In this years National CMV Awareness Month, Sarah Hogan, Senior Auditory Verbal Therapist and Research Co-ordinator at Auditory Verbal UK, shares with us her thoughts on the additional difficulties faced by children who have cCMV related deafness.
“What I’m learning about cCMV”
Although congenital cytomegalovirus (cCMV) infection has long been recognised as a cause of sensorineural hearing loss (SNHL) in children, there is currently less awareness of the many associated difficulties for children deafened through cCMV. If additional difficulties associated with cCMV are overlooked or misinterpreted by professionals, then this will impact the support offered to the child and their family.
Over the last twenty years, therapists at the charity Auditory Verbal UK (AVUK), have supported children with hearing loss to develop spoken language thorough listening with a highly specialist early intervention programme called Auditory Verbal therapy. Here we reflect on what we have learnt and what we are learning about supporting children with cCMV and hearing loss.
Gemma’s parents found Auditory Verbal UK when their daughter was 5 months old. The Newborn Hearing Screening Programme had indicated that a full hearing assessment for their daughter was required and further testing revealed a bilateral, severe hearing loss. Gemma was fitted with hearing aids by 3 months of age and went on to have bilateral cochlear implants. Through our parent coaching programme which guides parents in strategies to support their child’s listening and spoken language development, we were able support Gemma’s parents in ensuring that she was supported in her listening, thinking and talking. Gemma had a wider range of needs than just her communication and her AV therapist was able to signpost Gemma’s parents to support across the range of her needs, including help with feeding issues, with her gross motor skills and with her need to be ‘on the go’. By the time she was ready to start school, she was fluent in her home language and went on to achieve spoken English language scores at an equal level to her hearing peers.
Handprints from children leaving our Auditory Verbal programme
Early identification and intervention
In their paper on Early Identification and Management of Congenital Cytomegalovirus, Drs Jenks, Mithal and Hoff (2021)1 wrote: “Hearing habilitation should be pursued for children with SNHL, regardless of cause, to minimize the impact of hearing loss on speech, language, and cognition. The first line of treatment is amplification for children with bilateral SNHL or unilateral SNHL of mild to moderate degree […]. Amplification should not be delayed by antiviral treatment. To maximize listening and spoken language, early intervention therapy that includes listening and spoken language therapy is beneficial.
Cochlear implantation (CI) is the only medical treatment of SNHL when amplification does not provide adequate access to spoken language. Benefits of CI in patients with cCMV are well established, with multiple series showing improvement in auditory thresholds, speech perception, and expression. Some studies have shown equivalent progress among implanted patients with cCMV compared with controls with other causes of SNHL, whereas others have shown comparatively slower or poorer progress.”
cCMV is now thought to account for as much as 20-30% of childhood SNHL but despite this, many professionals supporting families with children with cCMV have incomplete knowledge of the medically recognised, or commonly observed, associated conditions. Some years ago, we looked at the additional challenges for children attending AVUK’s early intervention family programme and found that, for our small sample, there were children who experienced difficulties well-described in the literature but that there were also common traits that were not highlighted to the same extent, for example, sensory integration difficulties.
Sensory Integration is a process through which external and internal sensory inputs are computed within the brain so as to provide information to a person about the external world and their internal sense of being. “Sensory information, including proprioceptive, vestibular, tactile, visual, auditory, taste, and olfactory input, when integrated effectively, allows children to participate to their full potential in learning and other childhood occupations. Together these senses contribute to the development of many abilities, including motor planning, body awareness, visual–motor skills, language development, and regulation of level of activity”. We now also consider interoception, the ability to acknowledge the status of our internal organs (e.g stomach, bladder, bowel etc) to be important among this sensory information.
We reviewed ten children with cCMV who had been enrolled on our Auditory Verbal family programme and had been diagnosed by a paediatric occupational therapist with advanced training in sensory integration (SI) as having sensory integration differences . Eight of the children had difficulty moving from one activity to another, seven children had difficulty maintaining their attention and six had difficulties regulating self-arousal, that is, their level of attention, motivation and readiness for a task. Half of the children had sequencing and planning difficulties. Seven of the ten children experienced balance problems in addition to hearing loss. Six children had oromotor difficulties; eight children were of low weight and four children had problems with sleep. Many families of children with cCMV will recognise these characteristics and further recognise the impact for their child and their family.
Developing spoken language
As Jenks, Mithal and Hoff (2021) have described, the outcomes for listening and spoken language for children with cCMV are mixed: Our outcomes suggest that just under half of the children (4 in 10 children with cCMV leave the programme with age-appropriate language after having Auditory Verbal therapy for more than one year. Approximately 1 in 4 children with cCMV leave the programme without age-appropriate language, but nonetheless, having made accelerated progress in spoken language after having Auditory Verbal therapy for more than one year. Around 3 in 10 children with cCMV are supported to investigate other approaches to developing communication before completing 1 year of their Auditory Verbal programme.
Auditory Verbal therapy is delivered through play
What we’re learning
Having worked with a large number of families whose child has hearing loss associated with cCMV we know that:
- We are learning all the time from the children we are supporting
- Regular audiology check-ups are essential including for children with unilateral hearing loss
- The neurodevelopmental implications of cCMV impinge on many aspects of the child’s life
- Early identification of sensory differences that impact on listening behaviours should be prioritised for children with hearing loss
- Children diagnosed with cCMV should be assessed by a specialist paediatric occupational therapist (OTs) with specific advanced training in diagnosing sensory integration difficulties. The difficulties that these children experience mean that additional skills in interpreting their behaviour must be applied by informed professionals.
- Specialist OTs can draw up ‘Sensory Diets’, a specific set of exercises for children to perform to meet their sensory requirements
- Implementing individually tailored exercises provided by a specialist paediatric occupational therapist helps children with sensory differences feel ‘grounded’ and enhances listening attention and spoken language
- A diagnosis of sensory integration difficulty and the commensurate increase in parental knowledge, helps parents to better understand their child’s world view
- Families find difficulty in accessing practitioners with an appropriate diagnostic skill set within the NHS
- The degree to which interventions such as PROMPT may help remediation of oro-motor difficulties in this group of children should be investigated. It can be useful to refer to Speech and Language therapists specialising in PROMPT therapy in order to work on oro-motor skills (the movement of the mouth for the development of speech).
- When children’s sleep patterns impact substantially on family life, help can be reached through NHS Sleep Clinics or from Cerebra, a charity working with children with brain related conditions.
Support from AVUK
As we continue to learn how best to support children with hearing loss as a result of cCMV, we’re pleased to be working with CMV Action UK and backing their work in raising awareness of the virus, its link to hearing loss and support for parents and caregivers with children who are born with it.
All year round, including during CMV Awareness Month, Auditory Verbal UK provides support and information about family programmes of Auditory Verbal therapy which equip parents and carers with the tools to support the development of their deaf child’s speech and language through play based, every-day activities. The programme is for families who want their deaf child to learn to listen and speak and supports children to learn how to make sense of this sound they receive through their hearing technology, such as cochlear implants or hearing aids.
Find out more about AVUK’s family programme online or call 01869 325000 to speak to our support team.