Publications by authors named "Rachelle C Dar Santos"

Objectives: The management options for otologic symptoms (i.e., hearing loss, otorrhea) in patients with fibrous dysplasia of the temporal bone (FDTB) include either observation or otologic surgery.

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Importance: Sinonasal disease and, specifically, nasal polyps, occur frequently in children with cystic fibrosis (CF). As survival rates have improved, it has become imperative that otolaryngologists become involved in the care of patients with CF to provide appropriate medical and surgical interventions for sinonasal disease. Despite significant variability in the subjective reporting of clinical symptoms, previous work has suggested there may be a relationship between clinical indicators and sinonasal disease in this population.

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Objective: To systematically review the literature describing the relationship between autism spectrum disorder (ASD) and peripheral hearing loss including literature recommendations for audiological assessment and auditory habilitation in cases where peripheral hearing loss and ASD coexist.

Data Sources: Published studies indexed in MEDLINE (1948-2011).

Review Methods: The search strategy identified 595 potential studies.

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Background: The Mucosal Atomization Device (MAD) distributes medication throughout the paranasal sinuses for patients with chronic rhinosinusitis (CRS). Determining the optimal head position is important to ensure maximal delivery of medication to the sinus cavities. The objective of this work was to determine the effect of the lying-head-back (LHB) and head-down and forward (HDF) position, on the distribution of topical nasal medication via MAD in cadaver specimens.

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Objective: To systematically review the literature and appraise the evidence reporting the effects of women's health, including pregnancy, postpartum, menstruation, oral contraception, menopause, and hormone replacement therapy, on common rhinological pathologies and nasal physiology.

Data Sources: Systematic search strategy using MEDLINE (1966-2012) and EMBASE (1980-2012) databases.

Review Methods: Title review, abstract screening, and then full paper analysis were undertaken by 2 authors independently.

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Facial emotion recognition impairments have been reported in Huntington's disease (HD). However, the nature of the impairments across the spectrum of HD remains unclear. We report on emotion recognition data from 344 participants comprising premanifest HD (PreHD) and early HD patients, and controls.

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Working memory deficits have been found in Huntington's disease (HD) and in a small group of premanifest (PreHD) gene-carriers. However, the nature and extent of these deficits are unknown. In a large cross-sectional study, we aimed to determine the degree of visuospatial working memory dysfunction across multiple stages of HD.

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Objectives: Visuomotor integration deficits have been documented in Huntington disease (HD), with disproportionately more impairment when direct visual feedback is unavailable. Visuomotor integration under direct and indirect visual feedback conditions has not been investigated in the stage before clinical onset ('premanifest'). However, given evidence of posterior cortical atrophy in premanifest HD, we predicted visuomotor integration would be adversely affected, with greater impairment under conditions of indirect visual feedback.

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