Klinik und Poliklinik für Hals-, Nasen- und Ohrenkrankheiten,Plastische und Ästhetische OperationenDirektor: Prof. Dr. R. Hagen

Ten years experience with ABR- newborn y pscreening using the BERAphon®Wafaa Shehata-Dieler 1), M. Cebulla 1), E. Stürzebecher 2), R. Hagen

1) ENT Clinic, Faculty of Medicine, University of Würzburg, Germany 2) WDH Denmark, c/o Petershagen, Germany

S ( )Screening program in Würzburg: (N= 13363)

• Information session and brochures for the parents prior to delivery

• BERAphon (TCSS) SSPBERAphon (TCSS) SSP

• measurements as of 24 hours after delivery

• database and tracking system (based on the birth register)

Two Stage screening protocol• Two Stage screening protocol

• Follow up screen for external babies (N = 1108)

• newborns who fail the test are followed up through the diagnosis and treatment

210 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

BERAphon

Transducer/headphone

activeground reference

310 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Screening ProtocolScreening Protocol

passscreen 1 +/ 2

fail

passscreen 1 +/- 2well-baby nursery

dischargel

failscreen

audiologypass normal hearing

gexternal babies

fail riskfactors

gy

paedaudiological passpaedaudiologicaldiagnosis

passfollow up

6 - 12 months

hearing losstreatment

410 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Stimulus and recording paradigmsStimulus and recording paradigms

A t 1997 Ti C St Sti l i l i• August 1997:Time Course Step Stimulus: visual scoring, threshold determination, latency and amplitude values

• 2002: SSP with clicks: automated scoring, broadband, 40 dB criteriadB criteria

• 2006: SSP with optimised chirp (CECTM) : automated• 2006: SSP with optimised chirp (CECTM) : automated scoring, broadband, 35 dB criteria

• SSP with frequency- and-limited stimuli, automated scoring, 35 dB criteria, “frequency specific” (2007)

510 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

35 dB criteria, frequency specific (2007)

Ti C St Sti l P diTime-Course-Step Stimulus Paradigm(Stimulus: 14 sequences/s, 6 clicks/sequence, 5 ms ISI, 10 dB steps)

2 03 0 40 50 6 0 dB c lickkein e Ant wort

10 20 3 0 40 50 600.0 5.0 10. 0 15.0 20.0 25 .0 30.0 35. 0 40.0ms

flexible, test time = 3 minutesSince 2002 used only for the diagnosis

610 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Since 2002 used only for the diagnosis

Pass

710 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Steady State Potentials (SSP)Steady State Potentials (SSP)(Stütrzebecher et al. 1999/ Cebulla et al.2007)

• Click evoked Potentials with a repetition rate 90/s

• Automated analysis based on a statistical method

(modified q sample test)(modified q sample test)

• 40 dB HL Criteria

• Extremely low possibility of false negative results

• Since 2006 modified Chirp (CECTM) at 35 dB HLp ( )

810 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

B db d ti li d f SSP diBroadband stimuli used for SSP recordingni

ts]

0

0.2

0.4lit

ude

[arb

. un

Click

-0.4

-0.2

Rel

ativ

e A

mp

0 0.005 0.01 0.015 0.02 0.025 0.03

R

Time [s]

nits

]

0

0.2

0.4

litud

e [a

rb. u

n

Chirp

-0.4

-0.2

0

Rel

ativ

e A

mpl Chirp

910 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

0 0.005 0.01 0.015 0.02 0.025 0.03

R

Time [s]

S i lt (fi t ) ith tiScreening results (first screen) with time-course-step-stimulus, SSP and SSP with CE chirp

N (ears) Pass Screening time

SSP with click (40dB) 1562 96.3% 48 s( )

Time-course-step-Stimulus (40 dB) 1158 93.6% 3:11 mStimulus (40 dB) 1158 93.6% 3:11 m

SSP with CECTM

(35dB) 3787 96 0% 24 s(35dB) 3787 96.0% 24 s(since 2006)

1010 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

The Würzburg Hearing Screening Program:The Würzburg Hearing Screening Program: Results (Following the control-screening)

Pass 99.4%

Fail 0.3%Fail 0.3%

D t 0 24%Dropouts 0.24%

Test time (SSP) 24 S

1110 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Diagnostic Model for Newborns who Fail the TestDiagnostic Model for Newborns who Fail the Test

well-baby nursery paediatrics

audiology screen outpatient births+/- ABR, OAE

phome births

maternity hospitalschildren hospitalschildren hospitalspaediatricians

otolaryngologistspaedaudiological y g gdiagnosis and

treatment

1210 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Results Würzburg (N = 13363)True positive N = 68p

Healthy (no risk) newborns: N = 51 Risk newborns: N = 17Risk newborns: N 17

24 newborns with unilateral hearing lossg44 newborns with bilateral hearing loss

27 fitted with hearing aids9 received a Cochlear Implant

1310 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Results: Würzburg external Newborns (N= 1108)Results: Würzburg external Newborns (N= 1108)

True positive: N = 196

65 i h il l h i l65 with unilateral hearing loss130 with bilateral hearing loss

79 fitted with hearing aids21 i d hl i l t21 received a cochlear implant

1410 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Aetiology of hearing lossAetiology of hearing loss

cleft palat9%

th

syndrome11%

ear deformity3%

others7%

heredofamilial4%

3%

No risk newborns66%

1510 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Aetiology of hearing lossAetiology of hearing loss Others (premat.,sepsis…)9%

Heredofamilial3%3%

Syndromes11%

Isolated ear deformity3%

No Risk65%

%

Cleftpalat/craniofacial anom.9%

1610 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

T f H i lType of Hearing loss

Bilateral moderate and severe SNHL 40%

Unilateral HL 32%

Bilateral mild SNHL and CHL 28%

1710 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

CHL 28%

T f H i lType of Hearing loss

Bilateral Severe to profound21%

Unilateral34%

66%

ater

al H

L

Bilateral Moderate27%

Bil

Bilateral Mild18%

1810 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

T t tTreatment

H i idHearing aid48%

Others 39%

CI13%

1910 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

13%

Age of identification diagnosis and intervention ( N= 53)Age of identification, diagnosis and intervention ( N= 53)

600 conductive and mild SN HL

d t SNHL534

515

500

moderate SNHL

severe and profound HL

unilateral HL

325

400

days

283

210229

200

300

Age

in

110

157143

113 120102

100

0

identification diagnosis treatment

2010 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Age of identification diagnosis and intervention:Age of identification, diagnosis and intervention:own newborns: N = 68external newborns: N = 196

234 234250

300

350 Wü newborns

ext ernal newborns

234

105

134

234

150

200

250

Age

in d

ays

7083

0

50

100

first visit primary diagnosis Hearing aid fitting

2110 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Age of identification diagnosis and intervention (N = 149)Age of identification, diagnosis and intervention (N = 149)600 conductive and mild SN HL

moderate SNHL

423

500

severe and profound HL

unilateral HL

324

3

400

days

229

179

266

200

300

Age

in d

132 138

95117

136112

136

100

0

identification diagnosis treatment

2210 years experience with ABR- newborn screening using the BERAphon®

03.04.2009

Early diagnosis of hearing loss in infantsEarly diagnosis of hearing loss in infants

Special considerations

Developmental stage and neural maturationCases with low frequency HLq y

Parents counselling

Difficulties in hearing aid fitting at a young ageDifficulties in hearing aid fitting at a young age

2310 years experience with ABR- newborn screening using the BERAphon®

03.04.2009