The DizzyQuest Combined with Accelerometry: Daily Physical Activities and Limitations among Patients with Bilateral Vestibulopathy Due to DFNA9.

BACKGROUND: DFNA9 is a genetic disease of the inner ear, causing progressive bilateral sensorineural deafness and bilateral vestibulopathy (BV). In this study, DizzyQuest, a mobile vestibular diary, and the MOX accelerometer were combined to assess the daily life functional limitations and physical activity of patients with DFNA9 suffering from BV. These parameters might be appropriate as potential candidacy criteria and outcome measures for new therapeutic interventions for BV. METHODS: Fifteen DFNA9 patients with BV and twelve age-matched healthy controls were included. The DizzyQuest was applied for six consecutive days, which assessed the participants' extent of functional limitations, tiredness, types of activities performed during the day, and type of activity during which the participant felt most limited. The MOX accelerometer was worn during the same six days of DizzyQuest use, measuring the participants intensity and type of physical activity. Mixed-effects linear and logistic regression analyses were performed to compare the DFNA9 patients and control group. RESULTS: DFNA9 patients with BV felt significantly more limited in activities during the day compared to the age-matched controls, especially in social participation (p < 0.005). However, these reported limitations did not cause adjustment in the types of activities and did not reduce the intensity or type of physical activity measured with accelerometry. In addition, no relationships were found between self-reported functional limitations and physical activity. CONCLUSIONS: This study demonstrated that self-reported functional limitations are significantly higher among DFNA9 patients with BV. As a result, these limitations might be considered as part of the candidacy criteria or outcome measures for therapeutic interventions. In addition, the intensity or type of physical activity performed during the day need to be addressed more specifically in future research.

Capturing nystagmus during vertigo attacks using a smartphone: adherence, characteristics, pearls and pitfalls.

OBJECTIVE: To investigate adherence, characteristics, and first clinical experiences of capturing ictal nystagmus at home, which can be performed to complement the diagnostic process in patients with episodic vestibular symptoms. METHODS: Patients were recruited at a tertiary referral center in case capturing ictal nystagmus could contribute to the diagnostic process (e.g., to detect or rule out BPPV). They were asked to capture ictal nystagmus with their own smartphone at home, using a smartphone-based adapter (Nystagmocatcher, Balansdiagnos, Stockholm, Sweden). All recordings were analyzed by the last author (RvdB), and the adherence, characteristics, and first clinical experiences were evaluated. RESULTS: Seventy patients with vestibular symptoms were asked to participate in this study. Sixty-two (89%) agreed to participate. The median period of participation was 86 days. Fifty-one patients experienced attacks during the study period. Eventually, 51% of them provided eye movement recordings sufficient for analysis. Different types of nystagmus were observed: positional nystagmus related to BPPV, positional nystagmus not related to BPPV, functional eye movements, and the absence of nystagmus or functional eye movements. Capturing ictal nystagmus could contribute to the diagnostic process in several ways, including to detect or rule out BPPV, to detect or rule out vestibular origin of symptoms, to determine the affected side, telemedicine, to monitor attack frequency, and to detect malingering. Furthermore, strict guidance of patients was necessary, which could be time-consuming. CONCLUSION: Capturing ictal nystagmus can contribute to the diagnostic process in several ways, which motivates to rethink current clinical workflow in vestibular medicine. However, strict guidance is necessary and not all patients provide ictal recordings. In an outpatient setting, it would be advised to use ictal nystagmus recordings on indication, to complement the diagnostic process.

Bilateral vestibulopathy patients' perspectives on vestibular implant treatment: a qualitative study.

OBJECTIVES: The aim of this study was to explore expectations of patients with bilateral vestibulopathy regarding vestibular implant treatment. This could advance the definition of recommendations for future core outcome sets of vestibular implantation and help to determine on which characteristics of bilateral vestibulopathy future vestibular implant research should focus. METHODS: Semi-structured interviews were conducted with 50 patients diagnosed with bilateral vestibulopathy at Maastricht UMC + . Interviews followed a semi-structured interview guide and were recorded and transcribed. Transcripts were analyzed thematically by two independent researchers. A consensus meeting took place to produce a joint interpretation for greater dimensionality and to confirm key themes. RESULTS: Overall, patient expectations centralized around three key themes: (physical) symptom reduction, functions and activities, and quality of life. These themes appeared to be interrelated. Patient expectations focused on the activity walking (in a straight line), reducing the symptom oscillopsia and being able to live the life they had before bilateral vestibulopathy developed. In general, patients indicated to be satisfied with small improvements. CONCLUSION: This study demonstrated that patient expectations regarding a vestibular implant focus on three key themes: symptom reduction, functions and activities, and quality of life. These themes closely match the functional improvements shown in recent vestibular implantation research. The results of this study provide a clear guideline from the patient perspective on which characteristics of bilateral vestibulopathy, future vestibular implant research should focus. TRIAL REGISTRATION: NL52768.068.15/METC.