Title: CONGENITAL CYTOMEGALOVIRUS (cCMV) DEAF CHILDREN USING COCHLEAR IMPLANTS The Nottingham experience
1CONGENITAL CYTOMEGALOVIRUS (cCMV) DEAF CHILDREN
USING COCHLEAR IMPLANTS The Nottingham
experience
- Jayne Ramirez Inscoe
- Speech and Language Therapist
- January 2013
2Some 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?
3Aims 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
long-term educational and linguistic outcomes
4What is known about cCMV?
- gt90 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
5Lot of research into CMV recently
- What can it tell us about the children we work
with??
6Most 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)
7Internal audit of cCMV deaf children 1999
- ChiP (Childrens Implant Profile (Hellman et al.,
1991 Edwards et al., 2003) - Face-to-face Interviews with TODs, Audiologists
and SLTs at NCIP - Parental telephone reports
8Four themes emerged
- Audiological issues
- Medical/developmental issues
- Speech/language/communication issues
- Behaviour issues
9Audiological issues
- Short attention span
- Challenging behaviour
- Intolerance of speech signal (ASD cases)
- Frequent illness missed appointments,
inconsistent wearing of speech processor
10Medical/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
11Speech/Language/Communication issues
- Over 70 rated as having specific concerns
- Notable problems with
- - Interaction difficulties/communication
style - - Speech production difficulties
12Behaviour issues
- 67 rated as having some concerns
- Attention control!
- Behaviour management issues
13Long-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
14NB. 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
15Details of children in NCIP study 2004
- Confirmed diagnosis of CMV
- 16 children 8 boys, 8 girls
- Mean age at implantation 309 years
- At least 12 months follow-up (range1-5 years)
- Implanted between Jan.90 and Jan.01
- All received Nucleus multi-channel cochlear
implants - Control group131 congenitally profoundly deaf,
mean age at implantation401years
16Results 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 (p0.04) - 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) (pgt0.05)
17Conclusion 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
18Trends 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
19Current educational placement
20(No Transcript)
21Average SIR rating after 10 years implant use is
2.8
22Possible 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
23Results
- 74 of cCMV deaf children have a significant
ongoing difficulty - 35 have 3 or more additional difficulties
24Behaviour 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
25Autistic Spectrum Disorders 17.6 have a formal
diagnosis
- he doesnt like changing routines
- he wont tolerate sounds he doesnt like
- he has difficulties with social interaction
- she is benefiting from being in a more
structured setting
26Cognitive difficulties (41 of group)
- maths is her worst subject she just cant
understand it - she cant grasp time concept
- I dont know how he will cope in mainstream with
poor organisational skills - she finds reading and handwriting very
difficult
27Language and Communication problems (56 of group)
- I cant 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
28NCIP specific findings or not?
- Remarkably similar proportions of cCMV deaf
children with these difficulties found by South
West England cochlear implant groups (2011 audit).
29Other difficulties
- Physical, not only gross motor skills (17.5) but
also - our children cant 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
30Ongoing 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
31Pilot study Working Memory training
- Clinical Psychologist
- CogMed
- 2 children with ongoing concentration and memory
difficulties - Home/school training package
32Working Memory
33Why 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, cant
keep it in there long enough! - Use visual clues to support learning if poor
phonological loop
34Attention
- Child needs to be able to
- Focus
- Divide
- Switch
- Inhibit
- Also need to increase processing speed!
35Areas to work on
- Attention
- Processing speed
- Rehearsal
- Use of visual clues
- Manipulating verbal information (eg. backwards,
after time delay)
36Early 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
37Conclusion
- 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 childs development aside
from hearing - These difficulties may require specific
structured rehabilitation
38- Thank you for listening!
- Any questions?