CONGENITAL CYTOMEGALOVIRUS c CMV DEAF CHILDREN USING COCHLEAR
- Slides: 38
CONGENITAL CYTOMEGALOVIRUS (c. CMV) 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 c. CMV deafness • All 3 children had significant additional difficulties affecting progress with a cochlear implant • Interest in c. CMV 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 c. CMV deaf children using a cochlear implant • Examine the impact of these difficulties on longterm educational and linguistic outcomes
What is known about c. CMV? • >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 c. CMV However, growing body of literature describing functional outcomes • UK c. CMV Association has carried out a survey of additional difficulties as reported by the childrens’ parents/carers • BATOD has published several articles about c. CMV deaf children (Nicky Povey-Howell, TOD; Jayne Ramirez Inscoe, SLT; Carmen Burton, parent of c. CMV deaf child) • Cochlear Implants International: additional difficulties in c. CMV deaf children using cochlear implants (Ramirez Inscoe 2011)
Internal audit of c. CMV deaf children 1999: • Chi. P (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; 01 years
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 c. CMV children (2010) • 34 confirmed c. CMV 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:
Average SIR rating after 10 years implant use is 2. 8
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 c. CMV 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 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 c. CMV 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 c. CMV 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 • Cog. Med • 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 c. CMV on a child’s development aside from hearing • These difficulties may require specific structured rehabilitation
• Thank you for listening! • Any questions?
- Cytomegalovirus
- Cmv
- Cmv retinitis
- Viracor cmv-avr
- Cmv microcephaly
- Cmv
- Cmv
- Cmv microcephaly
- Best viruses
- Cmv klierkoorts
- Roxanne
- Vestibular membrane
- "cochlear implant"
- Retinal layers
- Carotid cochlear dehiscence
- "cochlear implant facts"
- James denison deaf
- Cochlear implant history
- Cochlear implant
- Cochlear duct
- Rp full form
- Pros and cons of cochlear implants quizlet
- Cochlear
- Nucleus 5 cochlear implant troubleshooting
- Canadian congenital heart alliance
- Congenital rubella syndrome triad
- Egg on a string heart
- Congenital
- Rds pathophysiology
- Von rosen view
- Farah garmany
- Kode icd palatoschisis
- Non classical adrenal hyperplasia
- Congenital voice disorders
- Triade di hutchinson
- Cyanotic congenital heart disease
- Bilateral macostomia
- Guyton
- Congenital glaucoma