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Prof. Gerard M. O’Donoghue University of Nottingham, UK
Paediatric cochlear implants: challenges and future directions
Current Developments and New Directions In
Pediatric Audiology, Istambul 2011
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The best of times ...
Late identification
Delayed diagnosis
Poor amplification
Education : low expectation
No cochlear implants
Public Health Policy
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Prostheses
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Prostheses - implantable
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Overview
Neurobiology of deafness Plasticity into Practice Early cochlear implantation Bilateral CI Medical and surgical aspects Electro-acoustic hearing / ABI etc Turkish Temple
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Human Auditory System
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Lost around time of birth Normal morphogenesis Critical gene product (Steel 2009)
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50-90% loss of CN neurones
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Quality and number of synapses Hyperexcitability (Eggermont 2008)
Ryugo et al 1997
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a
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a
No sensory system exists in isolation
Non-auditory effects of deprivation – attention, working memory, executive functioning etc
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Conel, 1939-1967
-1 Mo. Birth 1 Mo. 3 Mo. 6 Mo.
15 Mo. 2 Years 4 Years 6 Years
Development Human Cortex
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Huttenlocher, Dabholkar 1997, J Comp Neurol 387:167-178.
Auditory cortex Visual cortex Prefrontal cortex
1 y. 2 y. 4 y. 6 y. 15 y. Adult Birth -3. M.
Age
Syn
ap
ses /
100 µ
m³
Synaptic Pruning
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Synaptic Maturation
Kral and O’Donoghue, New Eng J Med 2010
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Cochlear Implants and Brain Plasticity
Cochlear Implants and Plasticity
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1.0
0.8
0.6
0.4
0.2
0.0
0.2
Coch
lea-t
o-c
ort
ex c
orr
ela
tion (
r)
Normal Deaf Stim.
Cochleotopic Organisation of Cortex - CI
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1.0
0.8
0.6
0.4
0.2
0.0
0.2
Coch
lea-t
o-c
ort
ex c
orr
ela
tion (
r)
Normal Deaf Stim.
(Fallon et al., 2009)
Cochleotopic Organisation of Cortex - CI
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Dorman et al 2009
Cross modal reorganisation after CI
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Overview
Neurobiology of deafness Plasticity into Practice Early cochlear implantation Bilateral CI Medical and surgical aspects Electro-acoustic hearing / ABI etc Turkish Temple
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Plasticity into Practice [ Carol Flexer]
Not about ears: its all about the brain
Prostheses: brain access tools
‘Its not what we hear, its what we do with what we hear’
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Plasticity in Practice
46 million words heard by age 4 (Hart and Risley)
20,000 hours listening for reading (Dehaene)
Hearing loss: 3 times exposure to learn new words / concepts (Pittman)
Acoustically favourable conditions
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Huttenlocher, Dabholkar 1997, J Comp Neurol 387:167-178.
Auditory cortex Visual cortex Prefrontal cortex
1 y. 2 y. 4 y. 6 y. 15 y. Adult Birth -3. M.
Age
Syn
ap
ses /
100 µ
m³
Synaptic Pruning
46 Million Words
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Huttenlocher, Dabholkar 1997, J Comp Neurol 387:167-178.
Auditory cortex Visual cortex Prefrontal cortex
1 y. 2 y. 4 y. 6 y. 15 y. Adult Birth -3. M.
Age
Syn
ap
ses /
100 µ
m³
Synaptic Pruning
46 Million Words
X3
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Huttenlocher, Dabholkar 1997, J Comp Neurol 387:167-178.
Auditory cortex Visual cortex Prefrontal cortex
1 y. 2 y. 4 y. 6 y. 15 y. Adult Birth -3. M.
Age
Syn
ap
ses /
100 µ
m³
Synaptic Pruning
Reading 20,000 hours listening
(normally hearing)
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Receptive and Expressive standardised language scores
as a function of age of identification for children with
'normal cognition' (Yoshinago-Itano, 1999)
Receptive
<6m
>6m
Expressive
<6m
>6m
Age of identification
40 60 80 100 120
Language quotient
Early intervention
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Receptive and Expressive standardised language scores
as a function of age of identification for children with
'low cognition' (Yoshinago-Itano, 1999)
Receptive
<6m
>6m
Expressive
<6m
>6m
Age of identification
40 60 80 100 120
Language quotient
Early intervention
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Overview
Neurobiology of deafness Plasticity into Practice Early cochlear implantation Bilateral CI Medical and surgical aspects Electro-acoustic hearing / ABI etc Turkish Temple
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Early Cochlear Implantation
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Age of implant 0-4 years 2000 to 2010
0%
20%
40%
60%
80%
100%
Benelux France Germany Italy Poland United
Kingdom
Benelux France Germany Italy Poland United
Kingdom
2000 2010
2 to 4 Years
1 to 2 Years
0 to 1 Years
Source: Cochlear Ltd database
Implanted < 1y
40%
60%
80%
100%
2000
Bex Fr Ger It Pol UK
20%
2000
0%
Age at implant 0-4 years 2000 to 2010
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0%
20%
40%
60%
80%
100%
Benelux France Germany Italy Poland United
Kingdom
Benelux France Germany Italy Poland United
Kingdom
2000 2010
2 to 4 Years
1 to 2 Years
0 to 1 Years
Source: Cochlear Ltd database
2010 Belx Fr Ger It Pol UK Belx Fr Ger It Pol UK
2000
40%
60%
80%
100%
20%
0%
Age at implant 0-4 years 2000 to 2010
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Audiological Certainty
What age?
What measures?
What validity?
Who decides?
Parameters of hearing aid trial
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Overview
Neurobiology of deafness Plasticity into Practice Early cochlear implantation Bilateral CI Medical and surgical aspects Electro-acoustic hearing / ABI etc Turkish Temple
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0%
10%
20%
30%
40%
50%
60%
70%
80%
90%
100%
19
92
19
93
19
94
19
95
19
96
19
97
19
98
19
99
20
00
20
01
20
02
20
03
20
04
20
05
20
06
20
07
20
08
Nu
mb
er
of
bila
tera
l
Nu
cle
us
rec
ipie
nts
European Data - Bilaterals
Source: Cochlear Ltd database
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Bilateral implants children 2005:2010
Source: Cochlear Ltd database
0%
20%
40%
60%
80%
100%
Benelux France Germany Italy Poland United Kingdom Benelux France Germany Italy Poland United Kingdom
2005 2010
Bilateral Unilateral
40%
60%
80%
100%
20%
0% Bex Fr Ger It Pol UK Bex Fr Ger It Pol UK
2005
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Source: Cochlear Ltd database
0%
20%
40%
60%
80%
100%
Benelux France Germany Italy Poland United Kingdom Benelux France Germany Italy Poland United Kingdom
2005 2010
Bilateral Unilateral
40%
60%
80%
100%
20%
0% Belx Fr Ger It Pol UK Belx Fr Ger It Pol UK
NICE 2009
2010 2005
Bilateral implants children 2005:2010
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Bilateral implantation recommended for children & some adults
Simultaneous
Additional contralateral
Bilaterals – UK Provision
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National Audit Bilaterals:UK
Localisation
Speech recognition in background noise
Speech
Language
Listening
Parental perception
Quality of life
Surgical data including complications
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Overview
Neurobiology of deafness Plasticity into Practice Early cochlear implantation Bilateral CI Medical and surgical aspects Electro-acoustic hearing / ABI etc Turkish Temple
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Surgical challenges - BICI
Minimal Access Surgery
Operating time
Anesthesia
Facial nerve injury
Co-morbidity – known or unknown
Electrocautery
Loss vestibular function / residual hearing
Meningitis risk
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Overview
Neurobiology of deafness Plasticity into Practice Early cochlear implantation Bilateral CI Medical and surgical aspects Electro-acoustic hearing / ABI etc Turkish Temple
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&
Electrical Stimulation Acoustical Stimulation
Hearing aids + Implants
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Frequency (kHz)
-10
0
10
20
30
40
50
60
70
80
90
100
110
120
0,125 0,25 0,5 1
Heari
ng L
oss
(dB
HL
)
Lower Limit Upper Limit
1 1,5 2 3 4 6 8 10
Electro-acoustic hearing
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Single Side Deafness and CI
CI CROS Aid
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Drug and Stem Cell delivery systems
Advances in Implant Technology
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Auditory Brainstem Implant
(Lenarz et al , Colletti et al 2006)
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Overview
Neurobiology of deafness Plasticity into Practice Early cochlear implantation Bilateral CI Medical and surgical aspects Electro-acoustic hearing / ABI etc Turkish Temple
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Temple of Artemis
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J. Gravel 2007
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J. Gravel 2007
Information and systems give meaning to everything we do; there is no point in going through the motions of providing a service if there are no systems on which to support, record or monitor the process and outcomes Sir Muir Gray 2007
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Deafness - not just about hearing loss
Prostheses as brain access tools
Earlier is best - but not a cure!
Bilateral CI - timely, safe, efficient
Combined acoustical and electrical hearing
Audiological certainty - central
Paediatric audiology ‘Temple’
Summary