EFFICACY OF SAFE AND SOUND PROTOCOL ON SENSORY PROCESSING DIFFICULTIES IN PATIENTS WITH CONGENITAL GLYCOSYLATION DISORDER: A CASE REPORT

D. Ragheb¹, A. Elfeky¹, S. Neair¹, M. Ahmed¹

¹4 Kids Therapy, Cairo, Egypt

Background: Congenital Glycosylation Disorder Type 1(CDG-I) is a rare congenital metabolic disease with an incidence of 1 in every 50,000 to 100,000 births. It is characterized by different signs and symptoms including but not limited to developmental delay, sensory processing difficulties and cognitive disabilities.
Safe and Sound Protocol (SSP) is a technique created to manage sensory processing and behavioral difficulties using acoustics of altered frequency that is, theoretically, the same as the frequency band and the weights associated with the articulation index and speech intelligibility index.
SSP uses computer modulated (filtered) vocal music and unfiltered vocal music in a specific sequence to exercise the muscles of the middle ear in a dynamically changing pattern which aims to improve auditory processing in children with auditory hypersensitivity.

Purpose: To investigate how SSP affects sensory processing and behavioral difficulties in a child with CDG-I who suffers from auditory hypersensitivity.

Methods: The subject is an 8-year-old female with CDG-I with a caregiver's complaint of sensory and behavioral challenges. The protocol was administered in 19 days in which she administered twelve hours of both filtered and unfiltered music divided into 2 hours of unfiltered music, 5 hours of filtered music, and 5 hours of less filtered music. During listening protocol, therapists encouraged her to engage in playing activities as puzzles, peg boards, and other fine motor games.
We used Brain Body Center Sensory Scale (BBCSS) which is a self-reported questionnaire based on Polyvagal theory to assess behaviors related to sensory processing before and after applying the protocol. It includes 67 across 4 subscales which are Auditory Processing, Visual Processing, Tactile Processing, and Eating and Feeding Behaviors.
Caregiver`s interviewing including any behavioral, social or emotional changes was used to address changes in correlation with BBCSS before and after applying the protocol.

Results: Caregiver`s interview revealed that she became less sensitive to high sounds, calmer in crowded areas, less hyperactive or messy, and more obedient to instruction. The caregiver also reported emerging new behaviors such as tolerating carrying her bag on her back for the first time and less fixation on certain types of food.
These findings correlate with BBCSS findings as BBCSS total score decreased from 93 to 76 with auditory processing section score decreasing from 37 to 22 and eating and feeding behaviors section score decreasing from 26 to 25. Touch processing and visual processing sections scores did not improve despite caregivers’ reports of sensation related changes.

Conclusions: SSP was beneficial in improving auditory processing in children with CDG-I who exhibited signs of auditory hypersensitivity. It also influences other sensory aspects including hypersensitivity to touch, eating and feeding behaviors.

Implications: SSP may be a beneficial line of intervention for children with rare diseases who exhibit signs of auditory processing disorder and behavioral issues. Further controlled studies relevant to this unique protocol are needed to determine an evidence-based methodology to use this protocol.

Funding acknowledgements: No funding

Keywords:
Sensory
Music
Glycosylation

Topics:
Paediatrics
Disability & rehabilitation
Neurology

Did this work require ethics approval? No

Reason: This is a case study that is based on a non invasive technique which does not require any ethical approval. There is an attachment of the mother's consent.