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Cogan's syndrome is a rare disorder first clinically defined in 1945, characterized by nonsyphilitic interstitial keratitis and progressive audiovestibular symptoms. Later, patients with audiovestibular dysfunction and various types of inflammatory eye disease were classified as having atypical Cogan's syndrome. The etiology and pathogenesis of Cogan's syndrome remain largely unknown. Here, we report a case of atypical Cogan's syndrome with a histological assessment of the temporal bone during the acute disease period. Temporal bone histology was compared to age- and gender-matched normal control, and our findings revealed endolymphatic hydrops and degenerative changes in various parts of the inner ear. Laryngoscope, 134:5135-5138, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Purpose: To report a case of Cogan-Like Syndrome following treatment with nivolumab for metastatic cutaneous melanoma., Methods: A case report., Results: A 54-year-old female sought a second opinion from us regarding the recently diagnosed uveitis in both eyes. She had a diagnosis of metastatic cutaneous melanoma in the right arm and was undergoing treatment with nivolumab. Four weeks following the initiation of nivolumab therapy, she experienced tinnitus and bilateral sensorineural hearing loss, which was treated with oral and intratympanic steroids. While tapering the oral steroids, she developed iridocyclitis with papillitis in both eyes. This combination of vestibuloauditory symptoms and ocular inflammation was strikingly reminiscent of Cogan's syndrome. Because of the timing in relation to the nivolumab therapy and the steroid responsiveness of her presentation, this was speculated to be due to immune overactivation from the nivolumab. Given her complex condition, which involved toxicity and multiple metastases, the patient was advised to consider either topical and/or local corticosteroids or intravenous immunoglobulin. The patient chose to persist with corticosteroid therapy., Conclusion: Nivolumab could potentially be linked to an immune-related condition resembling Cogan syndrome. In cases involving patients with a complex condition necessitating nivolumab treatment, the use of topical and/or local corticosteroids or intravenous immunoglobulin, might constitute the sole viable treatment options.

Background: Cogan syndrome is a rare autoimmune systemic vasculitis presenting with interstitial keratitis and audiovestibular symptoms. The atypical form, characterized by more extensive ocular lesions with audiovestibular symptoms appearing with a longer delay and more frequent systemic features, is usually underdiagnosed, delaying treatment., Case Presentation: We report the case of a 30-year-old Mediterranean female who presented recurrent left red and painful eye. The evolution in flare-ups during 3 years, associated with a nonspecific biological inflammatory response, motivated ocular biopsies demonstrating diffuse inflammatory changes from the cornea to the sclera. Since Morocco is an endemic region for tuberculosis, the laboratory tests were expanded to identify the latent forms based on the tuberculin skin test and QuantiFERON TB Gold in tube assay, both positive for Mycobacterium tuberculosis infection. Thus, antibacillary chemotherapy was started for 6 months, with reappearance of the symptoms at the end of treatment. Three years later, the patient presented isolated and fluctuating audiovestibular Ménière-like symptoms, with progressive sensorineural hearing loss. The nonstabilization under usual medical treatment along with her diffuse inflammatory ocular lesions led to the diagnosis of atypical Cogan syndrome. The patient received long-term corticosteroid with notable improvement., Conclusion: Cogan syndrome is a rare autoimmune disease that should be considered when faced with ocular and audiovestibular manifestations, even in its atypical form, to provide early and adequate treatment, which is the main prognosis factor in the control of irreversible lesions. Thus, interdisciplinary collaboration is fundamental along with screening for other infectious and systemic disorders that should include tuberculosis., Competing Interests: Declarations Ethics approval and consent to participate Obtained. Consent for publication Written informed consent was obtained from the patient’s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Conflict of interest None., (© 2024. The Author(s).)

Behçet's syndrome and Cogan's syndrome constitute the group of variable vessel vasculitides in the Chapel-Hill Nomenclature. They involve arteries and veins of all sizes. As reflected in the name "syndrome", both diseases can manifest with different individual symptoms. Both formally are rare diseases, but the Cogan syndrome is much rarer than Behçet`s. For the latter, there are diagnosis and classification criteria as well as European (EULAR, European Alliance of Associations for Rheumatology) treatment recommendations. The symptomatology, diagnostic measures and treatment as well as some considerations about pathogenesis will be discussed in this article., Competing Interests: Votragshonorare, Reisekosten: Abbvie, Amgen, Boehringer, GSK, Janssen, Lilly, Medac, MSD, Novartis, Pfizer, Sobi, (Thieme. All rights reserved.)

Cogan's syndrome is a rare autoimmune inflammatory disease, characterized by interstitial keratitis and audio-vestibular signs. The syndrome was first described in 1945 by David G. Cogan. Then, it was only in 1980 when Haynes et al. proposed diagnostic criteria for patients with other symptoms and was qualified as atypical form of Cogan's syndrome. Herein, we report a case of a 28-year-old woman with atypical Cogan's syndrome. The patient was treated with corticosteroids and received a cochlear implant., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Paediatric Cogan Syndrome is a rare and underrecognised autoimmune vasculitis characterised by ocular inflammation and sensorineural hearing loss. Its etiopathogenesis, diagnosis, and management are not well defined. We report a 12-year-old girl who initially presented with symptoms of IgA vasculitis formerly called Henoch Schoenlein Purpura (HSP) and eventually developed anterior uveitis and bilateral sensorineural hearing loss leading to the diagnosis of atypical Cogan Syndrome. The workup for infectious etiologies and other systemic rheumatologic disorders was negative. The management was multidisciplinary involving Rheumatology, Ophthalmology, Otorhinolaryngology, and Audiology. The anterior uveitis responded well to systemic glucocorticoids and Methotrexate, but the hearing loss was grossly progressive warranting a cochlear implant. We are not aware of Paediatric Cogan Syndrome being reported as a mimicker of IgA vasculitis previously in the literature. It is an important finding as IgA vasculitis is prevalent in the paediatric age group and new-onset ocular or vestibular symptoms after IgA vasculitis should alert the clinician to the possibility of Cogan Syndrome. In the absence of well-defined diagnostic criteria, it is crucial to recognise the clinical symptoms of Paediatric Cogan Syndrome for early diagnosis and treatment since the delay in diagnosis can lead to permanent disability., (© Japan College of Rheumatology 2023. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Competing Interests: The authors report no conflicts of interest.

A 61-year-old man presented with weight loss, bilateral ocular redness, blurred vision, and sensorineural hearing loss. Fluorodeoxyglucose-position emission tomography/computed tomography demonstrated an uptake in the ascending and descending aorta, abdominal aorta and femoral arteries. Atypical Cogan's syndrome complicated with large-vessel vasculitis (LVV) was diagnosed. He was treated with high-dose prednisolone and subcutaneous tocilizumab (162 mg/week), resulting in successful improvements in his ocular and vascular involvements. Although there is currently no established treatment strategy for LVV associated with Cogan's syndrome, our case and literature review suggest that tocilizumab is a viable treatment option for this rare but life-threatening complication.

Cogan syndrome is an autoimmune disease characterized by vestibular symptoms, bilateral sensorineural hearing loss, and inflammatory ocular manifestations, which may be accompanied by systemic vasculitis. We herein present the case of a patient with bilateral sensorineural hearing loss who presented with pain over her cochlear implantation incision site. She was later found to have evidence of ocular disease and underlying vasculitis leading to a diagnosis of Cogan syndrome.

Introduction: Cogan's syndrome is a rare form of vasculitis mainly affecting young subjects of whatever gender, associating cochleovestibular and ophthalmological damage. Despite medical treatment, auditory prognosis is uncertain, with 50-60% of patients showing irreversible severe to profound hearing loss, thus being candidates for cochlear implantation. Following CARE guidelines, we report 10 cases of cochlear implantation in Cogan's syndrome, with assessment of speech reception threshold and maximum intelligibility after a minimum 1 year's experience with the implant., Case Series: Ten patients from 4 centers received cochlear implants (14 implants) for Cogan's syndrome between 2005 and 2020. After 1 year's experience, there was significant improvement in speech reception threshold (P=0.0002) and maximum intelligibility (P=0.0002)., Conclusion-Discussion: Audiovestibular signs associated with ophthalmological manifestations should suggest Cogan's syndrome. Hearing impairment is usually irreversible. Cochlear implantation may be necessary and improves hearing performance., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Background: Cogan´s syndrome is a rare, presumed autoimmune vasculitis of various vessels characterized by interstitial keratitis and vestibular impairment accompanied by sensorineural hearing loss. Due to the rarity of Cogan´s syndrome in children, therapeutic decision making may be challenging. Therefore, a literature search was performed to collect all published paediatric Cogan´s syndrome cases with their clinical characteristics, disease course, treatment modalities used and their outcome. The cohort was supplemented with our own patient., Main Text: Altogether, 55 paediatric Cogan´s syndrome patients aged median 12 years have been reported so far. These were identified in PubMed with the keywords "Cogan´s syndrome" and "children" or "childhood". All patients suffered from inflammatory ocular and vestibulo-auditory symptoms. In addition, 32/55 (58%) manifested systemic symptoms with musculoskeletal involvement being the most common with a prevalence of 45%, followed by neurological and skin manifestations. Aortitis was detected in 9/55 (16%). Regarding prognosis, remission in ocular symptoms was attained in 69%, whereas only 32% achieved a significant improvement in auditory function. Mortality was 2/55. Our patient was an 8 year old girl who presented with bilateral uveitis and a history of long standing hearing deficit. She also complained of intermittent vertigo, subfebrile temperatures, abdominal pain with diarrhoea, fatigue and recurrent epistaxis. The diagnosis was supported by bilateral labyrinthitis seen on contrast-enhanced magnetic resonance imaging. Treatment with topical and systemic steroids was started immediately. As the effect on auditory function was only transient, infliximab was added early in the disease course. This led to a remission of ocular and systemic symptoms and a normalization of hearing in the right ear. Her left ear remained deaf and the girl is currently evaluated for a unilateral cochlear implantation., Conclusions: This study presents an analysis of the largest cohort of paediatric Cogan´s syndrome patients. Based on the collected data, the first practical guide to a diagnostic work-up and treatment in children with Cogan´s syndrome is provided., (© 2023. The Author(s).)

Background: Cogan's syndrome (CS) is a rare autoimmune disorder characterized by non-syphilitic interstitial keratitis (IK) and Menière-like cochlear vestibular symptoms, which may also have systemic effects. Corticosteroids are first-line treatment. DMARDs and biologics have been used to treat ocular and systemic symptoms of CS., Case Presentation: This is a case of a 35-year-old female who reported hearing loss, eye redness and photophobia. Her condition progressed to a sudden sensorineural hearing loss, tinnitus, and constant vertigo accompanied by cephalea. CS was diagnosed after excluding other diseases. The patient still developed bilateral sensorineural hearing loss after receiving hormone, methotrexate, cyclophosphamide, and a variety of biological agents. Joint symptoms were relieved after treatment with a JAK inhibitor (tofacitinib), and hearing did not deteriorate further., Conclusions: CS should be involved in the differential diagnosis of keratitis. Early identification and intervention of this autoimmune disease can minimize disability and irreversible damage., (© 2023. The Author(s).)

Purpose: To systematically compare idiopathic and non-idiopathic ocular motor apraxia (OMA) in children., Methods: A retrospective chart review was conducted of all children (< 18 years) diagnosed as having OMA from 2010 to 2020. Demographics, clinical characteristics, and oculomotor outcomes were compared for children with idiopathic and non-idiopathic OMA., Results: Thirty-seven children were included, 17 (46%) with idiopathic OMA and 20 (54%) with non-idiopathic OMA. Among patients with non-idiopathic OMA, Joubert syndrome was the most frequent underlying diagnosis (30%). Strabismus (45% vs 12%, P = .04), nystagmus (30% vs 0%, P = .02), and vertical saccade involvement (25% vs 0%, P = .049) were significantly more common in non-idiopathic than idiopathic OMA, respectively. Neuroimaging abnormalities (90% vs 18%, P < .0001) and developmental delays (100% vs 59%, P = .002) were also more frequent in non-idiopathic than idiopathic OMA, respectively. Endocrine disorders (most commonly growth hormone deficiency) were diagnosed in 12% and 20% of children with idiopathic and non-idiopathic OMA, respectively ( P = .67). On survival curve analysis, improvement in OMA occurred faster and more frequently in children with idiopathic than non-idiopathic OMA (median time to improvement 56 vs 139 months, respectively, P = .034)., Conclusions: Non-idiopathic OMA is associated with a higher rate of vertical saccade involvement, nystagmus, and developmental delays. These findings should prompt neuroimaging in children with OMA. Additionally, endocrine disorders may be more frequent in children with OMA than the general pediatric population. [ J Pediatr Ophthalmol Strabismus . 2022;59(5):326-331.] .

Background: Cogan syndrome is a rare inflammatory condition that mainly affects adults and is characterised by inflammation of various ocular structures and by audiovestibular symptoms such as hearing loss and vertigo., Case Description: A 63-year old woman recently diagnosed with an anterior uveitis presented at A&E with vertigo, nausea, vomiting, tinnitus and headache, and she developed bilateral sudden deafness within days. Blood testing revealed elevated inflammatory parameters, without signs of infection. Additional laboratory and imaging tests showed no abnormalities. We finally diagnosed her with Cogan syndrome. Our patient started oral prednisolon and methotrexate and she gradually improved., Conclusion: Cogan syndrome is a rare inflammatory condition that warrants a multidisciplinary approach by an ophthalmologist, neurologist, ENT-physician, and rheumatologist / immunologist for swift diagnosis and treatment with immunosuppressive medication. A timely recognition of the syndrome at first presentation and with new flares improves the chances of full or partial recovery.

Cogan's syndrome (CS) is a rare autoimmune vasculitis of unknown aetiology characterised by non-syphilitic interstitial keratitis, audiovestibular symptoms, sometimes systemic symptoms, and multi-organ involvement. Atypical CS has other ocular features, such as scleritis, episcelritis, retinitis, and optic neuritis. Diagnosis of CS is purely clinical without a confirmatory test. Hereby, we report a case of atypical CS presenting with features of encephalitis who was treated successfully with intravenous pulse methylprednisolone with cyclophosphamide. It is important to consider CS in the differential diagnosis of encephalitis with ocular and vestibular symptoms in young patients, as high morbidity and mortality rates are effectively lowered by early immunosuppressive treatment., (© Japan College of Rheumatology 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Cogan I syndrome is a rare disease consisting of vestibulocochlear symptoms and non-syphilitic interstitial keratitis. Although this disease was first described in 1945, its pathogenesis is still unknown. An autoimmune vasculitis etiology is currently discussed. Atypical manifestations are characterized by delayed ocular symptoms or variability of inflammatory eye symptoms. Physical examination often reveals bilateral sensorineural hearing loss. Intratympanic corticosteroid application can be successful., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

BACKGROUND Cogan syndrome is a rare autoimmune disorder associated most frequently with ocular, vestibular, and auditory involvement from presumed small vessel vasculitis. Cogan syndrome, in a significant proportion of patients, can progress to systemic symptoms, including gastrointestinal, neurologic, and musculoskeletal manifestations. Large-vessel involvement has also been described in some cases (eg, aortitis), but acute limb ischemia in the setting of this illness has been infrequently reported. CASE REPORT We present a rare case of Cogan syndrome complicated by acute vascular ischemia of the left upper extremity. A 50-year-old man presented with symptoms of severe acute pain and weakness of the left arm. The patient endorsed a diagnosis of Cogan syndrome 4 years prior in the setting of unilateral left-sided hearing loss and bilateral uveitis. A physical examination revealed pallor of the left forearm and pulselessness at the wrist. Computed tomography angiography was suggestive of vasculitis and concerns for embolic occlusion of several arterial structures of the left upper limb. After consultation with various specialists, the patient was treated with high-dose steroids, anticoagulants, and topical nitroglycerin and experienced significant clinical improvement. CONCLUSIONS Treatment of Cogan syndrome with severe systemic manifestations depends on the organ involvement and degree of extension. Our patient's presentation serves as an impressive example of systemic vasculitis with subsequent acute ischemia in the setting of this rare autoimmune disorder. In such a case, given the potential for life- or limb-threatening systemic vascular catastrophes, emergent interventions (including imaging, anticoagulation, and specialist involvement) are required to prevent untoward outcomes.

A 49-year-old Japanese man with a 2-month history of a fever, headache, and bilateral conjunctival hyperemia was admitted. His condition fulfilled the giant cell arteritis classification criteria (new headache, temporal artery tenderness, elevated ESR) and atypical Cogan's syndrome (CS) with scleritis and sensorineural hearing loss (SNHL). The interleukin (IL)-6 serum level was extremely high. Two weeks after his insufficient response of SNHL and scleritis to oral prednisolone, we administered tocilizumab (TCZ); rapid improvements in scleritis and SNHL occurred. Early IL-6 target therapy can help prevent irreversible CS-induced sensory organ damage.

Purpose: To report the histological findings and clinical course of 2 patients with microcyst-like epithelial keratopathy (MEK) associated with antibody-drug conjugate, depatuxizumab mafodotin., Methods: Case series., Results: Two patients with glioblastoma multiforme participating in a phase 3 clinical trial of the antibody-drug conjugate, depatuxizumab mafodotin, presented with bilateral MEK. Confocal imaging showed multiple large, round, hyperreflective lesions in the epithelium. Epithelial debridement was performed for symptomatic relief in both patients. Along with aggressive lubrication, bandage contact lenses, and reduction in the chemotherapeutic dose to maintenance levels, both patients experienced symptomatic improvement. However, MEK lesions recurred after re-epithelialization. Immunohistochemistry of the diseased epithelium showed immunoglobulin (Ig)G-positive granular cytoplasmic inclusions and increased cell apoptosis., Conclusions: Depatuxizumab mafodotin accumulates in the basal corneal epithelium resulting in MEK because of increased apoptosis. Frequent lubrication and bandage contact lenses can provide symptom relief., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

A 39-year-old male presented with bilateral hearing loss and progressive left eye vision loss over a 14-month period. The development of systemic symptoms including arthralgias, enlarged lymph nodes, and profound leg weakness, prompted a workup for lymphoproliferative disease, infection, and autoimmune inflammatory conditions which was unrevealing. Subsequently, the right visual acuity declined from 20/25 to 20/70 and the left to hand motions due to corneal interstitial keratitis. There was limitation of left infraduction. Neuroimaging revealed dural thickening of the internal auditory canals, cavernous sinuses, cerebellum, and along the optic nerves. There was fusiform enhancing enlargement of the left inferior and medial rectus muscles and pathologic enlargement of the left lacrimal gland. Biopsy of the left lacrimal gland and left inferior rectus revealed fibrosis and lymphocytic infiltration. The patient was diagnosed with atypical Cogan syndrome and treated with oral prednisone, with improvement in visual acuity of the right eye, motility of the left eye, and systemic weakness., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

In the present case we report on a 51-year-old patient diagnosed with Cogan syndrome. This vasculitis of variable vessel size is a rare disease that poses a major challenge for the correct diagnostics and therapy. In the classic setting, it comprises a triad of non-syphilitic interstitial keratitis as well as hearing loss with vestibular dysfunction. A vascultis-related aortitis, an uncertain, more likely degenerative structure in combination with strongly elevated inflammation parameters was misinterpreted as infective endocarditis for a long time and treated with anti-infective medications. After diagnosis the patient recovered following treatment with high-dose steroids and in the further course cyclophosphamide and tumor necrosis factor‑α blockers.

Cogan's syndrome (CS), a rare vasculitis characterized by non-syphilitic, interstitial keratitis and Ménière-like attacks, is classified into "typical" and "atypical" forms, while Takayasu arteritis (TAK) is a rare large-vessel vasculitis associated with human leukocyte antigen (HLA)-B*52. Very few cases meet both the CS and TAK classification criteria. We herein report a 53-year-old woman diagnosed with atypical CS and aortitis similar to TAK. Her 25-year-old daughter manifested TAK without symptoms of CS, and both are HLA-B*52 positive. Our case highlights the difficulties of distinguishing aortitis with atypical CS from aortitis with TAK.

Purpose of Review: Cogan's syndrome (CS) is a rare systemic vasculitis that can severely affect vision and hearing, which may also have significant systemic effects. Early recognition of this autoimmune disorder and intervention can minimize disabling and irreversible damage., Recent Findings: This article will review the varying clinical presentations of CS and emerging information of systemic disease associated with CS. We will also review recently published promising treatment outcomes using immune modulating medications. As our framework for recognizing the markers of CS and the associated systemic disorders expands, more effective guidelines and treatment options may emerge.

Cogan syndrome (CS) is a rare vasculitis seen primarily in young adults. It predominantly affects eyes, ears and the heart with characteristic findings of interstitial keratitis, sensorineural hearing loss and vestibular dysfunction. A high index of suspicion is required to diagnose this rare disorder. It is one of the few vasculitis which can involve vessels of all sizes: small, medium and large. Coexistence of inflammatory bowel disease (IBD) in Cogan syndrome has been described in the literature. Immunosuppressive agents such as corticosteroids with or without steroid sparing agents are the standard of care. Early diagnosis and treatment are the cornerstone of treatment to prevent permanent damage to the ears and eyes. We describe a patient with Cogan syndrome with large vessel vasculitis and IBD. Our patient was treated with glucocorticoids and methotrexate., Competing Interests: None

Cogan's syndrome is a rare autoimmune disease that usually affects young Caucasian adults and is classically defined as the combination of nonsyphilitic interstitial keratitis and audiovestibular symptoms resembling Meniere's disease, both of them developed in an interval of less than two years. Nevertheless, cases with atypical ophthalmologic and audiovestibular features, with systemic manifestations or affecting children and older patients have also been reported, expanding the clinical spectrum of Cogan's syndrome. Herein, we present the case of a late-onset Cogan's syndrome associated with a large-vessel vasculitis., (Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

We describe two patients in whom postsurgical refractive error occurred after routine refractive lens exchange cataract surgery with multifocal intraocular lens insertion most likely attributable to the presence of underlying corneal epithelial basement membrane dystrophy (EBMD). In Case 1, there was an unexpected hyperopic postoperative spherical equivalent refractive error of +1.50 diopters and in Case 2, a cylindrical refractive error of 2.75 diopter cylinder. We examine the possible causes of error and discuss potential management strategies to prevent and address these unpredictable postoperative outcomes. The importance of comprehensive and careful ocular surface assessment before cataract or refractive surgery as part of the presurgical workup is reemphasized. EBMD can be subtle and if overlooked, can affect the validity of biometric keratometric measurements preoperatively, resulting in an inaccurate biometry measurement, incorrect IOL selection, and reduced visual performance and patient satisfaction., (Copyright © 2019 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.)

Objective: To explore the diagnostic utility of normal tear osmolarity in patients with symptoms suggestive of dry eye disease (DED)., Methods: Prospective observational cohort study of 100 patients that underwent tear osmolarity testing (TearLab ™ ) if they endorsed one or more symptoms of potential DED. Patients were included for the study if they had a normal tear osmolarity test (value <308 mOsm/L in each eye, and an inter-eye difference <8 mOsm/L). The main outcome measure was the presence of any alternate diagnosis to explain the patient's symptoms. Results were recorded and descriptive and univariate statistics were employed., Results: Mean tear osmolarity was 293.40 mOsms/L (±6.82), with a mean absolute difference of 2.85 mOsms/L (±1.98) between the eyes. A possible alternate diagnosis was established in 89% of patients with normal tear osmolarity testing. The most frequent diagnoses included anterior blepharitis (26%) and allergic conjunctivitis (21%)., Conclusions: Common symptoms of DED overlap significantly with a wide variety of other ocular surface diseases, and a normal tear osmolarity test should increase clinical suspicion for alternate causes of those symptoms. Anterior blepharitis and allergic conjunctivitis were the most common diagnoses made in symptomatic patients with normal tear osmolarity who may have otherwise been misdiagnosed and treated for DED., (Copyright © 2018 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved.)

We describe a case of Cogan syndrome in patients with clinical manifestations, related examinations and diagnosis,treatment,and review of relevant literature. Both patients had bilateral moderate to severe sensorineural hearing loss. The collateral function test showed that there were bilateral semicircular canal paresis,and the head impulse test had a bilateral gain value between 0.09 and 0.55. It showed obvious compensatory saccade wave, the head impulse suppression test bilateral no anti-compensation saccade wave.Cogan syndrome is a rare syndrome with unspecific symptoms and early diagnosis is difficult. However, the involvement of the auditory vestibular systems are often poor prognosis and needs to be diagnosed by early diagnosis and treatment., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

We report a case of Cogan's syndrome presenting as fever of unknown origin in a 31-year-old woman who was admitted to the hospital with a 7-week history of fever, night sweats and other constitutional symptoms. The diagnosis remained elusive despite numerous investigations, and the patient subsequently developed rash, episcleritis, dizziness and sensorineural hearing loss. While initially thought to be a postinflammatory response to a previous infection, confirmation of the rash as a vasculitis together with the audiovestibular and ocular involvement led to a clinical diagnosis of Cogan's syndrome. This was further corroborated by resolution of her symptoms once immunosuppressive therapy was instituted. Early recognition of Cogan's syndrome is crucial to reducing the risk of serious complications through the timely initiation of treatment., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

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.

The constellation of ocular symptoms known as Balint syndrome is a rare disorder seen in bilateral parieto-occipital lesions and is most frequently due to arterial occlusive disease or acute hypertension. Here we present the case of a patient with tacrolimus-induced posterior reversible encephalopathy syndrome (PRES) who presented with optic ataxia, simultanagnosia, and ocular apraxia. These ocular findings, consistent with Balint syndrome, are rarely the initial presentation of PRES. This case highlights the importance of early recognition of this unusual phenomenon, as well as the importance of an individualized rehabilitation plan to maximize functional independence in these patients., Level of Evidence: V., (Copyright © 2018 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.)

Degenerative or hereditary corneal diseases are sometimes difficult to discriminate. Corneal dystrophies affect approximately 0.09 % of the population. They are identified by the IC3D classification based on their phenotype, genotype and evidence gathered for their diagnosis. Practically, the ophthalmologist manages functional symptoms, such as recurrent erosions, visual loss and amblyopia, photophobia, foreign body sensation, and sometimes pain and aesthetic concerns. Medical treatments consist of drops to promote healing, ointments, hyperosmotic agents and bandage contact lenses. Less invasive surgical treatments are used as second line therapy (phototherapeutic keratectomy, lamellar keratectomy). More invasive procedures may eventually be utilized (lamellar or penetrating keratoplasty). Anterior lamellar or endothelial keratoplasty are now preferred to penetrating keratoplasty, although the latter still remains the only possible option in some cases. Some rare dystrophies require coordinated and comprehensive medical care., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Vasculitis, characterized by inflammation of vessel walls, is comprised of heterogeneous clinicopathological entities, and thus poses a diagnostic challenge. The most widely used approach for classifying vasculitides is based on the International Chapel Hill Consensus Conference (CHCC) nomenclature system. Based on the recently revised CHCC 2012, we propose computed tomography (CT) features of vasculitides and a differential diagnosis based on location and morphological characteristics. Finally, vasculitis mimics should be differentiated, because erroneous application of immunosuppressive drugs on vasculitis mimics may be ineffective, even deteriorating. This article presents the utility of CT in the diagnosis and differential diagnosis of vasculitides.

The police brought a 65-year-old female patient to the EADU after being found 'roaming the streets' in an apparent state of confusion. This was her third admission under the same circumstances during the last 3 years. Neurological examination revealed (1) cognitive impairment, (2) oculomotor apraxia, (3) abnormal cancellation of vestibular ocular reflex, (4) mild ataxia and (5) mild hypotonia. Renal function was abnormal and liver function was normal. No retinal disturbance was found. The head CT on admission was normal for stroke and the lumbar puncture was negative for encephalitis. Her brain MRI showed 'molar tooth sign', suggestive of Joubert syndrome, which was confirmed by genetic testing showing anomalous NPHP1 gene., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Objective: The aim of this study was to systematically evaluate the evidences for treatment of Cogan's syndrome (CS), with reference to adult and younger patients described in the literature., Systematic Review Methodology: DATA SOURCES: Studies reviewed were English language original articles ranging from 1990 to 2016 reporting data for subjects with CS (typical and atypical) undergoing any systemic treatment other than steroids alone. Medline/EMBASE, Cochrane Library were searched. The full text of articles meeting selection criteria was reviewed for: study type, diagnosis, number of subjects, treatment type and duration, clinical and/or functional outcomes, possible systemic manifestation or other autoimmune syndromes combined., Results and Conclusions: The authors identified 76 relevant reports: 4 prospective studies, 2 retrospective studies, 12 case series and 58 case reports. The studies included a total of 141 new patients: based on the available data, 46 men and 50 women with a mean age of 33years (range 5-69). In the descriptive analysis adult patients (N=87) were separated from pediatric patients (<18years old) (N=17). Concerning treatment strategies, except for a first-line approach to check for a rapid remission, or for a drug sensibility often supporting the diagnosis, a long-term steroidal monotherapy is no longer recommended in CS. As in other autoimmune and rheumatologic diseases, combined treatment with steroid-sparing immunosuppressant agents, also considering "biological" drugs, is nowadays preferred. However, the evidence of clearly effective or preferable treatment options for CS remains lacking, mostly due to the rarity of the disease and to the consequent difficulty in organizing high-quality prospective trials., (Copyright © 2017. Published by Elsevier B.V.)