Newborn Screening and Middle Ear Problems

Transcription

1 Lisa L. Hunter 1, Douglas H. Keefe 2, M. Patrick Feeney 3;4, David K. Brown 1, Jareen Meinzen-Derr and Denis F. Fitzpatrick 2 1 Cincinnati Children's Hospital Medical Center, 2 Boys Town National Research Hospital, 3 National Center for Rehabilitative Auditory Research, 4 Oregon Health & Science University Relevant Financial Disclosure: Supported by NIH/NIDCD R01 DC Relevant Nonfinancial Disclosure: None Approximately 3 in 1,000 babies are born with permanent hearing loss - the most common birth defect - affects 12,000 children born in the United States each year. (White, 1997; Ross et al., 2008) Until the 1990s, children born with permanent hearing loss typically would not have been identified and diagnosed until 2 ½ to 3 years of age. Newborn hearing screening and EHDI programs have decreased the average age of hearing loss identification to 2 3 months of age (White, 2008; Hoffman & Beauchine, 2007; Harrison et al., 2003) Early intervention for congenital hearing loss results in better developmental outcomes (Holt & Svirsky, 2008; Moeller, 2000; Nicholas & Geers, 2006) Newborn Screening and Middle Ear Problems Refer rate for Newborn Hearing Screening (NHS) averages 8% at Stage I screening but improves to 2% at re-screening % of NHS referrals are due to temporary middle-ear dysfunction (Sanford et al., 2009; Hunter et al, 2010) Maturational differences in ear canal and middle ear acoustic transfer affect screening and diagnosis. Positive Predictive Value of NHS 10% since incidence of mild or greater congenital permanent hearing loss is approx. 2/1000. Sensitivity: Screening Auditory Brainstem Response (ABR) misses approximately 23% of infants with mild permanent hearing loss (Johnson et al., 2005). Limitations of Hearing Screening Tests in Newborns OAE and ABR screening does not distinguish between temporary hearing loss due to middle ear fluid and permanent congenital hearing loss. Standard tympanometry cannot detect middle ear effusion in newborns. Standard tympanometry tests only one frequency at a time, missing important frequency regions for pathology detection. Limitations of Diagnostic Hearing Tests in Infants Standard acoustic reflex tests carry risk of iatrogenic hearing loss. Standard click ABR tests can miss sloping high frequency or mild hearing loss, as well as conductive hearing loss. tests are affected by fine structure peaks and valleys in OAE and noise levels. Click-evoked OAE tests perform more poorly at high frequencies than tests. 1

2 Energy Reflectance 10/19/2012 Early Diagnosis of Ears in Newborns Aims: Improve accuracy of NHS to identify SNHL and CHL, and determine risk for recurrent OME in screening refers Overall Goal: Improve accuracy of hearing screening to detect temporary conductive hearing loss, and to determine risk for recurrent/chronic Otitis Media with Effusion (OME) Study Design: Longitudinal, prospective study of infants screened in normal nurseries and neonatal intensive care units Clinical Performance: Ambient and tympanometric wideband acoustic absorbance, TEOAE and acoustic reflex tests in newborns and infants Wideband acoustic tests performed along with clinical OAE and ABR at hospital screening and outpatient follow-up visits Clinical standard validation: Diagnostic tone burst threshold ABR at age 1 month and VRA at 9 months Determine whether WB immittance test battery improves the accuracy of detecting SNHL and CHL Gold standard validation is diagnostic ABR at age 1 months and VRA at 9 months Prospectively study OME in infants who refer on UNHS compared to those who pass Down syndrome and other craniofacial anomalies enrolled to determine feasibility of wideband reflectance coupled with OAEs to detect conductive hearing loss Wideband reflectance (WBR) Keefe et al., 2008 Energy Reflectance at Ambient Pressure WBR on infants and children 6 mos. to 10 years just prior to tube surgery Wideband reflectance is poorer in newborns with abnormal OAE Hunter et al., Refer = Normal (N=59) MEE (N=53) f (khz) Wideband reflectance improves with OAE improvement Hunter et al., Hunter in et prep. al., Refer = 141 Test performance for reflectance compared to tympanometry Hunter et al., in prep. Hunter Refer = et 141 al., % Sens. 90% Spec. Refer = 141 Area under curve = 90% 2

3 Wideband ART Wideband Screening Tests A Triple Play Wideband Absorbance Tympanometry Chirp evoked OAEs Middle ear, cochlear and efferent middle ear muscle reflex tests Same probe/tip and instrumentation for 3 tests; total time ranges from 10 sec to 1 min per test. 13 Wideband tympanometry research system (Keefe et al.) Produced by Interacoustics, Inc. Wideband Tympanometry in Normal Newborn Ear Wideband Reflexes in Normal Newborn Ear 15 Smart Algorithm Calculation of Wideband ASR in Normal Newborn Ear Calculation of ASR response presence (top) Relative ASR level compared to baseline (middle) Correlation of ASR responses (bottom) Pressurized Chirp-Evoked OAEs Based on Middle Ear Absorbance Wideband stimulation of entire cochlea Improve stimulus by using chirps rather than clicks Improve signal by correcting for middle ear pressure Improve signal by correcting for absorbed sound power Ultimate goal to decrease over-referral rate 3

4 Latency Wave V (msec) Current Study 10/19/2012 Auditory Brainstem Response (ABR) Toneburst Air and Bone Conduction Threshold ABR at 1 month Cond/Rare waveform correlation >=.7 for wave V threshold Results Typical Tone-burst 4000 Hz, 30 ABR db nhl Recording R= 0.87 R= Hz, 10 db nhl R= 0.91 R= Hz, 30 db nhl 4000 Hz, 10 db nhl High forehead to ipsilateral mastoid recording montage R= 0.86 R= Hz, 0 db nhl 1000 Hz, 30 db nhl R= Hz, 30 db nhl Hand-held bone vibrator at temporal bone for adequate force and placement R= 0.76 AC TB-ABR 1000 Hz, 10 db nhl R= 0.77 BC TB-ABR 1000 Hz, 10 db nhl Discussion Comparison of the Mean AC & BC TB-ABR latencies BC TB Intensity (db nhl) *significant 500 Hz: no significant difference between AC and BC latencies Hz: no significant difference except at 30 db nhl and 4000 Hz: significant differences at db nhl AC TB Intensity (db nhl) 500 Hz 1000 Hz 2000 Hz 4000 Hz Wideband Test Combinations for Diagnostic Interpretation Diagnosis WB Tymp WB Reflex Chirp OAE Air/Bone ABR Normal Normal Normal Normal Normal Conductive Flattened Elevated Threshold Absent Elevated air threshold, normal bone Cochlear Normal Normal Absent Elevated air, elevated bone Neural Normal Absent Normal Absent or markedly abnormal 23 4

5 EDEN Study Enrollment Follow-up Evaluations 299 newborns enrolled at screening in birth hospital or NICU; 159 completed one or more follow-up visits Screening in Hospital N=299 TEOAE + AABR Risk Factors Visit 1 (1 mo.) N=159 ABR Visit 2 (6 mo.) N=48 Visit 3 (9 mo.) N=46 VRA Ambient and Absorbance Tympanometry WB Acoustic Reflex Test Research TEOAE Otoscopy & OM records Visit 4 (12 mo.) N=9 Tympanometric Peak Pressure Measured in Newborns Normal Newborns Newborns in NICU Development of Ambient Absorbance in Normal Ears Large decreases in absorbance below 1 khz around 6 months of age due to ear canal wall stiffening and middle ear development. Absorbance also decreases at 8 KHz. Overall, absorbance rapidly develops and becomes adult-like by 6 months of age. 28 Development of Wideband Absorbance & OAE/ABR Screening Status Birth 1 month 6 months 9 months 1.5 to 8 khz frequency region shows less absorbance at all ages in refer ears Development of ASR Thresholds Across First Year Low frequency increase is due to increased ear canal volume High frequency decrease may reflect development of MOC reflex 5

6 Averaged Acoustic Reflexes (<2.8 khz): Screening and Diagnostic Outcomes Birth Two Stage Screening 1 mo - ABR Thresholds are elevated by > 10 db in ears that refer on 2-stage NHS exam Possible to classify threshold by SPL in 2-cc coupler or using in-the-ear SPL. More variability for in-the-ear SPL due to variability of in-the-ear measurements. Preliminary Area under ROC Curve (AUC): Prediction of Screening and Diagnostic Outcomes Two-Stage OAE-ABR Screening N=265 Pass N=84 Refer Diagnostic ABR at 1 month N=200 Pass N=18 Conductive HL 35 Summary/Conclusions Ears that refer on NHS have less absorbance above 1 khz and higher ART than ears that pass NHS. Development of absorbance shows adult-like responses by 6 months. At multiple ages up to 1 year, less absorbance was associated with abnormal OAE. Lower absorbance was also found for ears with conductive hearing loss on diagnostic ABR. Absorbance at ambient and TPP can accurately identify conductive hearing loss in newborns and infants. 6

7 Conclusions ART thresholds were higher in Refer ears, indicating a main effect of OAE Test result on ART levels. Pressurization at TPP for WB-ART improved thresholds for both Pass and Refer groups. Wideband ART were relatively resistant to effects of temporary conductive hearing loss at birth. Pressurized OAEs did not improve pass rates in newborns ears. Chirp OAEs showed higher stimulus levels than click OAEs Absorbed power chirp OAEs show efficacy in adult ears, currently being tested in newborn ears. Acknowledgments We are grateful to the families and infants who participated. Kelly Baroch, Candice Dixon, Alaa Elsayed, Kara Francis, Jennifer Wright, and Erin Hegner provided infant assessment at CCHMC. Maureen Sullivan-Mahoney and Candice Dixon provided infant assessment at Good Samaritan Hospital (Cincinnati). Research supported by NIH grant R01 DC