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

Disclosure

• No conflicts of interest related to any research

• Phonak sponsorship for travel/accommodations for this conference

• 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

Age at Diagnosis by Degree and Route

Durieux-Smith, Fitzpatrick & Whittingham, 2008

1980-2003: n=709

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.

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…

New challenges from newborn hearing screening: Children with mild bilateral and unilateral hearing loss (MUHL)

E. Fitzpatrick, CASLPA 2011

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)

Profile of HL for children diagnosed at CHEO n=598

25.8%

36.0%

38.2%

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.

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

Amplification recommendation by HL at identification (n=255)

Fitzpatrick et al., 2010, Ear and Hearing

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

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.

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

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

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

Why the uncertainty?

• Lack of evidence of benefit

• Concerns about masking ‘good’ hearing’ particularly in young children

• Not so successful with amplification use

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

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)

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

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

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

New challenges from newborn hearing screening: Children with mild bilateral and unilateral hearing loss (MUHL)

E. Fitzpatrick, CASLPA 2011

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

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

Amplification recommendations/use

UHL Age fitting = 12.2 months (IQR: 4.6, 30.0) 79% fitted with amplification

Amplification use at age 4 years – UHL

Age of fitting = 12.2 months (IQR: 4.6, 30.0) 79% fitted with amplification

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

Does amplification affect auditory/ language outcomes for preschool-age children with UHL?

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

Does degree in impaired ear affect outcomes for preschool age children with UHL?

UHL effect of severity of hearing loss -MUHL preschool study

• Results showed no relationship (p=0.12) – small sample

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.

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…

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...

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

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

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…"

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”

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

“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

PresenterPresentation Notes.

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�����