Sudden hearing loss as an early detector of multiple sclerosis: a systematic review.

OBJECTIVE: To evaluate whether Sudden Sensorineural Hearing Loss (S-SNHL) may be an early symptom of Multiple Sclerosis (MS). MATERIALS AND METHODS: A systematic review was conducted using the following keywords: "Multiple sclerosis, hearing loss, sudden hearing loss, vertigo, tinnitus, magnetic resonance imaging, otoacoustic emission, auditory brainstem responses, white matter lesions, sensorineural hearing loss, symptoms of MS and otolaryngology, nerve disease and MS". Only the articles that included results of at least one auditory test and MRI were considered. We evaluated the prevalence of SNHL in patients with MS, the presence of different forms of SNHL (S-SNHL and Progressive SNHL (P-SNHL)) and their correlation with the stage of MS, the results of electrophysiological tests, and the location (if any) of MS lesions as detected by white matter hyperintensities in the MRI. RESULTS: We reviewed a total of 47 articles, which included 29 case reports, 6 prospective studies, 6 cohort studies, 4 case-control studies, and 2 retrospective studies. 25% of patients suffered from SNHL. S-SNHL typically occurred in the early stage of the disease (92% of patients) and was the only presenting symptom in 43% of female subjects. Instead, P-SNHL occurred in the late stage of MS (88% of patients). Auditory Brainstem Responses (ABR) were abnormal in all MS patients with S-SNHL. When S-SNHL appeared during the early stage of the disease, MS lesions were found in the brain in 60% of patients and in the Internal Auditory Canal in 40% of patients. ABR remained abnormal after recovery. CONCLUSIONS: S-SNHL can be an early manifestation of MS and should always be considered in the differential diagnosis of this condition, especially in women. The pathophysiology can be explained by the involvement of microglia attacking the central and/or peripheral auditory pathways as indicated by WMHs.

The efficacy of telerehabilitation in dysphagic patients: a systematic review.

SUMMARY: Telerehabilitation is the use of telecommunications technology for rehabilitation. Recently, some studies have shown positive effects of telerehabilitation of swallowing disorders, yet there are no systematic reviews verifying the evidence. The aim of this review is to assess the effects of telerehabilitation in the field of dysphagia as an alternative to face-to-face patient care, considering swallowing recovery and/or quality of life in different patient populations. We searched the Cochrane Library, MEDLINE, EMBASE, Google Scholar, Google Search and the grey literature from inception until December 2016 for publications written in English (keywords: telerehabilitation, telemedicine, dysphagia, swallowing disorders), which resulted in 330 records. Abstract screening and data extraction was carried out independently by two reviewers. Four papers were selected to read in full, and the methodological quality of the studies included was evaluated using Cochrane Collaboration's tool for assessing risk of bias. One study met our inclusion criteria (Wall et al. 2016), which showed that telerehabilitation improves adherence to treatment compared to patient-directed intervention. Although adherence is an important factor that influences the treatment outcome, clinical outcomes have to be examined in randomised controlled trials in order to reach evidence in this field. Lastly, this systematic review did not demonstrate the efficacy of telerehabilitation compared with face-to face therapy.

Re-assessing acalculia: Distinguishing spatial and purely arithmetical deficits in right-hemisphere damaged patients.

Arithmetical deficits in right-hemisphere damaged patients have been traditionally considered secondary to visuo-spatial impairments, although the exact relationship between the two deficits has rarely been assessed. The present study implemented a voxelwise lesion analysis among 30 right-hemisphere damaged patients and a controlled, matched-sample, cross-sectional analysis with 35 cognitively normal controls regressing three composite cognitive measures on standardized numerical measures. The results showed that patients and controls significantly differ in Number comprehension, Transcoding, and Written operations, particularly subtractions and multiplications. The percentage of patients performing below the cutoffs ranged between 27% and 47% across these tasks. Spatial errors were associated with extensive lesions in fronto-temporo-parietal regions -which frequently lead to neglect- whereas pure arithmetical errors appeared related to more confined lesions in the right angular gyrus and its proximity. Stepwise regression models consistently revealed that spatial errors were primarily predicted by composite measures of visuo-spatial attention/neglect and representational abilities. Conversely, specific errors of arithmetic nature linked to representational abilities only. Crucially, the proportion of arithmetical errors (ranging from 65% to 100% across tasks) was higher than that of spatial ones. These findings thus suggest that unilateral right hemisphere lesions can directly affect core numerical/arithmetical processes, and that right-hemisphere acalculia is not only ascribable to visuo-spatial deficits as traditionally thought.

Comparison of the cough reflex test and water swallowing test in healthy participants and neurological patients.

Comparison of the cough reflex test and water swallowing test in healthy participants and neurological patients. BACKGROUND: Silent aspiration is poorly identified by traditional clinical swallowing evaluations. Recently, sevral studies have proposed the use of a cough reflex test (CRT) for screening patients at risk of aspirations. The first aithis study is to investigate the CRT thresholds of citric acid concentration for identifying cough responses in healthy participants and neurological patients. The second aim is to compare the results of the CRT with the water swallowing test (WST), a standard screening test for identifying cough responses in neurological patients. METHODS: The CRT and then the WST were administered to 100 neurological patients and 100 healthy participants. For the CRT, we administered incremental solutions of citric acid interspersed with placebo doses. We used the results of the CRT in healthy participants to define a citric acid concentration cut-off, which could be used with neurological patients as a screening for aspirations. RESULTS: As all controls coughed at a concentration of 0.1 mol/L, this was used as a cut-off in patients to identify coughing as a screening for aspiration risk. Patients showed cough reflexes at concentrations significantly higher than controls (p=0 .001). The WST was not administered to 17 patients, due to cognitive deficits and severe clinical conditions. Thirty six patients had a cough response above the screening cut-off (> 0.1 mol/L), 25 of which (30.1%) also had a positive cough response during the WST. CONCLUSION: The CRT correlated significantly with the WST. Unlike the WST, the CRT could be easily administered to severely impaired patients. Our results indicate the use of the CRT as a screening test for silent aspirators.

Masked myoclonus in corticobasal degeneration: neurophysiological study of a case.

A single case study of a 58 year-old male with right asymmetric apraxia and akinetic-rigid syndrome is described. Brainimaging scans (MRI, SPECT) indicated asymmetric cortical atrophy compatible with the diagnosis of Corticobasal Degeneration. Reflex myoclonus was absent and myoclonic discharges only appeared in response to pharmacological treatment of limb dystonia and rigidity. Electromyographic evidence of jerky movements was recorded only in the affected right hand and forearm after muscle relaxation, and myoclonus was not preceded by an EEG paroxysm. The cortical components of the correspondent SEPs were not increased in amplitude while LLRs recordings showed a late response over the muscles of the affected side. Furthermore, the duration of post MEP silent period was bilaterally reduced. This single case study report points out that sometimes myoclonus in Corticobasal Degeneration can be masked by the presence of increased muscle tone.

Deficit of auditory space perception in patients with visuospatial neglect.

There have been many studies of visuospatial neglect, but fewer studies of neglect in relation with other sensory modalities. In the present study we investigate the performance of six right brain damaged (RBD) patients with left visual neglect and six RBD patients without neglect in an auditory spatial task. Previous work on sound localisation in neglect patients adopted measure of sound localisation based on directional motor responses (e.g., pointing to sounds) or judgement of sound position with respect to the body midline (auditory midline task). However, these measures might be influenced by non-auditory biases related with motor and egocentric components. Here we adopted a perceptual measure of sound localisation, consisting in a verbal judgement of the relative position (same or different) of two sequentially presented sounds. This task was performed in a visual and in a blindfolded condition. The results revealed that sound localisation performance of visuospatial neglect patients was severely impaired with respect to that of RBD controls, especially when sounds originated in contralesional hemispace. In such condition, neglect patients were always unable to discriminate the relative position of the two sounds. No difference in performance emerged as a function of the visual condition in either group. These results demonstrate a perceptual deficit of sound localisation in patients with visuospatial neglect, suggesting that the spatial deficits of these patients can arise multimodally for the same portion of external space.

Left tactile extinction following visual stimulation of a rubber hand.

In close analogy with neurophysiological findings in monkeys, neuropsychological studies have shown that the human brain constructs visual maps of space surrounding different body parts. In right-brain-damaged patients with tactile extinction, the existence of a visual peripersonal space centred on the hand has been demonstrated by showing that cross-modal visual-tactile extinction is segregated mainly in the space near the hand. That is, tactile stimuli on the contralesional hand are extinguished more consistently by visual stimuli presented near the ipsilesional hand than those presented far from it. Here, we report the first evidence in humans that this hand-centred visual peripersonal space can be coded in relation to a seen rubber replica of the hand, as if it were a real hand. In patients with left tactile extinction, a visual stimulus presented near a seen right rubber hand induced strong cross-modal visual-tactile extinction, similar to that obtained by presenting the same visual stimulus near the patient's right hand. Critically, this specific cross-modal effect was evident when subjects saw the rubber hand as having a plausible posture relative to their own body (i.e. when it was aligned with the subject's right shoulder). In contrast, cross-modal extinction was strongly reduced when the seen rubber hand was arranged in an implausible posture (i. e. misaligned with respect to the subject's right shoulder). We suggest that this phenomenon is due to the dominance of vision over proprioception: the system coding peripersonal space can be 'deceived' by the vision of a fake hand, provided that its appearance looks plausible with respect to the subject's body.