First report of the efficacy of vestibular rehabilitation in improving function in patients with Neurofibromatosis type 2: an observational cohort study in a clinical setting

Abstract

Purpose: Patients with Neurofibromatosis type 2 often experience debilitating neuro-otological problems which affect their mobility and balance. This study examined the efficacy of a personalised program of vestibular rehabilitation for patients with Neurofibromatosis type 2.

Materials and methods: An observational cohort study analysing routinely collected data for 21 patients in a highly specialised Neurofibromatosis type 2 service. Vestibular rehabilitation comprised an initial one-hour assessment followed by a patient-specific exercise program reviewed in person and by email consultations. Patients were subsequently followed-up at 9 months. The vestibular rehabilitation efficacy was assessed using the Dynamic Gait Index score.

Results: Nineteen of 21 patients were assessed as impaired and at risk of falls pre-rehabilitation (Dynamic Gait Index <19/24), of which 79% showed clinical improvement post-rehabilitation. There was a significant improvement in the Dynamic Gait Index scores pre-rehabilitation to post-rehabilitation (p < 0.001) and outcomes were subsequently maintained at the 9-month follow-up assessment. Whilst the pre-rehabilitation Dynamic Gait Index scores of patients with more severe genotype were lower compared to other patients, the beneficial effect of vestibular rehabilitation was similar amongst genetic severity groups.

Conclusions: Personalised vestibular rehabilitation significantly improves function in Neurofibromatosis type 2, sustaining benefits for 9 months, irrespective of patients’ age or genetic severity.

Implications for rehabilitation

• Patients with Neurofibromatosis type 2 experience debilitating neuro-otological problems which affect their mobility and balance.

• A patient-tailored program of vestibular rehabilitation was offered in a highly specialised clinic for six months with a follow-up assessment at 9 months post-treatment.

• All patients improved from baseline, with 79% of them achieving clinically significant improvement in function and with statistically significant benefits sustained for 9 months.

• The beneficial effect of vestibular rehabilitation was similar for all patients, regardless of age or genetic severity, suggesting vestibular rehabilitation could be incorporated in routine clinical care in Neurofibromatosis type 2 clinics internationally.

Keywords:

• Neurofibromatosis type 2
• vestibular rehabilitation
• dynamic gait index
• genetic severity

Acknowledgements

The authors would like to thank the NF2 patients and the Oxford NF2 research team, in particular, Rosie Crabtree, Wendy Howard, Samuel MacKeith, Anne May, and Carolyn Redman.

Disclosure statement

The authors report no conflicts of interest.