Overview of Behavioral and Clinical Overview of Behavioral and Clinical Research at the Boys Town Research at the Boys Town National Research HospitalNational Research Hospital

Nebraska Biomedical Research Nebraska Biomedical Research Retreat 2006Retreat 2006

Overview of BTNRHOverview of BTNRH 40,000 outpatients per year.40,000 outpatients per year.

Clinical programs focused on Clinical programs focused on pediatrics and communication pediatrics and communication disorders.disorders.

One of the world’s largest One of the world’s largest communication disorders communication disorders research programs. research programs.

Hospital: 1977; Lied Center: Hospital: 1977; Lied Center: 2004; BTNRH - West : 20062004; BTNRH - West : 2006

Three Major Areas of ResearchThree Major Areas of Research

Clinical and Behavioral Studies of Human Clinical and Behavioral Studies of Human CommunicationCommunication

Hereditary Communication Disorders Hereditary Communication Disorders

Auditory, Vestibular and Retinal Auditory, Vestibular and Retinal NeurobiologyNeurobiology

For talks this morning, our work in genetics For talks this morning, our work in genetics has been subdivided into clinical studies has been subdivided into clinical studies and molecular studies.and molecular studies.

Current grant-funded Behavioral and Clinical Current grant-funded Behavioral and Clinical Research at BTNRH is focused on early Research at BTNRH is focused on early identification and remediation of hearing loss.identification and remediation of hearing loss.

Infant hearing screeningInfant hearing screening

Better measurement proceduresBetter measurement procedures

Communication development Communication development

Hearing aids and cochlear implantsHearing aids and cochlear implants

Hereditary communication disordersHereditary communication disorders

Infant Hearing ScreeningInfant Hearing Screening

Universal newborn hearing screening is now Universal newborn hearing screening is now mandated in Nebraska and most other states.mandated in Nebraska and most other states.

Screening is done using evoked potentials (EPs) Screening is done using evoked potentials (EPs) and/or oto-acoustic emissions (OAEs).and/or oto-acoustic emissions (OAEs).

– Normal inner ears produce a faint echo or Normal inner ears produce a faint echo or OAE that can be recorded in the ear canal.OAE that can be recorded in the ear canal.

– OAEs reflect inner ear mechanics.OAEs reflect inner ear mechanics.

False positives can be reduced with use of new False positives can be reduced with use of new measures of middle ear function.measures of middle ear function.

Infant Hearing ScreeningInfant Hearing Screening

Norms for some EP and OAE measures were Norms for some EP and OAE measures were developed at BTNRH.developed at BTNRH.

Some OAE measurement and screening devices Some OAE measurement and screening devices use software developed at BTNRH.use software developed at BTNRH.

3 BTNRH laboratories are doing research to 3 BTNRH laboratories are doing research to develop better EP, OAE and middle-ear develop better EP, OAE and middle-ear measurement procedures.measurement procedures.

Clinical Sensory Physiology LabClinical Sensory Physiology LabMichael GorgaMichael Gorga

Goal:Goal: To gain greater insight into cochlear processing in To gain greater insight into cochlear processing in both normal and impaired human ears through the use of both normal and impaired human ears through the use of noninvasive acoustical and electro-physiological noninvasive acoustical and electro-physiological techniques.techniques.   

Current Projects:Current Projects:– Understanding sources of variability in normal cochlear Understanding sources of variability in normal cochlear

responses.responses.

– Developing more accurate techniques for identifying and Developing more accurate techniques for identifying and quantifying hearing loss in infants, young children, and quantifying hearing loss in infants, young children, and patients with developmental disabilities. patients with developmental disabilities.

– Determining whether cochlear processing in humans Determining whether cochlear processing in humans differs for low and high frequencies. differs for low and high frequencies.

Physical Acoustics Laboratory Physical Acoustics Laboratory Douglas KeefeDouglas Keefe

Goal:Goal: To better understand: (1) the inter-relationships of To better understand: (1) the inter-relationships of the acoustic functioning of the external, middle and inner ear the acoustic functioning of the external, middle and inner ear in children and adults, and (2) the impact of this functioning in children and adults, and (2) the impact of this functioning on the processes of hearing in both normal-hearing and on the processes of hearing in both normal-hearing and hearing-impaired listeners. hearing-impaired listeners.     

Current Projects:Current Projects:– Studies of a new technique to detect middle-ear Studies of a new technique to detect middle-ear

dysfunction in infants and adults .dysfunction in infants and adults .

– Measurement of spectral and temporal processes in Measurement of spectral and temporal processes in human hearing using OAEs.human hearing using OAEs.

– Use of OAEs to monitor hearing at high frequencies.Use of OAEs to monitor hearing at high frequencies.

Communication Engineering LabCommunication Engineering LabStephen NeelyStephen Neely

Goal:Goal: Understanding the mechanisms by which the inner Understanding the mechanisms by which the inner ear processes sound information.     ear processes sound information.    

Current Projects:Current Projects:– Development of mathematical models of cochlear Development of mathematical models of cochlear

mechanics that provide a correct account of the inner mechanics that provide a correct account of the inner ear’s ability to amplify quiet sounds. ear’s ability to amplify quiet sounds.

– Development of models of auditory perception that take Development of models of auditory perception that take into account the non-linear response of the inner ear.into account the non-linear response of the inner ear.

– Development of software to facilitate the measurement Development of software to facilitate the measurement and analysis of otoacoustic emissions. and analysis of otoacoustic emissions.

– Development of better software to present and record Development of better software to present and record digital audio waveforms.digital audio waveforms.

Better Measurement ProceduresBetter Measurement Procedures

Behavioral tests of hearing measure the Behavioral tests of hearing measure the threshold of hearing (audiogram) or speech threshold of hearing (audiogram) or speech perception.perception.

Measures of frequency and temporal analysis Measures of frequency and temporal analysis are not used clinically because they are too are not used clinically because they are too variable.variable.

Labs working on better measurement Labs working on better measurement procedures include the first 3, plus the procedures include the first 3, plus the Psychoacoustics Laboratory.Psychoacoustics Laboratory.

Psychoacoustics Laboratory Psychoacoustics Laboratory Walt JesteadtWalt Jesteadt

Goal:Goal: To understand the rules governing detection and To understand the rules governing detection and discrimination of simple sounds by human listeners and to discrimination of simple sounds by human listeners and to use that information to refine models of how the ear use that information to refine models of how the ear processes sounds.processes sounds.

Current Projects:Current Projects:– Studies of how subjects make decisions about the Studies of how subjects make decisions about the

sounds they hear in simple intensity resolution tasks.  sounds they hear in simple intensity resolution tasks. 

– We once thought that decisions were based on the We once thought that decisions were based on the amount of energy in specific frequency regions, but we amount of energy in specific frequency regions, but we now know that most decisions are more complex.now know that most decisions are more complex.

Communication DevelopmentCommunication Development

Infant hearing screening is allowing us to identify Infant hearing screening is allowing us to identify hearing loss at birth so that we can provide hearing loss at birth so that we can provide some form of remediation.some form of remediation.

The goal is to achieve normal communication The goal is to achieve normal communication development.development.

Word learning is an important component of Word learning is an important component of communication development that we can communication development that we can measure.measure.

Communication development is obviously Communication development is obviously closely linked to more general cognitive closely linked to more general cognitive development.development.

Infant Development LabInfant Development LabMary Pat MoellerMary Pat Moeller

Goals: Goals: – To characterize early word learning strategies of To characterize early word learning strategies of

infants with normal and impaired hearing. infants with normal and impaired hearing.

– To identify perceptual, social-cognitive and linguistic To identify perceptual, social-cognitive and linguistic factors influencing word learning.factors influencing word learning.

Current ProjectsCurrent Projects– Analysis of maternal responsiveness to words, Analysis of maternal responsiveness to words,

possible words and other vocalizations.possible words and other vocalizations.

– Study of word learning through overhearing.Study of word learning through overhearing.

– Examination of continuity between babble and the first Examination of continuity between babble and the first word stage. word stage.

Hearing Aids and Cochlear ImplantsHearing Aids and Cochlear ImplantsBoth technologies rely heavily on digital Both technologies rely heavily on digital signal processing.signal processing.

Both require unique procedures when Both require unique procedures when working with infants and young children, working with infants and young children, including long-term follow-up.including long-term follow-up.

Infants with significant hearing loss are Infants with significant hearing loss are fitted with a hearing aid before they are fitted with a hearing aid before they are 1-year old.1-year old.

BTNRH is one of the few centers with BTNRH is one of the few centers with NIH-funded research programs in both NIH-funded research programs in both areas. Work is underway in 3 labs.areas. Work is underway in 3 labs.

Hearing Aid Research LaboratoryHearing Aid Research LaboratoryPatricia StelmachowiczPatricia Stelmachowicz

Goal: Goal: To explore ways to optimize amplification for To explore ways to optimize amplification for infants and young children with hearing loss. infants and young children with hearing loss.

Current Projects:Current Projects:

– Improved hearing aid signal processing (extended Improved hearing aid signal processing (extended bandwidth, single microphone noise reduction, and bandwidth, single microphone noise reduction, and frequency compression/transposition).frequency compression/transposition).

– Structured auditory learning paradigms using high Structured auditory learning paradigms using high variability training (e.g., multiple talkers) to improve variability training (e.g., multiple talkers) to improve perception in noise or to accelerate the normal course perception in noise or to accelerate the normal course of acclimatization.of acclimatization.

Cochlear Implant Cochlear Implant Research LabResearch LabMichelle HughesMichelle Hughes

Goal: Goal: To determine whether physiological measures of To determine whether physiological measures of implant performance can be used to predict behavioral implant performance can be used to predict behavioral measures such as judgments of pitch and loudness or measures such as judgments of pitch and loudness or speech perception.speech perception.

Current Projects:Current Projects:– Physiological and behavioral measures of channel Physiological and behavioral measures of channel

interaction in cochlear implants.interaction in cochlear implants.

– Clinical outcomes for individuals who were borderline Clinical outcomes for individuals who were borderline cochlear implant candidates.cochlear implant candidates.

– Efficacy of bilateral cochlear implants.Efficacy of bilateral cochlear implants.

Auditory Perception Laboratory Auditory Perception Laboratory Donna NeffDonna Neff

Goal:Goal: To explore how more central auditory processes To explore how more central auditory processes such as selective attention affect the ability of adult and such as selective attention affect the ability of adult and child listeners to perceive sounds that are degraded, child listeners to perceive sounds that are degraded, unpredictable or otherwise non-optimal. unpredictable or otherwise non-optimal.

Current Projects:Current Projects:– Use of non-speech sounds to determine pattern Use of non-speech sounds to determine pattern

recognition abilities in children and adults with normal recognition abilities in children and adults with normal hearing and cochlear implants.   hearing and cochlear implants.  

– Development of procedures to measure selective Development of procedures to measure selective auditory attention in children and adults. auditory attention in children and adults.

Hereditary Communication DisordersHereditary Communication Disorders

At least 50% of the occurrences of childhood At least 50% of the occurrences of childhood hearing loss are genetic in origin.hearing loss are genetic in origin.

Identification of specific genes provides an entry Identification of specific genes provides an entry point for studies of molecular mechanisms.point for studies of molecular mechanisms.

BTNRH was one of the first research centers to BTNRH was one of the first research centers to localize genes associated with hearing loss.localize genes associated with hearing loss.

For the past 10 years, most of our research in For the past 10 years, most of our research in this area has focused on Usher syndrome, the this area has focused on Usher syndrome, the leading cause of deafness followed by blindness.leading cause of deafness followed by blindness.

Center for the Study and Treatment Center for the Study and Treatment of Usher Syndromeof Usher Syndrome

William KimberlingWilliam KimberlingGoal: Goal: To identify novel genes associated with Usher To identify novel genes associated with Usher syndrome, develop efficient methods of screening and syndrome, develop efficient methods of screening and early diagnosis, and collaborate on clinical trials to early diagnosis, and collaborate on clinical trials to determine the effective treatment that would delay or determine the effective treatment that would delay or stop the progression of blindness. stop the progression of blindness.

Current Projects:Current Projects:– Development of state-wide screening programs using Development of state-wide screening programs using

chip technology.chip technology.

– Genetic and environmental factors that influence the Genetic and environmental factors that influence the severity of Usher syndrome.severity of Usher syndrome.

– Identification of novel Usher genes.Identification of novel Usher genes.

Other Areas of InterestOther Areas of InterestExpanded clinical trials in otolaryngology Expanded clinical trials in otolaryngology and pediatrics.and pediatrics.

Boys Town Institute for Child Health Boys Town Institute for Child Health ImprovementImprovement– Directed by Thomas Tonniges, MDDirected by Thomas Tonniges, MD– Policy and outcome studies for children and Policy and outcome studies for children and

youth with special healthcare needs.youth with special healthcare needs.

Behavioral HealthBehavioral Health