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Behcet's disease (BD) is an autoimmune vasculitis of unknown aetiology that is characterised by relapsing episodes of oral aphthous ulcers, genital ulcers, ocular lesions, skin lesions, and other manifestations, including vascular, gastrointestinal and neurological involvement. Behcet's disease exists worldwide, although there are significant regional differences, with the highest number of incidences in the Mediterranean, Middle East, and Far East. Behcet's disease occurs mainly between 18 and 40 years of age, and the male-to-female ratio is 7:1. The cause of Behcet's disease is unknown. It is believed to be due to an autoimmune process triggered by an infectious or environmental agent (possibly local to a geographic region) in a genetically predisposed individual. T cell homeostasis perturbation, especially Th1 and Th17 expansions and decrease regulation by Tregs are now supposed to be the cornerstone of BD pathogenesis. Inflammatory cytokine such as IL21 plays a critical role in pathogenesis of BD. Inner ear involvement in Behcet's disease was first reported by Alajouanine in 1961. Thereafter, many cases of inner ear involvement were described by other authors. The otological features of Behcet's disease can be divided into hearing loss and disequilibrium. The incidence of hearing loss in Behcet's disease has been reported to be 12 to 80% in several studies in the literature. Recently, some authors observed a significant percentage (59.2%) of hearing loss associated with Behcet's disease. According to other studies, hearing loss can be the fourth or even the third most common manifestation of the disease. The treatment of inner ear lesions remains unsatisfactory and is based on immunosuppressive agents and will not be reviewed here. This review focuses on the etiophatogenesis and otological manifestations of Behcet's disease and specifies the role of the otorhinolaryngologist as an integral member of the multidisciplinary team for clinical management of these patients.

In this study, we review our current knowledge of the autoimmune etiopathogenesis of chronic rhinosinusitis with nasal polyps including bacterial infections, viral infections and immunomediated mechanisms and to discuss pathogenesis with relevance for pharmacotherapy. Relevant publications on the etiopathogenesis and treatment of chronic rhinosinusitis with nasal polyps (CRSwNP) from 1977 to 2013 were analyzed. The characteristic signs and symptoms include appearance of relapsing nasal polyps, with typical symptoms such as nasal obstruction, nasal discharge and, usually, loss of the sense of smell. The etiology and pathogenesis remain unknown. Proposed theories of causation include bacterial or viral infections and immunomediated mechanisms. The autoimmune aetiology of of unknown origin or failure to respond to classic pharmacological treatments with nasal and oral steroids is now suspected. At present, the nature of the antigen trigger, the exact role played by B/T cells and anti-dsDNA autoantibodies in the pathogenesis of nasal polyposis remains unclear. Corticosteroids and surgery are the first line of treatment in CRSwNP. In the case of corticosteroid treatment failure, other drugs can be used such as rituximab, belimumab or omalizumab which have demonstrated clinical efficacy in the treatment of nasal polyposis with comorbid asthma. Immunosuppressive drugs such as methotrexate, and cyclophosphamide have also been used with varying degrees of success.

Clin. Otolaryngol. 2012, 37, 376–381 Objectives: To determine the prevalence of radiation-induced carotid stenosis, in patients who were treated for head and neck malignancies, using colour-flow duplex scanning. Design: Prospective controlled study at a single medical centre. Participants: We enrolled two groups of patients. The first (radiotherapy group) consisted of patients who received surgical treatment and adjuvant radiotherapy of the neck. The control group consisted of patients with head and neck malignancies who received only surgical treatment. Main outcome measures: All patients were evaluated with carotid artery ecoDoppler imaging 1 week before and 36 months after the surgical procedure. Intima-media thickness was measured bilaterally at the internal carotid artery and at the bifurcation. Carotid obstruction was classified as low (0–30%), moderate (31–49%) or severe (≥50%). Results: The preoperative stenosis grade did not differ between groups. In 15/25 patients (60%) in the radiotherapy group, mild stenosis evolved to moderate stenosis, while only 6/37 (16%) of the controls did (P = 0.004). Additionally, 9/39 (23%) patients in the radiotherapy group progressed to severe stenosis compared with only 3/54 (6%) controls (P = 0.029). The overall evolution showed that stenosis worsened in 24/32 (62%) patients in the radiotherapy group and 9/54 (17%) patients in the control groups (P

Granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is pathologically characterised by an inflammatory reaction pattern (necrosis, granulomatous inflammation and vasculitis) that occurs in the upper and lower respiratory tracts and kidneys. Although the aetiology of GPA remains largely unknown, it is believed to be autoimmune in origin and triggered by environmental events on a background of genetic susceptibility. In Europe, the prevalence of GPA is five cases per 100,000 population, with greater incidence in Northern Europe. GPA can occur in all racial groups but predominantly affects Caucasians. Both sexes are affected equally. GPA affects a wide age range (age range, 8–99 years). Granulomatosis with polyangiitis is characterised by necrotising granulomatous lesions of the respiratory tract, vasculitis and glomerulonephritis. Classically, the acronym ELK is used to describe the clinical involvement of the ear, nose and throat (ENT); lungs; and kidneys. Because the upper respiratory tract is involved in 70–100% of cases of GPA, classic otorhinolaryngologic symptoms may be the first clinical manifestation of disease. The nasal cavity and the paranasal sinuses are the most common sites of involvement in the head and neck area (85–100%), whereas otological disease is found in approximately 35% (range, 19–61%) of cases. Diagnosis of GPA is achieved through clinical assessment, serological tests for anti-neutrophil cytoplasmic antibodies (ANCA) and histological analysis. The 10-year survival rate is estimated to be 40% when the kidneys are involved and 60–70% when there is no kidney involvement. The standard therapy for GPA is a combination of glucocorticoids and cyclophosphamide. In young patients, cyclophosphamide should be switched to azathioprine in the maintenance phase. A multidisciplinary approach, involving otorhinolaryngologists, oral and maxillofacial surgeons, oral physicians, rheumatologists, renal and respiratory physicians, and ophthalmologists, is necessary for the diagnosis and therapeutic treatment of GPA. ENT physicians have a determining role in recognising the early onset of the disease and starting an appropriate therapy.

Background: Both the immediate beneficial physiological changes in a laboratory setting and the long-term clinical outcomes of heat and moisture exchanger (HME) use are well described. So far, there has not been any research published that provides detailed insight in the pattern of changes in both respiratory function and patients’ experiences with HMEs in the first weeks of use. Methods: A multicenter time-series study design with a 2-week double baseline period. All patients used the XtraHME for 12 weeks afterward. Data were collected 2 weeks, 6 weeks, and 12 weeks after the start of HME use. Results: Data of 30 patients were analyzed. Pulmonary symptoms decreased significantly during the 12 weeks of HME use. After 2 weeks, a significant decrease in daily coughs and daily forced expectorations was seen. The general quality of life showed a significant increase throughout the study. More general physical complaints also significantly decreased with HME use. Patient satisfaction with the HME was high. Conclusions: This study shows that there is a significant influence of the XtraHME on pulmonary status that can already be observed after 2 weeks of using the XtraHME and continues to improve further after 6 weeks of XtraHME use.

Objectives The short-term and long-term beneficial effects of HME use by laryngectomees are well described in literature. In this study, we document how laryngectomised patients, who previously did not use an HME, get accustomed to the use of HME and attachments. Participants Thirty patients, who were at least 3 months post-laryngectomy and previously did not use an HME, were followed for 12 weeks and were asked to complete questionnaires about their experiences with the HME and attachments. Results Results show that when patients start using an HME, they report some difficulties with breathing resistance during the first 2 weeks of use. However, after 6 weeks, they have become accustomed to the breathing resistance and after 12 weeks over 96% reports that breathing was equal or less strenuous compared with breathing though an open stoma. Only a small proportion of patients experienced problems with increased coughing when starting HME use. Conclusions This study provides insight in the way laryngectomised patients are experiencing the use of HMEs in the first weeks. These outcomes can contribute to a better knowledge of HME use by healthcare providers and help them to manage patient expectations and improving support to patients in achieving compliant HME use.

Background Squamous cell carcinoma (SCC) is the most common oral cavity malignant tumor. Surgery, radiotherapy, and chemotherapy have been the major options for its treatment. Electrochemotherapy (ECT) is a novel local treatment successfully used in secondary or primary skin or subcutaneous tumors. This new cancer treatment is a modality in which a locally applied electrical field enhances cell membrane permeability, thereby allowing greater intracellular accumulation of a chemotherapeutic agent. Methods We report a case of a man affected by an ulcerated SCC. The man was treated with ECT with good results. Results In our case, ECT was successful in the management of extensive metastasis of SCC in clinical conditions, whereas other approaches were rejected. Conclusion This case shows good clinical results; however, other studies are necessary to show that ECT should be considered as a promising treatment option. © 2014 The Authors. Head & Neck Published by Wiley Periodicals, Inc. Head Neck 36: E86–E90, 2014

Summary Objective/Hypothesis Provox 2 voice prosthesis requires periodic replacement due to biofilm proliferation which causes malfunctioning of the valve. The aim of this study was to show that Provox 2 voice prosthesis malfunctioning is due not only to valve obstruction caused by biofilm but also to the silicone variations. Design Methods Prospective study on the malfunction of Provox2 voice prostheses. Methods Through photographic and electron microscopic assessment, the authors studied nine Provox 2 voice prostheses, which were removed due to malfunctioning. Results Findings revealed that the silicone undergoes a degenerative process, thus causing the surface to become rough, deformed, swollen, and translucent. Furthermore, electron microscopy confirmed the presence of immune system cells and biofilm on the prosthesis surface and their role in creating a structural nonhomogenous structure in the silicone, which is deformed due to the presence of “crests” caused by material degeneration. Conclusion The degenerative process of the silicone seems to be related to the oxygen present in the trachea and esophagus and to the production of oxygen-free radicals on the biofilm's part and the immune system.

Objectives. To review the current knowledge of the aetiology of vestibular neuritis including viral infections, vascular occlusion, and immunomediated mechanisms and to discuss the pathogenesis with relevance to pharmacotherapy.Systematic Review Methodology. Relevant publications on the aetiology and treatment of vestibular neuritis from 1909 to 2013 were analysed.Results and Conclusions. Vestibular neuritis is the second most common cause of peripheral vestibular vertigo and is due to a sudden unilateral loss of vestibular function. Vestibular neuronitis is a disorder thought to represent the vestibular-nerve equivalent of sudden sensorineural hearing loss. Histopathological studies of patients who died from unrelated clinical problems have demonstrated degeneration of the superior vestibular nerve. The characteristic signs and symptoms include sudden and prolonged vertigo, the absence of auditory symptoms, and the absence of other neurological symptoms. The aetiology and pathogenesis of the condition remain unknown. Proposed theories of causation include viral infections, vascular occlusion, and immunomediated mechanisms. The management of vestibular neuritis involves symptomatic treatment with antivertiginous drugs, causal treatment with corticosteroids, and physical therapy. Antiviral agents did not improve the outcomes.

Objectives The objectives of this study are to review our current knowledge of the aetiopathogenesis of Vogt–Koyanagi–Harada syndrome, including viral infection, genetic factors and immunomediated mechanisms, and to discuss pathogenesis and its relevance to pharmacotherapy. Systematic review methodology Relevant publications from 1965 to 2012 on the aetiopathogenesis and pharmacotherapy of VKHS were analysed. Results and conclusion Vogt–Koyanagi–Harada syndrome (VKHS) is a rare multisystemic autoimmune disease that affects tissues containing melanin, including the eye, inner ear, meninges, and skin. The disease is characterised by bilateral uveitis associated with a varying constellation of auditory, neurological and cutaneous manifestations. The disease occurs more frequently among people with darker skin pigmentation. Asians, Native Americans, and Hispanics are most frequently affected. It predominates in patients aged between 20 and 50 years, and females are affected more frequently, with a female:male ratio of 2:1. The classic clinical course is characterised by bilateral panuveitis, hypoacusis, and meningitis, in addition to cutaneous involvement with poliosis, vitiligo, and alopecia. Although the exact cause of VKH disease remains unknown, it is thought to be a T-cell-mediated autoimmune process directed against melanocytes. VKHS classically begins with vague systemic symptoms suggestive of a viral infection, although a clear association between a specific viral agent and the disease has not been established. Genetic factors may play an important role in the loss of self-tolerance in VKHS. The HLA-DRB1*0405 allele is the main susceptibility allele for VKHS. Early and aggressive systemic corticosteroids are still the primary initial therapy for VKHS. Ocular complications may require an intravitreous injection of corticosteroids. Despite proper treatment with steroids, a number of patients experience recurrent attacks or steroid-associated complications. Thus, non steroid immunomodulatory therapy (IMT) has become necessary for the treatment of VKHS.

Renal cell carcinoma is the most common renal tumor in adults. Clear cell carcinoma represents 85% of all histological subtypes. In February 2012 a 72-year-old woman came to our department due to the appearance of massive hemoptysis and pharyngodinia. Previously, this patient was diagnosed with a renal cell carcinoma treated with left nephrectomy. We observed an exophytic, grayish, and ulcerated mass in the left tonsillar lodge and decided to subject the patient to an immediate tonsillectomy. Postoperative histology showed nests of cells with highly hyperchromatic nuclei and clear cytoplasm. These features enabled us to make the diagnosis of renal clear cell carcinoma metastasis. Only few authors described metastasis of renal cell carcinoma in this specific site.

Objectives The objective of our study was to review our current knowledge of the aetiopathogenesis of Cogan's syndrome, including viral infection and autoimmunity, and to discuss disease pathogenesis with relevance to pharmacotherapy. Systematic review methodology Relevant publications on the aetiopathogenesis and pharmacotherapy of Cogan's syndrome from 1945 to 2012 were analysed. Results and conclusions Cogan's syndrome is a rare autoimmune vasculitis, and its pathogenesis is unknown. Infection, but primarily autoimmunity, may play contributing roles in the pathogenesis of this disease. It is characterised by ocular and audiovestibular symptoms similar to those of Meniere's syndrome. Approximately 70% of patients have systemic disease, of which vasculitis is considered the pathological mechanism. The immunologic theory is based on the release of auto-antibodies against corneal, inner ear and endothelial antigens, and of anti-nuclear cytoplasmic auto-antibodies (ANCA). Corticosteroids are the first line of treatment, and multiple immunosuppressive drugs have been tried with varying degrees of success. Tumour necrosis factor (TNF)-alpha blockers are a category of immunosuppressive agents representing a recent novel therapeutic option in Cogan's syndrome.

Rheumatoid arthritis rarely involves the cricoarytenoid joint. The possible consequent symptom includes hoarseness, dysphagia, odynophagia, dysfunctional dysphonia, and acute dyspnea. Etiologic diagnosis is possible with high-resolution computed tomography, which can show spacing of the articular cartilage, density and volume alterations, and subluxation of the cartilage. However, these radiologic signs are not pathognomonic for rheumatoid arthritis, and they should be combined with anamnestic data.

Objectives To review our current knowledge of the pathogenesis of Meniere's disease, including viral infection and immune system-mediated mechanisms, and to discuss the pathogenesis as it relates to pharmacotherapy. Systematic review methodology Relevant publications on the aetiopathogenesis, molecular biology, genetics and histopathology of Meniere's disease from 1861 to 2011 were analysed. Results and conclusions Meniere's disease is characterised by intermittent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural pressure. The aetiology and pathogenesis remain unknown. Proposed theories of causation include viral infections and immune system-mediated mechanisms. The immune response in Meniere's disease is focused on inner ear antigens. Approximately one-third of Meniere's disease cases seem to be of an autoimmune origin although the immunological mechanisms involved are not clear. The diagnosis of autoimmune inner ear disease is based either on clinical criteria or on a positive response to steroids. The antiviral approach has virtually eliminated the use of various surgical methods used in the past. Steroid responsiveness is high, and with prompt treatment, inner ear damage may be reversible. The administration of etanercept improves or stabilises symptoms in treated patients. Treatment of antiphospholipid syndrome can be directed toward preventing thromboembolic events by using antithrombotic medications. Only warfarin has been shown to be effective. Gene therapy can be used to transfer genetic material into inner ear cells using viral vectors and to protect, rescue, and even regenerate hair cells of the inner ear.

Objectives To review our current knowledge of the etiopathogenesis of Bell's palsy, including viral infection or autoimmunity, and to discuss disease pathogenesis with respect to pharmacotherapy. Systematic review methodology Relevant publications on the etiopathogenesis, clinical presentation, diagnosis and histopathology of Bell's palsy from 1975 to 2012 were analysed. Results and conclusions Bell's palsy is an idiopathic peripheral nerve palsy involving the facial nerve. It accounts for 60 to 75% of all cases of unilateral facial paralysis. The annual incidence of Bell's palsy is 15 to 30 per 100,000 people. The peak incidence occurs between the second and fourth decades (15 to 45 years). The aetiology of Bell's palsy is unknown but viral infection or autoimmune disease has been postulated as possible pathomechanisms. Bell's palsy may be caused when latent herpes viruses (herpes simplex, herpes zoster) are reactivated from cranial nerve ganglia. A cell-mediated autoimmune mechanism against a myelin basic protein has been suggested for the pathogenesis of Bell's palsy. Bell's palsy may be an autoimmune demyelinating cranial neuritis, and in most cases, it is a mononeuritic variant of Guillain–Barre syndrome, a neurologic disorder with recognised cell-mediated immunity against peripheral nerve myelin antigens. In Bell's palsy and GBS, a viral infection or the reactivation of a latent virus may provoke an autoimmune reaction against peripheral nerve myelin components, leading to the demyelination of cranial nerves, especially the facial nerve. Given the safety profile of acyclovir, valacyclovir, and short-course oral corticosteroids, patients who present within three days of the onset of symptoms should be offered combination therapy. However it seems logical that in fact, steroids exert their beneficial effect via immunosuppressive action, as is the case in some other autoimmune disorders. It is to be hoped that (monoclonal) antibodies and/or T-cell immunotherapy might provide more specific treatment guidelines in the management of Bell's palsy.

Objectives To review our current knowledge of the pathogenesis of sudden sensorineural hearing loss, including viral infection, vascular occlusion and immune system-mediated mechanisms, and to discuss the pathogenesis as it relates to pharmacotherapy. Systematic review methodology Relevant publications on the pathogenesis of sudden sensorineural hearing loss from 1944 to 2010 were analysed. Results and conclusions Sudden sensorineural hearing loss is defined as hearing loss of 30 dB in three sequential frequencies over 3 days or less. It can be an isolated symptom or the presenting symptom of a systemic disease. The aetiology and pathogenesis remain unknown. Detailed investigation typically reveals a specific cause in about 10% of patients. Proposed theories of causation include viral infections, vascular occlusion and immune system-mediated mechanisms. A variety of therapies have been proposed based on the various proposed aetiologies.

E-Mail: antonio.grecoo@uniroma1.it Objective: The aim of our study was to evaluate prevalence of carotid stenosis in patients with head and neck malignant tumours treated with radiotherapy. Methods: In our study we included 137 patients with head and neck cancers treated during a period of about 5 years. The larynx was the most common affected site of primary tumour. Only 93 patients were alive at the moment of our observation. A group of 39 patients underwent neck radiation. A control group of 54 patients were not treated with radiotherapy. All patients undergoing radiotherapy received a local dose of 50-70 Gy for 6 weeks. An ecodoppler scan of the carotid arteries was performed in all patients with estimation of common and internal carotid artery Intimal Medial Thickness (IMT). Stenosis grading was divided into low (0-30%), moderate (31-49%) and severe (more than 50%). In addition, we considered hematochimic and flogistic parameters. Statistic evaluation was performed with Chi-square test. Results: We found that severe carotid stenosis was higher (41%) in patients who had undergone radiotherapy than in the control group (16.6 %) (P = 0.008). The ecodoppler examination demonstrated that the most commonly affected site was the internal carotid artery, just past the carotid fork.