A phase 2a study investigating the effects of ritlecitinib on brainstem auditory evoked potentials and intraepidermal nerve fiber histology in adults with alopecia areata.

Reversible axonal swelling and brainstem auditory evoked potential (BAEP) changes were observed in standard chronic (9-month) toxicology studies in dogs treated with ritlecitinib, an oral Janus kinase 3/tyrosine kinase expressed in hepatocellular carcinoma family kinase inhibitor, at exposures higher than the approved 50-mg human dose. To evaluate the clinical relevance of the dog toxicity finding, this phase 2a, double-blind study assessed BAEP changes and intraepidermal nerve fiber (IENF) histology in adults with alopecia areata treated with ritlecitinib. Patients were randomized to receive oral ritlecitinib 50 mg once daily (QD) with a 4-week loading dose of 200 mg QD or placebo for 9 months (placebo-controlled phase); they then entered the active-therapy extension and received ritlecitinib 50 mg QD (with a 4-week loading dose of 200 mg in patients switching from placebo). Among the 71 patients, no notable mean differences in change from baseline (CFB) in Waves I-V interwave latency (primary outcome) or Wave V amplitude on BAEP at a stimulus intensity of 80 dB nHL were observed in the ritlecitinib or placebo group at Month 9, with no notable differences in interwave latency or Wave V amplitude between groups. The CFB in mean or median IENF density and in percentage of IENFs with axonal swellings was minimal and similar between groups at Month 9. Ritlecitinib treatment was also not associated with an imbalanced incidence of neurological and audiological adverse events. These results provide evidence that the BAEP and axonal swelling finding in dogs are not clinically relevant in humans.

Corrigendum: Analysis on the MRI and BAEP results of neonatal brain with different levels of bilirubin.

[This corrects the article DOI: 10.3389/fped.2021.719370.].

Monitoring surgery around the cranial nerves.

Intraoperative neurophysiologic monitoring (IONM) of cranial nerve (CN) function is an essential component in multimodality monitoring of surgical procedures where CNs are at risk for injury. In most cases, IONM consists of localizing and mapping CNs and their pathways, and monitoring of CN motor function during surgery. However, CN VIII, which has no motor function, and is at risk for injury in many surgical procedures, can be easily and accurately monitored using brainstem auditory evoked potentials. For motor CNs, the literature is clear that function can be safely and adequately performed using basic electromyographic (EMG) techniques, such as recording of continuous EMG activity and electrically evoked compound muscle actions potentials. Newer techniques, such as corticobulbar motor evoked potentials and reflex studies, show good potential for a greater degree of functional assessment but require further study to determine their clinical utility. EMG remains the basic clinical neurophysiologic technique with the greatest clinical research supporting its utility in IONM of motor CN function and should be used as part of a comprehensive multimodality IONM protocol. Understanding the physiologic basis of EMG and the changes associated with altered motor function will allow the practitioner to alter surgical course to prevent injury and improve patient safety.

Brainstem auditory evoked potential combined with high resolution cranial base CT can optimize the diagnosis of auditory nerve injury.

PURPOSE: Auditory nerve injury is one of the most common nerve injury complications of skull base fractures. However, there is currently a lack of auxiliary examination methods for its direct diagnosis. The purpose of this study was to find a more efficient and accurate means of diagnosis for auditory nerve injury. METHODS: Through retrospectively analyzing the results of brainstem auditory evoked potential (BAEP) and high-resolution CT (HRCT) in 37 patients with hearing impairment following trauma from January 1, 2018 to July 31, 2020, the role of the two inspection methods in the diagnosis of auditory nerve injury was studied. Inclusion criteria were patient had a clear history of trauma and unilateral hearing impairment after trauma; while exclusion criteria were: (1) severe patient with a Glasgow coma scale score ≤5 because these patients were classified as severe head injury and admitted to the intensive care unit, (2) patient in the subacute stage admitted 72 h after trauma, and (3) patient with prior hearing impairment before trauma. According to Goodman's classification of hearing impairment, the patients were divided into low/medium/severe injury groups. In addition, patients were divided into HRCT-positive and negative groups for further investigation with their BAEP results. The positive rates of BEAP for each group were observed, and the results were analyzed by Chi-square test (p < 0.05, regarded as statistical difference). RESULTS: A total of 37 patients were included, including 21 males and 16 females. All of them were hospitalized patients with GCS score of 6-15 at the time of admission. The BAEP positive rate in the medium and severe injury group was 100%, which was significantly higher than that in the low injury group (27.27%) (p < 0.01). The rate of BEAP positivity was significantly higher in the HRCT-positive group (20/30, 66.7%) than in the HRCT-negative group (1/7, 14.3%) (p < 0.05). Twenty patients (54.05%) were both positive for BEAP and HRCT test, and considered to have auditory nerve damage. Six patients (16.22%) were both negative for BEAP and HRCT test, and 10 patients (27.03%) were BAEP-negative but HRCT-positive: all the 16 patients were considered as non-neurological injury. The rest 1 case (2.70%) was BAEP-positive but HRCT-negative, which we speculate may have auditory nerve concussion. CONCLUSION: By way of BAEP combining with skull base HRCT, we may improve the accuracy of the diagnosis of auditory nerve injury. Such a diagnostic strategy may be beneficial to guiding treatment plans and evaluating prognosis.

Diagnostic accuracy of intraoperative brainstem auditory evoked potential for predicting hearing loss after vestibular schwannoma surgery.

Objective: This meta-analysis evaluated the diagnostic value of intraoperative brainstem auditory evoked potential (BAEP) for predicting post-operative hearing loss. Methods: Research articles in MEDLINE, Embase, and Cochrane Library databases were searched and selected up to 20 January 2022, and data were extracted following a standard procedure. A diagnostic accuracy test meta-analysis was performed using a mixed-effect binary regression model. Results: A total of 693 patients from 15 studies were extracted. The change in intraoperative BAEP showed high sensitivity (0.95) but low specificity (0.37), with an area under the curve of 0.83. Diagnostic accuracy of the loss of potentials showed high sensitivity (0.82) and specificity (0.79). The area under the curve was 0.88. No factor was found to account for the heterogeneity of the results according to the meta-regression and subgroup analyses (all P-values > 0.05). Conclusions: Our results showed that the loss of BAEP has meaningful value for predicting hearing loss after vestibular schwannoma surgery. The change in BAEP is also important for its high sensitivity during hearing preservation surgery.

Brainstem auditory evoked potential combined with high resolution cranial base CT can optimize the diagnosis of auditory nerve injury / 中华创伤杂志(英文版)

PURPOSE@#Auditory nerve injury is one of the most common nerve injury complications of skull base fractures. However, there is currently a lack of auxiliary examination methods for its direct diagnosis. The purpose of this study was to find a more efficient and accurate means of diagnosis for auditory nerve injury.@*METHODS@#Through retrospectively analyzing the results of brainstem auditory evoked potential (BAEP) and high-resolution CT (HRCT) in 37 patients with hearing impairment following trauma from January 1, 2018 to July 31, 2020, the role of the two inspection methods in the diagnosis of auditory nerve injury was studied. Inclusion criteria were patient had a clear history of trauma and unilateral hearing impairment after trauma; while exclusion criteria were: (1) severe patient with a Glasgow coma scale score ≤5 because these patients were classified as severe head injury and admitted to the intensive care unit, (2) patient in the subacute stage admitted 72 h after trauma, and (3) patient with prior hearing impairment before trauma. According to Goodman's classification of hearing impairment, the patients were divided into low/medium/severe injury groups. In addition, patients were divided into HRCT-positive and negative groups for further investigation with their BAEP results. The positive rates of BEAP for each group were observed, and the results were analyzed by Chi-square test (p < 0.05, regarded as statistical difference).@*RESULTS@#A total of 37 patients were included, including 21 males and 16 females. All of them were hospitalized patients with GCS score of 6-15 at the time of admission. The BAEP positive rate in the medium and severe injury group was 100%, which was significantly higher than that in the low injury group (27.27%) (p < 0.01). The rate of BEAP positivity was significantly higher in the HRCT-positive group (20/30, 66.7%) than in the HRCT-negative group (1/7, 14.3%) (p < 0.05). Twenty patients (54.05%) were both positive for BEAP and HRCT test, and considered to have auditory nerve damage. Six patients (16.22%) were both negative for BEAP and HRCT test, and 10 patients (27.03%) were BAEP-negative but HRCT-positive: all the 16 patients were considered as non-neurological injury. The rest 1 case (2.70%) was BAEP-positive but HRCT-negative, which we speculate may have auditory nerve concussion.@*CONCLUSION@#By way of BAEP combining with skull base HRCT, we may improve the accuracy of the diagnosis of auditory nerve injury. Such a diagnostic strategy may be beneficial to guiding treatment plans and evaluating prognosis.

Electrophysiological Evaluation of Audiovestibular Pathway Dysfunction in Parkinson's Disease and Its Correlates: A Case Control Study.

BACKGROUND: Parkinson's disease (PD) is associated with brainstem dysfunction causing non-motor symptoms. Vestibular evoked myogenic potential (VEMP) and brainstem auditory evoked potential (BAEP) are electrophysiological tests to assess the vestibular and auditory pathways in the brainstem. OBJECTIVES: To study the abnormalities of cervical VEMP (cVEMP) and BAEP in PD and to correlate the findings with the symptoms related to brainstem involvement. PATIENTS AND METHODS: cVEMP and BAEP were recorded in 25 PD patients and compared 25 age matched controls. The PD patients were assessed with the following clinical scales: REM Sleep Disorder Screening Questionnaire (RBD-SQ), Epworth Sleepiness Scale (ESS), mini-BESTest, Geriatric Depression Scale (GDS-15) and MMSE (Mini-mental state examination). The P13 and N23 peak latencies and the P13/N23 amplitude of cVEMP, the latencies of waves I, III and V, and the inter-peak latencies (IPL) of waves I-III, III-V and I-V of BAEP were measured. RESULTS: The PD patients showed prolonged latencies and reduced amplitude in cVEMP responses. They had abnormal BAEP in the form of prolonged absolute latencies of wave V, followed by wave III and I-V IPL with no significant difference in waves I and I-III IPL. The cVEMP abnormality was correlated directly with RBD-SQ and inversely with mini-BESTest scores. There were no correlations between cVEMP/BAEP abnormality and disease severity, GDS-15, ESS and MMSE. CONCLUSION: PD is associated with cVEMP and BAEP abnormalities that suggest auditory and vestibular pathway dysfunction in the brainstem and cVEMP correlates with the symptoms of brainstem degeneration like RBD and postural instability.

Gentamicin-induced sensorineural auditory loss in healthy adult horses.

BACKGROUND: Irreversible sensorineural auditory loss has been reported in humans treated with aminoglycosides but not in horses. OBJECTIVE: Investigate if auditory loss occurs in horses treated using the recommended IV daily dosage of gentamicin for 7 consecutive days. ANIMALS: Ten healthy adult horses (7-15 years; females and males, 5 each). METHODS: Prospective study. Physical and neurological examinations and renal function tests were performed. Gentamicin sulfate was administered at a dosage of 6.6 mg/kg via the jugular vein on alternating sides for 7 days. Gentamicin peak and trough concentrations were measured. Horses were sedated using detomidine hydrochloride IV to perform brainstem auditory evoked responses (BAER) before the first dose, immediately after the last dose, and 30 days after the last dose. Peaks latencies, amplitudes, and amplitude ratios were recorded. Data from the second and last BAER were compared to results at baseline. Bone conduction was performed to rule out conduction disorders. RESULTS: Seven horses had auditory loss: complete bilateral (N = 1), complete unilateral (N = 2), and partial unilateral (N = 4). Based on physical examination and BAER results, sensorineural auditory loss was suspected. Absent bone conduction ruled out a conduction disorder and further supported sensorineural auditory loss in horses with completely absent BAER. Auditory dysfunction was reversible in 4 of 7 horses. CONCLUSIONS AND CLINICAL IMPORTANCE: Gentamicin at recommended doses may cause sensorineural auditory loss in horses that might be irreversible. Follow-up studies are needed to investigate if other dosing protocols present a similar risk.

Brainstem auditory evoked responses and bone conduction assessment in alpacas.

Auditory loss has been reported in camelids using brainstem auditory evoked responses (BAER). Differentiation between conductive versus sensorineural dysfunction has not been investigated. Therefore, the objective of the study was to investigate auditory function using BAER and bone conduction (BC). Twenty-four alpacas: 15 females, 9 intact males (2-16 years of age) were included in a randomized clinical trial. BAER and BC were recorded using two derivations (vertex to mastoid and vertex to cranial aspect of second cervical vertebra). All alpacas underwent complete physical examinations and were sedated with xylazine hydrochloride at 0.6 mg/kg IM. Peaks, when present, were identified and latencies, amplitudes, and amplitude ratios were determined. Eleven alpacas had normal responses and 13 had auditory loss based on BAER. The latter consisted of complete absence of peaks bilaterally (n = 3), absence of peaks unilaterally (n = 1), delayed latencies bilaterally (n = 4), and delayed latencies unilaterally (n = 5). Distinct peaks on BC supported conductive auditory loss in 6 alpacas, difficult to interpret due to stimulus artifact and additional undefined peaks in 4, and absent peaks in 3 alpacas. The cause of auditory loss was presumed to be due to otitis in 6, aging in 4 (10-16 years old), and congenital sensorineural (absent peaks on BAER and BC) in 3 alpacas with unpigmented fiber and irises. BAER and BC are useful and non-invasive to perform techniques for the investigation of auditory loss in alpacas, and further characterization as conductive or sensorineural.

Analysis on the MRI and BAEP Results of Neonatal Brain With Different Levels of Bilirubin.

BACKGROUND: To explore whether there is abnormality of neonatal brains' MRI and BAEP with different bilirubin levels, and to provide an objective basis for early diagnosis on the bilirubin induced subclinical damage on brains. METHODS: To retrospectively analyze the clinical data of 103 neonatal patients, to conduct routine brain MRI examination and BAEP testing, and to analyze BAEP and MRI image results of the neonatal patients, who were divided into three groups based on the levels of total serum bilirubin concentration (TSB): 16 cases in mild group (TSB: 0.0-229.0 emol/L), 49 cases in moderate group (TSB: 229.0-342.0 emol/L), and 38 cases in severe group (TSB ≥ 342.0 emol/L). RESULTS: We found the following: A. Comparison of the bilirubin value of the different group: The bilirubin value of the mild group is 171.99 ± 33.50 emol/L, the moderate group is 293.98 ± 32.09 emol/L, and the severe group is 375.59 ± 34.25 emol/L. The comparison of bilirubin values of the three groups of neonates (p < 0.01) indicates the difference is statistically significant (p < 0.01). B. The weight value of the <2,500 g group is 2.04 ± 0.21 and the ≥2,500 g group is 3.39 ± 0.46; the weight comparison of the two groups indicates that the difference is statistically significant (p < 0.01). C. Comparison of the abnormal MRI of the different groups: The brain MRI result's abnormal ratio of the mild group is 31.25%, the moderate group is 16.33%, and the severe group is 21.05%, but the comparison of brain MRI results of the three neonates groups indicates that the difference is not statistically significant (p > 0.05). D. Comparison of abnormal MRI signal values of globus pallidus on T1WI in different groups: 1. The comparison of normal group signal values with that of mild group (p < 0.05), with that of moderate group, and with that of severe group (p < 0.01) indicates that the difference is statistically significant. CONCLUSION: At low level of bilirubin, central nervous system damage may also occur and can be detected as abnormality by MRI and BAEP. Meanwhile, MRI and BAEP can also provide early abnormal information for the judgment of central nervous system damage of the children with NHB who have no acute bilirubin encephalopathy (ABE) clinical features, and provide clues for early treatment and early intervention.

Prediction of Delayed Neurodevelopment in Infants Using Brainstem Auditory Evoked Potentials and the Bayley II Scales.

Background: Brainstem auditory evoked potentials (BAEP) provide an objective analysis of central nervous system function and development in infants. This study proposed to examine the relationship between infant BAEP values at age 6 months, and their neurodevelopment at age 2 years assessed by the mental development indices (MDI), a form of Bayley Scales of Infant Development. We hypothesized that in infants with BAEP values outside normal range, there may be neurodevelopmental delays, as shown by their MDI scores. Methods: An exploratory investigation was conducted using preterm (28-36 weeks gestation; 95 cases) and term infants (≥37 weeks gestation; 100 cases) who were born with specific perinatal conditions. BAEP values were recorded in these infants from 1 to 8 months of age, and compared with MDI scores in these infants at age 2 years. A multivariate linear regressions model was performed to test the associations between all variables and MDI scores. Stratified linear regression was used to test the interactions between gestational age and BAEP values with MDI scores. Significance was determined at a p < 0.05. Results: We found that BAEP values were inversely associated with MDI scores in premature infants (ß = -1.89; 95% confidence interval = -3.42 to -0.36), and that the effect of gestational age and BAEP values on the MDI scores is decreased by 1.89 points due to the interaction between these two variables. In premature babies, the lower the BAEP value below the mean, the greater the decrease in MDI score at age 2 years. Asphyxia and lower socioeconomic status in the family were also covariates associated with lower MDI scores at age 2 years. Conclusion: The data provided evidence that BAEP values outside the normal range in premature infants at age 6 months may predict developmental delays in cognitive and motor skills, as shown by MDI scores. We propose that BAEP assessment may be utilized as a potential indicator for neurodevelopment, and suggest that early intellectual and public health interventions should be encouraged to enrich neurodevelopment in premature babies with BAEP values outside the normal range.

Fully endoscopic microvascular decompression for hemifacial spasm.

OBJECTIVE: Hemifacial spasm (HFS) is characterized by involuntary tonic and/or clonic contractions of facial nerve muscles. Fully endoscopic microvascular decompression (E-MVD) for HFS has not been widely adopted. This paper aims to illustrate the safety and efficacy of the fully endoscopic technique for HFS treatment. METHODS: The authors conducted a single-center retrospective study of 27 patients (28 separate E-MVD cases; 1 patient had bilateral E-MVD) diagnosed with HFS who underwent fully E-MVD from January 2013 to October 2016. Intraoperative brainstem auditory evoked potentials and lateral spread resolution were reviewed. Outcome was based on the clinical status of the patient at the last contact point with the senior author. Complications were categorized as facial weakness, hearing loss, ataxia, dysphagia, or any adverse event able to be attributed to the surgical procedure. RESULTS: HFS was relieved either completely or partially in the majority of cases (24 of 28, 85.7%). Of the 28 separate procedures, 17 (60.7%) resulted in complete resolution of symptoms, 4 (14.3%) resulted in near-complete resolution, 2 (7.1%) resulted in 50% reduction of symptoms, 1 (3.6%) resulted in minimal reduction, and 4 (14.3%) resulted in no relief. Of the 27 patients, 26 (96%) had no permanent postoperative complications. In multivariate logistic regression, the best predictor of greater than 50% resolution of spasm was resolution of intraoperative lateral spread response. CONCLUSIONS: A fully E-MVD for HFS provides a safe and comprehensive view of the neurovascular conflict. Exclusive use of the endoscope in MVD is both safe and feasible in the treatment of HFS. Attention to lateral spread response monitoring remains an integral part of comprehensive neurosurgical management.

Effect of granulocyte colony-stimulating factor on the cochlear nuclei after creation of a partial nerve lesion: an experimental study in rats.

OBJECTIVE The risk of injury of the cochlear nerve during angle (CPA) surgery is high. Granulocyte colony-stimulating factor (G-CSF) has been found in various experimental models of peripheral and CNS injury to have a neuroprotective effect by inhibiting apoptosis and inflammation. However, to the authors' knowledge, the influence of G-CSF on cochlear nerve regeneration has not been reported. This study investigated the neuroprotective effect of G-CSF after a partial cochlear nerve lesion in rats. METHODS A lesion of the right cochlear nerve in adult male Sprague-Dawley rats was created using a water-jet dissector with a pressure of 8 bar. In the first group (G-CSF-post), G-CSF was administrated on Days 1, 3, and 5 after the surgery. The second group (G-CSF-pre/post) was treated with G-CSF 1 day before and 1, 3, and 5 days after applying the nerve injury. The control group received sodium chloride after nerve injury at the various time points. Brainstem auditory evoked potentials (BAEPs) were measured directly before and after nerve injury and on Days 1 and 7 to evaluate the acoustic function of the cochlear nerve. The animals were sacrificed 1 week after the operation, and their brains were fixed in formalin. Nissl staining of the cochlear nuclei was performed, and histological sections were analyzed with a light microscope and an image-processing program. The numbers of neurons in the cochlear nuclei were assessed. RESULTS The values for Waves 2 and 4 of the BAEPs decreased abruptly in all 3 groups in the direct postoperative measurement. Although the amplitude in the control group did not recover, it increased in both treatment groups. According to 2-way ANOVA, groups treated with G-CSF had a significant increase in BAEP Wave II amplitudes on the right side (p = 0.0401) after the applied cochlear nerve injury. With respect to Wave IV, a trend toward better recovery in the G-CSF groups was found, but this difference did not reach statistical significance. In the histological analysis, higher numbers of neurons were found in the G-CSF groups. In the statistical analysis, the difference in the numbers of neurons between the control and G-CSF-post groups reached significance (p = 0.0086). The difference in the numbers of neurons between the control and G-CSF-pre/post groups and between the G-CSF-post and G-CSF-pre/post groups did not reach statistical significance. CONCLUSIONS The use of G-CSF improved the function of the eighth cranial nerve and protected cochlear nucleus cells from destruction after a controlled partial injury of the nerve. These findings might be relevant for surgery that involves CPA tumors. The use of G-CSF in patients with a lesion in the CPA might improve postoperative outcomes.

Deep brain stimulation for the early treatment of the minimally conscious state and vegetative state: experience in 14 patients.

OBJECTIVE An effective treatment of patients in a minimally conscious state (MCS) or vegetative state (VS) caused by hypoxic encephalopathy or traumatic brain injury (TBI) is not yet available. Deep brain stimulation (DBS) of the thalamic reticular nuclei has been attempted as a therapeutic procedure mainly in patients with TBI. The purpose of this study was to investigate the therapeutic use of DBS for patients in VS or MCS. METHODS Fourteen of 49 patients in VS or MCS qualified for inclusion in this study and underwent DBS. Of these 14 patients, 4 were in MCS and 10 were in VS. The etiology of VS or MCS was TBI in 4 cases and hypoxic encephalopathy due to cardiac arrest in 10. The selection criteria for DBS, evaluating the status of the cerebral cortex and thalamocortical reticular formation, included: neurological evaluation, electrophysiological evaluation, and the results of positron emission tomography (PET) and MRI examinations. The target for DBS was the centromedian-parafascicular (CM-pf) complex. The duration of follow-up ranged from 38 to 60 months. RESULTS Two MCS patients regained consciousness and regained their ability to walk, speak fluently, and live independently. One MCS patient reached the level of consciousness, but was still in a wheelchair at the time the article was written. One VS patient (who had suffered a cerebral ischemic lesion) improved to the level of consciousness and currently responds to simple commands. Three VS patients died of respiratory infection, sepsis, or cerebrovascular insult (1 of each). The other 7 patients remained without substantial improvement of consciousness. CONCLUSIONS Spontaneous recovery from MCS/VS to the level of consciousness with no or minimal need for assistance in everyday life is very rare. Therefore, if a patient in VS or MCS fulfills the selection criteria (presence of somatosensory evoked potentials from upper extremities, motor and brainstem auditory evoked potentials, with cerebral glucose metabolism affected not more than the level of hypometabolism, which is judged using PET), DBS could be a treatment option.

Adult-onset of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome with hypothyroidism and psychiatric disorders.

Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a clinical syndrome associated with mitochondrial disorders (MIDs). This report illustrates a case of MELAS syndrome with hypothyroidism and psychiatric disorders, which is different from the common clinical manifestations of MELAS syndrome, such as exercise intolerance, migraine-like headaches, hearing loss and seizures etc. There are considerable interests in the possibility that mitochondrial dysfunction may play a role in the pathogenesis of endocrine dysfunctions and psychiatric disorders in MELAS syndrome.

Intraoperative neurophysiological monitoring during resection of infratentorial lesions: the surgeon's view.

OBJECTIVE Methods of choice for neurophysiological intraoperative monitoring (IOM) within the infratentorial compartment mostly include early brainstem auditory evoked potentials, free-running electromyography, and direct cranial nerve (CN) stimulation. Long-tract monitoring with somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) is rarely used. This study investigated the incidence of IOM alterations during posterior fossa surgery stratified for lesion location. METHODS Standardized CN and SEP/MEP IOM was performed in 305 patients being treated for various posterior fossa pathologies. The IOM data were correlated with lesion locations and histopathological types as well as other possible confounding factors. RESULTS Alterations in IOM were observed in 158 of 305 cases (51.8%) (CN IOM alterations in 130 of 305 [42.6%], SEP/MEP IOM alterations in 43 of 305 [14.0%]). In 15 cases (4.9%), simultaneous changes in long tracts and CNs were observed. The IOM alterations were followed by neurological sequelae in 98 of 305 cases (32.1%); 62% of IOM alterations resulted in neurological deficits. Sensitivity and specificity for detection of CN deficits were 98% and 77%, respectively, and 95% and 85%, respectively, for long-tract deficits. Regarding location, brainstem and petroclival lesions were closely associated with concurrent CN IOM and SEP/MEP alterations. CONCLUSIONS The incidence of IOM alterations during surgery in the posterior fossa varied widely between different lesion locations and histopathological types. This analysis provides crucial information on the necessity of IOM in different surgical settings. Because MEP/SEP and CN IOM alterations were commonly observed during posterior fossa surgery, the authors recommend the simultaneous use of both modalities based on lesion location.

Efficacy of microsurgical tumor removal for treatment of patients with intracanalicular vestibular schwannoma presenting with disabling vestibular symptoms.

OBJECTIVE The aim of this study was to analyze the efficacy and risks of microsurgery via the hearing-preserving retrosigmoid approach in patients with intracanalicular vestibular schwannoma (VS) suffering from disabling vestibular symptoms, with special attention to vertigo. METHODS This is a retrospective analysis of 19 patients with intracanalicular VS and disabling vestibular dysfunction as the main or only symptom (Group A). All of the patients reported having had disabling vertigo attacks. Subjective evaluation of the impairment of patients was performed before surgery, 3 weeks after surgery, 3 months after surgery, and 1 year after surgery, using the Dizziness Handicap Inventory (DHI). The main outcome measures were improvement in quality of life as measured using the DHI, and general and functional outcomes, in particular facial function and hearing. Patient age, preoperative tumor size, preoperative DHI score, and preservation of the nontumorous vestibular nerve were tested using a multivariate regression analysis to determine factors affecting the postoperative DHI score. The Mann-Whitney U-test was used to compare the postoperative DHI score at 3 weeks, 3 months, and 1 year after surgery with a control group of 19 randomly selected patients with intracanalicular VSs, who presented without vestibular symptoms (Group B). The occurrence of early postoperative discrete vertigo attacks was also compared between groups. RESULTS The preoperative DHI score was ≥ 54 in all patients. All patients reported having had disabling rotational vertigo before surgery. The only significant factor to affect the DHI outcome 3 weeks and 3 months after surgery was the preoperative DHI score. The DHI outcome after 1 year was not affected by the preoperative DHI score. Compared with the control group, the DHI score at 3 weeks and 3 months after surgery was significantly worse. There was no significant difference between the groups after 1 year. Vertigo was improved in all patients and completely resolved after 1 year in 17 patients. CONCLUSIONS Disabling vestibular dysfunction that affects quality of life should be considered an indication for surgery, even in otherwise asymptomatic patients with intracanalicular VS. Surgical removal of the tumor is safe and very effective in regard to symptom relief. All patients had excellent facial nerve function within 1 year after surgery, with a very good chance of hearing preservation.

Intraoperative auditory steady-state monitoring during surgery in the cerebellopontine angle for estimation of postoperative hearing classes.

OBJECTIVE Brainstem auditory evoked potentials (BAEPs) have been used for intraoperative monitoring of the auditory nerve for many years. However, BAEPs yield limited information about the expected postoperative hearing quality and speech perception. The auditory steady-state response (ASSR) enables objective audiograms to be obtained in patients under anesthesia. These ASSRs could be used for intraoperative estimation of hearing classes to improve the postoperative outcome and quality of life. Studies investigating the clinical use of ASSRs during total intravenous anesthesia are currently lacking. The work presented in this article therefore investigates the application of ASSRs for intraoperative estimation of hearing classes. METHODS In 43 patients undergoing surgery for vestibular schwannoma, ASSR measurements were performed at the beginning and end of the surgical procedure. ASSR stimuli consisted of 80-dB hearing level amplitude-modulated tones with 5-minute duration, 90-Hz modulation, and 3 different carrier frequencies: 500, 1000, and 2000 Hz. Stimulation was performed unilaterally with and without contralateral masking, using single and combined carriers. Evoked responses were recorded and analyzed in the frequency domain. ASSRs were compared with extraoperative hearing classes and BAEPs using ANOVA, correlation, and receiver operating characteristic statistics. RESULTS ASSRs yielded high and consistent area under the curve (AUC) values (mean 0.83) and correlation values (mean -0.63), indicating reliable prediction of hearing classes. Analysis of BAEP amplitude changes showed lower AUC (mean 0.79) and correlation values (0.63, 0.37, and 0.50 for Waves I, III, and V, respectively). Latencies showed low AUC values (mean 0.6) and no significant correlation. Combination of several carriers for simultaneous evaluation reduced ASSR amplitudes and respective AUC values. Contralateral masking did not show a significant effect. CONCLUSIONS ASSRs robustly estimate hearing class in patients under total intravenous anesthesia, even when using short measurement durations. The method provides a diagnostic performance that exceeds conventional BAEP monitoring and enables objective and automated evaluation. On the basis of these findings, continuous intraoperative auditory monitoring could become a promising alternative or adjunct to BAEPs.

Are evoked potentials clinically useful in the study of patients with Chiari malformation Type 1?

OBJECTIVE In this study, the authors describe the brainstem auditory evoked potential (BAEP) and somatosensory evoked potential (SSEP) alterations found in a large cohort of patients with Chiari malformation Type 1 (CM-1), the relationship between the BAEPs/SSEPs and the clinical findings, the abnormalities in patients with associated syringomyelia, and the clinical and neuroradiological risk factors that are associated with abnormal evoked potentials (EPs). METHODS A prospectively collected database containing 545 patients with CM-1 was queried to search for patients satisfying the following criteria: 1) an age of at least 14 years, 2) neuroradiological criteria of CM-1, 3) no prior Chiari-related surgeries, and 4) preoperative EP studies conducted at the authors' institution. The 200 patients included in this cohort were classified into CM-0, CM-1, and CM-1.5 subtypes. Linear, planimetric, and angular measurements of the posterior fossa were conducted, as well as syringomyelia measurements. Two separate multiple logistic regression models were used, one to predict the covariates associated with abnormal BAEPs, and a second model to explore the variables associated with an abnormal SSEP. In these models, the BAEPs and SSEPs were dichotomized as being normal or abnormal. RESULTS Headaches were the main symptom in 70.5% of the patients, and Valsalva-induced headaches were most frequent in patients with CM-1 and CM-1.5 compared with patients with CM-0 (p = 0.031). BAEPs were abnormal in 38.5% of patients, and abnormal SSEPs were found in 43.5% of the entire cohort. Syringomyelia was most frequent in patients with CM-0 (64.3%) and CM-1 (51.1%) compared with those with CM-1.5 (34.7%; p = 0.03). Age (OR 1.03, 95% CI 1.00-1.06), the degree of tonsillar herniation (OR 1.08, 95% CI 1.01-1.16), and lower cranial nerve dysfunction (OR 3.99, 95% CI 1.29-14.01) had a statistically significant correlation with abnormal BAEPs. Only age (OR 1.07, 95% CI 1.04-1.10) and the degree of tonsillar herniation (OR 1.11, 95% CI 1.04-1.19) had a statistically significant correlation with abnormal SSEPs. CONCLUSIONS A high percentage of patients with CM-1 exhibited EP alterations regardless of their clinical or radiological findings. These findings suggest that EPs do not add any clinically relevant information nor are they helpful in establishing which symptomatic patients with CM should undergo surgical treatment. However, BAEP and SSEP studies clearly play an important role in incidentally detected patients with CM and may help to establish objective evidence of subclinical dysfunctions. In addition, neurophysiological studies may help to define subgroups of patients who require further testing and follow-up to personalize strategies for the management of incidental and oligosymptomatic patients.

Diagnostic accuracy of motor evoked potentials to detect neurological deficit during idiopathic scoliosis correction: a systematic review.

OBJECTIVE The goal of this study was to evaluate the efficacy of intraoperative transcranial motor evoked potential (TcMEP) monitoring in predicting an impending neurological deficit during corrective spinal surgery for patients with idiopathic scoliosis (IS). METHODS The authors searched the PubMed and Web of Science database for relevant lists of retrieved reports and/or experiments published from January 1950 through October 2014 for studies on TcMEP monitoring use during IS surgery. The primary analysis of this review fit the operating characteristic into a hierarchical summary receiver operating characteristic curve model to determine the efficacy of intraoperative TcMEP-predicted change. RESULTS Twelve studies, with a total of 2102 patients with IS were included. Analysis found an observed incidence of neurological deficits of 1.38% (29/2102) in the sample population. Of the patients who sustained a neurological deficit, 82.8% (24/29) also had irreversible TcMEP change, whereas 17.2% (5/29) did not. The pooled analysis using the bivariate model showed TcMEP change with sensitivity (mean 91% [95% CI 34%-100%]) and specificity (mean 96% [95% CI 92-98%]). The diagnostic odds ratio indicated that it is 250 times more likely to observe significant TcMEP changes in patients who experience a new-onset motor deficit immediately after IS correction surgery (95% CI 11-5767). TcMEP monitoring showed high discriminant ability with an area under the curve of 0.98. CONCLUSIONS A patient with a new neurological deficit resulting from IS surgery was 250 times more likely to have changes in TcMEPs than a patient without new deficit. The authors' findings from 2102 operations in patients with IS show that TcMEP monitoring is a highly sensitive and specific test for detecting new spinal cord injuries in patients undergoing corrective spinal surgery for IS. They could not assess the value of TcMEP monitoring as a therapeutic adjunct owing to the limited data available and their study design.