Reliability of Functional Outcome Measures in Neurofibromatosis 2

NCT03617276 | Completed

• 1 other identifier: 16/NW/0504
• Study Type: observational
• Target: 30
• Locations: 1 country
• Sites: 1

Brief Summary

Neurofibromatosis 2 (NF2) is a rare inherited condition. Those with NF2 usually present with tumours (schwannomas) in their inner ear, or vestibular organ, with common symptoms including problems with hearing, balance, dizziness and vision. This study aims to evaluate the reliability (stability) of a range of functional standardised outcome measures in adults with NF2.

Conditions

Neurofibromatosis 2

Outcome Measures

Primary Outcomes (3)

• Modified nine hole peg test

  Participant is sitting comfortably in a chair in front of a desk. The 9 hole peg test equipment is set up so that the bowl containing pegs is directly behind the pegboard and in front of the participant. Participants can rest the forearms on the desk but must use only 1 hand for each test. If not the test is invalid. The assessor measures from the time the fingers pick up the first peg from the bowl until the fingers place the last peg in the hole of the pegboard. Time will be recorded in seconds and milliseconds.

  Up to 3 minutes

• Four square step test

  This is a test of balance when moving. Participants are asked to step forwards, sideways and backwards in a specific pattern. The rater times how long it takes to complete the test

  Up to 30 seconds

• Modified Clinical Test of Sensory Integration and Balance

  This is a test of standing balance. Participants are asked to stand upright with the feet together, first on a hard surface (the floor) and then on a soft surface (some foam). The rater will time the participant standing in each position with the eyes open and shut.

  Up to 10 minutes

Secondary Outcomes (2)

• Dynamic Visual Acuity test

  Up to 3 minutes

• The NFTI-QOL: A Disease-Specific Quality of Life Questionnaire for Neurofibromatosis 2

  Up to 2 minutes

Study Arms (1)

Outcome measurement

Each participant will be asked to complete each standardised outcome measure (SOM) three times and each trial will be videotaped by the researcher. The selected SOM's are the modified nine-hole peg test, the four square step test and the modified clinical test of sensory integration and balance. A doctor will watch the video on 2 separate occasions to evaluate intra-rater reliability. Inter-rater reliability will be assessed through asking three other neurofibromatosis specialist professionals (two NF2 consultants and one NF2 specialist nurse) to review the video and to score each measure completed. Once the filmed sessions have been analysed by the relevant clinician's the data will be destroyed in line with Trust policy. Each participant will also be required to complete the INFI-QOL questionnaire, the dynamic visual acuity test and provide information about the number of falls/near misses they have had over the past 12 months

Eligibility Criteria

• Age: 16 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

All adults (over the age of 16 years) with a clinical diagnosis of neurofibromatosis 2 who attend the neurofibromatosis clinic for appointments at Guys hospital, London and who fit the inclusion/exclusion criteria for the trial are eligable to participate in the study.

You may qualify if:

• Fulfill diagnostic criteria for NF2 Aged 16 years or older Attend the national NF service at Guys and St Thomas' NHS Foundation Trust Are able to provide informed consent Do not have any non-NF2 related comorbidities that affect mobility or balance (confirmed by the treating clinician) Are able to walk more than 10m without assistance of a person Do not have unstable vascular or orthopaedic pathology at the cervical spine (confirmed by consultant) Have not had a stroke within the last 3 months

You may not qualify if:

• Do not fulfill diagnostic criteria for NF2 Aged under 16 years or older Are unable to provide informed consent Have non-NF2 related comorbidities that affect mobility or balance (confirmed by the treating clinician) Are unable to walk more than 10m without assistance of a person Have unstable vascular or orthopaedic pathology at the cervical spine (confirmed by consultant) Have had a stroke within the last 3 months

Sponsors & Collaborators

• Guy's and St Thomas' NHS Foundation Trust: lead

Study Sites (1)

Neurofibromatosis Unit, Guys Hospital.

London, SE1 9RT, United Kingdom

Location

MeSH Terms

Conditions

Neurofibromatosis 2

Condition Hierarchy (Ancestors)

Neuroma, Acoustic; Neurilemmoma; Neuroendocrine Tumors; Neuroectodermal Tumors; Neoplasms, Germ Cell and Embryonal; Neoplasms by Histologic Type; Neoplasms; Neurofibromatoses; Neurofibroma; Nerve Sheath Neoplasms; Neoplasms, Nerve Tissue; Neuroma; Neoplastic Syndromes, Hereditary; Vestibulocochlear Nerve Diseases; Retrocochlear Diseases; Ear Diseases; Otorhinolaryngologic Diseases; Otorhinolaryngologic Neoplasms; Cranial Nerve Neoplasms; Cranial Nerve Diseases; Nervous System Diseases; Neurocutaneous Syndromes; Heredodegenerative Disorders, Nervous System; Neurodegenerative Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: August 1, 2018
• First Posted: August 6, 2018
• Study Start: September 7, 2016
• Primary Completion: May 17, 2017
• Study Completion: May 17, 2017
• Last Updated: August 6, 2018

Record last verified: 2018-08

Data Sharing

• IPD Sharing: Will not share

Locations

GB (1)