A. De Virgilio

Cogan's syndrome: An autoimmune inner ear disease

OBJECTIVES The objective of our study was to review our current knowledge of the aetiopathogenesis of Cogans 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 Cogans syndrome from 1945 to 2012 were analysed. RESULTS AND CONCLUSIONS Cogans 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 Menieres 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 Cogans syndrome.

Vogt–Koyanagi–Harada syndrome

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 50years, 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.

Robotic sialoadenectomy of the submandibular gland via a modified face-lift approach

The purpose of this study was to describe and analyse the advantages and disadvantages of submandibular gland (SMG) resection using a robotic surgical system through a modified face-lift approach. The authors performed robotic sialoadenectomy of the SMG on 5 patients using the daVinci robot system through a modified face-lift approach. Three robotic arms were inserted through a modified face-lift incision; a face-down 30-degree endoscopic arm and two operative arms. The right arm was equipped with a harmonic scalpel and the left arm with a Maryland forceps. In all patients, robotic sialoadenectomy of the SMG was completed successfully. Diagnoses were sialolithiasis in two patients, pleomophic adenoma in two patients, and ranula in one patient. The mean robotic operative time was 90.2 min (range 62-185 min) and that for setting the robotic system was 8.2 min (range 5-15 min). No significant intra-operative or postoperative complications were observed. All patients were satisfied with the outcome and especially the cosmetic results at their last follow-up visit. In the authors opinion robotic sialoadenectomy of the SMG is technically feasible and secures a better cosmetic outcome than endoscopic submandibular resection.

Oncological results of the surgical salvage of recurrent laryngeal SCC in a multicentric retrospective series. The emerging role of supracricoid partial laryngectomy

Several studies in the last decade evaluated conservative surgical procedures and, in particular, supracricoid operations as an alternative to total laryngectomy for the salvage of recurrences of laryngeal squamous cell carcinoma (SCC) after a first attempt of organ preservation.

Transoral robotic surgery for the resection of parapharyngeal tumour: Our experience in ten patients

below the mandibular notch is resectable with a favorable outcome. Oral Oncol.43, 570–579 3 Greene F.L., Page D.L., Fleming I.D. et al. (2002) AJCC Cancer Staging Manual, 6th edn. Springer-Verlag, New York 4 Karakousis C.P., Kontzoglou K. & Driscoll D.L. (1998) Anterior compartment resection of the thigh in soft-tissue sarcomas. Eur. J. Surg. Oncol.24, 308–312 5 Stotter A., Fallowfield M., Mott A. et al. (1990) Role of compartmental resection for soft tissue sarcoma of the limb and limb girdle. Br. J. Surg.77, 88–92 6 Calabrese L., Giugliano G., Bruschini R. et al. (2009) Compartmental surgery in tongue tumours: description of a new surgical technique. Acta Otorhinolaryngol. Ital.29, 259–264 7 Calabrese L., Bruschini R., Giugliano G. et al. (2011) Compartmental tongue surgery: long term oncologic results in the treatment of tongue cancer. Oral Oncol.47, 174–179

Carotid stenosis after adjuvant cervical radiotherapy in patients with head and neck cancers: a prospective controlled study

Clin. Otolaryngol. 2012, 37, 376–381

Clinic manifestations in granulomatosis with polyangiitis.

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.

Is vestibular neuritis an immune related vestibular neuropathy inducing vertigo

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.

Chemical ear peeling: a simple technique for the treatment of chronic external otitis: how we do it.

Dear Editor, Few conditions encountered by otolaryngologists are as frustrating as chronic otitis externa and recurrent exacerbation presents a special challenge for the attending physician. The disease may be extremely therapy-resistant: a great many patients report chronic suffering from otitis externa with inadequate and inefficacious treatment attempts. Current medical treatment is based on topical application of steroids, antibiotics and external auditory canal cleansing. Moreover, there are no long-term outcome data on medical management. In this study we present, showing the shortand longterm clinical outcomes, a new, safe and simple therapeutic technique for the management of chronic otitis externa: chemical ear peeling (CEP).

Reinke's Edema: investigations on the role of MIB-1 and hepatocyte growth factor

Reinkes edema is a benign disease of the human vocal fold, which mainly affects the sub-epithelial layer of the vocal fold. Microscopic observations show a strongly oedematous epithelium with loosened intercellular junctions, a disruption of the extracellular connections between mucosal epithelium and connective tissue, closely adherent to the thyroarytenoid muscle. Thickening of the basal layer of epithelium, known as Reinkes space, high deposition of fibronectin and chronic inflammatory infiltration it is also visible. We analyzed, together with the hepatocyte growth factor (HGF), the expression level of MIB-1 in samples harvested from patients affected by Reinkes edema, in order to define its biological role and consider it as a possible prognostic factor in the follow-up after surgical treatment. We observed a moderate expression of HGF in the lamina propria of the human vocal fold and in the basal membrane of the mucosal epithelium. Our finding suggests that this growth factor acts as an anti - fibrotic agent in Reinkes space and affects the fibronectin deposition in the lamina propria. MIB-1, on the contrary, showed a weak expression in the basement membrane of the mucosal epithelium and a total absence in the lamina propria deep layer, thus suggesting that only the superficial layer is actively involved in the reparatory process with a high regenerative capacity, together with a high deposition of fibronectin. The latter is necessary for the cellular connections reconstruction, after the inflammatory infiltration.