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.

Emmanouil B1, Browne K1,2, Halliday D1,3, Parry A1.

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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.