Treating auditory problems in children with neurofibromatosis type 1

Not Applicable

Recruiting

• Conditions: eurofibromatosis type 1; Neurofibromatosis type 1; Human Genetics and Inherited Disorders - Other human genetics and inherited disorders; Ear - Other ear disorders; Neurological - Other neurological disorders

• Registration Number: ACTRN12622001328763
• Lead Sponsor: Murdoch Children's Research Institute

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Results First Posted: 1900-01-01
• Study Start: 2022-10-14
• Last Update Posted: 8 May 2023

Recruitment & Eligibility

• Status: Recruiting
• Sex: All
• Target Recruitment: 124

Inclusion Criteria

• Children aged 6-12 years
• Satisfy the revised diagnostic criteria for NF1
• 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
• Demonstrate a functional hearing difficulty on the Listening in Spatialized Noise - Sentences (LiSN-S) test [Note: defined as 2 standard deviations below the normative age range for the Different Voice 90°, Same Voice 90° and/or spatial advantage conditions]
• School/teachers willing to participate in the study (of any age)

Exclusion Criteria

• School/teachers unwilling to participate
• Active ear infection at the time of screening
• 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.
• Plan to commence or change medication (or dosage) for ADHD symptoms during the 4-week RCT. In these cases, children will be considered eligible if they have been on a stable dose of ADHD medication for 4 weeks prior to screening (and they meet all other eligibility requirements).
• 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.

Study & Design

• Study Type: Interventional
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Performance on the Consonant-Nucleus-Consonant test, measured by % correct phonemes[Assessments will be undertaken with the device (aided) and without the device (unaided), conducted during the pre-intervention baseline assessment in a randomised order by the study audiologist. ];Total score on the Listening Inventory for Education-Revised questionnaire (child version)[Baseline, end of treatment period 1 (2 weeks post-randomisation), end of treatment period 2 (4-weeks post-randomisation)];Total score on the Listening Inventory for Education-Revised questionnaire (teacher version)[Baseline, end of treatment period 1 (2 weeks post-randomisation), end of treatment period 2 (4-weeks post-randomisation)]

Secondary Outcome Measures

Name	Time	Method
Integrated Visual and Auditory-Quick Screen test, in background noise[Assessments will be undertaken with the device (aided) and without the device (unaided), conducted during the pre-intervention baseline assessment in a randomised order by the study audiologist. ];Adverse events after short term use, assessed using a short Tolerability and Adverse Events survey written by the investigator team. While uncommon, possible adverse events may include: <br>- discomfort related to the positioning of the device on the child's ear<br>- loud volume of signal transmitted to the child’s ear[Baseline, end of treatment period 1 (2 weeks post-randomisation), end of treatment period 2 (4-weeks post-randomisation)]