University of Edinburgh
 

Working with children with Cytomegalovirus, Auditory Neuropath Spectrum Disorders or Autistic Spectrum disorders

Presented on Monday 2 November 2009

Cytomegalovirus (CMV)

Jackie Salter, The Ear Foundation

What is CMV?

  • Virus often 'silent'
  • Like a mild cold/flu part of the herpes family
  • Remains with you for life
  • Person to person contact
  • Present in body fluids
  • Centre for Diseases Control and Prevention in USA estimate up to 90% age over 40 are infected

How is it spread?

  • Present in body fluids
  • Can only exist outside the body for a short time
  • Person to person contact
  • A pregnant woman can pass it to her unborn child
  • Blood transfusions and organ transplants

How to prevent infection

  • Wash hands with soap and water
  • Do not share food, drink, cutlery, cups, dummies etc with young children
  • Do not kiss young children on the mouth
  • Nursery workers should wear gloves to change nappies
  • Toys should be washed regularly

CMV and Pregnancy

  • Initial infection during pregnancy or if pregnancy occurs within 6 months of infection
  • Approx one third infected women pass on virus to unborn child
  • Of which 90% will be asymptomatic
  • A recurrence of the infection may be passed on
  • Can cause a number of different disabilities

Symptomatic/ Asymptomatic

  • Symptomatic - born with the virus active
  • Asymptomatic - no signs of the virus being active therefore often goes undiagnosed

Symptoms of CMV Infection at birth

  • Low birth weight
  • Enlarged liver
  • Enlarged spleen
  • Jaundice
  • Fits
  • Small head
  • Red spots under the skin
  • Patches of calcium in the brain
  • Eye problems
  • Deafness

Natalie

  • Full term/ premature?
  • Birth weight 2.1 kg
  • Symptomatic of CMV
  • Jaundice, enlarged liver and spleen, bleed and water on the brain, petechial rash, blood would not clot, heart murmur
  • Not expected to survive
  • Blood transfusion
  • 10 weeks in SCUBU
  • Subsequent developmental display

Natalie Today

  • Profound bilateral deafness cochlear implant at 3 years
  • Gross motor skills delay (sat 15 months, walked at 3 years)
  • Poor fine motor skills and mixed tone
  • Difficulties with feeding and weight gain
  • Asthma, eczema and allergies
  • Dyscaculia diagnosed at 7 years
  • Behaviour issues although these are improving with professional guidance
  • Concentration, memory and organisation
  • Epilepsy developed at puberty
  • 11 A-C Grade GCSE equivalents

Megan 22.9.96

  • Apparently normal pregnancy until approximately 6 months
  • Delivered early, 32 weeks, by emergency caesarean as twin was becoming distressed
  • Low birth weights 1.2kg and 1.6 kg
  • Asymptomatic
  • CMV discovered during a full screen when twin had respiratory problems
  • Blood test indicated virus in same state in both twins and myself but not active Heather had feeding and breathing difficulties and spent 3 months in neonatal unit
  • At 5 weeks Megan contracted RSV bronchiolitus and spent 12 days on a ventilator
  • Heather continued to have feeding and breathing difficulties at 18 months she weighed just 6 kg and contracting recurrent pneumonias that led to 40-50% tissue loss in her lungs and oxygen dependency for approximately 18 months
  • Severe gastro-oesophageal reflux was diagnosed leading to a regime of continual feeding

Megan today

  • Profound unilateral hearing loss (badly affected by glue ear when younger causing a lot of difficulties)
  • Visual perceptual difficulties
  • Finds maths difficult and rapidly becomes disheartened
  • Concentration variable although this is improving
  • Unable to sit still! Although this too is improving
  • Attends local mainstream secondary
  • KS2 Sats: English Level 5 (reading 5, writing 4) Science 5 Maths 4
  • Plays Flute
  • School Production
  • Swims

How big a problem is it?

  • Approx. 1 in 150 live births
  • 11% Symptomatic
  • 89% Asymptomatic
  • 90% Symptomatic experience permanent disability
  • Estimated up to 65 % with a HL
  • 10% Asymptomatic experience permanent disability
  • Estimated up to 23% with a HL

Type of hearing Loss

  • Symptomatic: 52% bilateral 48% unilateral
  • Asymptomatic: 33% bilateral 67% unilateral
  • 66% of all hearing losses are severe or profound
  • Hearing loss not always present at birth so not detected by NHSP
  • Is often progressive
  • Can fluctuate
  • Can improve
  • May occur several years after birth

Chimes Study

  • CMV was recognised as aeitiology of HL in USA in 2006
  • Recent study in USA estimates 21% of HL at birth and 25% Hl in 4 year olds is caused by CMV
  • Screen 100 000 new borns for CMV
  • Follow up all those CMV positive with structured systematic hearing screening programme
  • Estimate should be able to profile approximately 9000 children

The impact of Congenital CMV

  • Hearing loss
  • Autism Yamashita et al, 2003; Enstrom et a., 2008
  • Visual impairment
  • Cerebral palsy
  • Learning difficulties
  • Feeding difficulties
  • Poor or no vestibular function
  • Physical and motor impairment
  • Behavioural problems
  • Epilepsy
  • The effects of congenital CMV may develop later

Details of children in NCIP study

  • 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
    Nottingham Cochlear Implant Programme

Cochlear Implantation in Children deafened by Cytomegalovirus: Speech Perception and Speech Intelligibility Outcomes. Ramirez Inscoe JM & Nikolopoulos TP. J Otology & Neurotology 25 (2004)

Notes: Let me now outline our study method. We actually implanted 17 children with known CMV in 10 years. (One child with progressive hearing loss was not included in the study, born with less severe hearing loss, which became profound at 3 years. Her rate of progress was faster than the rest of the group).

Control group

Progress of group was compared with 131 congenitally deaf children who did not have a diagnosis of CMV . Mean age at implantation=4.1 years. same follow-up period. Interestingly in the period Jan '01 to Jan '04 we have implanted a further 7 CMV children. Total number implanted=420=24 CMV.

Conclusion of study

  • Wide variation in outcomes
  • For many rate of progress appears to be slower than other CI users in the first 3 years
  • Have a long time-scale
  • Presence of co-existing central disorders affects prognosis in speech development
  • Progressive hearing loss can produce different outcomes
  • Promote dialogue across CI team professionals

Notes: Wide variation, they don't all progress slower than non CMV group. Also Paediatric CI research suggests variation is the norm rather than the exception. Presence of a child with suspected 'asymptomatic' CMV in group outcomes

Presence of co-morbid factors that don't affect spoken language outcomes
Presence of co-morbid factors that do affect outcomes, particularly motor coordination problems and cognitive impairments.

Pyman found these were both present significantly more commonly in CMV children compared to all other aetiologies studied, eg; 3 children with ASD Group outcomes may not identify those children who appear to progress at the expected or slower rate in the early stages of speech development, and then fail to make the jump from speech that is intelligible to very familiar listeners to intelligible to strangers and over the phone.

Congenital CMV Association

  • Family and parental support group
  • Linked with international associations
  • Pushing for awareness

Where from here?

  • Screening?
  • Awareness?
  • Investigation for CMV in all cases of HL
  • Management strategies and protocols
  • Drug treatments anti viral therapies and vaccines are in trial
  • CMV-related SNHL may become preventable

Useful Websites

What do we need to bear in mind when working with these children?

  • May be a progressive element to their hearing loss
  • Other difficulties may present themselves
  • Concentration and behaviour
  • Protect yourself when working with young children
  • Remain vigilant

One Family's Story

The Very Early Days

  • Normal pregnancy until 6 months
  • She is an identical twin
  • Delivered 32 weeks
  • Low birth weights 1.2kg and 1.6 kg
  • CMV discovered
  • Blood test reveal CMV in same state in both twins and myself
  • Heather remained in SCBU with breathing and feeding difficulties
  • Eventually came home at 10 weeks old

3-12 months

  • Heather failed to thrive
  • She was hospitalised with a chest infection at 9 months
  • A nasal gastric tube placed to supplement her feeding
  • At 12 months she weighed 5.5 kg (average expected weight is 9.7 kg)
  • Gross motor skills were significantly delayed; she did not sit independently until after her first birthday.
  • Diagnosed with profound bilateral hearing loss

12-22 months

  • Recurrent Pneumonia extended stay in hospital
  • Became oxygen dependent
  • Diagnosed with gastro-oesophageal reflux - fed via a nasal gastric tube
  • A CT scan revealed between 40 and 50% tissue loss in her lungs
  • At 18 months she weighed 5.8kg (average expected weight 11.2kg)
  • She came home from hospital trailing a feed pump and oxygen tubing
  • Numerous medications
  • Referred for a cochlear implant

The Consequences

  • The first 2 years were a struggle to survive
  • She had no energy to devote to development
  • Significant delays in all areas especially gross motor skills
  • Unable to determine the impact these delays would have on her future

22-36 months

  • Significant weight gain 9.5 kg by 22 months
  • Started to walk at 28 months
  • Required oxygen until 28 months
  • Tube feeding continued a gastrostomy sited at 32 months
  • We continued to pursue funding for a CI at a centre that could cater for her additional needs

Consequences

Entered her fourth year with significant developmental delays with:

  • Language skills
  • Gross motor skills
  • Fine motor skills
  • Social skills
  • When she was well and able to do so she was alert, interactive and involved in her environment
  • Her needs were varied and complex and it was difficult to predict how her development would proceed

We were involved with:

  • Paediatric Respiratory Consultant
  • Dietician
  • ENT Consultant
  • Audiologist
  • Physiotherapist
  • Speech and Language Therapist
  • Teacher of the Deaf

Then...

  • Initially attended local resource provision
  • Received first CI aged 3
  • Made steady progress
  • Concentration a big challenge
  • Moved to local mainstream school
  • Really struggled with Maths ...

Was it successful?

  • Her oral language development improved significantly
  • Her academic progress overall improved significantly
  • Received a bilateral implant 18 months ago
  • KS2 SATs English Level 4 (Reading 4, Writing 3)
  • Mathematics Level 4 Science Level 4
  • Plays Guitar -played a duet in concert last Sat
  • School production
  • Is very keen to develop her sign language skills
  • Has maintained friendships with deaf peers

The Future

  • She has a new phase of her education
  • Seems to have settled well
  • CMV has not gone away
  • How will she cope?