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CHIMES STUDY: CONGENITAL CYTOMEGALOVIRUS INFECTION AND HEARING MULTICENTER SCREENING STUDY

CHIMES STUDY: CONGENITAL CYTOMEGALOVIRUS INFECTION AND HEARING MULTICENTER SCREENING STUDY. Karen Fowler, DrPH University of Alabama in Birmingham Faye P. McCollister, EdD University of Alabama, Emeritus Diane Sabo, PhD University of Pittsburgh.

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CHIMES STUDY: CONGENITAL CYTOMEGALOVIRUS INFECTION AND HEARING MULTICENTER SCREENING STUDY

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  1. CHIMES STUDY: CONGENITAL CYTOMEGALOVIRUS INFECTION AND HEARING MULTICENTER SCREENING STUDY Karen Fowler, DrPH University of Alabama in Birmingham Faye P. McCollister, EdD University of Alabama, Emeritus Diane Sabo, PhD University of Pittsburgh

  2. Confusion Regarding Congenital CMV Infection & Hearing Loss • Studies/Reports of etiology of pediatric hearing loss seldom include routine screening for congenital CMV infection • Most congenital CMV infection is asymptomatic (90%) • No profile for audiometric configuration is available • Hearing may be normal; loss may be unilateral or bilateral; present at birth or delayed in onset; may be fluctuating and/or may be progressive

  3. Review of Congenital CMV Infection • Common virus although not easily spread person to person • Diagnosis needs to be made in the first 3 weeks of life • Clinical observation of infection in the newborn period identifies < 5% of all infants with congenital CMV infection • Delayed onset sequelae may occur over a long period with sensorineural hearing loss being the most common

  4. Review of Congenital CMV and Hearing Loss Research • 1960s - CID/symptomatic CMV infection and hearing loss first reported. Medearis, l964; McCracken, et al. 1969 • 1970s - inapparent / asymptomatic CMV infection and HL was first reported. Reynolds, et al. 1974; Dahle, et al. 1974; Hanshaw, et al. 1976; Stagno, et al. 1977 • 1970s & 1980s, progression and delayed onset HL first described. Dahle, et al, 1979; Williamson et al. 1982 • 3.9% at birth will have hearing loss; 8.3% at 6 yrs Symptomatic infection – 16.5% HL at birth; 36.4% at 6 yrs Asymptomatic infection – 2.9% HL at birth; 11.3% at 6 yrs

  5. NIH/NIDCD Contract The Natural History of CMV-Related Hearing Loss and the Feasibility of CMV Screening as Adjunct to Hearing in the Newborn

  6. CHIMES Study

  7. NIDCD CMV Grant, UAB • Multi-site investigation, 7 sites • 100,000 newborns to be screened for congenital CMV infection • Link newborn hearing results • Evaluate methods for CMV screening • Saliva vs. dried blood spots (DBS) • Audiological follow up of CMV positive infants for 4 years

  8. Project Design • Screen at least 100,000 newborns for CMV infection who currently undergo newborn hearing screening • Audiological follow-up of all CMV positive infants • Compare the accuracy of two diagnostic methods for CMV screening (traditional rapid saliva cell culture vs. real time PCR on DBS)

  9. Objectives • Define the long-term audiologic/otologic outcome in children with congenital CMV infection • Determine the clinical validity and utility of CMV screening: • in the detection of hearing impairment in the newborn • in the prediction of hearing impairment with onset during infancy or in the early years of life

  10. Project Staff • The project is directed by Drs. Suresh Bopanna & Karen Fowler • Each site will have a person designated as a co-principal investigator • Site audiologists direct audiological assessments • Project consultants: Faye McCollister, Judy Gravel, Karl White

  11. Site Audiologists • Julie Woodruff Birmingham, AL • Belinda Blackstone Birmingham, AL • William Mustain Jackson, MS • Marci Schwab New Brunswick, NJ • Edie Cox Charlotte, NC • David Brown Cincinnati, OH • Dan Choo Cincinnati, OH • John Greinwald Cincinnati, OH • Diane Sabo Pittsburgh, PA • Angela Shoup Dallas, TX • Kris Owen Dallas, TX

  12. Participating Hospitals Birmingham, AL University Hospital & Cooper Green Hospital Jackson, MS University of Mississippi Medical Center New Brunswick, NJ Saint Peters University Hospital Charlotte, NC Carolinas Medical Center Cincinnati, OH Good Samaritan Hospital Pittsburgh, PA Magee Women’s Hospital Dallas, TX Parkland Memorial Hospital

  13. Selected Hospital Populations 38% Caucasian, Non Hispanic 34% Caucasian, Hispanic 24% African American 4% Asian

  14. Challenges to the CHIMES Study (1) • Establishing consistency in protocols across 7 sites, getting everyone on the same page • Challenges inherent in longitudinal investigation: 6 year period of study, equipment changes, staff changes, protocol changes • Age of subjects, birth through age 4 years, more difficult to evaluate audiologically • Multiple disabilities, including developmental, vision, and motor for children with symptomatic infection

  15. Challenges to the CHIMES Study (2) • Variability of hearing loss: progression, delay in onset, and fluctuation • Subject ethnic background with language differences, cultural differences, social differences • Otitis media resulting in conductive overlay for sn hearing loss, delay in getting assessment data • Subject compliance with study protocol • Subject retention

  16. Challenges to the CHIMES Study (3) • Specified protocol modified to accommodate clinical needs with rapidly progressive hearing loss • Accuracy/consistency in data collection, recording, transfer, and storage • Interpretation of data and clinical judgment • Ensuring client confidentiality

  17. Addressing the Challenges to the CHIMES Study • Standardized collection of data: developed data forms using barcoded labels with unique study identifier for all data sent from 7 sites (scanned and processed) • Developed detailed Manual of Procedures (MOP) for study policies and procedures • Developed audiology protocols establishing optimal and minimal goals for audiology data at visits • Site Visits and Review of protocols • Detailed retention plan with computerized database at each site and data forms (Visit Forms & Missed Visit Forms) to collect information on amount of contact each site had with a study participant during the intervals between visits

  18. Addressing the Challenges to the CHIMES Study

  19. Subject Variables • Additional Disabilities (symptomatic infection) • Vision • Can not see visual reinforcers • Can not process visual instructions • Need glasses for assessment, if prescribed • Seizure disorder • Flicker stimulation with lighted reinforcer • Absence, petit mal, and grand mal seizures

  20. Cultural Diversity • Racial, cultural, socioeconomic differences exist among individuals from same country • Interpreters may have difficulty explaining medical / technical information • May be difficult for family to understand • will not qualify for public assistance medical and technical services (hearing aids) • finding financial assistance challenging, at best

  21. Cultural Diversity • Project informational materials will be provided in English and Spanish for parents and at understandable reading levels. • Communication options chosen by families for their child will be respected and supported.

  22. CHIMES STUDY Audiological Assessment Protocol Diane Sabo, PhD University of Pittsburgh

  23. Components of Study Audiological Assessment • ABR tone bursts, bone conduction, • Otoacoustic emissions, (DPs) • Immittance with high frequency probe for subjects less than 7 months of age, only when conductive involvement needs greater definition • Behavioral assessment • VRA • Play

  24. Screening Data Obtained • Physiologic data • ABR • Automated-ABR • OAEs • Automated OAEs • Combinations of the tests

  25. Objective Hearing Diagnostic Methods • Auditory Brainstem Response (ABR) • Tone Bursts –air and bone conduction • Auditory Steady State Response (ASSR) not required, optional • Acoustic Immittance • Evoked Otoacoustic Emissions (EOAEs) • Distortion Product OAEs (DPOAEs)

  26. Entry or Baseline Data • Physiologic tests • ABR • Tone bursts at .5, 1, 2 and 4k Hz • Bone conduction if air conduction abnormal (>25) • DPOAEs • 2-8k Hz • 60/45 • Signal and noise levels recorded

  27. Physiologic Assessment with ABR and EOAEs • Advantages • “Objective” • Reliable • Correlate well with hearing • Age not a factor (but sometimes an issue)

  28. Physiologic Assessment with ABR and EOAEs • Limitations • Not completely “objective” • Not tests of hearing • Not always good at predicting threshold • Not independent of infant’s behavior, state, or other factors

  29. Click Spectrum Frequency Frequency

  30. Comparison of Frequency Range for ABR Clicks and Tone Bursts

  31. What We Know About ABR Clicks • Click commonly used, but……can give misleading information • Click has energy at all frequencies transduced by earphone • Within limits, regions of normal hearing will generate ABR, even with regions of hearing loss • ABR tests avoid problems associated with reverse middle ear transmission, but potential still exists for missing hearing loss with click-evoked ABR

  32. Progressive HL Secondary to SX CMV in Six Month Period

  33. ABR Assessment at Enrollment Visit • Enrollment visit at 3-6 weeks of age • Objective is to obtain valid/accurate estimates of ear specific, frequency specific hearing thresholds for each ear and to characterize the type of any permanent loss present as baseline information • Case history/parent observation report • Otoscopic inspection • DPOAE • Medical referral if testing deferred because of otologic problems

  34. Physiologic Assessment with ABR and EOAEs • Advantages • “Objective” • Reliable • Correlate well with hearing • Age not a factor (but sometimes an issue)

  35. Optimal Goal for ABR Assessment at Enrollment Visit (3-6 Weeks) • ABR minimum response levels at .5, 1.0, 2.0, and 4.0 kHz for each ear • DPOAE information at five frequency bands centered at 2,3,4,6, and 8 • Bone conduction, tympanometry, and ipsilateral reflexes may be required if loss suspected • Record on ABR data form CF107 • Record on OAE data form • Record on immittance data form CF110

  36. Minimal Goal for ABR Assessment at Enrollment Visit at 3-6 Weeks of Age • Due to the importance of obtaining baseline information that is a clear and accurate reflection of infant’s hearing level a minimum goal was set: • Obtain MRL at 0.5, 2.0, and 4.0 kHz for each ear • Bone conduction, tympanometry, and ipsilateral acoustic reflexes may be necessary if loss is suspected • If additional assessment apt necessary, use sequence system of 1,2, and 3 on forms for enrollment visit • Prolonged delays between assessments avoided if possible

  37. ABR Protocol • Order for tone bursts: 2000, 4000, 500 and 1000 • Use 2-0-2 for 500 Hz • Use 4-0-4 for • Rate 27-29 • Filter setting of 30-3000 • Two replications • <1000 sweeps for 2.0 and 4.0 • No less than 2000 sweeps for 0.5 and 1.0 kHz

  38. ABR Parameters • 15-20 msec window • At least two replications at no response level and next level up, using a 10dB step above the last response level; threshold determination is midpoint between these two levels • Electrode configuration of high forehead to earlobe, with low forehead as ground; assuming a two channel recording • Insert earphones

  39. Entry Data • Acoustic Immittance Data • Tympanometry • High frequency tymps (1k) < 7 months • Acoustic reflex thresholds • IPSI reflexes @ 1k only, if necessary

  40. Use Insert Earphones • Separation of stimulus artifact from the onset of the response makes wave I more visible • Prevent ear canal collapse • Increase interaural attenuation • Provide greater comfort for long periods of time • Can attenuate surrounding environmental noise more efficiently • Absolute latencies delayed by 0.9ms • Affect amplitude of wave I, lower

  41. CHIMES STUDY Faye P. McCollister, EdD University of Alabama, Emeritus Behavioral Audiological Assessment Protocols

  42. Request Parental Help in Preparing for Behavioral Diagnostic Assessment • Try to have child rested and feed, comfortable and attentive for test • Schedule appointment away from nap time • Bring diapers for diaper change • Bring bottle for comforting • Bring pacifier, if used • Bring familiar car seat for test

  43. Manage Baby/Mom/Diagnostic Environment • Quietest environment possible, no talking, no noisy toys • Reduce undesirable visual, auditory, tactile distractions • calm, alert child preferred, but not after heavy feeding, will fall asleep • Do not separate child from parent and create crying, agitated child • Wait for child to calm prior to initiating test

  44. Child/Parent Handling Issues • Child State • Activity level • Comfort level • Infection control • Clinic protocol • Gloves, disposables, cross contamination • Parent inclusion • Pre-test information sharing • Time during testing • Time spent informing about procedure • Informing about the results

  45. Visual Reinforcement Audiometry • Developmentally appropriate technique that gives valid estimate of hearing • Gives ear specific, frequency specific information • Reliable method for early years • Practical for repeat use

  46. Schedule for Behavioral Audiological Assessment • Visual Reinforcement Audiometry scheduled at 7,12, 18, and 24 month follow-up visits • Play Audiometry scheduled at 24, 30, 36, 42, and 42 month follow-up visit

  47. VRA Assessment Information • Beginning stimulus type • warble tone, speech • Beginning transducer • earphone, speaker • # beginning conditioning trials • # reconditioning trials • # stimulus trials • # control trials, if greater than 0, # correct

  48. VRA Assessment Information • Test time • Number of breaks taken • DPs completed, 2,3,4,6,and 8 • Acoustic Immittance Measures Done? • Acoustic reflex threshold • tympanometry • Follow up/ reschedule, no, yes- mark reason • Fussy scared, wax occluding canal, time, refused earphones/inserts, habituated, test reliability poor, failed to condition, mrl > 20, cnd if sn loss present, other- specify • Medical Follow-up Recommended- yes, no- mark reason • Conductive component, tymps abnormal, ab gap, draining ear, hearing aid clearance

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