CONGENITAL CYTOMEGALOVIRUS
(cCMV) DEAF CHILDREN USING
COCHLEAR IMPLANTS
The Nottingham experience
Jayne Ramirez Inscoe –
Speech and Language Therapist
January 2013
Some background information
• In 1994 I started work as SLT on Nottingham cochlear implant
programme
• 3/50 (6%) children on my caseload had known cCMV deafness
• All 3 children had significant additional difficulties affecting progress
with a cochlear implant
• Interest in cCMV grew
- trends in types of additional difficulties?
- long-term outcomes following implantation?
Aims of this presentation:
• Investigate prevalence
and co-occurrence of
additional difficulties of
cCMV deaf children
using a cochlear implant
• Examine the impact of
these difficulties on longterm educational and
linguistic outcomes
What is known about cCMV?
• >90% infants who survive active CMV infection
will develop late complications eg. hearing loss,
delayed psychomotor development, learning
disabilities, expressive language delays (Lee et
al., 2005)
• Following cochlear implantation, hearing loss
may not present the biggest challenge for
rehabilitation
Lot of research into CMV
recently …
• What can it tell us about the children
we work with??
Most recent research looks at
diagnosis and treatment of cCMV
However, growing body of literature describing
functional outcomes
• UK cCMV Association has carried out a survey of
additional difficulties as reported by the childrens’
parents/carers
• BATOD has published several articles about cCMV deaf
children (Nicky Povey-Howell, TOD; Jayne Ramirez
Inscoe, SLT; Carmen Burton, parent of cCMV deaf child)
• Cochlear Implants International: additional difficulties in
cCMV deaf children using cochlear implants (Ramirez
Inscoe 2011)
Internal audit of cCMV deaf children
1999:
• ChiP (Children’s
Implant Profile
(Hellman et al., 1991;
Edwards et al., 2003)
• Face-to-face
Interviews with TODs,
Audiologists and
SLTs at NCIP
• Parental telephone
reports
Four themes emerged
•
•
•
•
Audiological issues
Medical/developmental issues
Speech/language/communication issues
Behaviour issues
Audiological issues
•
•
•
•
Short attention span
Challenging behaviour
Intolerance of speech signal (ASD cases)
Frequent illness – missed appointments,
inconsistent wearing of speech processor
Medical/developmental issues
• Almost 50% had multiple disabilities
• CMV leads to immune deficiency problems
- nearly all CMV children have frequent
illnesses – ‘winter’ very badly
• Problems reported with major organs other
than ears
• Many have other difficulties affecting early
development
Speech/Language/Communication
issues
• Over 70% rated as having specific
concerns
• Notable problems with:
- Interaction difficulties/communication
style
- Speech production difficulties
Behaviour issues
• 67% rated as having some concerns
• Attention control!
• Behaviour management issues
Long-term outcomes
• Follow up children and young people up to
15 years post implantation – generally
slower progress; some difficulties can
resolve
• Compare outcomes with trends following
cochlear implantation
• Will inform expectations counselling
• Long-term ongoing needs …
NB. Wide variation in outcomes!
• Cochlear Implantation in Children deafened by
Cytomegalovirus: Speech Perception and
Speech Intelligibility Outcomes. Ramirez Inscoe
JM & Nikolopoulos TP. J Otology & Neurotology
25 (2004)
• Pyman et al. Am J Otol (2000) concluded that
those with significant cognitive impairment had a
poorer prognosis in spoken language
development following cochlear implantation
than if this was not present
Details of children in NCIP study
2004
• Confirmed diagnosis of CMV
• 16 children: 8 boys, 8 girls
• Mean age at implantation: 3;09
years
• At least 12 months follow-up
(range=1-5 years)
• Implanted between Jan.’90
and Jan.’01
• All received Nucleus multichannel cochlear implants
• Control group=131
congenitally profoundly deaf,
mean age at
implantation=4;01years
Results of NCIP study
• IOWA Test of Speech Perception (Tyler &
Holstad,87) -level A
At the last follow-up interval, 6% scored better, 38%
worse and 56% the same as the median score of the
non-CMV congenitally deaf children at the same
interval (p=0.04)
• Speech Intelligibility Rating (SIR)
At the last follow-up interval, 19% developed speech
intelligibility better than the median of the congenitally
deaf group, (50% worse and 31% the same) (p>0.05)
Conclusion of NCIP study
• Wide variation in outcomes
• But significant auditory benefit from CI (also found by
Lee)
• For many, rate of progress appears to be slower than
other CI users in the first 3 years
• Presence of co-existing central (cognitive) disorders
affects prognosis in speech development
• Co-incidental CMV infection can exist! (deaf sibling)
• Progressive hearing loss can produce different
outcomes
Need for follow up to assess long-term benefits of
cochlear implants
Trends in the long-term for cCMV
children (2010)
• 34 confirmed cCMV deaf children implanted by
NCIP
• CI experience= 2-15 years
• Mean age at CI= 51 months (14-187 months)
• 27 of these have used a cochlear implant for
more than 5 years
Current educational placement:
Educational placement after 5 yrs cochlear implant use
for CMV group (N=27)
100%
80%
spec sch
sch for deaf
40%
unit
%
60%
M/S
20%
0%
CMV group
%
% CMV children needing sign 10 years post
cochlear implantation (N=14)
100
80
60
40
20
0
% CMV children
1
Need sign
2
Oral
Average SIR rating after 10 years implant
use is 2.8
Children with 10 or more years cochlear implant
use (N=14)
5
SIR rating
4
3
2
1
0
0
10
5
CMV children
15
Possible reasons for these
outcomes
•
•
•
•
•
•
•
Presence of additional difficulties (BCS database,
Robbins)
Behaviour
Autistic Spectrum Disorders
Cognitive difficulties
Language and communication problems
Physical difficulties
Visual impairment
Oro-motor problems
Results:
• 74% of cCMV deaf children have a
significant ongoing difficulty
• 35% have 3 or more additional difficulties
Behaviour difficulties - 32% continue to have
significant problems with attention control,
distractibility and behaviour outbursts
• ‘he still has very limited
concentration and he
distracts others’
• ‘her behaviour is fine if
the world is following her
agenda’
• ‘I am struggling to get
help in managing her
behaviour’
• ‘her behaviour is very
volatile and challenging’
Autistic Spectrum Disorders – 17.6%
have a formal diagnosis
• ‘he doesn’t like
changing routines’
• ‘he won’t tolerate
sounds he doesn’t like’
• ‘he has difficulties with
social interaction’
• ‘she is benefiting from
being in a more
structured setting’
Cognitive difficulties (41% of group)
• ‘maths is her worst
subject – she just can’t
understand it’
• ‘she can’t grasp time
concept’
• ‘I don’t know how he will
cope in mainstream with
poor organisational skills’
• ‘she finds reading and
handwriting very difficult’
Language and Communication problems
(56% of group)
• ‘I can’t say long words’
• ‘there is still a huge gap
between her receptive
and expressive
language’
• ‘her signing is often
bizarre and inaccurate’
• ‘talking is too hard for
me’ (signed statement)
• ‘he has specific
problems processing
spoken language’
• ‘her speech deteriorates
when she is excited or
upset’
NCIP specific findings or not?
• Remarkably similar proportions of cCMV
deaf children with these difficulties found
by South West England cochlear implant
groups (2011 audit).
Other difficulties:
• Physical, not only gross motor skills (17.5%) but also
…
‘our children can’t tie shoe laces, ride a bike or do
up their clothes correctly’
• Sensory Integration difficulties, ‘she will only eat
beige food’ (%?)
• Visual, (5%)
• Oro-motor problems (11%) often presenting as
dyspraxic tendencies
Ongoing needs of cCMV deaf children
with cochlear implants:
• Tease out the difficulties
• Refer to other agencies, eg. Occupational therapy, (SI),
behaviour management specialists, CAMHS, dyspraxia,
dyscalculia specialists
• Prioritise needs and provide structured support and
therapy
• Acknowledge child may be better placed in a more
specialised educational setting
• Recognise child may need signing to aid language
processing and expression
Pilot study Working Memory training
• Clinical Psychologist
• CogMed
• 2 children with ongoing concentration and
memory difficulties
• Home/school training package
Working Memory
Why working memory is so
important
• Central executive function controls attention!
• Phonological loop holds memory trace of
speech, sub-vocal rehearsal keeps it in there
long enough to process it
• If got speech or learning difficulties, can’t keep it
in there long enough!
• Use visual clues to support learning if poor
phonological loop
Attention
•
•
•
•
•
Child needs to be able to:
Focus
Divide
Switch
Inhibit
Also need to increase processing speed!
Areas to work on
•
•
•
•
•
Attention
Processing speed
Rehearsal
Use of visual clues
Manipulating verbal information (eg.
backwards, after time delay)
Early outcomes of pilot study
• Child A
Parent reported inability to improve memory at
level child was struggling at, found it difficult to
motivate child
• Child B
Over-reaction to rewards given after successful
improvement significantly affected subsequent
progress
Conclusion
• Longitudinal studies have shown that there
are clear trends in the ongoing presence
and impact of additional cognitive and
motor difficulties in this population
• Parents and professionals should be
aware of the impact of cCMV on a child’s
development aside from hearing
• These difficulties may require specific
structured rehabilitation
• Thank you for
listening!
• Any questions?