Hearing loss in Takayasu's arteritis: a role for hyperbaric oxygen therapy?

In the scientific community, there is growing interest regarding associated symptoms in Takayasu’s arteritis (TA), an autoimmune condition that mainly affects the medium and large arteries. Hearing loss (HL) is a rare complication of TA that is often overlooked and has severe consequences on the quality of life. HL mainly presents as sudden sensorineural hearing loss (SSNHL) and responds to corticosteroid therapy. In the authors’ opinion, the reasons for misdiagnosing HL in patients with TA are lack of evidence re- garding this rare complication and because TA involves large caliber arteries, instead of small vessels that are typical of the inner ear. We recently used HBOT for a 36-year-old woman with TA, who had two SSNHL episodes in different ears in an 11-month period and observed a significant improvement in the patient's hearing. Although this was a single case finding and HBOT was administered together with steroid therapy, the significant recovery of HL in both ears following this therapeutic approach may be worth sharing with the scientific community. In conclusion, we recommend that awareness for inner ear involvement in TA should increase and that HL should be considered a possible complication of TA and should be treated with corticosteroid therapy and, after collecting further evidence, HBOT

Optimal management of Cogan’s syndrome: a multidisciplinary approach

Cogan's syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. CS affects mainly young Caucasian adults, mostly during their first three decades of age, and may develop into typical and atypical variants. Typical CS manifests primarily with IK and hearing loss, whereas atypical CS usually presents with inflammatory ocular manifestations in association with audio-vestibular symptoms but mostly different Ménière-like symptoms and, more frequently, with systemic inflammation (70%), of which vasculitis is the pathogenic mechanism. CS is considered as an autoimmune- or immune-mediated disease supported mainly by the beneficial response to corticosteroids. Using well-developed assays, antibodies to inner ear antigens, anti-Hsp70, and antineutrophil cytoplasmic antibodies were found to be associated with CS. Corticosteroids represent the first line of treatment, and multiple immunosuppressive drugs have been tried with variable degrees of success. Tumor necrosis factor-alpha blockers and other biological agents are a recent novel therapeutic option in CS. Cochlear implantation is a valuable rescue surgical strategy in cases with severe sensorineural hearing loss unresponsive to intensive and/or innovative immunosuppressive regimens

Autoimmunity and otolaryngology diseases

Many systemic autoimmune diseases have otolaryngology manifestations that could represent a diagnostic challenge for clinicians, as they often constitute an early sign of an otherwise asymptomatic autoimmune condition and may lead to delayed diagnosis and treatment. In other cases, otolaryngology manifestations can be overlooked in patients with previously diagnosed autoimmune diseases. The pres- ence of concomitant conditions, the heterogeneity of studies available in the literature, and the lack of randomized trials are factors that may limit the prompt diagnosis of otolaryn- gology manifestations in systemic autoimmune diseases, with underestimation of the problem and undertreatment of the related condition

Somatosensory tinnitus: current evidence and future perspectives

In some individuals, tinnitus can be modulated by specific maneuvers of the temporomandibular joint, head and neck, eyes, and limbs. Neuroplasticity seems to play a central role in this capacity for modulation, suggesting that abnormal interactions between the sensory modalities, sensorimotor systems, and neurocognitive and neuroemotional networks may contribute to the development of somatosensory tinnitus. Current evidence supports a link between somatic disorders and higher modulation of tinnitus, especially in patients with a normal hearing threshold. Patients with tinnitus who have somatic disorders seems to have a higher chance of modulating their tinnitus with somatic maneuvers; consistent improvements in tinnitus symptoms have been observed in patients with temporomandibular joint disease following targeted therapy for temporomandibular disorders. Somatosensory tinnitus is often overlooked by otolaryngologists and not fully investigated during the diagnostic process. Somatic disorders, when identified and treated, can be a valid therapeutic target for tinnitus; however, somatic screening of subjects for somatosensory tinnitus is imperative for correct selection of patients who would benefit from a multidisciplinary somatic approach

Nasal manifestations in granulomatosis with polyangiitis: a case report and review of the literature

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 coexist- ing 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 func- tional 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 diagno- sis of GPA is important to initiate therapy which may be life-saving and organ sparing

Letter to the editor: autoimmune pathogenic mechanisms in Huntington’s disease

Letter to the Editor: Autoimmune pathogenic mechanisms in Huntington's disease

Large parapharyngeal mass: a challenging differential diagnosis

Tumors of the parapharyngeal space are rare entities that represent less than 1 % of all head and neck neoplasms. Parapharingeal masses may represent a relevant differential diagnostic challenge for clinicians. The common benign character of these conditions does not exclude malignancy, such as in this case in which malignant transformation was observed

An atypical case of trigeminal trophic syndrome: a legal medicine perspective in medical responsibility

BACKGROUND: Trigeminal trophic syndrome is a rare complication of peripheral or central damage to the trigeminal nerve characterized by anesthesia, paresthesia and a secondary persistent facial ulceration. METHODS: We describe the case of a 40-year-old woman with previous history of Le Fort I osteotomy for a class III malocclusion who developed trigeminal trophic syndrome. Atypically, the cutaneous symptoms appeared bilaterally and 8 years after surgery. RESULTS: Differential diagnosis was based on clinical history, tissue biopsy and serologic evaluation. Atypical findings could be linked to the surgical burdens of Le Fort I osteotomy, a procedure characterized by a bilateral incision on the maxillofacial bones with a reasonable probability of causing a bilateral injury of the peripheral branches of the trigeminal nerve. CONCLUSION: Although the long delay between trigeminal trophic syndrome onset and surgery and the absence of adequate medical evidence cannot confirm a link with previous surgery in this case, the increasing number of maxillofacial surgery cases suggests that this complication may be more frequent in the next decades, and thus, involved specialists should be aware of this condition as a possible complication of maxillofacial surgery procedures

Sialendoscopic management of autoimmune sialadenitis: a review of literature

Autoimmune diseases of major salivary glands include Sjögren's syndrome and a complex of disorders classified as immunoglobulin G4-related diseases. These pathologies are characterised by an autoimmune reaction mediated by T-helper lymphocytes that targets the ducts of exocrine glands in Sjögren's syndrome and glandular parenchyma in immunoglobulin G4-related diseases. Immunoglobulin G4-related diseases represent recently introduced multi-organ diseases that also involve the salivary glands. However, the morbid conditions once known as Mikulicz's disease and Kuttner's tumour were recently considered as two variants of immunoglobulin G4-related diseases affecting the major salivary glands ( immunoglobulin G4-related sialadenitis). This review briefly summarises the pathogenesis and clinical features of autoimmune diseases of the major salivary glands, focusing on the diagnostic and therapeutic role of sialendoscopy