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? • >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

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 (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

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: 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

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 (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)

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:

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 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’

26. 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’

27. 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’

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 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

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, can’t 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 child’s development aside from hearing • These difficulties may require specific structured rehabilitation

38. Thank you for listening! • Any questions?