Long-term efficacy of vestibular neurotomy in disabling Ménière’s disease and Tumarkin drop attacks

OBJECTIVE When Ménière’s disease (MD) becomes disabling due to the frequency of attacks or the appearance of drop attacks (i.e., Tumarkin otolithic crisis) despite "conservative" medical and surgical treatments, a radical treatment like vestibular neurotomy (VN) is possible. An ideal MD treatment would relieve symptoms immediately and persist after the therapy. The aim of this study was to identify if VN was effective after 10 years of follow-up regarding vertigo and drop attacks, and to collect the immediate complications. METHODS The authors report a retrospective, single-center (i.e., in a single tertiary referral center with otoneurological surgery activity) cohort study conducted from January 2003 to April 2020. All patients with unilateral disabling MD who had received a VN with at least 10 years of follow-up were included. The therapeutic efficacy was defined by complete disappearance of vertigo and drop attacks. The postoperative complications (CSF leak, total deafness, meningitis, death) were determined immediately after the surgery, and the hearing thresholds were determined during the patient follow-up with the pure tone average (PTA). RESULTS A total of 74 patients (of 85 who were eligible), average age 51.9 ± 11.1 years, including 38 men (51.4%), with disabling MD and/or Tumarkin drop attacks (24.3%) received VN, with at least 10 years of follow-up after surgery. After an average follow-up of 12.4 ± 1.7 years (range 10.0–16.3 years), 67 patients (90.5%) no longer presented any vertiginous attacks, and no patient experienced drop attack. The mean variation in early pre- and postoperative PTA was not statistically significant (n = 64, 2.2 ± 10.3 decibels hearing level [range −18 to 29], 95% CI [−0.4 to 4.37]; p = 0.096), and 84.4% of the patients evaluated had unchanged or improved postoperative PTA. Three significant complications were noted, including two surgical revisions for CSF leak. There was no permanent facial paralysis, meningitis, or death. CONCLUSIONS In case of disabling MD (disabling vertigo refractory to conservative vestibular treatments—Tumarkin drop attacks), VN via the retrosigmoid approach must be the prioritized proposal in comparison to intratympanic gentamicin injections, because of the extremely low complication rate and the immediate and long-lasting effect of this treatment on vertigo and falls.

Surgical Aspects of Corpus Callosotomy

Corpus callosotomy (CC) is one of the options in epilepsy surgeries to palliate patient seizures, and is typically applied for drop attacks. The mechanisms of seizure palliation involve disrupting the propagation of epileptic activity to the contralateral side of the brain. This review article focuses on the surgical aspects of CC. As a variations of CC, anterior two-thirds, posterior one-third, and total callosotomy are described with intraoperative photographs. As less-invasive surgical variations, recent progress in endoscopic CC, and CC without craniotomy, is described. CC remains acceptable under the low prevalence of complications, and surgeons should make the maximum effort to minimize the complication rate.

Corpus Callosotomy for Controlling Epileptic Spasms: A Proposal for Surgical Selection

In 1940, van Wagenen and Herren first proposed the corpus callosotomy (CC) as a surgical procedure for epilepsy. CC has been mainly used to treat drop attacks, which are classified as generalized tonic or atonic seizures. Epileptic spasms (ESs) are a type of epileptic seizure characterized as brief muscle contractions with ictal polyphasic slow waves on an electroencephalogram and a main feature of West syndrome. Resection surgeries, including frontal/posterior disconnections and hemispherotomy, have been established for the treatment of medically intractable ES in patients with unilaterally localized epileptogenic regions. However, CC has also been adopted for ES treatment, with studies involving CC to treat ES having increased since 2010. In those studies, patients without lesions observed on magnetic resonance imaging or equally bilateral lesions predominated, in contrast to studies on resection surgeries. Here, we present a review of relevant literature concerning CC and relevant adaptations. We discuss history and adaptations of CC, and patient selection for epilepsy surgeries due to medically intractable ES, and compared resection surgeries with CC. We propose a surgical selection flow involving resection surgery or CC as first-line treatment for patients with ES who have been assessed as suitable candidates for surgery.

Hydropic Ear Disease: Correlation Between Audiovestibular Symptoms, Endolymphatic Hydrops and Blood-Labyrinth Barrier Impairment

Research Objective: To investigate the correlation between clinical features and MRI-confirmed endolymphatic hydrops (EH) and blood-labyrinth barrier (BLB) impairment.Study Design: Retrospective cross-sectional study.Setting: Vertigo referral center (Haga Teaching Hospital, The Hague, the Netherlands).Methods: We retrospectively analyzed all patients that underwent 4 h-delayed Gd-enhanced 3D FLAIR MRI at our institution from February 2017 to March 2019. Perilymphatic enhancement and the degree of cochlear and vestibular hydrops were assessed. The signal intensity ratio (SIR) was calculated by region of interest analysis. Correlations between MRI findings and clinical features were evaluated.Results: Two hundred and fifteen patients with MRI-proven endolymphatic hydrops (EH) were included (179 unilateral, 36 bilateral) with a mean age of 55.9 yrs and median disease duration of 4.3 yrs. Hydrops grade is significantly correlated with disease duration (P < 0.001), the severity of low- and high-frequency hearing loss (both P < 0.001), and the incidence of drop attacks (P = 0.001). Visually increased perilymphatic enhancement was present in 157 (87.7%) subjects with unilateral EH. SIR increases in correlation with hydrops grade (P < 0.001), but is not significantly correlated with the low or high Fletcher index (P = 0.344 and P = 0.178 respectively). No significant differences were found between the degree of EH or BLB impairment and vertigo, tinnitus or aural fullness.Conclusion: The degree of EH positively correlates with disease duration, hearing loss and the incidence of drop attacks. The BLB is impaired in association with EH grade, but without clear contribution to the severity of audiovestibular symptoms.

Reiterating the role of corpus callosum in generalization of interictal and ictal epileptiform discharges: a case report with post-callosotomy intracranial electroencephalography in Lennox–Gastaut syndrome

Lennox–Gastaut syndrome (LGS) is an epileptic encephalopathy characterized by delayed mental development and intractable multiple seizure types, predominantly tonic. Drop attacks are the commonest and the most disabling type of seizures. Resective surgery is often not possible in LGS as the electroencephalogram (EEG) abnormalities are usually multifocal and generalized, and magnetic resonance image is often either normal or multilesional. We report a case of LGS with bilateral parieto-occipital gliosis where EEG before and after callosotomy demonstrated synchronized bilateral interictal epileptiform discharges and ictal discharges becoming desynchronized and running down. This phenomenon emphasizes the role of the corpus callosum in secondary bilateral synchrony.

Ictal Syncope or Isolated Syncope? A case report highlighting the overlap

Background Ictal bradyarrhythmia is a rare condition defined by temporal lobe epilepsy resulting in bradycardia or asystole and can result in syncope. This needs to be differentiated from isolated syncope in patients with seizure disorder, as treatment strategies differ. Case Summary A 50-year-old female with well-controlled temporal epilepsy and a 20-year seizure-free period presented to her neurologist with abrupt onset of sudden drop attacks, thought to be ictal events with potential underlying ictal bradyarrhythmia and was initially treated with escalation of anti-convulsant therapy. However, her workup was consistent with a diagnosis of cardiac syncope. She subsequently underwent successful insertion of a pacemaker, with no recurrence of her presenting episodes at a 13-month Follow-up. Discussion Ictal syncope and isolated syncope may share a common terminal pathway and may have similar presenting symptoms. In patients with known seizure disorder, loss of consciousness may be attributable to epileptic events, ictal syncope, or isolated syncope—which can be difficult to differentiate. This case highlights the ambiguous nature of such episodes and the importance of simultaneous EEG/ECG monitoring, as this can have implications on treatment.

Evaluation and outcomes of paediatric epilepsy surgery in Singapore: A single-centre audit

Background: Paediatric epilepsy surgery reduces seizure burden in drug-refractory epilepsy reducing long-term neurocognitive damage. Methods: Single-centre retrospective audit of pre-surgical evaluations and outcomes of the paediatric epilepsy and epilepsy surgery programme over eleven years at KK Women’s and Children’s Hospital, Singapore. Data were collected based on National Institute of Neurological Disorders and Stroke Common Data Elements guidelines. Outcome was categorized using Engel classification scale, and favourable outcome defined as greater than 50% decrease in seizure frequency or drop attacks. Results: Thirty-three children underwent epilepsy surgery, with mean follow-up 3.8±3.1 years. Median age at surgery was 10.9 years. Twenty-four children with focal epilepsy underwent resection of the epileptogenic focus, including lesionectomy (n=8), anterior temporal lobectomy (n=7), extratemporal lobectomy (n=7) and hemispherectomy (n=2). Nine children underwent corpus callosotomy for Lennox Gastaut Syndrome (n=8) and West Syndrome (n=1). Median hospital stay duration was ten days. All twenty-three focal epilepsy patients with minimum three-month follow-up achieved greater than 50% seizure reduction. Fifteen (65%) focal epilepsy patients achieved seizure-freedom (Engel Class IA) after first surgeries. Four patients required second surgeries, with two achieving seizure-freedom. Intraoperative MRI (iMRI) is beneficial. All nine corpus callosotomy patients (100%) achieved greater than 50% decrease in drop attacks. Number of antiepileptic drugs was weaned for 21/32 (66%) patients. Post-operative complications were low and some patients had anticipated neurological deficits. Outcomes were comparable to current literature. Conclusions: In well-selected candidates with tailored evaluation, paediatric epilepsy surgery is a safe therapeutic option with favourable outcomes and can be performed across the entire paediatric age range.