The necessity of electrodiagnostic studies and ultrasound in ulnar nerve entrapment according to surgeons in the Netherlands.

BACKGROUND: Ulnar nerve entrapment at the elbow (UNE) is the second most prevalent entrapment neuropathy after carpal tunnel syndrome. The objective of this study was to evaluate the expert opinion of different surgical disciplines regarding the need for electrodiagnostic or ultrasound confirmation of UNE and, if so, which test was preferred for confirmation. METHODS: A questionnaire was sent to all neurosurgeons and plastic or hand surgeons in the Netherlands to evaluate the current practice in planning surgical treatment of UNE. RESULTS: The response rate was 36.4 % (134 out of 368). 94 % of surgeons reported that > 95 % of their patients had EDX or ultrasound studies before surgery. 80.6 % of all surgeons who responded reported that they seldom operated on UNE without electrodiagnostic confirmation. Hand surgeons (25.9 %) were more willing to operate on clinically diagnosed UNE without EDX than neurosurgeons (9.4 %) CONCLUSIONS: Dutch surgeons prefer diagnostic confirmation of UNE either by ultrasound or EDX, with a preference for EDX and the vast majority of operated patients do have either EDX or ultrasound or both before surgery. Compared to neurosurgeons, hand surgeons are more willing to operate on patients with clinically defined UNE but normal electrodiagnostic studies.

Dutch injection versus surgery trial in patients with carpal tunnel syndrome (DISTRICTS): protocol of a randomised controlled trial comparing two treatment strategies.

INTRODUCTION: Carpal tunnel syndrome (CTS) is the most common peripheral neuropathy. The optimal treatment strategy is still unknown. The objective of the Dutch Injection versus Surgery TRIal in patients with CTS (DISTRICTS) is to investigate if initial surgery of CTS results in a better clinical outcome and is more cost-effective when compared with initial treatment with corticosteroid injection. METHODS AND ANALYSIS: The DISTRICTS is an ongoing multicenter, open-label randomised controlled trial. Participants with CTS are randomised to treatment with surgery or with a corticosteroid injection. If needed, any additional treatments after this first treatment are allowed and these are not dictated by the study protocol. The primary outcome is the difference between the groups in the proportion of participants recovered at 18 months. Recovery is defined as having no or mild symptoms as measured with the 6-item carpal tunnel symptoms scale. Secondary outcome measurements are among others: time to recovery, hand function, patient satisfaction, quality of life, additional treatments, adverse events, and use of care and health-related costs. ETHICS AND DISSEMINATION: The study was approved by the Medical Ethical Committee of the Amsterdam University Medical Centers (study number 2017-171). Study results will be disseminated in peer-reviewed journals and conferences. TRIAL REGISTRATION NUMBER: ISRCTN Registry: 13164336.

Congenital Bilateral Anomaly of the Flexor Pollicis Longus Tendon Without Thenar Atrophy: A Case Report.

In this case report, we describe an adult patient with bilateral congenital absence of the m. flexor pollicis longus (FPL) without any other anomalies or thenar atrophy. The FPL muscle acts to flex the thumb and is innervated by the anterior interosseous nerve. Impaired function of the FPL muscle may have several causes. A bilateral anomaly of the FPL tendon is a rare phenomenon, with only a few cases having been reported. This case report does not only demonstrate the diagnosis of bilateral FPL tendon anomaly by physical examination and magnetic resonance imaging, it also includes the embryological development of the FPL muscle and tendon.

Self-reported work productivity in people with multiple sclerosis and its association with mental and physical health.

PURPOSE: This study aimed to identify mental health, physical health, demographic and disease characteristics relating to work productivity in people with multiple sclerosis (MS). METHODS: In this cross-sectional study, 236 employed people with MS (median age = 42 years, 78.8% female) underwent neurological and neuropsychological assessments. Additionally, they completed questionnaires inquiring about work productivity (presenteeism: reduced productivity while working, and absenteeism: loss of productivity due to absence from work), mental and physical health, demographic and disease characteristics. Multiple linear and logistic regression analyses were performed with presenteeism and absenteeism as dependent variables, respectively. RESULTS: A model with mental and physical health factors significantly predicted presenteeism F(11,202) = 11.33, p < 0.001, R2 = 0.38; a higher cognitive (p < 0.001) and physical impact (p = 0.042) of fatigue were associated with more presenteeism. A model with only mental health factors significantly predicted absenteeism; χ2(11)=37.72, p < 0.001, with R2 = 0.27 (Nagelkerke) and R2 = 0.16 (Cox and Snell). Specifically, we observed that more symptoms of depression (p = 0.041) and a higher cognitive impact of fatigue (p = 0.011) were significantly associated with more absenteeism. CONCLUSIONS: In people with MS, both cognitive and physical impact of fatigue are positively related to presenteeism, while symptoms of depression and cognitive impact of fatigue are positively related to absenteeism.Implications for rehabilitationMultiple sclerosis (MS) affects people of working age, significantly interfering with work productivity.Higher cognitive and physical impact of fatigue were associated with more presenteeism in workers with MS.A higher cognitive impact of fatigue and more depressive symptoms were associated with absenteeism in workers with MS.Occupational and healthcare professionals should be aware of the impact of both physical and mental health on work productivity in workers with MS.

Development of a patient-reported outcome measure for patients who have recovered from a subarachnoid hemorrhage: the "questionnaire for the screening of symptoms in aneurysmal subarachnoid hemorrhage" (SOS-SAH).

BACKGROUND: Patients who have been successfully treated for an aneurysmal subarachnoid hemorrhage (aSAH) often retain multiple health complaints, including mood disorders, cognitive complaints, fatigue, and problems with social participation. These problems are not always fully addressed during hospital visits or in current outcome measures, such as the modified Rankin score and the Glasgow Outcome Scale. Here, we present the development of the "Questionnaire for the Screening of Symptoms in aneurysmal Subarachnoid Hemorrhage" (SOS-SAH), which screens for the self-reported symptoms of patients with mild disabilities. METHODS: During the development of the SOS-SAH we adhered to the PROM-cycle framework for the selection and implementation of patient-reported outcome measures (PROMs). The SOS-SAH was developed in an iterative process informed by a literature study. Patients and healthcare professionals were involved in the development process through participating in a working group, interviews, and a cognitive validation study. RESULTS AND CONCLUSIONS: Relevant patient-reported outcomes (PROs) were identified for patients with aSAH. The SOS-SAH was developed primarily using domains and items from existing PROMs and, if necessary, by developing new items. The SOS-SAH consists of 40 items and covers 14 domains: cognitive abilities, hypersensitivity to stimuli, anxiety, depression, fatigue, social roles, personality change, language, vision, taste, smell, hearing, headache, and sexual function. It also includes a proxy measurement for use by family members to assess cognitive functioning and personality change.

Wrist Circumference-Dependent Upper Limit of Normal for the Cross-Sectional Area Is Superior Over a Fixed Cut-Off Value in Confirming the Clinical Diagnosis of Carpal Tunnel Syndrome.

Introduction: In confirming the clinical diagnosis of carpal tunnel syndrome (CTS), ultrasonography (US) is the recommended first diagnostic test in The Netherlands. One of the most important parameters for an abnormal US result is an increase of the CSA of the median nerve at the carpal tunnel inlet. An earlier study showed that a wrist-circumference dependent cut-off for the upper limit of normal of this CSA might be superior to a fixed cut-off of 11 mm2. In this study we compared three ultrasonography (US) parameters in three large Dutch hospitals. Methods: Patients with a clinical suspicion of CTS and with reasonable exclusion of other causes of their symptoms were prospectively included. A total number of 175 patients were analysed. The primary goal was to compare the number of wrists with an abnormal US result while using a fixed cut-off of 11 mm2 (FC), a wrist circumference-dependent cut-off (y = 0.88 * x-4, where y = ULN and x = wrist circumference in centimetres; abbreviated as WDC), and an intraneural flow related cut-off (IFC). Results: The WDC considered more US examinations to be abnormal (55.4%) than the FC (50.3%) did, as well as the IFC (46.9%), with a statistically significant difference of p = 0.035 and p = 0.001, respectively. The WDC detected 12 abnormal median nerves while the FC did not, and 18 while the IFC did not. The wrist circumference of the patients of these subgroups turned out to be significantly smaller (p < 0.001) when compared with the rest of the group. Conclusion: According to these study results, the wrist-circumference dependent cut-off value for the CSA of the median nerve at the wrist appears to have a higher sensitivity than either a fixed cut-off value of 11 mm2 or cut-off values based on intraneural flow, and may add most value in patients with a smaller wrist circumference.

Neurovestibular Dysfunction and Falls in Parkinson's Disease and Atypical Parkinsonism: A Prospective 1 Year Follow-Up Study.

Our primary aim was to determine whether neurovestibular laboratory tests can predict future falls in patients with either Parkinson's disease (PD) or atypical parkinsonism (AP). We included 25 healthy subjects, 30 PD patients (median Hoehn and Yahr stage 2.5, range 1-4), and 14 AP patients (6 multiple system atrophy, 3 progressive supranuclear palsy, and 5 vascular parkinsonism) in a case-control study design (all matched for age and gender). At baseline, all subjects underwent clinical neurological and neurotological assessments, cervical and ocular vestibular evoked myogenic potentials (VEMP), brainstem auditory evoked potentials (BAEP), subjective visual vertical measurements (SVV), and video nystagmography with caloric and rotary test stimulation. After 1 year follow-up, all subjects were contacted by telephone for an interview about their fall frequency (based upon fall diaries) and about their balance confidence (according to the ABC-16 questionnaire); only one participant was lost to follow-up (attrition bias of 1.4%). Cervical and ocular VEMPs combined with clinical tests for postural imbalance predicted future fall incidents in both PD and AP groups with a sensitivity of 100%. A positive predictive value of 68% was achieved, if only one VEMP test was abnormal, and of 83% when both VEMP tests were abnormal. The fall frequency at baseline and after 1 year was significantly higher and the balance confidence scale (ABC-16) was significantly lower in both the PD and AP groups compared to healthy controls. Therefore, VEMP testing can predict the risk of future fall incidents in PD and AP patients with postural imbalance.

Augmented Diagnostic Accuracy of Ultrasonography for Diagnosing Carpal Tunnel Syndrome Using an Optimised Wrist Circumference-Dependent Cross-Sectional Area Equation.

Introduction: In diagnosing carpal tunnel syndrome (CTS) there is no consensus about the upper limit of normal (ULN) of the cross-sectional area (CSA) of the median nerve at the carpal tunnel inlet. A previous study showed wrist circumference is the most important independent predictor for the ULN. In this study we optimised a wrist circumference-dependent ULN equation for optimal diagnostic accuracy and compared it to the generally used fixed ULN of 11 mm2. Methods: CSA and wrist circumference were measured in a prospective cohort of 253 patients (clinically defined CTS) and 96 healthy controls. An equation for the ULN for CSA was developed by means of univariable regression analysis. We calculated z-scores for all patients and healthy controls, and analysed these scores in a ROC curve and a decision plot. Sensitivity and specificity were determined and compared to fixed ULN values. Results: We found augmented diagnostic accuracy of our newly developed equation y = 0.88 * x -4.0, where y = the ULN of the CSA and x = wrist circumference. This equation has a corresponding sensitivity and specificity of 75% compared to a sensitivity of 70% while using a fixed cut-off value of 11 mm2 (p = 0.015). Conclusion: Optimising the regression equation for wrist circumference-dependent ULN cross-sectional area of the median nerve at the wrist inlet might improve diagnostic accuracy of ultrasonography in patients with carpal tunnel syndrome and seems to be more accurate than using fixed cut-off values.

The use of WhatsApp Messenger: an important tool in modern neurological examination.

WhatsApp Messenger is one of the most popular and still growing mobile applications worldwide in telemedicine. WhatsApp Messenger can be used as a communication tool and an adjunctive tool within telemedicine, but it is the ability or inability to use WhatsApp Messenger correctly that provides a powerful clinical neurological function tool. The easy use and the application's popularity make it extremely useful in obtaining clues in patients' neurological history. Dystextia or dystypia, the inability to send correct text messages, can be used as an indicator of neurological deficits, and is an upcoming term in neurological vascular pathology. In this case series, we present three cases in which the inability to send text messages were the clue for neurological pathology. This easy and useful evaluation of the ability to use a text message program should be incorporated in standard neurological history taking and examination, as it can provide clues for underlying neurological pathology.

Validation of the Dutch Version of the Boston Carpal Tunnel Questionnaire.

The Boston Carpal Tunnel Questionnaire (BCTQ) is a scale that has been developed specifically for carpal tunnel syndrome (CTS). It consists of the Functional Status Scale (FSS) and the Symptom Severity Scale (SSS). It is the most widely used patient reported outcome measure in CTS and has been validated in many languages. Although already widely used, psychometric properties of the Dutch version of the BCTQ are yet unknown. The aim of this study was to assess the validity, reliability, responsiveness, and acceptability of the Dutch version. Moreover, this paper focuses the longitudinal validity (the use after an intervention) of the BCTQ, which has not been investigated before. A total of 180 patients completed the BCTQ in addition to a six-point Likert scale for perceived improvement, before and about 6-8 months after carpal tunnel release (CTR). Principal factor analysis revealed that the FSS is unidimensional, consisting of a single latent factor ("functionality") and has a high internal consistency (Cronbach's α = 0.825). However, the SSS has three dimensions, which are all highly internally consistent: "daytime symptoms" (Cronbach's α = 0.805), "nighttime symptoms" (Cronbach's α = 0.835), and "operational capacity" (Cronbach's α = 0.723). Post-treatment, the FSS still consisted of one factor, but the SSS changed in dimensionality, as it had only two factors left post-treatment. The ΔFSS and ΔSSS had good correlation with the six-point Likert scale for perceived improvement (r = 0.524; p < 0.01 and r = 0.574; p < 0.01, respectively), a moderate correlation between FSS and pinch grip (r = 0.259; p < 0.01) was found, and a weak correlation between SSS and pinch grip (r = 0.231; p < 0.01) was found. Standard Response Mean for FSS and SSS was 0.76 and 1.49, respectively. Effect size was 0.92 and 1.96, respectively, both indicating a good responsiveness. Response rate was high (82-84%). We concluded that the Dutch version of the BCTQ has a proper reliability, validity, responsiveness, and acceptability to assess the symptom severity and functional disabilities of CTS patients. Because of multidimensionality, we would recommend to create sum scores of the four different dimensions instead of two. Caution is required when interpreting the results postoperatively, due to the insufficient longitudinal validity of the SSS.

Cognitive functioning as a predictor of employment status in relapsing-remitting multiple sclerosis: a 2-year longitudinal study.

BACKGROUND: Cognitive functioning has been linked to employment outcomes in multiple sclerosis (MS) in cross-sectional studies. Longitudinal studies are however lacking and previous studies did not extensively examine executive functioning. OBJECTIVES: We examined whether baseline cognitive functioning predicts a change in employment status after 2 years, while taking into account mood, fatigue and disability level. METHODS: A total of 124 patients with relapsing-remitting MS (pwMS) and 60 healthy controls were included. They underwent neurological and neuropsychological examinations and completed online questionnaires. PwMS were divided into a stable and deteriorated employment status group (SES and DES), based on employment status 2 years after baseline. We first examined baseline differences between the SES and DES groups in cognitive functioning, mood, fatigue and disability level. A logistic regression analysis was performed, with change in employment status (SES/DES) as dependent variable. RESULTS: The DES group included 22% pwMS. Group differences were found in complex attention, executive functioning, self-reported cognitive functioning, fatigue and physical disability. More physical disability (OR = 1.90, p = 0.01) and lower executive functioning (OR = 0.30, p = 0.03) were retained as independent predictors of DES (R2 = 0.22, p ≤ 0.001). CONCLUSIONS: Baseline physical disability and executive functioning, but none of the other variables, moderately predicted a deterioration in employment status 2 years later. TRIAL REGISTRATION: This observational study is registered under NL43098.008.12: 'Voorspellers van arbeidsparticipatie bij mensen met relapsing-remitting Multiple Sclerose'. This study is registered at the Dutch CCMO register (https://www.toetsingonline.nl).

Patient-reported outcome measures in subarachnoid hemorrhage: A systematic review.

OBJECTIVE: Patient-reported outcomes (PROs) are aspects of a patient's health status and are considered important for stimulating patient-centered care. Current outcome measures in clinical care for patients with aneurysmal subarachnoid hemorrhage (aSAH) are insufficient to capture PROs. In this systematic review, we aimed to summarize the evidence regarding the quality of patient-reported outcome measures (PROMs) in aSAH patients. METHODS: We performed a systematic review of the literature published from inception until October 29, 2018, in PubMed, the Cochrane Central Register of Controlled Trials, and EMBASE. Eligible studies had to evaluate measurement properties and capture PROs in aSAH patients. The quality of the studies and measurement properties were assessed using the consensus-based standards for the selection of health status measurement instruments (COSMIN) checklist. The review protocol was registered with PROSPERO (CRD42018058566). RESULTS: We identified 9 articles that reported the assessment of 7 different disease-specific and generic PROMs used for aSAH patients, including 5 that focused on the Stroke-Specific Quality of Life Scale (SS-QoL). The methodologic quality of the validation processes used was generally doubtful. None of the PROMs complied with current standards for content validity. CONCLUSIONS: Due to the low quality of evidence for the measurement properties, the evidence base for selecting a suitable PROM for use with aSAH patients is insufficient. Given the specific long-term consequences of aSAH, we consider a disease-specific PROM the most appropriate, with SS-QoL the most suitable PROM currently available.

Outcome after carpal tunnel release: effects of learning curve.

INTRODUCTION: In carpal tunnel release, it is yet unclear whether a learning curve exists among surgeons. The aim of our study was to investigate if outcome after carpal tunnel release is dependent on surgeon's experience and to get an impression of the learning curve for this procedure. METHODS: A total of 188 CTS patients underwent carpal tunnel release. Patients completed the Boston Carpal Tunnel Questionnaire at baseline and 6-8 months postoperatively together with a six-point scale for perceived improvement. RESULTS: Patients operated by an experienced resident or certified surgeon reported a favorable outcome more often than patients operated by an inexperienced resident (adjusted OR 3.23 and adjusted OR 3.16, respectively). In addition, a negative association was found between surgeon's years of experience and postoperative Symptom Severity Scale and Functional Status Scale scores. DISCUSSION: Outcome after carpal tunnel release seems to be dependent on surgical experience, and there is a learning curve in residents.

The Diagnostic Sensitivity for Ulnar Neuropathy at the Elbow Is Not Increased by Addition of Needle EMG of ADM and FDI When Nerve Conduction Studies Are Normal.

Introduction: The main objective of this study was to investigate whether electromyography (EMG) has additional value in the confirmation of the clinical diagnosis of ulnar nerve entrapment at the elbow (UNE) if nerve conduction studies (NCS) are normal. Methods: A prospective cross-sectional cohort observational study was conducted among patients with the clinical suspicion of UNE. A total of 199 arms were included, who were examined according to a standard neurophysiological protocol, i.e., NCS and EMG relevant to the ulnar nerve. Results: NCS were normal in 76 (38.2%) arms. No abnormal spontaneous muscle fiber activity was found with EMG in any of these cases. In 9 arms with normal NCS (11.8%), isolated abnormal MUAP configurations were found with EMG. Of these nine arms one UNE was diagnosed clinically, in which additional ultrasound and repeated NCS/EMG were negative. One had already been diagnosed with neuralgic amyotrophy and one with CTS. The other 6 arms had additional diagnostics which did not reveal an UNE. Conclusion: EMG as part of the standard neurophysiological protocol exclusively in the confirmation of the clinical diagnosis of UNE has limited added value if NCS are normal in a high prior-odds setting. However, removing EMG may prevent detecting concomitant and/or additional differential diagnoses.

Motor Nerve Conduction Tests in Carpal Tunnel Syndrome.

Background: For the preoperatively often required confirmation of clinically defined carpal tunnel syndrome (CTS), sensory as well as motor nerve conduction studies can be applied. The aim of this study was to test the sensitivity of specific motor nerve conduction tests in comparison with, as well as in addition to, sensory nerve conduction tests. Methods: In 162 patients with clinically defined CTS, sensory and motor nerve conduction tests were performed prospectively. Sensitivity and specificity of all tests were computed. Also, Receiver Operating Characteristic (ROC) analyses were conducted. Results: Sensitivity for all sensory tests was at least 79.4% (DIG1). All tests had a specificity of at least 95.7%. The motor conduction test with the highest sensitivity was the TLI-APB (81.3%); its specificity was 97.9%. Conclusion: In the electrophysiological confirmation of CTS, sensory nerve conduction tests and terminal latency index have a high sensitivity. If, however, sensory nerve action potentials cannot be recorded, all motor nerve conduction tests have a high sensitivity.

The controversy of the normal values of ultrasonography in carpal tunnel syndrome: diagnostic accuracy of wrist-dependent CSA revisited.

INTRODUCTION: No consensus exists about the upper limit of normal (ULN) of the cross-sectional area (CSA) of the median nerve in diagnosing carpal tunnel syndrome (CTS). Previously, we demonstrated a strong positive correlation between wrist circumference and CSA. ULN depending on wrist circumference turned out to have a low sensitivity, which was hypothesized to be caused by an age mismatch. The aim of this study was to re-evaluate the found invariance by augmentation of the healthy control group, adding older subjects, and to determine the diagnostic accuracy of the updated normal values. METHODS: CSA and wrist circumference were measured in an additional 42 healthy controls in the ages of 40-60. Univariable and multivariable linear regression analyses were applied to determine predicting factors for CSA. Diagnostic accuracy was assessed in a prospective cohort of 253 patients. RESULTS: A strong correlation was found between wrist circumference and CSA (r = 0.61). Wrist circumference is the most important independent predictor for ULN (r2 = 0.37). We managed to simplify our newly derived regression equations, which turned out to be unrelated to age. Sensitivity of our new equations is low, but higher than a general fixed cut-off value (53.4% and 47.4%, respectively). DISCUSSION: Wrist circumference is the most important independent predicting factor of CSA. By using our updated equations and taking wrist circumference into account, one can determine a more precise ULN for each individual, which will lead to the improvement of the diagnostic accuracy of ultrasonography (US). Sensitivity for US in diagnosing CTS remains low and it can therefore not replace EDX.

Long-term outcome after carpal tunnel release and identification of prognostic factors.

BACKGROUND: The effectiveness of the surgical treatment of carpal tunnel syndrome (CTS) is well known on short term. However, limited data is available about long-term outcome after carpal tunnel release (CTR). The aims of this study were to explore the long-term outcome after CTR and to identify prognostic factors for long-term outcome. METHODS: Patients with clinically defined CTS underwent CTR and completed the Boston Carpal Tunnel Questionnaire at baseline (T0), at about 8 months (T1), and after a median follow-up of 9 years (T2), as well as a 6-point scale for perceived improvement (at T1 and T2). Potentially prognostic factors were identified by logistic regression analysis and correlation. RESULTS: At long-term follow-up, 87 patients (40.3%) completed the questionnaires. Mean score on Symptom Severity Scale (2.87 to 1.54; p < 0.001) and Functional Status Scale (2.14 to 1.51; p < 0.001) improved at 8 months and did not change significantly after 8 months. A favorable outcome was reported in 81.6%. A good treatment outcome after 8 months and to a lesser extent a lower FSS score at T0 were associated with a better long-term outcome. CONCLUSIONS: CTR is a robust treatment for CTS and its effect persists after a period of 9 years. The most important factor associated with long-term outcome is treatment outcome after about 8 months and to a lesser extent functional complaints preoperatively. Outcome is independent of patient characteristics, electrodiagnostic test results, or findings at the initial neurological examination.

Minimal clinically important difference for the Boston Carpal Tunnel Questionnaire: new insights and review of literature.

No consensus exists about the minimal clinically important difference for the Boston Carpal Tunnel Questionnaire, which hampers its clinical application. This study assessed the minimal clinically important difference of this questionnaire. The Boston Carpal Tunnel Questionnaire was completed by 180 patients, with clinically defined carpal tunnel syndrome, preoperatively and at about 8 months follow-up after carpal tunnel release, together with a six-point scale for perceived improvement. Receiver operator characteristics curves showed that relative changes in Symptom Severity Scale and Functional Status Scale scores correspond better to a clinically relevant improvement than absolute changes. The minimal clinically important difference should be individually calculated from baseline Symptom Severity Scale and Functional Status Scale scores, as patients experiencing more symptoms require more improvement to notice a clinically important difference. By taking this into account, the Boston Carpal Tunnel Questionnaire is more meaningful as an outcome measure in research and clinical practice.

Psychophysical Evaluation of Sensory Reweighting in Bilateral Vestibulopathy.

Perception of spatial orientation is thought to rely on the brain's integration of visual, vestibular, proprioceptive, and somatosensory signals, as well as internal beliefs. When one of these signals breaks down, such as the vestibular signal in bilateral vestibulopathy, patients start compensating by relying more on the remaining cues. How these signals are reweighted in this integration process is difficult to establish, since they cannot be measured in isolation during natural tasks, are inherently noisy, and can be ambiguous or in conflict. Here, we review our recent work, combining experimental psychophysics with a reverse engineering approach, based on Bayesian inference principles, to quantify sensory noise levels and optimal (re)weighting at the individual subject level, in both patients with bilateral vestibular deficits and healthy controls. We show that these patients reweight the remaining sensory information, relying more on visual and other nonvestibular information than healthy controls in the perception of spatial orientation. This quantification approach could improve diagnostics and prognostics of multisensory integration deficits in vestibular patients, and contribute to an evaluation of rehabilitation therapies directed toward specific training programs.

Hearing impairment in patients with myotonic dystrophy type 2.

OBJECTIVE: To systematically assess auditory characteristics of a large cohort of patients with genetically confirmed myotonic dystrophy type 2 (DM2). METHODS: Patients with DM2 were included prospectively in an international cross-sectional study. A structured interview about hearing symptoms was held. Thereafter, standardized otologic examination, pure tone audiometry (PTA; 0.25, 0.5, 1, 2, 4, and 8 kHz), speech audiometry, tympanometry, acoustic middle ear muscle reflexes, and brainstem auditory evoked potentials (BAEP) were performed. The ISO 7029 standard was used to compare the PTA results with established hearing thresholds of the general population according to sex and age. RESULTS: Thirty-one Dutch and 25 French patients with DM2 (61% female) were included with a mean age of 57 years (range 31-78). The median hearing threshold of the DM2 cohort was higher for all measured frequencies, compared to the 50th percentile of normal (p < 0.001). Hearing impairment was mild in 39%, moderate in 21%, and severe in 2% of patients with DM2. The absence of an air-bone gap with PTA, concordant results of speech audiometry with PTA, and normal findings of BAEP suggest that the sensorineural hearing impairment is located in the cochlea. A significant correlation was found between hearing impairment and age, even when corrected for presbycusis. CONCLUSIONS: Cochlear sensorineural hearing impairment is a frequent symptom in patients with DM2, suggesting an early presbycusis. Therefore, we recommend informing about hearing impairment and readily performing audiometry when hearing impairment is suspected in order to propose early hearing rehabilitation with hearing aids when indicated.