Various predictors and outcomes of corrected congenital aural atresia

Various predictors and outcomes of corrected
congenital aural atresia: a model of maximum
unilateral conductive hearing loss.
Lincoln Gray, Ph.D.
No Relevant Financial Disclosure
(2009-10 grant from Richmond Eye & Ear Fndn to Kesser & Gray)
No Relevant Non-financial Disclosures
Congenital
Aural Atresia
In this talk only
considering
unilateral atresia;
one normal ear
~30 dB gain from ~ 60 dB at birth to
near normal at ages ~ 6 to 53
I will be presenting the work of
several outstanding collaborators
Brad Kesser, MD
Brian Nicholas, MD Kaitlin Krook, MD
Erica Cole, AuD
We owe it all to Robert Jahsdoerfer
Dr.
J
?
Various predictors and outcomes of corrected
congenital aural atresia: a model of maximum
unilateral conductive hearing loss.
1. Introduction and brief review of what we knew.
2. Does preoperative hearing predict postoperative hearing in
patients undergoing primary aural atresia repair?
–
Nicholas BD, Krook K, Gray L, Kesser BW., Otol Neurotol , 2012
3(6):1002-6
3. Impact of Unilateral Conductive Hearing Loss Due to Aural
Atresia on Academic Performance in Children
– Kesser BW, Krook K, Gray L, in revision
4. Hearing speech in noise from a single source before and
after surgical improvement of congenital conductive
hearing loss.
– Kesser BW, Cole, E, Gray L. ARO 2013
Part I: Introduction
Audiometric Threshold
Change in PTA
PTA
Wilmington et al. 1994
Several different components of binaural
(stereo) hearing, and normal benefit:
• Redundancy (2 ears better than 1), ~3dB
• Head Shadow, 4-7 dB
• Squelch, varies up to ~10 dB
• Localization ~1 degree of azimuth
(redundancy to be discussed in Part IV of
this talk)
Use of new head-shadow cues.
Mean ~ 4 dB
No age effect
Gray et al., 2009
Implication: Ears seem to be forgiving of
early unilateral deprivation
Got
better
Signal
Improvement Hearing a Signal in Noise
Noise
Noise to atretic ear
Binaural
squelch for
simple stimuli
decreases
with age.
Got
Implication:
worse
critical period
(amblyaudia?)
perhaps
Gray et al., 2009
Improvement Hearing a Signal in Noise
Binaural squelch for
complex stimuli is
complicated
r2=.75
Got
better
Got
worse
Got
worse
Gray et al., 2009
Implication: A learning curve for binaural hearing,
or like multitasking good for teens
RMS Error in Degrees
Lots of variance in pre- and post-op localization.
Significant improvement, but still not very good
Horizontal Sound
Localization
Wilmington et al, 94
Limit of Normal (+1 95% CI)
13
Part II: . Does preoperative hearing
predict postoperative hearing in
patients undergoing primary aural
atresia repair?
Nicholas BD, Krook KA, Gray LC, Kesser BW. Otol
Neurotol. 2012 Aug;33(6):1002-6. PMID: 22772017
OBJECTIVE: The purpose of this study is to explore the
correlation between preoperative hearing and early
postoperative hearing results in patients undergoing primary
aural atresia repair. CONCLUSION: Among patients undergoing
primary atresia repair, better preoperative hearing strongly
predicts better postoperative hearing and correlates with ear
anatomy. Preoperative hearing status should be factored when
counseling atresia patients on hearing rehabilitation options.
Problem: lots of variability in results
Jahrsdoerfer et al , 1992, proposed a 10
point scale based on radiological findings
ABSOLUTE REQUIREMENTS
1. Normal inner ear
2. Normal cochlear function
‘J Score’ from HRCT
</= 5/10: poor
6/10: marginal
7/10: fair
8/10: good
9/10: very good
10/10: excellent
~30 dB gain in audiometric
measures after surgery.
Pre- and post-op audiometric
measures are correlated
Preoperative Pure Tone Average (in quartiles)
Worst Quartile
(69 to 110)
3rd Quartile
(63 to 68)
2nd Quartile
(59 to 62)
Best Quartile
(28 to 58)
Probability of ‘normal’ (PTA ≤30dB HL) Result
Pre-op Audiogram Predicts Post-op Result
p=.002; r2=7%; almost a ‘medium’ effect size
Worst Quartile
(61 to 100)
3rd Quartile
(56 to 60)
2nd Quartile
(51 to 55)
p=.05 (one-tailed); r2=2%; a ‘small’ effect size
Best Quartile
(15 to 50)
Probability of ‘normal’ (SRT ≤30dB HL) Result
Pre-op Speech Testing Predicts Post-op Result
Preoperative Speech Reception Thresholds (in quartiles)
No significant (p<.05) correlation of post-op results with J-score
Conclusion:
• Among patients undergoing primary
atresia repair, better preoperative
hearing correlates with ear anatomy
and strongly predicts better
postoperative hearing.
• Preoperative hearing status should
be used as an important adjunct to
Jahrsdoerfer score when counseling
atresia patients and families on
hearing rehabilitation options.
Part III: The impact of unilateral
conductive hearing loss due to
aural atresia on academic
performance in children
Bradley W. Kesser, MD
Kaelyn Krook, MD
Lincoln Gray, PhD
Laryngoscope (in revision)
Bess and Tharpe, 1986, did a classic study on the
effects of unilateral hearing loss on academic
performance. They surveyed clients with
unilateral sensorineural loss (USNHL).
(n=60)
35% repeated a grade
13% required some resource
48%
Academic effects of unilateral
sensorineural hearing loss (USNHL) in
children
Study
Failed
Resource Help
Combined
Bess and Tharpe,
1986
35%
13%
48%
Oyler et al., 1988
27%
41%
68%
Bovo et al., 1988
22%
12%
34%
Jensen et al., 1989
18%
60%
78%
Lieu, JE., 2004
22-35%
12-41%
34-76%
Adapted from Tharpe, AM. Trends in Amplification. 2008;12:7-15
Prevalence of UNSNHL
• 6-12 per 1,000 with USNHL
• 0-5 per 1,000 with mod.-profound
USNHL
• 391,000 school-aged children in the
US
•
Lee, DJ et al. Ear and Hearing. 1998;19:329-32
Prevalence of congenital aural atresia
• 1 per 10,000
Study question
Does unilateral conductive hearing loss
(secondary to congenital aural atresia)
confer the same academic disabilities on
school age children as unilateral
sensorineural hearing loss?
• 132 families of children with aural atresia
surveyed (Bess and Tharpe survey) June – Nov.
2011 (UVA IRB #15369)
– 91 returned (69%)
– 23 excluded for prior atresia surgery
– 26 excluded child < 5 yrs
– 2 excluded for bilateral atresia
– N=40
• 48 families of children with USNHL surveyed June
– Nov. 2011
– 12 returned (25%)
– 1 excluded for child < 5 yrs
– N=11
Demographics
Atresia Group
(n=40)
SNHL Group
(n=11)
23:17 (56% M)
7:4 (64% M)
27:33 (45% M)
0.36
Mean age
(range)
8.9 (5-31)
12.6 (7-19)
13 (6-18)
0.034
Right:Left
29:11
4:7
NR
0.04
30:4:6:0
9:0:2:0
46:0:0:14
0.72
0
NR
0.02
Male:Female
Race (C:A:H:AA)
Syndromic:
HFM/Goldenhar
14
Bess and Tharpe P value
(n=60)
Audiometric data
Atresia Group
SNHL Group
Atretic ear
Normal ear
SNHL ear
Normal ear
AC PTA
67.2 (46-91)
8.1 (0-23)
74.9 (25-114)
7.9 (0-25)
BC PTA
9.9 (0-38)
NR
NR
NR
SRT
63.9 (45-75)
NR
52.4 (10-110)
NR
Bess and Tharpe: PTA > 45 dB HL = poorer ear
< 15 dB HL = better ear
Results: Academic Progress
Resource
Atresia
Group
(n=40)
Repeated a
0 (0%)
grade
Any
26 (65%)
resource
Behavior
5 (12.5%)
problem
SNHL Group
(n=11)
Bess and
Tharpe
(n=60)
2 (18.2%)
21 (35%)
7 (63.6%)
8 (13.3%)
3 (27.3%)
12 (20%)
Analysis – Grade retention
Atresia is better than either SNHL group
Study Comparison
p Value
Atresia (0%) vs. Bess and Tharpe (35%)
<0.0001
Atresia (0%) vs. SNHL (18%)
0.04
SNHL (18%) vs. Bess and Tharpe (35%)
0.32
Fisher’s exact test
Historical Data on Grade Retention
(nothing as far back as 1986, unfortunately)
Planty M, et al. The Condition of Education (NCES 2009-081). Washington,
D.C.: National Center for Education Statistics, Institute of Education
Sciences, U.S. Department of Education; 2009
Resource assistance
Resource
Atresia
Group
(n=40)
SNHL Group
(n=11)
Bess and
Tharpe
(n=60)
Any resource
26 (65%)
7 (63.6%)
8 (13.3%)
Amplification
5 (12.5%)
3 (27.2%)
Speech Rx
18 (45%)
4 (36.4%)
FM System
13 (32.5%)
3 (27.2%)
IEP
19 (47.5%)
5 (45.5%)
Special Ed.
8 (20%)
3 (27.2%)
Analysis – Need for resource
More utilization in 2011 than 1986
Study Comparison
p Value
Atresia (65%) vs. Bess and Tharpe
(13%)
Atresia (65%) vs. SNHL (64%)
<0.0001
SNHL (64%) vs. Bess and Tharpe (13%)
0.001
Fisher’s exact test
1
Analysis – Behavior Problems
No differences.
Study Comparison
p Value
Atresia (12%) vs. Bess and Tharpe
(20%)
Atresia (12%) vs. SNHL (27%)
0.42
SNHL (27%) vs. Bess and Tharpe
(20%)
Pooled SNHL+Atresia vs. Bess and
Tharpe
0.69
Fisher’s exact test
0.35
0.63
Discussion
Children with atresia use resources as much or
more than their SNHL peers but are less likely to
repeat a grade.
Perhaps because aural atresia is seen.
Classrooms are different now:
• Children in small groups at
tables
• Projects
So why aren’t grade retention
rates better for today’s USNHL
children than those in 1986?
CHL may not be as significant a disability as SNHL
Stimulation of both
central auditory
pathways by the
good ear and via
bone conduction
from the atretic
ear
from Chapter 12: Auditory System:
Structure and Function, Lincoln Gray,
Ph.D.
http://nba.uth.tmc.edu/neuroscience/
Which resource is best?
• Preferential seating
• FM system
• IEP
• Bone conducting hearing device
• BAHA
• Atresia surgery
Limitations of survey study
•
•
•
•
Selection bias
Recall bias
No socioeconomic data
Unable to collect data on degree of
hearing loss and academic
performance
Conclusion
• congenital conductive hearing loss (atresia) causes
fewer academic delays than sensorineural hearing
loss.
Implications for Clinical Practice
• Optimize resources – Preferential seating, FM
system, Speech Rx, IEP, Amplification
• Role of surgery?
Implications for future research
• Longitudinal follow-up
• Especially detecting speech in noise
Part IV: Hearing speech in noise from a
single source before and after surgical
improvement of congenital conductive
hearing loss.
From Cole, E.D. JMU AuD Dissertation
2009.
Single-speaker HINT tests.
• Speech in quiet
• Speech in multi-talker babble
• All from a single central loud-speaker
Results – Hearing in quiet
Binaural summation
7
Improved
2
Worse
1
No change
Preoperative mean 31.8 dB(A)
RTS
Postoperative mean 28.4 dB(A)
RTS
= 3.4 dB gain
Summary: After surgery,
patients repeated speech
better in quiet by 3.4 dB
(normal summation)
Sound source
Signal
dB above normal
Reception Threshold for Speech in Quiet
} +3dB
Pre-op
Post-op
A puzzle is why the patients are so poor at this task?
Tentative suggestion: might have something to do with
typical signal to noise ratios if you only have one ear
Signal
Noise
dB above normal
Signal to Noise Ratio (in Noise)
}<1dB
Pre-op
Post-op
Maybe their world is noisy, thus harder to learn?
z above normal
Reception Threshold for Speech in Quiet
Pre-op
Post-op
Tentative conclusions:
• Expected redundancy effect in quiet, +3dB from
2nd ear.
• Did relatively better in noise compared to normals
than in quiet.
• Atresia patients likely get much practice listening
in noise, because without any binaural squelch the
world is a much noisier place.
• Almost normal in noise pre-operatively, thus
maybe can’t get much better because already
near normal.
• We don’t understand why so far away from
normal, both pre- and post op in the quiet. Maybe
having two ears helps you learn to hear out of one
1981
Thanks Roger!
With grateful appreciation to the one who
lead me to an interest in clinical audiology.
Who told me about this job at JMU.
Wishing we were still working together.
Acknowledgements:
Current Atresia Research Team
Joan Bessing
Tanvir Battu
Steve Colburn
Caitlin Cook
Erica Cole
Cliff Cutchins
Robert Jahrsdoerfer
Sofia Ganev
Bradley Kesser
Brittany Harwell
Janet Koehnke
Michael Kesler
Brian Nicholas
Bradley Kesser
Kaitlin Krook
Megan Klingenberg
Roger Ruth
Brandon Lancaster
Debra Wilmington
Robert Nagel
NIH
Jonathan Smith
DRF
Richmond Eye & Ear Foundation
References:
Gray, L., Kesser, B., and Cole, E. Detection of Speech in Noise after Correction of
Congenital Unilateral Aural Atresia: Effects of age in the emergence of binaural squelch
but not in use of head-shadow. International Journal of Pediatric Otorhinolaryngology
73: 1281–1287, 2009.
Nicholas. B., Krook, K., Gray, L., Kesser, B. Does preoperative hearing predict
postoperative hearing in patients undergoing primary aural atresia repair? Otology
Neurotology, 33(6):1002-6, 2012.
Wilmington D., Gray L. and Jahrsdoerfer R., 1994, Binaural processing after corrected
congenital unilateral conductive hearing loss. Hearing Research 74:99-114
Other:
Breier, J., Hiscock, M., Jahrsdoerfer, R., and Gray, L.: Ear Advantage in Dichotic
Listening After Correction for Early Congenital Hearing Loss, Neurophychologia 36:209216, 1998.
Jahrsdoerfer RA. Congenital atresia of the ear. Laryngoscope 1978; 88:1-48.
Jahrsdoerfer, R.A., Yeakley, J.W., Aguilar, E.A., Cole, R.A., and Gray, L.C.: A grading
system for the selection of patients with congenital aural atresia. American Journal of
Otolology 13:6-12, 1992.