None listed
Conditions
Brief summary
Learning disorders are one of the greatest causes of morbidity in children with the genetic syndrome, neurofibromatosis type 1 (NF1) and result in academic underachievement, reduced quality of life, and are of significant concern to families and their teachers. There are minimal evidence-based interventions for these problems in NF1, and there is an urgent need for trials targeting this area of clinical need. This registration form details the extension study from the Treating Auditory Problems in NF1 "TAP-iN" trial. The extension study described on this registry form will enable us to establish the efficacy of an RML device in treating central auditory deficits in children with NF1 and will allow us to determine whether treatment benefits extend to broader areas of learning and behaviour. Outcomes are clinically meaningful and include measures of speech perception, literacy skills, attention, fatigue, social function and quality of life. If realized, this study will provide powerful evidence for a novel, non-invasive intervention targeting a common and impairing problem in NF1.
Interventions
This is a multisite clinical trial evaluating the use of a remote microphone listening (RML) device in treating speech perception in noise difficulties in children with neurofibromatosis type 1 (NF1).
The investigational device is the Phonak Roger Touchscreen Microphone paired with Phonak Roger Focus Receivers with SlimTubes and open domes. The receivers are designed for children with normal hearing thresholds, so they provide a safe, comfortable, and adjustable volume for wearers. The receiver
This is a multisite clinical trial evaluating the use of a remote microphone listening (RML) device in treating speech perception in noise difficulties in children with neurofibromatosis type 1 (NF1). The investigational device is the Phonak Roger Touchscreen Microphone paired with Phonak Roger Focus Receivers with SlimTubes and open domes. The receivers are designed for children with normal hearing thresholds, so they provide a safe, comfortable, and adjustable volume for wearers. The receivers are small devices that sit behind each pinna and are held in place by a soft, vented rubber earpiece inserted into the ear canal. They are minimally visible, do not block the ear and allow the wearer access to environmental sound. The Touchscreen Microphone is a compact device, worn by the teacher on a lanyard. Children enrolled in this trial will have consented for participation in the parent study, which is a shorter randomised controlled trial (RCT) of RML devices. After completing participation in the parent study, participants will be invited into this extension trial where they will wear the RML device for a longer period of time (approximately 3 school terms). This will enable us to determine whether longer-term device use can improve learning outcomes for children with NF1. Children and teachers will be asked to wear the RML device Monday - Friday for the time the child is in the classroom. To determine the amount of time the device is used, we will ask the teacher to complete a simple weekly compliance check.
Sponsors
Murdoch Children's Research Institute
Study design
Allocation
Non-randomised trial
Intervention model
Parallel
Primary purpose
Treatment
Masking
Open (masking not used)
Eligibility
Sex/Gender
All
Age
6 Years to 12 Years
Healthy volunteers
No
Inclusion criteria
• Children aged 6-12 years. • Satisfy the revised diagnostic criteria for NF1. • Consented to and screened for the parent RCT trial. • Participant and at least one caregiver have sufficient English to complete study outcomes, understand and comply with study requirements and to communicate any adverse effects. • Has a legally acceptable parent/guardian capable of understanding the informed consent document and providing consent on the participant’s behalf. • School/teacher willing to participate in the study (of any age).
Exclusion criteria
• School/teachers unwilling to participate. • Consent and screening not undertaken for the parent RCT. • Evidence of sensory hearing loss (defined by a 4-frequency average hearing loss (average of 0.5-, 1-, 2-, and 4 kHz) of >20dbHL in both ears, or use of corrective hearing device such as a hearing aid or cochlear implant. • Full Scale IQ (FSIQ) <70 on standardised test of intellectual functioning. • Starting secondary school/high school within 9 months of commencing this extension phase of the study • Symptomatic or progressive intracranial pathology that may affect scores on audiological, cognitive, or behavioural outcome measures (e.g., acquired brain injury, or hydrocephalus). Asymptomatic or stable low-grade gliomas that are not thought to impact on outcome measures will not result in exclusion.
Outcome results
None listed