-
Elizabeth Fitzpatrick University of Ottawa CHEO Research
Institute Unilateral Hearing Loss in Children Conference
Philidelphia, October 22-24, 2017
Children with unilateral hearing loss: A glimpse at clinical
practice, outcomes, and parent experiences
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Disclosure
• No conflicts of interest related to any research
• Phonak sponsorship for travel/accommodations for this
conference
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• Child Hearing Lab group JoAnne Whittingham, Flora Nassrallah,
Viviane Grandpierre,
Eunjung Na, Dorie Noll, Mina Salamatmanesh, Huidan Sun
• Children’s Hospital of Eastern Ontario (CHEO) Audiology
Team
Ontario Ministry of Industry and Innovation Early Researcher
Award
Acknowledgements
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Age at Diagnosis by Degree and Route
Durieux-Smith, Fitzpatrick & Whittingham, 2008
1980-2003: n=709
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Audiologists’ perspectives
I would say that my concept of the impact of unilateral hearing
loss has really changed. I treat unilaterals more seriously than I
ever used to We used to kinda say, oh, unilateral, he’ll
compensate, he’ll compensate…
Now I tell parents, … they will develop speech and language just
like a child with two ears, generally speaking. but I do tell the
parents that they are more at risk, certainly at school and in
acquiring language because they are going to have much more trouble
in noise and much more trouble with distance.
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Audiologists’ perspectives
And the old standard [applies]… a hearing loss is a hearing
loss, is a hearing loss to the parent.
I need more [information] on unilaterals, I feel more
comfortable with bilaterals than I do with unilaterals. In terms of
saying this is what I should do. I have a harder time…
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New challenges from newborn hearing screening: Children with
mild bilateral and unilateral hearing loss (MUHL)
E. Fitzpatrick, CASLPA 2011
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Ottawa context
• Screening 2002/2003 • ~14,000 babies annually
• > 95% coverage
• Ontario protocol – 2 stage hospital and/or community
screen
• Diagnostic audiology: Children’s Hospital of Eastern
Ontario
(CHEO)
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Profile of HL for children diagnosed at CHEO n=598
25.8%
36.0%
38.2%
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Degree of HL (impaired ear) for 154 children with UHL at
diagnosis (2003-2016)
Graphique1
high frequency
mild
moderate
mod-severe
severe
profound
Series 1
7.1
30.5
18.8
19.5
9.1
14.9
Sheet1
Series 1
high frequency7.1
mild30.5
moderate18.8
mod-severe19.5
severe9.1
profound14.9
To resize chart data range, drag lower right corner of
range.
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2010 – what we learned
• Cohort identified 1990-2006 – 291 of 670 = UHL/mild bilateral
– 255 with chart info - n=46 with UHL
• Age diagnosis = 60.4 months (IQR: 50.3–82.3) – only 20%
screened
Fitzpatrick, Durieux-Smith, & Whittingham (2010), Ear and
Hearing
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Amplification recommendation by HL at identification (n=255)
Fitzpatrick et al., 2010, Ear and Hearing
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Amplification practices
• 91.4% overall received a recommendation for amplification over
time….
• For mild bilateral: Amplification decision related to: Age of
identification (p
-
Amplification use
37% of UHL not used
Fitzpatrick et al., 2010, Ear and Hearing
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2014 – what we learned
• Cohort identified 1990-2010 – 46.3% (381 of 823) presented
with UHL/mild bilateral loss
(n=62 UHL)
Fitzpatrick et al (2014), Ear and Hearing
Chart1
high frequency
mild
moderate
mod-severe
severe
profound
Series 1
8.1
35.5
16.1
14.5
16.1
9.7
Sheet1
Series 1
high frequency8.1
mild35.5
moderate16.1
mod-severe14.5
severe16.1
profound9.7
To resize chart data range, drag lower right corner of
range.
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Age at diagnosis pre vs post-UNHS
Age
in y
ears
Chart1
Pre-UNHSPre-UNHS
Post-UNHSPost-UNHS
Unilateral
Mild Bilateral
5.4
4.9
0.3
0.8
Sheet1
UnilateralMild Bilateral
Pre-UNHS5.44.9
Post-UNHS0.30.8
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Amplification recommendation by HL at identification (n=337)
21%
Chart1
mildmild
HFHF
unilateralunilateral
no initial amp
amp at identification
Percentage of Children
41.5
58.5
43.6
56.4
79
21
Sheet1
mildHFunilateral
no initial amp41.543.679
amp at identification58.556.421
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Amplification recommendation for children with unilateral HL at
confirmation (n=62)
P > .05
Chart1
Pre UNHSPre UNHS
UNHS (>2002)UNHS (>2002)
no initial amp
amp at identification
Percentage of Children
73.9
26.1
93.8
6.3
Sheet1
Pre UNHSUNHS (>2002)
no initial amp73.993.8
amp at identification26.16.3
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Why the uncertainty?
• Lack of evidence of benefit
• Concerns about masking ‘good’ hearing’ particularly in young
children
• Not so successful with amplification use
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Amplification decisions Does age of HL identification
matter?
• Mild bilateral HL better ear 24% greater chance of amp rx for
each additional year
older at diagnosis (OR=1.24 ; 95% CI: 1.13 to 1.36) 263% greater
chance if diagnosed > age 4 vs < age 4 (OR
3.63; CI 2.10 to 6.27)
• Did not apply to UHL group
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Amplification decisions Does severity of HL matter?
• Mild bilateral HL – 8% greater chance of amp rx for every
decibel increase in
better ear. OR=1.08 (95% CI: 1.04 to 1.12) – Did not apply to
poorer ear
• Did not apply to UHL group (impaired ear)
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UHL 2017 what we learned
• Cohort diagnosed 2003 to 2015 (n=108)
• Onset = 54.6% congenital/early onset
• Age diagnosed = 13.9 months (IQR: 2.8, 49.0)
• Age amplification = 42.9 months (IQR: 20.1, 63.1)
Fitzpatrick et al. 2017. Characteristics of children with
unilateral hearing loss. Int’l J Audiol
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Unilateral hearing loss 2003-2015 (n=108)
Per
cent
age
Time to amplification recommendation
Graphique1
3 mos
6 mos
12 mos
24 mos
36 mos
48 mos
60 mos
> 60 mos
n
20
29
35
45
52
62
69
71
Feuil1
n
3 mos20
6 mos29
12 mos35
24 mos45
36 mos52
48 mos62
60 mos69
> 60 mos71
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Children with UHL N=108
Insufficient information N=16 (14.8%)
In study N=92 (85.2%)
Stable hearing loss N=53 (57.6%)
Deterioration > 20 N=8
Bilateral HL N=16 (17.4%)
Progressive HL N=39 (42.4%)
Deterioration > 10 dB impaired ear
N=15
Deterioration normal ear only
N=4
Deterioration Both N=12
Figure 1. Children with progressive hearing loss in impaired and
normal hearing ear
Impaired ear only N=23 (25.0%)
Deterioration > 20 dB impaired ear
N=6
Deterioration > 10 dB impaired ear
N=6
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New challenges from newborn hearing screening: Children with
mild bilateral and unilateral hearing loss (MUHL)
E. Fitzpatrick, CASLPA 2011
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Towards an Understanding of the Consequences of Mild Bilateral
and Unilateral Childhood Hearing Loss (MUHL)
12 months 24 months 36 months 48 months
Auditory Functioning
ELF - - - - CHILD
PEACH
Speech-Language
MacArthur-Bates CDI - Language sample
IDI CDI - - - - PPVT - - - PLS - - - GFTA
Child-Family Functioning
- CBCL - CBCL - PSI - PSI
Parents’ perspectives interview
Amplification use
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2017 – MUHL multi-center study (n=69)
N=38 UHL; 31 mild bilateral ; 50 normal hearing UHL group
characteristics: •Age diagnosed: 3.4 months (2.0, 5.5)
•Age amp recommended: 6.7 months (IQR: 4.6, 30.0)
•Age amp fitted: 12.2 months (IQR: 7.2, 29.9)
•23 with
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Amplification recommendations/use
UHL Age fitting = 12.2 months (IQR: 4.6, 30.0) 79% fitted with
amplification
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Amplification use at age 4 years – UHL
Age of fitting = 12.2 months (IQR: 4.6, 30.0) 79% fitted with
amplification
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PEACH
-
40
50
60
70
80
90
100
PEACH in quiet PEACH in noise
Scor
e (p
erce
ntag
e)
Unilateral Mild bilateral Hearing
PEACH scores – UHL, mild bilateral, normal hearing at age 4
*
*p=0.002
-
0
10
20
30
40
50
60
70
80
90
100
70 121 79 99 67 85 46 83 116
101 69 56 39 18 91 87 59 45 84 1 92 7 120 68 111 62 102 57
100
PEAC
H s
core
-noi
se (p
erce
ntag
e)
Child number
Unilateral Mean: Hearing group
0
10
20
30
40
50
60
70
80
90
100
73 93 81 2 55 71 98 77 110 89 107 74 25 75 12 82 72 94 95 64 106
63 80 112
PEAC
H s
core
-noi
se (p
erce
ntag
e)
Child number
Mild bilateral Mean: Hearing group
-
0
20
40
60
80
100
120
140
PPVT PLS-AC PLS-EC GFTA
Stan
dard
sco
re
Unilateral Mild bilateral Hearing
*
Language scores – UHL, mild bilateral, normal hearing at age
4
*
* p=0.02; p= 0.04
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Does amplification affect auditory/ language outcomes for
preschool-age children with UHL?
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UHL effect of amplification – MUHL preschool study
• Results showed no relationship (p=0.49) – small sample –
parent report
• May see effect as children age or on other types of
outcomes
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Does degree in impaired ear affect outcomes for preschool age
children with UHL?
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UHL effect of severity of hearing loss -MUHL preschool study
• Results showed no relationship (p=0.12) – small sample
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Parent Perspectives
• Interviews completed with 20 parents
• Themes: – Lacking information at diagnosis – Need professional
support (emotional care) – Support from other parents is important
– Support for hearing aid use
Fitzpatrick et al., 2016. Journal of Deaf Studies and Deaf
Education.
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Parent perspectives
She wears it (hearing aid) all the time now. At the beginning to
be honest, it was me forgetting, because as the parent you have to
remember…
The audiologist said, if we like, we can go ahead and get the
hearing aid, he’s doing fine, but just to be sure, go ahead and get
a hearing aid. But we started his daycare, and that was already a
big transition for him, so we didn’t want to introduce the hearing
aid right then…
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Parent perspectives
So, they said - adequate hearing for speech, and sent us away.
And it’s hard because I know… they have a huge caseload, and yes
the hearing losses are more severe, but it’s my kid, but to them,
it’s just one ear...
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2017 Preliminary data: Early School Years
• N=16 UHL; 16 mild bilateral :
• Age diagnosed 5.0 months (IQR: 4.0, 35.3)
• Age amp fitted: 37.4 months (IQR: 28.8, 44.0) – 7 Hearing aids
– 7 FM – school – 1 no amplification, 1 unknown
• Assessed: 6 – 8 years
PresenterPresentation Notes
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40
50
60
70
80
90
100
110
120
PEACHQuiet
PEACHNoise
Mea
n pe
rcen
tage
sco
re
Outcome measure Unilateral Bilateral
PEACH scores
-
0
10
20
30
40
50
60
70
80
90
100
1 2 3 4 5 6 7 8 9 1011121314151617181920212223242526272829
PE
AC
H -
Sco
re in
Noi
se (%
)
Case ID
PEACH - Noise Indvidual scores
No relationship with degree in impaired ear p=0.23
-
40
50
60
70
80
90
100
110
120
130
PPVT CELFCL
CELFRL
CELFEL
CTOPPPA
CTOPPPM
CTOPPRSN
WIATWR
WIATPD
GFTASW
Mea
n st
anda
rd s
core
Unilateral Bilateral
Language / Literacy Scores
-
0
20
40
60
80
100
120
140
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24
25 26 27 28
CTO
PP -
Com
posit
e Sc
ore
Case ID
No relationship with degree in impaired ear p=0.32
CTOPP Phonological Memory Individual scores
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Parents perspectives – MUHL School age
Adjusting to/learning about hearing loss “So some of the early
things that I thought we needed, I was nervous, I was scared for
her… I needed that education to set my mind at ease, and ...sort of
take some time to get the hearing aid, and get used to that she has
to wear this thing potentially for the rest of her life” “I was
surprised that I wasn’t offered anything to help out, to get her
hearing out of her other ear better, like the Bahas, the cochlear
implants and stuff. Because she was doing so well, I guess they
didn’t feel she needed it…"
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Parents perspectives MUHL School age
Adjusting to hearing technology ”Oh [we noticed a difference]
right away! Because she is in the French program, it really helped
her with some of the words, we speak English at home….but French
was a completely different language...the hearing aid made a huge
difference”
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What we know / don’t know about children with UHL
• represent 20-25% diagnosed with permanent HL • do not perform
like their peers • are now fitted early with amplification • use of
amplification in early years is a big challenge • don’t know the
impact of amplification use on
auditory/language development • We need long-term studies on
early-identified children
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“It was mostly unclear from studies if hearing aids or school
support mechanisms such as FM systems or
IEPs were utilized or evaluated...”
Anne, Lieu, & Cohen, 2017. Speech and language consequences
of unilateral hearing loss: A systematic review, Otol-Head Neck
Surg., 57, 572-579, p. 573
PresenterPresentation Notes
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PresenterPresentation Notes.
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Towards understanding the consequences of mild bilateral
and unilateral hearing loss (MUHL)
Thank you to Collaborating Centers
Children’s Hospital of Eastern Ontario, Ottawa
Pinecrest-Queensway Health Centre, Ottawa, Ontario
Western University Clinic, London, Ontario Erin Oaks Centre,
Toronto, Ontario
Preschool Services Branch, Ministry of Education, Ontario Voice
for Hearing-Impaired Children, Hamilton, Ontario
Slide Number 1DisclosureSlide Number 3Age at Diagnosis by Degree
and RouteAudiologists’ perspectivesAudiologists’ perspectivesNew
challenges from �newborn hearing screening:��Children with mild
bilateral �and unilateral hearing loss (MUHL)Ottawa contextProfile
of HL for children diagnosed at CHEO n=598Degree of HL (impaired
ear) for 154 children with UHL at diagnosis (2003-2016)2010 – what
we learnedAmplification recommendation by HL at identification
(n=255)�Amplification practicesAmplification use 2014 – what we
learnedAge at diagnosis pre vs post-UNHSAmplification
recommendation by HL at identification (n=337)Amplification
recommendation for children with unilateral HL at confirmation
(n=62)Slide Number 19Why the uncertainty?Amplification
decisions�Does age of HL identification matter?Amplification
decisions�Does severity of HL matter?UHL 2017what we
learnedUnilateral hearing loss 2003-2015 (n=108)Slide Number 25New
challenges from �newborn hearing screening:��Children with mild
bilateral �and unilateral hearing loss (MUHL)Towards an
Understanding of the Consequences of Mild Bilateral and Unilateral
Childhood� Hearing Loss (MUHL)2017 – MUHL multi-center study
(n=69)Amplification recommendations/useAmplification use at age 4
years – UHL PEACHPEACH scores – UHL, mild bilateral, normal hearing
at age 4Slide Number 33Language scores – UHL, mild bilateral,
normal hearing at age 4���Does amplification affect auditory/
language outcomes for preschool-age children with UHL?��UHL effect
of amplification – MUHL preschool study���Does degree in impaired
ear affect outcomes for preschool age children with UHL?����UHL
effect of severity of hearing loss�-MUHL preschool studyParent
PerspectivesParent perspectives Parent perspectives 2017
Preliminary data:�Early School Years PEACH scoresPEACH - Noise
Indvidual scoresSlide Number 45CTOPP Phonological Memory�Individual
scoresParents perspectives – MUHL School ageParents
perspectives�MUHL School ageWhat we know / don’t know about
�children with UHLSlide Number 50Slide Number 51����� � Towards
understanding the consequences �of mild bilateral �and unilateral
hearing loss (MUHL)���Thank you to Collaborating
Centers���Children’s Hospital of Eastern Ontario,
Ottawa�Pinecrest-Queensway Health Centre, Ottawa, Ontario�Western
University Clinic, London, Ontario�Erin Oaks Centre, Toronto,
Ontario�Preschool Services Branch, Ministry of Education,
Ontario�Voice for Hearing-Impaired Children, Hamilton,
Ontario�����