Hearing Loss and Down Syndrome: Assessment and Rehabilitation

Symposium Aim

The prevalence of hearing loss in both adults and children with Down syndrome is many times that of the wider population. Awareness and research of both the presentation and management of hearing loss in individuals with Down syndrome within the audiology profession has increased notably over recent years. This symposia aims to consider some recent developments and their possible impact for users of audiology services with Down syndrome

As this symposia aims to consider audiological care throughout the pathway from initial identification to rehabilitation, it is proposed that the presentations are presented in chronological order along this pathway.

The initial presentation considers 2 studies, one complete and one ongoing, looking at the assessment of 2 aspects of the auditory system; middle ear function and temporal processing. The outcomes of these assessments and adaptations required for clinical practice are discussed.

Building on the discussion of assessment of the middle ear in the first presentation, the second will explore the approach to otitis media which is the most common pathology that affects the middle ear and is highly prevalent in children with Down syndrome. This presentation considers the identification of otitis media and different approaches to management adopted across England.

Moving then to rehabilitation, Auditory Eurhythmic Programs are presented and their effectiveness in developing individuals' relationships with sound using a wide range of stimuli.

It is widely reported that family and carers can have a critical impact on the success of rehabilitation so this final presentation explores families experiences and examples of good practice.

Tympanometry and Temporal Integration

Siobhán Brennan; Marie Kirkland; and Eleanor Nunns

Contact: siobhan.brennan@manchester.ac.uk


Part 1: Tympanometry

Background: Tympanometry is regularly used to assess middle ear function however reduced specificity in individuals with Down syndrome has been reported. Middle ear resonant frequency is critical in the parameters used. This is not currently known for adults with Down syndrome.

Research Question: What is the resonant frequency of the middle ear in individuals with Down syndrome?

Method: Using a GSI Tympstar ear canal volume, compliance and resonant frequency was measured in 10 adults with Down syndrome, and 10 without.

Results: Results indicate smaller mean canal volume and compliance values in adults with Down Syndrome but no significant difference in resonant frequency. Further data collection is in progress.

Part 2: Temporal Processing

Background: Temporal processing is critical in processing speech. Given the reported differences in linguistic abilities reported in individuals with Down syndrome, it is possible that differences in temporal processing may contribute.

Research Questions:

  1. Is a short interactive test of temporal resolution containing pauses between stimuli viable for ascertaining gap detection thresholds in adults with Down syndrome?
  2. Are gap detection thresholds in adults with Down syndrome significantly different from adults without?

Method: 7 adults with Down syndrome and 11 adults without were recruited and carried out an adapted gap detection test. Mann-Whitney U test was used to compare results from each group.

Results: All participants were able to complete the gap detection test. No significant differences between gap detection thresholds were found.

Conclusion: The study suggests that a modified gap detection test can be used for adults with Down syndrome and their temporal resolution appears to be within the expected range.

A mixed-methods study of the management of hearing loss associated with otitis media with effusion in children with Down syndrome

Amanda Hall; Helen Pryce; Iain Bruce; Peter Callery; Monica Lakhanpaul; and Anne Schilder

Contact: a.hall@aston.ac.uk


Objectives: To scope current service provision across England for management of otitis media with effusion and hearing loss in children with Down syndrome; to explore professional decision-making about managing otitis media with effusion and hearing loss; and to explore patient and public views on the direction of future research.

Design: Mixed methods including a service evaluation of NHS clinical practice through a structured telephone survey; a qualitative study of professional decision making with in-depth interviews collected and analysed using grounded theory methods; patient/public involvement consultations.

Participants: Twenty-one audiology services in England took part in the evaluation; 10 professionals participated in the qualitative study; 21 family members, 10 adults with Down syndrome and representatives from two charities contributed to the consultations.

Results: There was variation across services in the frequency of routine hearing surveillance, approaches to managing conductive hearing loss in infancy and provision of hearing aids and grommets. There was variation in how professionals describe their decision-making, reflecting individual treatment preferences, differing approaches to professional remit and institutional factors. The consultations identified that research should focus on improving practical support for managing the condition and supporting decision-making about interventions.

Conclusions: There is system-level variation in the provision of services and individual-level variation in how professionals make clinical decisions. As a consequence, there is inequity of access to hearing health care for children with Down syndrome. Future research should focus on developing core outcomes for research and care, and on improving decision support for families.

Auditory Eurhythmic Programs

Jane Douglas, Seashell Trust

Contact: jane.douglas@seashelltrust.org.uk


Children and young people with Down's syndrome can find it very difficult to extract meaning from sound and speech, and filter out competing multisensory information. They may be unable to consistently process visual and auditory stimuli at the same time and this is compounded where there may be a diagnosis of co-morbidity (such as Autistic Spectrum Disorder or deafness) which results in complex neurological deficits.

Sound is a transient stimuli and can be meaningless for Down's syndrome children and young people who struggle with auditory processing and the temporal nature of hearing. Individuals will often actively avoid challenging acoustic environments.

Auditory Eurhythmic Programs at Seashell Trust are highly individualized interventions which are tailor made for a range of students with complex intellectual disabilities and hearing impairment (either peripheral hearing system or abnormal central auditory processing) in order to explore and understand sound.

The sessions are delivered with live music which incorporates several instruments and a singer and are very structured and consistent in order to develop listening skills and expressive communication.

The music is specifically selected according to the student's needs and the melodies, musical patterns and dynamics are manipulated to engage the student and develop interaction with the musicians and singer. Intensive interaction is incorporated into the program whereby the student responds to the musicians initially but this is then turned round to follow the student's actions, which may be mirroring vocalizations, copying a drum beat, putting an action to a sound.

The program focusses primarily on music awareness, detection, discrimination and interaction. As part of this we aim to develop intentional vocalizations and oral communication, choice-making, turn-taking, steady beat competency, pitch perception, use of musical LING sounds, and shared attention.

Supporting Parents to Check Listening

Contact: sarah.hogan@avuk.org


Over three quarters of children with Down Syndrome experience hearing problems at some point. The hearing loss may be temporary and mild or of a greater degree and permanent. Even a mild hearing loss can have a significant effect on learning for children with Down Syndrome 1 . Historically, there has been a tendency for caregivers to underestimate the degree of loss 2 and its developmental impact and a recommendation for regular hearing assessment is made within the protocols given within Best Practice Guidance for the Management of Hearing Issues in Down Syndrome (2017).

Parents can be coached in assessing functional listening using the Ling 6-Sound check 3 . This presentation focuses on the essential requirements for, and benefits of, a functional listening assessment in pre-school aged children. We consider how listening underpins spoken language development and learning. We share the views of parents of children with Down Syndrome who have used the Ling 6-Sound check as part of their Auditory Verbal program and will use video-recorded examples of good practice. We consider the benefits and challenges in the use of amplification for children with Down Syndrome and hearing loss.

  1. Information about Down Syndrome - Early Support for children young people and families (2012)
  2. Best Practice for the Management of Hearing Issues in Down Syndrome (2017) McNeill, E., & Puri, S. https://www.dsmig.org.uk/information-resources/by-topic/hearing/ (accessed 8 Jan 2021)
  3. Listening with Ling Six https://www.jtc.org/listening-with-ling-six/ (accessed 8 Jan 2021)