihara & Shiraishi (2014). Correlations between word Fujihara & Shiraishi (2014). Correlations between word intelligibility under reverberation and speech ABRs in elderly intelligibility under reverberation and speech ABRs in elderly listeners. listeners. Clin Neurophysiol, Epub Clin Neurophysiol, Epub (Japan)(Japan)

Malveri et al (2014). Brainstem response to speech and non-Malveri et al (2014). Brainstem response to speech and non-speech stimuli in children with learning problemsspeech stimuli in children with learning problems. Hear Res, . Hear Res, 313, Epub (313, Epub (Iran)Iran)

Krisnamurti et al (2013). A case study of the changes in the Krisnamurti et al (2013). A case study of the changes in the speech-evoked ABR associated with auditory training in speech-evoked ABR associated with auditory training in children with auditory processing disorders. children with auditory processing disorders. Int J Pedi ORL, Int J Pedi ORL, 77, 77, 594-604594-604

Auditory Brainstem Response:Speech Evoked ABR Assessment of Auditory Processing

(www.brainvolts.northwestern.edu)

Skoe, Krizman, Spitzer & Skoe, Krizman, Spitzer & Kraus Kraus (2013). The auditory (2013). The auditory brainstem is a barometer of brainstem is a barometer of rapid auditory learning. rapid auditory learning. Neuroscience, 243Neuroscience, 243, 104-114, 104-114

Hornickel, Lin & Hornickel, Lin & Kraus Kraus (2013). Speech-evoked (2013). Speech-evoked ABRs reflect familial and ABRs reflect familial and cognitive influences. cognitive influences. Dev Dev Sci, 16, Sci, 16, 101-110101-110

Frequency (Hz)

Am

plitu

de

Speech-Elicited ABR: Nina Kraus at Northwestern University Auditory Neuroscience Laboratory

(www.brainvolts.northwestern.edu)

Experience-Dependence

Fidelity to the Stimulus

Auditory Brainstem Response:Investigations of New Technology for Measurement

Neumann & Indermark (2012). Validation of a new TEOAE-Neumann & Indermark (2012). Validation of a new TEOAE-AABR device for newborn hearing screening. AABR device for newborn hearing screening. Int J Audiol, 51, Int J Audiol, 51, 570-575570-575

Valderrama et al (2012). Recording of ABR at high stimulation Valderrama et al (2012). Recording of ABR at high stimulation rates using randomized stimulation and averaging. rates using randomized stimulation and averaging. JASA, 132, JASA, 132, 3856-3865 (Spain)3856-3865 (Spain)

Muhler et al (2013). ABRs to broad-band chirps: Amplitude Muhler et al (2013). ABRs to broad-band chirps: Amplitude growth functions in sedated and anaesthetised infantsgrowth functions in sedated and anaesthetised infants. Int J . Int J Pedi ORL, Pedi ORL, 77, 49-5377, 49-53

:ABR Investigations of New Technology for Measurement:

Chirp Stimuli

Cebulla & Shehata-Dieler (2012). ABR-based newborn hearing Cebulla & Shehata-Dieler (2012). ABR-based newborn hearing screening with MB11 BERAphone using an optimized chirp for screening with MB11 BERAphone using an optimized chirp for acoustical stimulationacoustical stimulation. Int J Pedi ORL, 76. Int J Pedi ORL, 76, 536-543 (N = 6866), 536-543 (N = 6866)

Rodrigues et al (2013). Comparing auditory brainstem Rodrigues et al (2013). Comparing auditory brainstem response to tone burst and narrow band CE-chirp in young response to tone burst and narrow band CE-chirp in young infants. infants. Int J Pedi ORL, 77Int J Pedi ORL, 77, 1565-60 (Brazil) Stuart & Cobb , 1565-60 (Brazil) Stuart & Cobb (2014). Effect of stimulus and number of sweeps on the (2014). Effect of stimulus and number of sweeps on the neonate ABR. neonate ABR. Ear Hear, 35Ear Hear, 35, 585-588 [chirp], 585-588 [chirp]

Xu et al (2014). Prediction of frequency-specific hearing Xu et al (2014). Prediction of frequency-specific hearing threshold using chirp ABR in infants with hearing losses. Int threshold using chirp ABR in infants with hearing losses. Int J J Pedi ORL, 78, Pedi ORL, 78, 812-816812-816

Child and family historyChild and family history Evaluation of risk factors for congenital hearing lossEvaluation of risk factors for congenital hearing loss Parental report of infantParental report of infant’’s responses to sounds responses to sound ““Clinical observation of infantClinical observation of infant’’s auditory behavior.s auditory behavior. Behavioral observation alone is not adequate for Behavioral observation alone is not adequate for

determining whether hearing loss is present in this age group, and is not adequate for the fitting of determining whether hearing loss is present in this age group, and is not adequate for the fitting of amplification devices.amplification devices.””

Audiological assessmentAudiological assessment Auditory brainstem response (ABR)Auditory brainstem response (ABR)

Click-evoked ABR with rarefaction and condensation single-polarity stimulation if there are risk Click-evoked ABR with rarefaction and condensation single-polarity stimulation if there are risk factors for auditory neuropathyfactors for auditory neuropathy

Frequency-specific ABR with air-conduction tone burstsFrequency-specific ABR with air-conduction tone bursts Bone-conduction stimulation (as indicated)Bone-conduction stimulation (as indicated)

Otoacoustic emissions (distortion product or transient OAEs)Otoacoustic emissions (distortion product or transient OAEs) Tympanometry with 1000 Hz probe toneTympanometry with 1000 Hz probe tone Supplemental procedures, e.g.,Supplemental procedures, e.g.,

Electrocochleography (ECochG)Electrocochleography (ECochG) Auditory steady state response (ASSR) Auditory steady state response (ASSR) Acoustic reflex measurement (for 1000 Hz probe tone)Acoustic reflex measurement (for 1000 Hz probe tone)

Year 2007 Joint Committee on Infant Hearing (JCIH): Protocol for Evaluation for Hearing Loss In Infants from Birth to 6 months

Documents

Update on Auditory Electrophysiology James W. Hall III, PhD Adjunct Professor Nova Southeastern University Salus University University of Florida Extraordinary