Elaine N. Rubinstein

Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients.

The management of chordomas and chondrosarcomas involving the cranial base remains controversial. The options for therapy include biopsy, partial resection, radical resection, and various forms of radiotherapy. In this article, we analyze the outcome of 60 patients with cranial base chordoma or chondrosarcoma treated with extensive surgical resection between 1984 and 1993. Forty-six patients had chordomas, and 14 had low-grade chondrosarcomas; 50% of these patients had been treated previously. Preoperative studies included computed tomography, magnetic resonance imaging, cerebral angiography, and balloon occlusion test of the internal carotid artery, as indicated. Magnetic resonance imaging was performed on all patients during follow-up. The surgical approaches used for tumor resection were predominantly the following: subtemporal, transzygomatic, transcavernous, and transpetrous apex; subtemporal and infratemporal; extended frontal; and extreme lateral transcondylar. Staged operations with a combination of approaches were used when necessary (52% of cases) to remove a tumor more completely. Statistical analysis was done by the chi 2 test and correlation matrix. Sixty-seven percent of the patients had total or near-total resection. Twenty percent of the patients received postoperative radiotherapy. Eleven patients died during the postoperative follow-up period, nine with chordomas and two with chondrosarcomas. Three patients died because of systemic complications within 3 months after surgery, five died because of tumor recurrence, one died from unrelated causes, and two died from late complications of radiotherapy. The recurrence-free survival rate for all tumors was 80% at 3 years and 76% at 5 years. Chondrosarcomas had a better prognosis than chordomas (recurrence-free survival rates, 90% at 5 years and 65% at 5 years, respectively; P = 0.09). Patients who had undergone previous surgery had a greater risk of recurrence (5-year recurrence-free survival rate, 64%) than did patients who had not undergone previous surgery (5-year recurrence-free survival rate, 93%; P < 0.05). Patients with total or near-total resection had a better 5-year recurrence-free survival rate (84%) than did patients with partial or subtotal resection (64%) (P < 0.05). Postoperative leakage of cerebrospinal fluid was the most frequent complication (30% of patients) and was found to increase the risk of permanent disability. Patients who had undergone previous radiotherapy had a greater risk of death in the postoperative period (within 3 months of their operations) and during follow-up. However, total or near-total resection did not increase the rate of postoperative disability.(ABSTRACT TRUNCATED AT 400 WORDS)

Summarizing Monte Carlo Results in Methodological Research: The One- and Two-Factor Fixed Effects ANOVA Cases

Meta-analytic methods were used to integrate the findings of a sample of Monte Carlo studies of the robustness of the F test in the one- and two-factor fixed effects ANOVA models. Monte Carlo results for theWelch (1947) and Kruskal-Wallis (Kruskal & Wallis, 1952) tests were also analyzed. The meta-analytic results provided strong support for the robustness of the Type I error rate of the F test when certain assumptions were violated. The F test also showed excellent power properties. However, the Type I error rate of the F test was sensitive to unequal variances, even when sample sizes were equal. The error rate of the Welch test was insensitive to unequal variances when the population distribution was normal, but nonnormal distributions tended to inflate its error rate and to depress its power. Meta-analytic and exact statistical theory results were used to summarize the effects of assumption violations for the tests.

A Patient-Specific in silico Model of Inflammation and Healing Tested in Acute Vocal Fold Injury

The development of personalized medicine is a primary objective of the medical community and increasingly also of funding and registration agencies. Modeling is generally perceived as a key enabling tool to target this goal. Agent-Based Models (ABMs) have previously been used to simulate inflammation at various scales up to the whole-organism level. We extended this approach to the case of a novel, patient-specific ABM that we generated for vocal fold inflammation, with the ultimate goal of identifying individually optimized treatments. ABM simulations reproduced trajectories of inflammatory mediators in laryngeal secretions of individuals subjected to experimental phonotrauma up to 4 hrs post-injury, and predicted the levels of inflammatory mediators 24 hrs post-injury. Subject-specific simulations also predicted different outcomes from behavioral treatment regimens to which subjects had not been exposed. We propose that this translational application of computational modeling could be used to design patient-specific therapies for the larynx, and will serve as a paradigm for future extension to other clinical domains.

Meta-analysis of variables that affect accuracy of threshold estimation via measurement of the auditory steady-state response (ASSR)

Reported are the results of meta-analyses of data derived collectively from a sample of 56 published research studies on electric response audiometry (ERA) using auditory steady-state responses (ASSRs). Several specific methodological issues were examined and hypotheses were posited to rigorously test common conclusions drawn from the ASSR literature on the accuracy of ASSR-ERA. Explanatory variables for analyses were type of population (normally hearing and hearing-impaired), type of modulation, number of sweeps acquired during response analysis, electrode montage, and modulation rate (80 vs. 40 Hz). No explanatory variables were found to be significantly related to the degree of disparity between thresholds obtained by ASSR-ERA versus behavioral audiometry in the normally hearing population. Conversely, all but one explanatory variable (i.e. electrode montage) was found to be significantly related to mean threshold differences in the hearing-impaired and combined populations. Results both substantiate some of common conclusions drawn from the literature but call others into question, helping to identify those methodological issues which appear to, or not to, significantly affect the accuracy of estimating threshold using ASSR measurement. In addition to these findings, another practical outcome of this study was the development of various summary tables of the data analysed from the literature sampled.

The Comparative Effectiveness of Combined Lumbrical Muscle Splints and Stretches on Symptoms and Function in Carpal Tunnel Syndrome

OBJECTIVE To compare the effectiveness of an intensive lumbrical splint/stretch combination with 3 less intensive lumbrical splint/stretch combinations on carpal tunnel symptoms and function. DESIGN Randomized Clinical Trial. SETTING Outpatient hand therapy clinics. PARTICIPANTS Volunteers (N=124) with mild to moderate carpal tunnel syndrome. INTERVENTIONS A 4-week home regimen of nocturnal splints (lumbrical splints or cock-up splints) combined with stretches (lumbrical intensive or general) performed 6 times daily. MAIN OUTCOME MEASURES The effect of the intervention on carpal tunnel symptoms and function was examined with the Carpal Tunnel Symptom Severity and Function Questionnaire (CTQ) and Disabilities of the Arm, Shoulder, and Hand (DASH). We also evaluated whether subjects obtained surgery at 24 weeks. RESULTS There were significant main effects over time for all outcome measures at 4, 12, and 24 weeks. There was a significant interaction effect for the CTQ-Function and DASH at 12 weeks. Post hoc analyses indicated significant differences between the lumbrical splint/general stretch and general splint/lumbrical stretch groups and the other 2 groups. At 24 weeks, a significantly greater percentage of subjects in the general splint/lumbrical stretch group achieved a clinically important improvement on the CTQ-Function. By 24 weeks, only 25.5% of subjects had elected to undergo surgery. CONCLUSIONS A combination of a cock-up splint with lumbrical intensive stretches was the most effective combination for improvements in functional gains at 24 weeks postbaseline. Our findings support further evaluation of this combination as a method of conservative carpal tunnel syndrome treatment.

A Study of the Effect of Nasal Steroid Sprays in Perennial Allergic Rhinitis Patients with Rhinitis Medicamentosa

OBJECTIVE: To determine if rebound congestion can be reduced with concomitant nasal steroid spray usage. STUDY DESIGN AND SETTING: Randomized, double blind, controlled single center study. PARTICIPANTS: Twenty subjects with perennial allergic rhinitis with nasal congestion. INTERVENTION: All subjects received 3 weeks of twice-daily oxymetazoline. After 2 weeks, subjects were randomized to 2 additional weeks of concomitant budesonide aqueous nasal spray (n = 9) or placebo (n = 10). In the sixth week, all sprays were stopped. RESULTS: Both groups showed subjective and objective evidence of rebound congestion 24 hours after cessation of oxymetazoline (P < 0.05). Subjective rebound congestion resolved in 48 hours in the budesonide aqueous nasal spray group but persisted for over 1 week in the placebo group. CONCLUSION: Rebound congestion is objectively present in patients with perennial allergic rhinitis after 3 weeks of oxymetazoline spray. Rebound congestion is reduced by concomitant budesonide aqueous nasal spray use. SIGNIFICANCE: This study supports the common clinical practice of nasal steroid sprays to ameliorate rebound congestion concomitant with and after cessation of topical decongestant sprays.

Cognitive and physical performance in patients with asymptomatic carotid artery disease

The purpose of this study was to determine if patients with asymptomatic carotid artery stenosis and occlusion demonstrate deficits in cognitive and physical performance. The relationship between cognitive measures and performance of instrumental activities of daily living was examined. Seventy-nine patients with asymptomatic carotid artery stenosis of moderate and severe degrees or occlusion were tested. Cognition was assessed via the repeatable battery for the assessment of neuropsychological status (RBANS) and the executive interview (EXIT). Physical performance was assessed via the physical performance test (PPT) and the Lawton instrumental activities of daily living (IADL). Deficits in the RBANS visuospatial/constructional, attention, language and delayed memory domains were found for patients with occlusion. Deficits in all RBANS domains were found for the moderate stenosis subgroup, and deficits in all domains except language were found in the severe subgroup. No deficit was found in executive function in any group. Additionally, deficits were related to sidedness of involvement with visuospatial/constructional deficits related to right sided disease, and deficits in all cognitive domains except language were found in left sided and bilateral disease. Decreased performance on the PPT was identified in all three subgroups with the lowest scores in the moderate stenosis subgroup and the highest scores in the severe stenosis subgroup. The Lawton IADL did not identify any decrease in performance. Deficits in cognitive and physical function were found in this observational study of patients with asymptomatic carotid artery stenosis and occlusion, indicating that asymptomatic patients may not be truly asymptomatic. These areas of function and the potential change in their status need to be considered when patients are being evaluated for interventions to manage their carotid artery disease.

Reliability of electric response audiometry using 80 Hz auditory steady-state responses

The reliability of the Auditory Steady State Response (ASSR) has not been thoroughly evaluated despite its recent application as a clinical tool for threshold estimation. The purpose of this study was to examine test-retest (TR) reliability of ASSR threshold estimates in an empirical research design. The ASSR, tested using modulation frequencies approximately 80 Hz and above, was evaluated against pure tone audiometry (PTA), and the slow vertex potential (SVP, N1-P2). Sixteen normal-hearing young female adults were tested twice, one week apart. Varying degrees of sensorineural hearing loss of a notched configuration were simulated with filtered masking noise. Test-retest reliability was assessed using Pearson-product moment correlation analysis, supplemented by other post-hoc analyses. Results demonstrated moderately strong TR reliability for ASSR at 1000, 2000 and 4000 Hz (r = 0.83–0.93); however, the reliability of ASSR at 500 Hz was weaker (r = 0.75). Results suggest that ASSR-ERA is a reliable test at mid–high frequencies, at least with the configuration and degrees of simulated sensorineural hearing loss examined in this study.

Efficacy of Sinonasal Simulator in Teaching Endoscopic Nasal Skills

Objectives/Hypothesis: To develop a nasal model (NM) which accurately simulates human texture and anatomy and to study the effect of training with NM on performance of video rigid nasal endoscopy and video flexible laryngoscopy. At the conclusion of this presentation, the participants should be able to demonstrate that training with nasal endoscopic simulation enhances efficiency and may improve comfort to the patient.

Comparison of Pediatric Voice Outcome Survey, Reflux Symptom Index, Reflux Finding Score, and Esophageal Biopsy Results

OBJECTIVE To examine correlations between the Pediatric Voice Outcome Survey (PVOS) score, the Reflux Symptom Index (RSI) score, the Reflux Finding Score (RFS), and esophageal biopsy findings in children undergoing upper aerodigestive tract endoscopy. DESIGN Retrospective review of pediatric voice quality-of-life and laryngopharyngeal reflux surveys. Blinded assessment of endoscopic laryngeal images. SETTING Tertiary care childrens hospital. PATIENTS The study included 36 children with the primary problem of dysphonia (n = 28) or cough (n = 8) who underwent endoscopy. INTERVENTIONS The PVOS and the RSI were administered to the patients parents before surgery. The patients underwent laryngotracheobronchoscopy and esophageal biopsy. Four raters independently assigned an RFS to the laryngeal photographs. MAIN OUTCOME MEASURES The assessment included (1) PVOS scores, RSI scores, and RFSs; (2) internal consistency of PVOS and RSI scores; (3) RFS intrarater and interrater reliability; and (4) correlations between PVOS score, RSI score, RFS, and esophageal biopsy findings. RESULTS The mean (SD) age of the patients was 7.5 (2.6) years; the mean (SD) PVOS score, 71.9 (21.4); and the mean (SD) RSI score, 16.2 (9.1). The PVOS and the RSI scores demonstrated good internal consistency (Cronbach alpha = 0.79 and 0.78, respectively). The RFS exhibited good intrarater reliability (r = 0.66-0.98) and moderate interrater reliability (r = 0.32-0.70). The PVOS and RSI instruments displayed significant correlation (r = -0.30; P = .04). There were no other significant correlations between RFSs, esophageal biopsy results, PVOS scores, or RSI scores (P > .05). CONCLUSIONS The RSI may be a useful parent-proxy instrument in addition to the PVOS for pediatric voice patients. The RFS is reliable in children, but its validity could not be demonstrated in this patient population.