Vestibular Dysfunction in Patients with Enlarged Vestibular Aqueduct.

OBJECTIVE: Enlarged vestibular aqueduct (EVA) is the most common inner ear malformation. While a strong correlative relationship between EVA and hearing loss is well established, its association with vestibular dysfunction is less well understood. In this study, we examine the effects of EVA on the vestibular system in patients with EVA. STUDY DESIGN: Prospective, cross-sectional study of a cohort ascertained between 1999 and 2013. SETTING: National Institutes of Health Clinical Center, a federal biomedical research facility. SUBJECTS AND METHODS: In total, 106 patients with unilateral or bilateral EVA, defined as a midpoint diameter greater than 1.5 mm, were referred or self-referred to participate in a study of the clinical and molecular aspects of EVA. Clinical history was ascertained with respect to the presence or absence of various vestibular signs and symptoms and history of head trauma. Videonystagmography (VNG), cervical vestibular evoked myogenic potential (cVEMP), and rotational vestibular testing (RVT) were performed to assess the vestibular function. RESULTS: Of the patients with EVA, 45% had vestibular signs and symptoms, and 44% of tested patients had abnormal VNG test results. An increased number of vestibular signs and symptoms was correlated with the presence of bilateral EVA (P = .008) and a history of head injury (P < .001). Abnormal VNG results also correlated with a history of head injury (P = .018). CONCLUSION: Vestibular dysfunction is common in patients with EVA. However, not all patients with vestibular signs and symptoms have abnormal vestibular test results. Clinicians should be aware of the high prevalence of vestibular dysfunction in patients with EVA.

Achieving valid patient-reported outcomes measurement: a lesson from fatigue in multiple sclerosis.

BACKGROUND: The increasing influence of patient-reported outcome (PRO) measurement instruments indicates their scrutiny has never been more crucial. Above all, PRO instruments should be valid: shown to assess what they purport to assess. OBJECTIVES: To evaluate a widely used fatigue PRO instrument, highlight key issues in understanding PRO instrument validity, demonstrate limitations of those approaches and justify notable changes in the validation process. METHODS: A two-phase evaluation of the 40-item Fatigue Impact scale (FIS): a qualitative evaluation of content and face validity using expert opinion (n=30) and a modified Delphi technique; a quantitative psychometric evaluation of internal and external construct validity of data from 333 people with multiple sclerosis using traditional and modern methods. RESULTS: Qualitative evaluation did not support content or face validity of the FIS. Expert opinion agreed with the subscale placement of 23 items (58%), and classified all 40 items as being non-specific to fatigue impact. Nevertheless, standard quantitative psychometric evaluations implied, largely, FIS subscales were reliable and valid. CONCLUSIONS: Standard quantitative 'psychometric' evaluations of PRO instrument validity can be misleading. Evaluation of existing PRO instruments requires both qualitative and statistical methods. Development of new PRO instruments requires stronger conceptual underpinning, clearer definitions of the substantive variables for measurement and hypothesis-testing experimental designs.

An investigation of factors supporting the psychological health of staff in a UK emergency department.

STUDY OBJECTIVE: Research indicates emergency department doctors experience high levels of stress. Poor psychological health affects staff well-being and patient care, with considerable organisational and financial cost. This study compares levels of psychological health in medical, nursing and administrative staff from a UK emergency department with an orthopaedic comparison department. The study investigates the influence of coping strategies and the support people receive from their colleagues (ie, social support). METHODS: Comparative design, using self-report questionnaires comparing emergency (n=73) and orthopaedic (n=63) staff. Measures included: General Health Questionnaire-12, Hospital Anxiety and Depression Scale, Brief COPE, and questions relating to social identity and social support. RESULTS: The proportion of staff experiencing clinically significant levels of distress was higher than would be expected in the general population. The increased risk of psychological distress previously shown for emergency doctors is not present here for other emergency staff members. Better psychological health was associated with greater use of problem-focused coping and less use of maladaptive coping. Social support was associated with better psychological health and greater use of problem-focused coping. CONCLUSIONS: Priority should be given to developing and evaluating interventions to improve psychological health for this group. Findings suggest that coping strategies and social support are important factors to incorporate into such interventions.

Dysgraphia in dementia: a systematic investigation of graphemic buffer features in a case series.

In this paper we report findings from a systematic investigation of spelling performance in three patients - PR, RH and AC - who despite their different medical diagnoses showed a very consistent pattern of dysgraphia, more typical of graphemic buffer disorder. Systematic investigation of the features characteristic of this disorder in Study 1 confirmed the presence of length effects in spelling, classic errors (i.e., letter substitution, omission, addition, transposition), a bow-shaped curve in the serial position of errors and consistency in substitution of consonants and vowels. However, in addition to this clear pattern of graphemic buffer impairment, evidence of regularity effects and phonologically plausible errors in spelling raised questions about the integrity of the lexical spelling route in each case. A second study was conducted, using a word and non-word immediate delay copy task, in an attempt to minimise the influence of orthographic representations on written output. Persistence of graphemic buffer errors would suggest an additional, independent source of damage. Two patients, PR and AC, took part and in both cases symptoms of graphemic buffer disorder persisted. Together, these findings suggest that damage to the graphemic buffer may be more common than currently suggested in the literature.

Does maintenance of colour categories rely on language? Evidence to the contrary from a case of semantic dementia.

Recent neuropsychological evidence, supporting a strong version of Whorfian principles of linguistic relativity, has reinvigorated debate about the role of language in colour categorisation. This paper questions the methodology used in this research and uses a novel approach to examine the unique contribution of language to categorisation behaviour. Results of three investigations are reported. The first required development of objective measures of category coherence and consistency to clarify questions about healthy control performance on the freesorting colour categorisation task used in previous studies. Between-participant consistency was found to be only moderate and the number of colour categories generated was found to vary markedly between individuals. The second study involved longitudinal neuropsychological examination of a patient whose colour categorisation strategy was monitored in the context of a progressive decline in language due to semantic dementia. Performance on measures of category coherence and consistency was found to be relatively stable over time despite a profound decline in the patient's colour language. In a final investigation we demonstrated that, for both the patient and controls, between- and within-participant consistency were higher than expected by (a) random sorting and (b) sorting perceptually similar chips together. These findings indicate that the maintenance of colour categorisation need not depend on language.

Measuring outcomes in cervical spine surgery: think twice before using the SF-36.

STUDY DESIGN: Psychometric evaluation of a patient-reported scale for measuring health status. OBJECTIVE: To evaluate the ability of the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36) to satisfy the clinical and research needs of cervical spine surgeons. SUMMARY OF BACKGROUND DATA: Although the SF-36 has been shown to be a reliable measure in the general population, the fundamental assumptions underpinning the generation of scale and summary scores must be rigorously tested before it can be considered a suitable measure for use in specific populations. METHODS: Data from 147 patients undergoing cervical spine surgery were examined. We examined the assumptions underpinning the generation of SF-36 scale and summary measures, targeting to the sample, and the scale's ability to detect change. RESULTS.: Evidence supports the reporting of SF-36 scale scores, but not SF-36 summary measures. Three of the 8 scales had notable floor/ceiling effects indicating poor targeting. Responsiveness was good for all scales except those with high floor/ceiling effects. CONCLUSIONS: The SF-36 fails to satisfy the measurement needs of cervical spine surgeons. Scale scores are valid, but floor and ceiling effects mean that changes in quality of life associated with surgery are underestimated. SF-36 summary scores are not valid. Neurosurgeons need better and more sophisticated scales to measure their outcomes.

Evidence for the involvement of a nonlexical route in the repetition of familiar words: A comparison of single and dual route models of auditory repetition.

In this paper, we attempt to simulate the picture naming and auditory repetition performance of two patients reported by Hanley, Kay, and Edwards (2002), who were matched for picture naming score but who differed significantly in their ability to repeat familiar words. In Experiment 1, we demonstrate that the model of naming and repetition put forward by Foygel and Dell (2000) is better able to accommodate this pattern of performance than the model put forward by Dell, Schwartz, Martin, Saffran, and Gagnon (1997). Nevertheless, Foygel and Dell's model underpredicted the repetition performance of both patients. In Experiment 2, we attempt to simulate their performance using a new dual route model of repetition in which Foygel and Dell's model is augmented by an additional nonlexical repetition pathway. The new model provided a more accurate fit to the real-word repetition performance of both patients. It is argued that the results provide support for dual route models of auditory repetition.