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Cogan's syndrome (CS) is a rare autoimmune disorder characterized by audiovestibular dysfunction and ocular inflammation. Currently, there is no specific serum autoantibody used in the diagnostic workup of CS. Treatment is based on immunosuppressive agents, mainly corticosteroids as first-line choice. Recently, novel therapeutic modalities in CS have emerged. These include tumor necrosis factor-α inhibitors and other biologicals. Despite medical treatment, hearing loss may progress to irreversible bilateral profound SNHL in approximately half of CS patients resulting in candidacy for cochlear implantation (CI). Due to the inflammatory nature of the disease that is causing endosteal reaction with partial obliteration or complete neoossification of the intracochlear ducts, early CI is recommended. CI provides excellent and stable hearing rehabilitation with high score of word and sentence recognition. In this review, we will discuss different aspects of CS including clinical presentation, diagnosis, treatment, and future directives. [ABSTRACT FROM AUTHOR]

Background Complications associated with long-term tracheotomy are obstruction of the distal end of the tube by granulation tissue and tracheomalacia. These complications have traditionally been surgically treated. Methods Prospective study in a chronic ventilator-dependent division, including 234 consecutive patients with tracheotomy and mechanical ventilation. Endoscopic evaluation was performed in patients in whom there was respiratory distress with difficulty in passing a suction catheter through the tube, and/or increased inspiratory resistance and increased peak inspiratory pressure. Results Nineteen patients were diagnosed with granulation or tracheomalacia. Two patients were treated by surgical removal of the obstructing tissue. Nonsurgical patients were conservatively managed with symptoms' resolution by bypassing the pathology with a longer tube than the previous one or by an adjustable flange tube under endoscopic visualization, with a median symptom-free period of 433 days (range, 55-1230 days). Conclusion In nonsurgical candidates, insertion of a longer tube is a conservative and feasible long-term treatment. © 2014 Wiley Periodicals, Inc. Head Neck 37: 716-721, 2015 [ABSTRACT FROM AUTHOR]

PMF of infancy is a recently described autosomal recessive disorder presenting with severe bone marrow failure, accelerated neutrophil apoptosis, and significant platelet dysfunction, caused by a mutation in the VPS45 gene. In this study, we update our group of patients with PMF, highlighting different aspects of this disease, and evaluating the effectiveness of HSCT for the treatment of this disorder. Update of clinical data, hematological features, molecular studies, treatment and final outcome of four children diagnosed with VPS 45-associated PMF of infancy. The patients described had clinical and hematological findings consistent with MF. Molecular studies showed that all patients were homozygous for the Thr224Asn mutation in the VPS 45 gene. HSCT was carried out in three patients and was successful in two. VPS 45-associated MF is a novel primary immune deficiency that can be successfully corrected by HSCT if applied early in the course of disease using appropriate conditioning. The diagnosis of VPS 45-associated PMF should be considered in all children presenting with SCN with subsequent development of pancytopenia. Long-term follow-up of these patients is necessary to identify extra-hematological manifestations of VPS45 deficiency. [ABSTRACT FROM AUTHOR]

Background Endoscopic anterior skull-base surgery has been previously suggested to cause a significant deterioration in olfactory function. Given the impact on quality of life, the objective of this study was to determine the effect of a unilateral middle-turbinate-sacrificing approach on olfactory function and sinonasal outcome. Methods Prospective cohort study comparing olfactory and sinonasal outcomes pre- and post-transsphenoidal skull-base surgery. Olfaction was the primary outcome and was measured prospectively using the University of Pennsylvania Smell Identification Test (SIT). Sinonasal symptoms were assessed subjectively via sinonasal-specific questions from the 22-item Sino-Nasal Outcome Test (SNOT-22), and objectively via the Lund-Kennedy Endoscopic Scoring system (LKES). Results Twenty-two patients met study inclusion criteria and completed all data acquisition. The mean preoperative SIT score was 34.8 and the mean postoperative SIT value was 35.1 ( p = 0.37). The average change in score (delta) was an increase of 0.3, with changes ranging from −3 to +4. When examined categorically, 91% of patients maintained their preoperative olfactory function classification. There was no significant difference in mean pre- and postoperative symptom scores. A small increase in the LKES was noted, from a mean of 0.6 to 2.5 ( p = 0.001) in the early postoperative period. Conclusion Olfactory function, as measured by the SIT test, was preserved with a middle-turbinate-sacrificing skull-base approach. Subjective sinonasal symptom scores were unaffected, but a slight worsening of objective endoscopic appearance was noted. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: Turban pin aspiration syndrome is a new clinical entity afflicting young Islamic girls wearing a turban.The goal of this study was to present our experience in diagnosis and treatment of this new entity, define its clinical and epidemiologic features, and shed a new light on the role of fashion in the increased incidence. Study Design: A retrospective study in a tertiary university hospital. Methods: Review of clinical parameters and epidemiologic features of 26 patients diagnosed with turban pin aspiration syndrome admitted to the Hadassah-Hebrew University Hospitals in Jerusalem from 1990 to 2010. Results: All patients were Muslim females with an average age of 16 years. In all cases, the history was positive for accidental aspiration. Most of the pins were located in the trachea (42%). In 20 cases, the pins were extracted by rigid bronchoscopy without major complications. Fluoroscopy-assisted rigid bronchoscopy was used successfully in three cases. In one case, the object was self-ejected by coughing before the bronchoscopy, and two patients were referred to the chest unit for thoracotomy. Conclusions: Clinicians should be aware of this distinct form of foreign body aspiration, its method of diagnosis, and extraction techniques. A cultural investigation showed a difference in the turban-fastening technique of young girls as compared with their mothers. Removal by rigid bronchoscopy is a safe method with a high success rate and should be considered as the preferred extraction method of choice. Laryngoscope, 2012 [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: To determine the effect of mouth breathing during childhood on craniofacial and dentofacial development compared to nasal breathing in malocclusion patients treated in the orthodontic clinic. Study Design: Retrospective study in a tertiary medical center. Methods: Clinical variables and cephalometric parameters of 116 pediatric patients who had undergone orthodontic treatment were reviewed. The study group included 55 pediatric patients who suffered from symptoms and signs of nasal obstruction, and the control group included 61 patients who were normal nasal breathers. Results: Mouth breathers demonstrated considerable backward and downward rotation of the mandible, increased overjet, increase in the mandible plane angle, a higher palatal plane, and narrowing of both upper and lower arches at the level of canines and first molars compared to the nasal breathers group. The prevalence of a posterior cross bite was significantly more frequent in the mouth breathers group (49%) than nose breathers (26%), (P = .006). Abnormal lip-to-tongue anterior oral seal was significantly more frequent in the mouth breathers group (56%) than in the nose breathers group (30%) (P = .05). Conclusions: Naso-respiratory obstruction with mouth breathing during critical growth periods in children has a higher tendency for clockwise rotation of the growing mandible, with a disproportionate increase in anterior lower vertical face height and decreased posterior facial height. Laryngoscope, 2010 [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to evaluate the routine use of packing or local hemostatic agents in endoscopic sinus surgery (ESS). Methods: Packing and/or hemostatic agents were used only when necessary in 100 consecutive adult ESS patients in a tertiary academic hospital. Necessity for packing the nose after excessive bleeding was analyzed in relation to demographic characteristics, medical history, previous surgeries, current surgical procedure, type of anesthesia, and amount of intraoperative bleeding. Results: Three patients who required packing because of other reasons (such as bolstering of mucosa) were excluded from the study. The remaining 97 patients included 61 males and 36 females between the ages of 16 to 86 (mean 44). Forty-nine patients underwent only ESS, 40 ESSs associated with nasal polypectomy, and 8 underwent other endoscopic procedures. Fifty-four underwent the operation under general anesthesia and 43 under local anesthesia. Intraoperative blood loss was less than 30 mL in 82 patients (85%), 30 to 50 mL in 11 (11%), and more than 50 mL in 4 (4%). In 89 patients (92%), packing or a hemostatic agent was not used. No patient had bleeding complications postoperatively. A comparison between patients who required packing to those who did not showed that the only statistically significant associations related to general anesthesia (P = 0.0082) and to the amount of intraoperative bleeding (P < 0.001). Conclusions: Most ESS procedures can be managed without packing or any other hemostatic measures. Local anesthesia, use of local vasoconstrictors, and careful operative technique minimize the need for nose packing, thus reducing patient’s discomfort, postoperative complications, and cost of surgery. EBM rating: C-4 [Copyright &y& Elsevier]

Background Foreign bodies traversing the aerodigestive tract lining into the surrounding soft tissues is a well-known entity. A 14-year-old girl was referred to our hospital after swallowing a pin that was not visualized by laryngoscopy, despite a neck film that localized it to the level of the larynx. Methods A CT scan demonstrated a bent pin piercing the left vertebral artery 'through and through,' and this was confirmed by angiography. The pin was extracted via an external approach to the neck without preoperative intravascular manipulation. Results Angiography immediately after the surgery demonstrated a stable thrombotic sleeve in the vertebral artery without further clot propagation or bleeding from the vessel wall. This is the first report of a 'through and through' thrombotic sleeve in an artery. Conclusions Simple surgical removal of a sharp foreign body from the vertebral artery without intravascular management may be a reasonable option. © 2010 Wiley Periodicals, Inc. Head Neck, 2012 [ABSTRACT FROM AUTHOR]

Acute upper airway obstruction is a potentially life-threatening event. Hemophilia-A is a coagulopathy with high risk for spontaneous bleeding. Here we describe for the first time a spontaneous epiglottic internal hemorrhage leading to upper airway narrowing in a hemophilia-A patient. The patient was admitted to the intensive care unit for airway observation and treatment with factor VIII supplementation. In the first 48 hours there was rapid respiratory improvement. The epiglottic swelling resolved on the 5th day. In this patient no oral intubation or surgical management was needed. Laryngoscope, 2010 [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: Sinonasal invasive aspergillosis (IA) is an aggressive fungal infection with high mortality rates. It commonly develops in immunocompromised patients, often after bone marrow transplantation (BMT). Aggressive surgical debridement by an external approach has been considered a central element of treatment. We describe our experience in endoscopic management of IA in BMT patients in a retrospective study., Methods: Charts of BMT patients with IA in the past 5 years were reviewed. Demographic data, primary disease, comorbidities, signs and symptoms, blood test results, preparation for surgery, surgical technique, and outcome were recorded., Results: Fourteen BMT patients, age ranging from 3 to 56 years, had sinonasal IA. The primary disease was acute myelogenous leukemia in 6, acute lymphoblastic leukemia in 3, chronic myeloblastic leukemia in one, severe combined immunodeficiency disease in 2, and myelodysplastic syndrome in 2 patients. Diagnosis was made by physical examination, biopsy, culture, and computed tomography scan. Treatment, including aggressive endoscopic debridement, a systemic antifungal medication, and local irrigations of amphotericin-B enabled eradication of IA in all patients. Seven patients required two or more operations. None required orbital exenteration or craniotomy. Six patients died of the primary illness or of comorbidities with no evidence of residual disease. Eight patients are alive., Conclusion: Early detection of IA in BMT patients enables aggressive treatment before the disease spreads into the orbit or brain. Proper preoperative preparation facilitates safe endoscopic surgery in patients with severe bleeding tendencies. Although sinonasal IA is lethal, endoscopic surgery is feasible and efficient, enabling excellent local control.

Objective: The objective of this study was to analyze the circumstance, demographic features, clinical findings, and complications of caustic ingestion in relation to the type and amount of caustic substance., Study Design: The authors conducted a retrospective cohort study in a tertiary medical center., Methods: The authors reviewed medical records from 1988 to 2003 of patients with a history of caustic ingestion and clinical signs of injury to the aerodigestive tract. Parameters examined included age at presentation, gender, demographic status, nature of the caustic substance ingested, amount of substance, circumstance of the event, diagnostic tools, degree of injury, and anatomic distribution of the injury, early and late complications, and requirement for ventilatory support., Results: A total of 50 cases of caustic ingestion were identified (age range, 5 months-71 years). A biphasic distribution of the patients was noted; half were children under 5 years old and the remainder was adults. The most common caustic agent ingested was alkaline (42%) followed by acidic (32%) and chlorine bleach (26%). The most frequent cause for ingestion was accidental (67%) as opposed to attempt suicide (33%). All cases of attempted suicide occurred in adults. Most of them reported ingestion of large amounts of caustic substance. In the pediatric group, an association between the caustic agent and ethnicity was observed. Among Jewish children, alkaline cleaning agents were the most common cause (82%). Acetic acid was the most common substance ingested by the Arab children (100%). The findings of rigid esophagoscopy in 36 patients were as follows: first-degree esophageal injury in 16 (44%), second-degree in 6 (17%), third-degree in 7 (19%), fourth-degree in 6 (17%), and one (3%) was normal. Mucosal injury to the esophagus was worse in the acidic ingestion group as compared with the alkaline substance ingestion group with marginally statistically significant difference in the median degree of injury between the three types of ingested substances (P = .054). Mucosal injury to the esophagus was worse among patients who attempted suicide as compared with accidental ingestion with a statistically significant difference in the median degree of injury between the two reasons for ingestion (P = .002)., Conclusion: Severity of injury from caustic ingestion damages depend on the type of ingested substance, which varies depending on ethnicity. Ingestion of caustic agents by children involves specific substances according to the season, cultural and religious festivals, and ethnicity. The majority of adult cases are intentional with more serious injuries and a higher rate of complications. In our series, ingestion of acidic substances and ingestion associated with suicide attempt had the most severe consequences.

Objectives/hypothesis: Conflicting data exist in the literature regarding the role of mitomycin in the prevention and treatment of laryngotracheal stenosis. The purpose of the study was to assess the value of mitomycin in preventing repeat stenosis after endoscopic dilation surgery of well-established stenoses., Study Design: Prospective controlled animal study., Methods: Laryngotracheal stenosis was induced in 16 dogs. After a period of 3 months the dogs underwent endoscopic dilation and were randomly divided into two groups. The control group received immediate topical application of normal saline. Dogs in the mitomycin group received immediate topical application of 0.5 mg mitomycin. The animals were then observed for 3 more months before euthanasia. The laryngeal lumens were measured endoscopically at baseline, before dilation, and before euthanasia. A comparison was made between the two study groups by means of the Wilcoxon rank-sum test for change in the percentage of stenosis attained by the endoscopic dilation., Results: Nine dogs were included in the control group, and seven in the mitomycin group. Group comparison for initial occlusion before endoscopic dilation using Wilcoxon rank-sum test showed no difference between the two groups (z = 0.16 [P =.87]). Three months after endoscopic dilation, no difference was observed between the two groups regarding the change in the percentage of occlusion (z = -0.21 [P =.83])., Conclusion: Mitomycin exerts a benefit equal to that of normal saline when applied to a well-established laryngotracheal stenosis in dogs and does not prevent repeat stenosis after endoscopic dilation surgery.