Angel Batuecas-Caletrio

Is Benign Paroxysmal Vertigo of Childhood a migraine precursor

INTRODUCTION Benign Paroxysmal Vertigo of Childhood (BPVC) is a common cause of vertigo in children and it is characterized by recurrent attacks of vertigo without warning resolving spontaneously after minutes to hours. It has been considered the equivalent of migraine in childhood. METHODS Twenty-seven patients diagnosed with BPVC were recruited between 1991 and 1997 with a follow up of at least 15 years. The incidence of migraine, inner ear disorders and family medical history is analyzed. RESULTS The average age for the onset of the attacks of BPVC was 3 years and 11 months, and for spontaneous resolution, it was around 5 years and 7 months. The average age for follow up was 18 years. Nine patients developed migraine during adulthood. Nineteen of them had a family history of migraine. CONCLUSION We have observed that the prevalence of migraine in patients that had been diagnosed with BPVC is higher than in the general population, which leads us to propose BPVC as a precursor of migraine during childhood.

Benign Paroxysmal Positional Vertigo in the Elderly

Background: Benign paroxysmal positional vertigo (BPPV) is the most frequent peripheral vertigo in the elderly. It is a well-characterized entity and generally easy to treat. Objective: To evaluate the main symptoms, time to consult for the problem, vertigo characteristics, treatment and follow-up in patients over 70 with BPPV. Methods: This was a retrospective cohort study. Four hundred and four patients were diagnosed to have BPPV (between January 2006 and December 2012); 211 of them were ≥70 years old (mean 77.7 years) and 193 <70 years old (mean 53.82 years). Results: Patients over 70 with BPPV took longer to consult for the problem (Spearman rho, p = 0.01). The frequency of a clinical presentation consisting of unsteadiness or imbalance without vertigo sensation is higher among elderly patients (χ2, p = 10-6). The effectiveness of the repositioning maneuver is lower than in patients under 70 (χ2, p = 0.002), and the recurrences are more frequent (χ2, p = 0.04). Conclusion: BPPV is a frequent entity in the elderly, and it is necessary to take it into account when older patients complain about imbalance. An appropriate treatment with repositioning maneuvers and prolonged follow-up are required in order to detect recurrences.

A novel missense variant in PRKCB segregates low-frequency hearing loss in an autosomal dominant family with Meniere’s disease

Menieres Disease (MD) is a complex disorder associated with an accumulation of endolymph in the membranous labyrinth in the inner ear. It is characterized by recurrent attacks of spontaneous vertigo associated with sensorineural hearing loss (SNHL) and tinnitus. The SNHL usually starts at low and medium frequencies with a variable progression to high frequencies. We identified a novel missense variant in the PRKCB gene in a Spanish family with MD segregating low-to-middle frequency SNHL. Confocal imaging showed strong PKCB II protein labelling in non-sensory cells, the tectal cells and inner border cells of the rat organ of Corti with a tonotopic expression gradient. The PKCB II signal was more pronounced in the apical turn of the cochlea when compared with the middle and basal turns. It was also much higher in cochlear tissue than in vestibular tissue. Taken together, our findings identify PRKCB gene as a novel candidate gene for familial MD and its expression gradient in supporting cells of the organ of Corti deserves attention, given the role of supporting cells in K+ recycling within the endolymph, and its apical turn location may explain the onset of hearing loss at low frequencies in MD.

The vestibulo‐ocular reflex and subjective balance after vestibular schwannoma surgery

To evaluate the vestibulo‐ocular reflex and its relationship with subjective balance in a long‐term follow‐up after vestibular schwannoma surgery.

Variable expressivity and genetic heterogeneity involving DPT and SEMA3D genes in autosomal dominant familial Meniere’s disease

Autosomal dominant (AD) familial Meniere’s disease (FMD) is a rare disorder involving the inner ear defined by sensorineural hearing loss, tinnitus and episodic vertigo. Here, we have identified two novel and rare heterozygous variants in the SEMA3D and DPT genes segregating with the complete phenotype that have variable expressivity in two pedigrees with AD-FMD. A detailed characterization of the phenotype within each family illustrates the clinical heterogeneity in the onset and progression of the disease. We also showed the expression of both genes in the human cochlea and performed in silico analyses of these variants. Three-dimensional protein modelling showed changes in the structure of the protein indicating potential physical interactions. These results confirm a genetic heterogeneity in FMD with incomplete penetrance and variable expressivity.

Clinical Subgroups in Bilateral Meniere Disease

Meniere disease (MD) is a heterogeneous clinical condition characterized by sensorineural hearing loss, episodic vestibular symptoms, and tinnitus associated with several comorbidities, such as migraine or autoimmune disorders (AD). The frequency of bilateral involvement may range from 5 to 50%, and it depends on the duration of the disease. We have performed a two-step cluster analysis in 398 patients with bilateral MD (BMD) to identify the best predictors to define clinical subgroups with a potential different etiology to improve the phenotyping of BMD and to develop new treatments. We have defined five clinical variants in BMD. Group 1 is the most frequently found, includes 46% of patients, and is defined by metachronic hearing loss without migraine and without AD. Group 2 is found in 17% of patients, and it is defined by synchronic hearing loss without migraine or AD. Group 3, with 13% of patients, is characterized by familial MD, while group 4, that includes 12% of patients, is associated by the presence of migraine in all cases. Group 5 is found in 11% of patients and is defined by AD. This approach can be helpful in selecting patients for genetic and clinical research. However, further studies will be required to improve the phenotyping in these clinical variants for a better understanding of the diverse etiological factors contributing to BMD.

Vestibular compensation after vestibular schwannoma surgery: normalization of the subjective visual vertical and disability

Abstract Conclusions: The degree of caloric weakness before surgery influences faster or slower recovery of patients undergoing vestibular schwannoma surgery. The Dizziness Handicap Inventory (DHI) is a good index to show the recovery of patients as it relates directly to an improvement or not of the subjective visual vertical (SVV). Objective: To evaluate the process of recovery of patients as measured by the SVV and the DHI after surgical removal of vestibular schwannoma. Methods: We studied 24 consecutive patients of the University Hospital of Salamanca who underwent vestibular schwannoma surgery. We assessed age, tumour size, degree of canalicular weakness and preoperative SVV, and their relationship with DHI and SVV at discharge and also at 1, 3 and 6 months postoperatively. Results: Patients with lesser degrees of caloric weakness took longer to normalize SVV than those with a higher caloric weakness before surgery (p < 0.05). There was a significant correlation between DHI and improvements in SVV with time. The differences disappeared in 6 months where all patients, with greater or lesser degree of caloric weakness, had the same results.

Vestibular function in cochlear implantation: Correlating objectiveness and subjectiveness.

To evaluate vestibular function before and after cochlear implantation (CI)

HITCal: a software tool for analysis of video head impulse test responses

Abstract Conclusion: The developed software (HITCal) may be a useful tool in the analysis and measurement of the saccadic video head impulse test (vHIT) responses and with the experience obtained during its use the authors suggest that HITCal is an excellent method for enhanced exploration of vHIT outputs. Objective: To develop a (software) method to analyze and explore the vHIT responses, mainly saccades. Methods: HITCal was written using a computational development program; the function to access a vHIT file was programmed; extended head impulse exploration and measurement tools were created and an automated saccade analysis was developed using an experimental algorithm. For pre-release HITCal laboratory tests, a database of head impulse tests (HITs) was created with the data collected retrospectively in three reference centers. This HITs database was evaluated by humans and was also computed with HITCal. Results: The authors have successfully built HITCal and it has been released as open source software; the developed software was fully operative and all the proposed characteristics were incorporated in the released version. The automated saccades algorithm implemented in HITCal has good concordance with the assessment by human observers (Cohen’s kappa coefficient = 0.7).

Extended phenotype and clinical subgroups in unilateral Meniere disease: A cross-sectional study with cluster analysis

To define clinical subgroups by cluster analysis in patients with unilateral Meniere disease (MD) and to compare them with the clinical subgroups found in bilateral MD.