Exploring the role of music therapy in the lives of children with Neuronal Ceroid Lipofuscinosis (Batten Disease)

Student thesis: Doctoral Thesis


Background: Batten disease is a rare neurodegenerative condition that affects approximately 150 – 200 children and young people in the UK. Children experience gradual loss of skill over time, and most do not live past their adolescent years. Present research focuses upon clinical trials in enzyme replacement therapy, however, leading research in the field recognises the need for improved education strategies. Although under-researched, music has been recognised as a key support mechanism for individuals affected by Batten disease. Research advances in neuroscience and music therapy suggest that music interventions are successful for neurodegenerative conditions. As yet, research has not investigated the impact of music or music therapy for childhood conditions.

Aim: The project aims to explore the role of music therapy for children affected by Batten disease, focusing on how children respond to long-term music therapy, and how this can be measured. Secondly, the project aims to explore how elements of music therapy can be transferred into educational environments through a novel music education program, ‘Music Speaks’. Finally, the project aims to capture experiences of professionals who support affected children in educational contexts and gather their experiences of delivering the Music Speaks program.

Methods: The project involved a three-phase mixed methods design involving observations over a four-year period; development and validation of a prototype music therapy assessment measure; and the creation and trial of a music education program for schools, Music Speaks. Semi-structured interviews captured the experiences of professionals, challenges and systemic structures surrounding support and education for children affected by Batten disease.

Findings: Psychometric measures confirmed strong face validity and inter-rater reliability of a new music therapy outcome measure. It was observed that music therapy facilitated sustained and, in some cases, improved functioning over time. The Music Speaks program created short-term improvements in expressive language. Professionals observed how music helped preserve skills, where in the absence of music, the skills of children with Batten disease might otherwise deteriorate. Interview data outlined how Music Speaks could enhance children’s self-expression and communication and complement pre-emptive teaching approaches to preserve the child’s independence in the future.

Conclusion: The project’s findings create a dialogue between the experiences of those in education and the observed impact of music, supporting the potential use of music therapy interventions for children affected by Batten disease. The research provides preliminary evidence to support the development and review of educational guidance and policy to incorporate music therapy interventions for childhood neurodegenerative diseases. Further research could optimise music interventions considering implementation, adaptability, transferability, and feasibility in other contexts, to support the use of music in wider care pathways for children with Batten disease.
Date of AwardJun 2022
Original languageEnglish
Awarding Institution
  • University of Brighton
SupervisorClaire Rosten (Supervisor) & Helen Johnson (Supervisor)

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