CONGENITAL CYTOMEGALOVIRUS (cCMV) DEAF CHILDREN USING COCHLEAR IMPLANTS The Nottingham experience - PowerPoint PPT Presentation

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CONGENITAL CYTOMEGALOVIRUS (cCMV) DEAF CHILDREN USING COCHLEAR IMPLANTS The Nottingham experience

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CONGENITAL CYTOMEGALOVIRUS (cCMV) DEAF CHILDREN USING COCHLEAR IMPLANTS The Nottingham experience Jayne Ramirez Inscoe Speech and Language Therapist – PowerPoint PPT presentation

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Title: CONGENITAL CYTOMEGALOVIRUS (cCMV) DEAF CHILDREN USING COCHLEAR IMPLANTS The Nottingham experience


1
CONGENITAL CYTOMEGALOVIRUS (cCMV) DEAF CHILDREN
USING COCHLEAR IMPLANTS The Nottingham
experience
  • Jayne Ramirez Inscoe
  • Speech and Language Therapist
  • January 2013

2
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?

3
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
    long-term educational and linguistic outcomes

4
What 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

5
Lot of research into CMV recently
  • What can it tell us about the children we work
    with??

6
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)

7
Internal 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

8
Four themes emerged
  • Audiological issues
  • Medical/developmental issues
  • Speech/language/communication issues
  • Behaviour issues

9
Audiological issues
  • Short attention span
  • Challenging behaviour
  • Intolerance of speech signal (ASD cases)
  • Frequent illness missed appointments,
    inconsistent wearing of speech processor

10
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

11
Speech/Language/Communication issues
  • Over 70 rated as having specific concerns
  • Notable problems with
  • - Interaction difficulties/communication
    style
  • - Speech production difficulties

12
Behaviour issues
  • 67 rated as having some concerns
  • Attention control!
  • Behaviour management issues

13
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

14
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

15
Details 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

16
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 (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)

17
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

18
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

19
Current educational placement
20
(No Transcript)
21
Average SIR rating after 10 years implant use is
2.8
22
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

23
Results
  • 74 of cCMV deaf children have a significant
    ongoing difficulty
  • 35 have 3 or more additional difficulties

24
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

25
Autistic 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

26
Cognitive 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

27
Language 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

28
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).

29
Other 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

30
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

31
Pilot study Working Memory training
  • Clinical Psychologist
  • CogMed
  • 2 children with ongoing concentration and memory
    difficulties
  • Home/school training package

32
Working Memory
33
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, cant
    keep it in there long enough!
  • Use visual clues to support learning if poor
    phonological loop

34
Attention
  • Child needs to be able to
  • Focus
  • Divide
  • Switch
  • Inhibit
  • Also need to increase processing speed!

35
Areas to work on
  • Attention
  • Processing speed
  • Rehearsal
  • Use of visual clues
  • Manipulating verbal information (eg. backwards,
    after time delay)

36
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

37
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 childs development aside
    from hearing
  • These difficulties may require specific
    structured rehabilitation

38
  • Thank you for listening!
  • Any questions?
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