Speech reception after cochlear implantation for Cogan's syndrome: Case series following CARE guidelines.

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.

Paediatric Cogan´s syndrome - review of literature, case report and practical approach to diagnosis and management.

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.

Cogan's Syndrome: Clinical Presentations and Update on Treatment.

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.

Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Aspects, and Therapeutic Modalities of Cogan's Syndrome.

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.

[Corneal dystrophies]. / Les dystrophies de cornée.

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.

Cogan syndrome--pathogenesis, clinical variants and treatment approaches.

Non-syphilitic keratitis, coexisting with vestiboloauditory symptoms namely hearing loss and dizziness was first reported by Morgan and Baumgartner back in 1934. It was then ten years later when D.G. Cogan, an ophthalmologist (1908-1993) described 4 patients having the same symptoms but in addition, attacks of vertigo, tinnitus, hearing loss and ocular symptoms. This was published in Archives of Ophthalmology in 1945 and later named Cogan's syndrome (CS). Almost 20years later on, bilateral recurrent episcleritis associated with vestiboloauditory symptoms were defined to be the atypical form of CS occurring in association with rheumatoid arthritis (RA). During the coming two decades the division of CS into typical (classical) and atypical variants, based mainly on the clinical presentation of this syndrome was accepted. Typical CS manifests primarily with interstitial keratitis and hearing loss, whereas atypical CS is usually presented with scleritis, chroiditis and more frequently with systemic inflammation. Approximately, 70% of these patients have systemic manifestations, of which vasculitis is considered the pathogenic mechanism and therefore carries a less favorable prognosis than typical CS. Since then, CS was considered by many to be autoimmune or immune mediated in origin, supported mainly by the beneficial response to corticosteroids. It was only later, using well developed assays such as western blotting and immunofluorescence (IF) when antibodies to inner ear antigens, anti neutrophil cytoplasmic antibodies (ANCA) and anti-endothelial antibodies were found and described to be associated with CS.

Cogan's syndrome--clinical guidelines and novel therapeutic approaches.

Cogan's syndrome (CS) is a rare chronic inflammatory disorder, classically characterized by interstitial keratitis and sensorineural hearing loss. Recurrent episodes of inner ear disease might result in deafness. In some patients, it may also be accompanied by systemic vasculitis. Diagnosis of CS is often missed or delayed due to its rarity, the nonspecific clinical signs at onset, and the lack of a confirmatory diagnostic test. The mechanisms responsible for CS are unknown; however, in the last decade, the pathogenesis has been somewhat elucidated, suggesting that the disease is a result of inner ear autoimmunity. The autoimmune hypothesis postulates the triggering of the disease by a viral infection via a number of mechanisms, which are mainly as follows: antigenic mimicry, self-perpetuating inflammation by cytokine release, and unveiling hidden epitopes. Aside from its clinical resemblance to other autoimmune disorders, some autoantigen has apparently been identified, namely, CD148 and connexine 26. Treatment should begin as early as possible. While treatment is based primarily on glucocorticoids, there is no standard alternative for patients who respond poorly. Failure of conventional treatment could lead to profound sensorineural hearing loss. From the limited data we have, infliximab seems to be the most promising biological remedy, enabling steroid tapering and leading to improvement in auditory/ocular disease, with better results when administered in early stages. Proposed guidelines for the use of infliximab in CS are found in the last table of the review, in an attempt to define the proper timing for initiating infliximab treatment in order to avoid permanent disability.

[Cogan syndrome]. / Syndrome de Cogan.

Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory ocular disease (mainly interstitial keratitis) and audiovestibular symptoms (mainly acute-onset sensorineural hearing loss) in the setting of a negative work-up for syphilis. The diversity of the ocular and audiovestibular manifestations reported in the literature in atypical Cogan's syndrome should make one cautious before accepting the diagnosis as the disease may mimic various other systemic disorders. Systemic corticosteroids are always the most widely used and successful therapy. For patients requiring high and prolonged doses, additional immunosuppression is appropriate. Methotrexate is the first-line steroid sparing agent. However, patients without systemic disease or severe eye disease unmanageable by topical corticosteroids should not be subjected to protracted courses of corticosteroids or immunosuppressive agents, particularly when little gain in hearing is obtained with their use. The effect of TNF-alpha blockers was recently investigated. Infliximab might be an alternative therapy in cases of failure of corticosteroids and immunosuppressive therapy. However, treatment might be more effective when started at an early stage of the disease, when the lesions are still reversible.

[Successful early treatment in a case of Cogan's syndrome].

We report a 53-year-old male with Cogan's syndrome. He was admitted to our hospital because of a fever of 2-weeks duration, blurred vision for 10 days, hypoacusis, and numbness of the left hand for 3 days. In addition to uveitis, hypoacusis, and aseptic meningitis, multiple mononeuropathy was diagnosed based on a nerve conduction study. Furthermore, positron emission tomography/computed tomography (PET/CT) revealed diffuse aortitis. Accordingly, the patient was diagnosed with Cogan's syndrome. After starting steroid-pulse therapy followed by 1 mg oral prednisolone/kg/day, the uveitis and hypoacusis improved immediately, while the peripheral neuropathy persisted until effectively treated with intravenous gamma globulin therapy. Prompt steroid therapy for Cogan's syndrome based on a diagnosis made using PET/CT prevented progression of the hypoacusis.

Complete restoration of auditory impairment in a pediatric case of Cogan's syndrome: report of a rare case with long-term follow-up and literature review.

Cogan's syndrome is characterized by interstitial keratitis, vestibular impairment and hearing loss, commonly bilateral. Many patients, especially children, experience a delay in proper diagnosis which may delay treatment and thus impact on prognosis of hearing restoration. Less than 10 pediatric cases of Cogan's syndrome have been reported in literature and only five of them were reported with long-term follow-up. We report an extremely rare pediatric case of Cogan's syndrome typical form with long-term follow-up and evaluation of hearing impairment. Cogan's syndrome must be familiar to otorhinolaryngologists, pediatricians and ophthalmologists because early diagnosis and rapid administration of the proper therapy increase the probability of recovering hearing loss.

[Cogan's syndrome--an interdisciplinary diagnostic challenge]. / Cogans syndrom--en tværdisciplinær diagnostisk udfordring.

Cogan's syndrome is a rare autoimmune-like disorder characterised by nonsyphilitic interstitial keratitis and Meniere's syndrome-like audiovestibular function disorder. David G. Cogan initially described the syndrome in 1945. In 1980 Haynes et al proposed diagnostic criteria for patients with other ocular symptoms and suggested this to be atypical Cogan's syndrome. We report a case of a 43 year-old woman with atypical Cogan's syndrome. The patient received a cochlear implant prior to diagnosis.

Cogan's syndrome with left main coronary artery occlusion.

Cogan's syndrome is a rare idiopathic chronic inflammatory disease of the eye and the inner ear in young adults. Cogan's syndrome can be associated with large vessel vasculitis. We report a young female, with a history of interstitial keratitis and audiovestibular disease, who presents with large vessel vasculitis with left main coronary artery occlusion and develops heart failure. Cogan's syndrome was diagnosed on the basis of the presence of large vessel vasculitis with the typical inner ear and ocular involvement.