Fibrous dysplasia of the temporal bone: systematic review of management and hearing outcomes.

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. The objective of this review is to describe the hearing outcomes in patients with FDTB to compare surgical intervention and conservative management to determine an evidence-based approach to patient management. DATA SOURCES: Cochrane Central Register of Controlled Trials (1995-April 5, 2013), MEDLINE (January 1948-April 5, 2013), EMBASE (January 1974-April 5, 2013), conference proceedings, and the reference lists of articles. STUDY SELECTION: Studies describing interventions or no intervention in patients with FDTB were included. Studies failing to report hearing outcomes were excluded. DATA EXTRACTION: Two authors independently reviewed titles and abstracts, read full-text articles, assessed quality, and extracted data. The Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines were followed. DATA SYNTHESIS: Seventy-two studies involving 215 patients were included. Thirty-one studies (n = 49 patients), including three case series and 28 case reports, had sufficient information for analysis of hearing outcomes (Level 4 evidence). The surgical intervention showing the best hearing outcomes was canaloplasty, with improvement in 84% of patients (n = 16 patients) and stable hearing thresholds in 18% (n = 3 patients). CONCLUSION: The literature lacks consistent reporting of hearing outcomes after otologic surgery in FDTB. Level 4 evidence suggests that an improvement in hearing thresholds is achievable in most patients when the disease involves the external ear canal. A management algorithm is proposed using the available evidence.

Autism and peripheral hearing loss: a systematic review.

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. After a review of the titles, 115 abstracts were reviewed and 39 articles were retrieved and assessed independently by at least two authors for possible inclusion. 22 articles pertained to children with ASD and peripheral hearing loss, hearing assessment in children with ASD, audiological habilitation for children with ASD or hyper-responsiveness in children with ASD. 17 further studies were garnered from the reference section of the 22 papers. RESULTS: Controversy exists in the literature regarding prevalence of hearing impairment among individuals with ASD. In cases where ASD and hearing impairment co-exist, diagnosis of one condition often leads to a delay in diagnosing the other. Audiological assessment can be difficult in children with ASD and test-retest reliability of behavioural thresholds can be poor. In cases where hearing impairment exists and hearing aids or cochlear implantation are recommended, devices are often fit with special considerations for the child with ASD. Hyper-responsiveness to auditory stimuli may be displayed by individuals with ASD. Evidence or the suspicion of hyper-responsiveness may be taken into consideration when fitting amplification and planning behavioural intervention. CONCLUSIONS: Prevalence rates of hearing impairment among individuals with ASD continue to be debated. At present there is no conclusive evidence that children with ASD are at increased risk of peripheral hearing loss. A complete audiological assessment is recommended in all cases where ASD is suspected so as not to delay the diagnosis of hearing impairment in the event that hearing loss and ASD co-exist. Objective assessment measures should be used to confirm behavioural testing in order to ensure reliability of audiological test results. Fitting of hearing aids or cochlear implantation are not contraindicated when hearing loss is present in children with ASD; however, success with these devices can be variable.

The effect of head position on the distribution of topical nasal medication using the Mucosal Atomization Device: a cadaver study.

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. METHODS: Twenty specimens having received complete functional endoscopic sinus dissection were chosen. The MAD was used to administer 2 mL of fluorescein-impregnated saline solution through the nose in both the LHB and HDF positions. Fluorescein was identified on 11 predetermined anatomical areas using a blue light filter. Three blinded investigators assessed endoscopic images to determine the presence of fluorescein. RESULTS: A total of 440 anatomical locations (n = 20 cadavers) received administration of the fluorescein nasal spray in the LHB or HDF position. LHB position had significantly greater total distribution to all pertinent anatomical sites than the HDF position (76% vs 41%; p < 0.001; 95% confidence interval [CI], 0.26-0.44). The proportion of staining was significantly greater for the ethmoid (p = 0.11; 95% CI, 0.05-0.66), frontal (p < 0.01; 95% CI, 0.20-0.80), and sphenoid sinuses (p = 0.03; 95% CI, 0.07-0.73) when compared to the HDF position. CONCLUSION: A greater distribution of medication to the sinonasal cavities was observed in the LHB position compared to the HDF position. These areas are of particular clinical relevance in postsurgical patients with refractory CRS.

The rhinological manifestations of women's health.

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. Level of evidence was graded according to the Oxford Centre of Evidence Based Medicine 2011 criteria and risk of bias assessment using the Jadad scale for randomized controlled trials and Newcastle-Ottawa Scale for cohort and case-controlled studies. RESULTS: Over the 46 years analyzed, the search strategy produced 2904 titles. In total, 314 abstracts were screened, from which 192 full-text articles were evaluated, and 145 research papers met all the criteria for inclusion in the study. Overall, the available evidence was of low quality. Seventy percent of studies (102 of 145) were case reports or case series from which only limited conclusions can be drawn. Only 3% of the included papers (4 of 145) were randomized controlled studies. The remaining data were mainly of a prospective cohort design. Study heterogeneity in design and measured outcomes resulted in data synthesis being limited to a descriptive/exploratory review. Study findings are presented by women's health category and then by rhinological manifestation with important clinical correlations highlighted. CONCLUSION: Physiological and hormonal changes occurring as a normal part of women's health have an important influence on rhinological function and disease.

Emotional face recognition deficits and medication effects in pre-manifest through stage-II Huntington's disease.

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. In a test of recognition of facial emotions, we examined responses to six basic emotional expressions and neutral expressions. In addition, and within the early HD sample, we tested for differences on emotion recognition performance between those 'on' vs. 'off' neuroleptic or selective serotonin reuptake inhibitor (SSRI) medications. The PreHD groups showed significant (p<0.05) impaired recognition, compared to controls, on fearful, angry and surprised faces; whereas the early HD groups were significantly impaired across all emotions including neutral expressions. In early HD, neuroleptic use was associated with worse facial emotion recognition, whereas SSRI use was associated with better facial emotion recognition. The findings suggest that emotion recognition impairments exist across the HD spectrum, but are relatively more widespread in manifest HD than in the premanifest period. Commonly prescribed medications to treat HD-related symptoms also appear to affect emotion recognition. These findings have important implications for interpersonal communication and medication usage in HD.

Visual Working Memory Impairment in Premanifest Gene-Carriers and Early Huntington's Disease.

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. Specifically, visuospatial working memory capacity and response times across various degrees of difficulty were examined, as well as the relationship between visuospatial working memory and motor dysfunction. We examined 62 PreHD-A gene-carriers (>10.8 years from estimated disease onset), 58 PreHD-B gene-carriers (<10.8 years from estimated disease onset), 77 stage-1 HD patients (HD1), 44 stage-2 HD patients (HD2), and 122 healthy controls. Participants viewed coloured squares (in sets of 3, 5 and 7) on a screen and were to decide whether on a subsequent screen the encircled square has changed colour. Accuracy and response times were recorded. Compared to controls, significant group differences in visuospatial working memory capacity (accuracy) were seen in PreHD-B, HD1 and HD2 groups across the difficulty levels. Significant group differences on response times were found for all groups (PreHD-A to HD2) compared to controls; the most difficult level producing the only group difference in speed between PreHD-A and controls. Accuracy and speed were positively correlated only in the HD groups. These findings suggest that visuospatial working memory impairments are detectable in both premanifest and manifest HD; the manifest HD showed evidence for a "worse-worse phenomenon" whereby reductions were present in both motor speed and accuracy.