Search

Abstract: The aim of this report is to describe the combination of Crouzon syndrome and acanthosis nigricans with fibrous dysplasia of the maxilla. The diagnosis of fibrous dysplasia was confirmed clinically and pathologically during Le Fort III osteotomy and midface advancement with distraction osteogenesis. Crouzon syndrome with acanthosis nigricans is a known syndrome with an incidence of 1:1,000,000. This is the first report in the literature of Crouzon syndrome and acanthosis nigricans combined with fibrous dysplasia. As all 3 pathologies are related to fibroblasts, they may be different manifestations of malfunction of a single molecular pathway. The detection of fibrous dysplasia in a patient with Crouzon syndrome and acanthosis nigricans is important because it may complicate midface osteotomies and fixation of the hardware on the bones during craniofacial surgery., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2020 by Mutaz B. Habal, MD.)

Objectives: The objective of the study was to compare the efficacy of transdermal scopolamine and cinnarizine in the prevention of seasickness and their adverse reactions., Methods: Seventy-six naval crew members participated in a double-blind, randomized, crossover study. On 2 voyages, they were administered either a transdermal scopolamine patch containing 1.5 mg scopolamine and placebo tablets or 25-mg cinnarizine tablets and a placebo patch. Subjects completed questionnaires for each voyage, reporting on the efficacy of the drugs, the severity of their adverse reactions, and the preferred treatment., Results: Subjects reported the scopolamine patch to be significantly more effective than the cinnarizine tablet (P = 0.029). A moderate to high degree of drowsiness was attributed more frequently to cinnarizine than to the scopolamine patch (34% and 17%, respectively; P < 0.02). Any adverse reaction, to at least a moderate degree, was more frequent with cinnarizine (38%) than with the scopolamine patch (22%), although the significance of this association was borderline. A significantly greater percentage of subjects preferred transdermal scopolamine to cinnarizine (41 vs 12%, P < 0.001)., Conclusions: Higher efficacy, a lower rate of adverse reactions, and convenience all led the participants of this study to prefer the scopolamine patch to cinnarizine. Considering the 2 therapeutic options assessed in this study, and in light of the findings of previous studies, it is recommended that the scopolamine patch be used as the drug of choice for the treatment of seasickness among naval crew in particular and probably also among all other sea travelers.

Objective: To follow changes in transient evoked and distortion product otoacoustic emissions (TEOAEs, DPOAEs) as they relate to pure-tone audiometry (PTaud) thresholds during the first 2 years of occupational noise exposure., Design: Prospective controlled., Methods: Pure-tone audiometry thresholds, TEOAE and DPOAE amplitudes, and contralateral medial olivocochlear reflex strength were repeatedly evaluated during 2 years and compared between and within a cohort of 135 ship engine room recruits and a control group of 100 subjects with no noise exposure., Results: Pure-tone audiometry thresholds for 2,000, 3,000 and 4,000 Hz in both ears were significantly elevated in the study group after 2 years of noise exposure. Significantly lower TEOAE amplitudes were found at 2,000 Hz in the right ear and 2,000 and 4,000 Hz in the left ear. Longitudinal intrasubject analysis of the study group revealed significant reductions of TEOAE amplitudes at 2,000 to 4,000 Hz in both ears and reduced DPOAE amplitudes for 5,957 Hz in the right ear and 3,809, 4,736, and 5,957 Hz in the left ear in the second follow-up evaluation. Baseline medial olivocochlear reflex strength showed no correlation to PTaud thresholds after 2 years of noise exposure. Poor to moderate negative linear correlations (r = -0.07 to -0.37) were found between the DPOAE-averaged amplitudes at 2,979 to 5,957 Hz and PTaud threshold means at 3,000 to 6,000 Hz. Abnormal TEOAE parameters after the first year of noise exposure had high sensitivity (86-88%) and low specificity (33-35%) for the prediction of noise-induced hearing loss (NIHL) after 2 years., Conclusion: The DP-gram is not significantly correlated with PTaud and cannot be used as an objective measure of pure-tone thresholds in early NIHL. Medial olivocochlear reflex strength before the beginning of chronic exposure to occupational noise has no relation to individual vulnerability to NIHL. Although TEOAEs changes after 1 year showed high sensitivity in predicting NIHL after 2 years of exposure, they cannot be recommended as an efficient screening tool due to high false-positive rates.

Objective: To present a case series of vestibular symptoms appearing after combined sailing and diving activity, and to discuss the differential diagnosis and the workup algorithm., Study Design: Case series., Setting: Tertiary referral center., Patients: Three patients aged 25 to 31 years suffering from unsteadiness and movement sensations after sailing and scuba diving., Interventions: Neurotologic evaluation and recompression therapy in a hyperbaric chamber., Main Outcome Measures: The increasing popularity of marine sports and leisure activities has resulted in the exposure of a growing number of people to unique abnormalities not encountered under terrestrial conditions. The otolaryngologist who is involved in the care of these patients is required to diagnose and treat diving-related sinus and ear injuries such as barotrauma and decompression sickness, and also to be familiar with sailing-related disorientation syndromes such as seasickness and mal de debarquement. Treatment modalities for the various abnormalities differ significantly, and early commencement of treatment is often crucial for a successful outcome., Conclusion: Whenever doubt exists, recompression treatment must be instituted as soon as possible because of the potential for severe sequelae if the patient is left untreated, and because the risks involved in this therapy are minimal.

Hypothesis: Sound conditioning might reduce cerebral oxygen toxicity., Background: Cerebral oxygen toxicity is related to high levels of reactive oxygen species. Noise-induced hearing loss has been shown to result from ischemia-reperfusion, in which reactive oxygen species play a major role. Repeated exposure to loud noise at levels below that which produces permanent threshold shift prevented noise-induced hearing loss and was associated with significant elevation of the antioxidant enzymes measured in the inner ear. We tested the hypothesis that sound conditioning might reduce cerebral oxygen toxicity., Methods: Forty-five guinea pigs were prepared for electroencephalography and auditory brainstem recording. The auditory brainstem recording detection threshold was determined to confirm baseline normal hearing. The animals were divided into three equal groups and subjected to the following procedures: Group 1, electroencephalography electrode implantation and auditory brainstem recording only; Group 2, exposure to oxygen at 608 kPa (the latency to the first electrical discharge in the electroencephalogram preceding the appearance of seizures was measured); and Group 3, sound conditioning followed by oxygen exposure. The animals were killed, and the brains were excised and homogenized. Brain levels of superoxide dismutase, catalase, glutathione peroxidase, glutathione transferase, glutathione reductase, glucose-6-phosphate dehydrogenase, and thiobarbituric acid reactive substances were compared among the groups., Results: Latency to the first electrical discharge was compared between Groups 2 and 3, and was found to be significantly longer in Group 3 (27.9 +/- 11 versus 20.4 +/- 7.6 min, p < 0.03). No significant changes were found in brain levels of superoxide dismutase, catalase, glutathione peroxidase, glutathione transferase, glutathione reductase, glucose-6-phosphate dehydrogenase, or thiobarbituric acid reactive substances., Conclusion: Our data show that sound conditioning prolongs the latency to oxygen-induced convulsions. This effect was not accompanied by significant changes in whole-brain antioxidant enzyme activity or the magnitude of lipid peroxidation.

Background: Traditional pharmacological therapies aiming to modify the abnormal electrophysiological substrate underlying cardiac arrhythmias may be limited by their relatively low efficacy, global cardiac activity, and significant proarrhythmic effects. We suggest a new approach, in which transfected cellular grafts expressing various ionic channels may be used to manipulate the local electrophysiological properties of cardiac tissue. To examine the feasibility of this concept, we tested the hypothesis that transfected fibroblasts expressing the voltage-sensitive potassium channel Kv1.3 can modify the electrophysiological properties of cardiomyocytic cultures., Methods and Results: A high-resolution multielectrode mapping technique was used to assess the electrophysiological and structural properties of primary cultures of neonatal rat ventricular myocytes. The transfected fibroblasts, added to the cardiomyocytic cultures, caused a significant effect on the conduction properties of the hybrid cultures. These changes were manifested by significant reduction in extracellular signal amplitude and by the appearance of multiple local conduction blocks. The location of all conduction blocks correlated with the spatial distribution of the transfected fibroblasts assessed by vital staining. All electrophysiological changes were reversed after the application of Charybdotoxin, a specific Kv1.3 blocker. In contrast, conduction remained uniform in the control hybrid cultures when nontransfected fibroblasts were used., Conclusions: Transfected fibroblasts are able to electrically couple with cardiac myocytes, causing a significant local and reversible modification of the tissue's electrophysiological properties. More broadly, this study suggests that transfected cellular grafts expressing various ionic channels may be used to modify cardiac excitability, providing a possible future novel cell therapy strategy.