Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several diagnostic criteria have been proposed. Besides, several diagnostic procedures have been used for the diagnosis, including indirect immunofluorescence, direct immunofluorescence, and ELISA. We reviewed the most recent literature, searching on PubMed “paraneoplastic pemphigus”. We included also papers in French, German, and Spanish. We found 613 papers for “paraneoplastic pemphigus”. Among them, 169 were review papers. Because of its varying clinical features, paraneoplastic pemphigus still represents a challenge for clinicians. Furthermore, diagnosis and management of paraneoplastic pemphigus requires close collaboration between physicians, including dermatologist, oncologist, and otorhinolaryngologist.
OBJECTIVE:Comparing the endoscopic and microscopic approaches in stapes surgery, and establishing the surgical approach that allows better results in terms of visualization of the middle ear structures and aids assistant's training. MATERIALS and METHODS:Twenty-one residents from the first to the fifth year of training in Otolaryngology were enrolled in this prospective study. A questionnaire specifically designed to investigate the understanding of middle ear anatomy, the surgical steps, and a personal opinion about endoscopic stapes surgery was submitted to each resident. RESULTS:Statistical differences between the endoscopic and microscopic approaches were evident about identification of stapes and long process of the incus (p=0.03) and stapes footplate and the oval window (p=0.03). No substantial difference between the two surgical approaches emerged regarding the visualization of the tympanic membrane and the other middle ear structures (p>0.5). A substantial difference in favor of the endoscopic approach emerged regarding the identification of the hole creation in the footplate of the stapes. CONCLUSION:Endoscopic stapes surgery could favor an easier understanding of the surgical technique for assistants in training with little knowledge of the anatomy and surgical steps.
ObjectiveDetermine in a cohort of patients with normal hearing and chronic tinnitus if self-reported history for temporomandibular joint (TMJ) dysfunction and a positive modulation of tinnitus in the TMJ region could be suggestive of an underlying TMJ disorder.Patients and methodsThe study included 226 patients presenting to the Head and Neck Service of our University Hospital. Following audiological and somatic tinnitus evaluation, patients were divided into two groups. The study group (n = 134) included subjects that met both the following criteria: A) a self-reported history for TMJ dysfunction and B) a positive modulation of tinnitus following somatic maneuvers in the TMJ region. The control group (n = 92) included patients with similar demographic and tinnitus characteristics that did not meet the proposed criteria for somatic tinnitus. Afterwards, patients underwent clinical TMJ evaluation in the Service of Clinical Gnathology of our University.ResultsOne hundred thirty-one patients (57.9%) received a clinical diagnosis of TMJ disorder according to DC/TMD Axis I; 79.1% in the study group and 27.2% in the control group. Ninety-five (42.1%) patients were negative for TMJ disorders; 20.9% in the study group and 72.8% in the control group. A significantly higher number of TMJ disorders was found in patients in the study group compared to the control group (p<0.0001). Most patients had joint disorders (67.2%), followed by other (29.8%) and pain disorders (29%). Logistic regression analysis in the study group showed that female gender was more prevalent in patients with TMJ disorders.ConclusionOur findings in patients with chronic tinnitus and normal hearing suggest that self-reported history for somatic dysfunction and modulation of tinnitus, when occurring simultaneously in the TMJ region, can be useful to preliminarily identify patients with TMJ disorders.
Hearing loss has been rarely reported in Takayasu's arteritis, may present as Sudden Sensorineural Hearing Loss, and usually responds well to corticosteroid therapy. Hyperbaric Oxygen Therapy is commonly used as a supplementary treatment to corticosteroids for Sudden Sensorineural Hearing Loss. We present the case of a 36-year-old woman with Takayasu's arteritis who had two episodes of sudden hearing loss involving one ear at a time with an 11-month delay between each episode. During the first episode, the patient was treated with high-dose intramuscular corticosteroids with a temporary improvement of auditory threshold that deteriorated 14 days after cessation of therapy. In the second episode, Hyperbaric Oxygen Therapy was associated with corticosteroids, with improvements in both ears, including the one that was unresponsive in the long term to previous pharmacologic therapy. In this case, Hyperbaric Oxygen Therapy could have acted synergically with corticosteroids playing a role in hearing restoration.
Granulomatosis with polyangiitis (GPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides of medium and small arteries, characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract with coexisting glomerulonephritis. We report a case of GPA in a patient presenting with a six-month history of spontaneous epistaxis, nasal obstruction and frontal headache. Nasal endoscopy showed a large nasal septum perforation and an anterior translucid mass in the right nasal fossa. Findings were confirmed by computed tomography (CT) scan with contrast. The patient underwent functional transnasal endoscopic removal of the mass; histological examination showed tissue features suggestive of GPA; dosage of c-ANCA e p-ANCA antibodies confirmed GPA diagnosis. Nasal septum perforation has long been recognized as a feature of GPA, in which granulomatous destruction of nasal cartilage can result in perforation and saddle-nose deformity. Prompt diagnosis of GPA is important to initiate therapy which may be life-saving and organ sparing.
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