Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients.

Importance: The development of a vestibular implant has reached milestones and seems to be a promising therapeutic tool for bilateral vestibulopathy (BV). Given the former lack of therapeutic options for BV, the disease has received scant attention in the previous research literature. It is therefore of major importance to gain more insight into the underlying pathology of BV. Furthermore, as some research groups specifically use a combined vestibulo-cochlear implant, the size of the group of BV patients with associated hearing loss is of special interest. Objectives: The study aimed to determine the definite and probable etiology in bilateral vestibulopathy (BV) patients and to report on their hearing status. Design: This study involves multicenter retrospective study design. Setting: The research setting is at tertiary referral centers. Participants: Consecutive BV patients diagnosed at the Antwerp University Hospital between 2004 and 2018 at the Maastricht University Medical Center between 2002 and 2015 and at the Geneva University Hospital between 2013 and 2018, who met the BV diagnostic criteria of the Bárány Society. Main outcome measures: Primary interests were the etiology and hearing status of BV patients. Moreover, the data of vestibular tests were examined (caloric irrigation, rotatory chair tests, and video-head impulse test). Results: The authors identified 315 BV patients, of whom 56% were male patients. Mean age at diagnosis was 58.6 ± 15.1 (range 7-91) years. The definite cause was determined in 37% of the patients and the probable cause in 26% of the patients. No cause was identified in 37% of BV patients. The largest subgroup included patients with genetic etiology (31%), most frequently COCH mutation. Only 21% of patients (n = 61) had bilateral normal hearing. Almost half of the patients (45%, n = 134) had profound hearing loss in at least one ear. Conclusion: BV is a heterogeneous condition, with over a third of cases remaining idiopathic, and nearly three-quarters affected by hearing loss. COCH mutation is the most common non-idiopathic cause of BV in our population. Only 21% of our BV patients presented with bilateral normal hearing.

Sound localization in patients with bilateral vestibulopathy.

PURPOSE: The goal of this study was to evaluate if bilaterally (partially) absent vestibular function during static sound localization testing, would have a negative impact on sound localization skills. Therefore, this study compared horizontal static sound localization skills of normal-hearing patients with bilateral vestibulopathy (BV) and healthy controls. METHODS: Thirteen normal-hearing patients with BV and thirteen age-matched healthy controls were included. Sound localization skills were tested using seven loudspeakers in a frontal semicircle, ranging from - 90° to + 90°. Sound location accuracy was analyzed using the root-mean-square error (RMSE) and the mean absolute error (MAE). To evaluate the severity of the BV symptoms, the following questionnaires were used: Dizziness Handicap Inventory (DHI), Oscillopsia severity questionnaire (OSQ), 12-item Spatial, Speech, and Qualities Questionnaire (SSQ12), and Health Utilities Index Mark 3 (HUI3). RESULTS: The RMSE and MAE were significantly larger (worse) in the BV group than in the healthy control group, with respective median RMSE of 4.6° and 0°, and a median MAE of 0.7° and 0°. The subjective reporting of speech perception, spatial hearing, and quality of life only demonstrated a moderate correlation between DHI (positive correlation) and HUI total score (negative correlation), and localization scores. CONCLUSION: Static sound localization skills of patients with BV were only mildly worse compared to healthy controls. However, this difference was very small and therefore most likely due to impaired cognitive function. The vestibular system does not seem to have a modulating role in sound localization during static conditions, and its impact is negligible in contrast to the impact of hearing impairment. Furthermore, the subjective reporting of speech perception, spatial hearing, and quality of life was not strongly correlated with localization scores.

Perforation of the excluded segment without pneumoperitoneum following Roux-en-Y gastric bypass surgery: case report and literature review.

BACKGROUND: Because of the increasing prevalence of obesity and bariatric surgery (Roux-en-Y gastric bypass (RYGB) as the gold standard), there is a still growing population of people with altered post-operative anatomy. Although the most common early and late complications following RYGB are well known, they can still be difficult to diagnose. The altered anatomy after RYGB can create a real diagnostic and therapeutic challenge since routine examinations can be negative. CASE REPORT: We present a rare case of a 38-year-old woman with acute abdominal pain and a history of RYGB who proved to have a duodenal perforation in the absence of free air on radiologic examination. The perforation was closed laparoscopically and proton pump inhibitors were administered. CONCLUSIONS: Perforations of the excluded segment in RYGB patients are rare and represent a diagnostic challenge, as pneumoperitoneum is usually absent and the excluded segment is difficult to access. Despite negative diagnostic findings, laparoscopic exploration should always be considered.

Impact of Bilateral Vestibulopathy on Spatial and Nonspatial Cognition: A Systematic Review.

OBJECTIVES: Hearing loss is considered an independent risk factor for dementia. Growing evidence in animal and human studies suggest that not only hearing loss but also vestibular loss might result in cognitive deficits. The objective of this study is to evaluate the presence of spatial and nonspatial cognitive deficits in patients with bilateral vestibulopathy. As different causes of bilateral vestibulopathy are associated with hearing loss, the objective is to evaluate if these cognitive deficits are due to the vestibular loss of the patients with bilateral vestibulopathy, or to their hearing loss, or both. DESIGN: We performed a systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. (1) Data sources: MEDLINE and the Cochrane Library. (2) Study selection: Cross-sectional studies investigating cognitive performances in human patients with bilateral vestibulopathy confirmed by quantitative vestibular testing. (3) Data extraction: Independent extraction of articles by three authors using predefined data fields, including patient- and control characteristics and cognitive outcomes. RESULTS: Ten studies reporting on 126 patients with bilateral vestibulopathy matched the inclusion criteria. Cognitive domains evaluated in patients with bilateral vestibulopathy included visuospatial abilities, memory, language, attention, and executive function. In only three studies, hearing performance of the included patients was briefly described. Nearly all studies demonstrated a significant impairment of spatial cognition in patients with bilateral vestibulopathy. In the few papers investigating nonspatial cognition, worse outcome was demonstrated in patients with bilateral vestibular loss performing cognitive tasks assessing attentional performance, memory, and executive function. CONCLUSIONS: Strong evidence exists that patients with bilateral vestibulopathy suffer from impaired spatial cognition. Recent studies even suggest impairment in other cognitive domains than spatial cognition. However, in all previous studies, conclusions on the link between cognitive performance and vestibular loss were drawn without taken hearing loss into consideration as a possible cause of the cognitive impairment.