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 long-term 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 multi-

channel 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)

0%

20%

40%

60%

80%

100%

CMV group

%

spec sch

sch for deaf

unit

M/S

% CMV children needing sign 10 years post cochlear implantation (N=14)

020406080

100

1 2

Need sign Oral

%

% CMV children

Average SIR rating after 10 years implant use is 2.8

Children with 10 or more years cochlear implant use (N=14)

0

1

2

3

4

5

0 5 10 15

CMV children

SIR

ra

tin

g

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?