Corneal autoimmunity in Cogan's syndrome? Report of two cases.

Autoimmune reactivity against corneal antigens is described in two patients with Cogan's syndrome, a nonsyphilitic deep interstitial keratitis with vestibuloauditory symptoms. In both cases corneal antibodies were found at the beginning or during an exacerbation of the disease. After administration of high doses of corticosteroids the corneal antibodies diminished. Interstitial keratitis can generally be controlled by local or systemic corticosteroids. The effect of corticosteroid therapy on the audiovestibular symptoms is variable. The possibility of an autoimmune pathogenesis of Cogan's syndrome is discussed.     

Cogan-I-syndrome. A rare differential diagnosis in progressive sensorineural hearing loss

We report on two patients affected by Cogan's syndrome since infancy.Cogan's syndrome is described as the association of abrupt or intermittent worsening of vestibulo-auditory function and non-syphilic interstitial keratitis or, in patients with "atypical" Cogan's syndrome, various severe, inflammatory eye diseases. The first patient was affected with different inflammatory eye diseases from her fifth year and became blind in one eye when she was 7 years old. At that time, a deterioration of her hearing ability was also diagnosed. The hearing loss progressed to a severe hearing disorder. The second patient suffered from different inflammatory eye diseases from his second year. When he was 3 years old,he was diagnosed with a severe hearing disorder that progressed.Cogan's syndrome was diagnosed when he was 13 years old. The recognition of Cogan's syndrome is particularly complex in young patients. The difficulty of the diagnosis requires a search for ophthalmic diseases in young children with acute and progressive vestibulo-auditory symptoms.     

Cogan syndrome: sudden, bilateral high-grade hearing loss

We report 2 cases of Cogan's syndrome. One female patient had a typical interstitial keratitis and bilateral hearing impairment, the other had dramatically progressive deafness, combined with failure of the left vestibule and severe conjunctivitis resistant to treatment and otitis externa. The typical Cogan's syndrome with interstitial keratitis developed only a few weeks later. The disease is caused by an auto-immune response. The hearing impairment is mainly due to endolymphatic hydrops. Corticosteroids are the treatment of choice.     

Side selection for cochlear implantation in a case of Cogan's syndrome

Cogan's syndrome is a rare clinical entity that is characterised by non-syphilitic interstitial keratitis and audiovestibular symptoms. The cause of Cogan's syndrome is considered to be autoimmune disease, which is supported by the resolution of hearing loss after steroid treatment, and the association with other autoimmune diseases. The sensorineural hearing loss of Cogan's syndrome is progressive over a few months, and sudden, bilateral deafness often occurs, which may be an indication for cochlear implantation. This paper presents the case of a young woman suffering from Cogan's syndrome and sudden, bilateral deafness. With reference to this case, we describe problems regarding cochlear implantation for Cogan's syndrome and radiological findings aiding selection of cochlear implantation side.     

Transtympanic gentamicin perfusion for the treatment of Meniere's disease

Meniere's syndrome is defined as the clinical disorder associated with the histopathological finding of endolymphatic hydrops (ELH). Clinically, Meniere's syndrome includes the following features: recurrent, spontaneous episodic vertigo; hearing loss; aural fullness; and tinnitus. Recognized causes of Meniere's syndrome include: 1) idiopathic, also known as Meniere's disease; 2) posttraumatic, following head injury or ear surgery; 3) post-infectious or delayed-onset Meniere's syndrome following a viral infection, usually mumps or measles; 4) late stage syphilitic; 5) classic Cogan's syndrome with episodic vertigo, hearing loss, interstitial keratitis, without syphilis; 6) variant Cogan's syndrome with episodic vertigo, hearing loss, uveitis or other ocular inflammation and without syphilis. Although Meniere's disease is by far the most common cause of Meniere's syndrome and the terms are often used interchangeably, it should be remembered that a patient has an idiopathic etiology only when the known causes have been excluded.     

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.     

The Cogan syndrome

Cogan's Syndrom is the clinical entity of non syphilitic interstitial keratitis with vestibuloauditory symptoms. Although the disease is not rate the pathohistological findings in temporal bones are only reported twice. Our communication deals with observations made on a temporal bone out of O. Mayer's collection. The most striking findings are: the endolymphatic hydrops, the degeneration of the organ of Corti, the pathology of the ductus reuniens, the fibrosis within the cisterna perilymphatica, the destruction of the maculae and the most uncommon ossification within the scala vestibuli of the basal turn. The vessels seem to show signs of angitis.     

Das Cogan-Syndrom

Background

Cogan’s disease is a diagnostic challenge. The typical form of this chronic inflammatory disease is characterized by non-syphilitic interstitial keratitis and acute audiovestibular impairment.

Case reports

The first patient had “typical” symptoms of Cogan’s syndrome with recurrent keratoconjunctivitis, severe hearing loss and vertigo. Systemic cardiovascular manifestations occurred in the further course of the disease with multiple vascular stenoses of the carotids and vessels of the lower limbs. The second patient had “atypical” symptoms of Cogan’s disease with progressive bilateral hearing loss and vestibular deficits, unilateral conjunctivitis and orbital swelling and unspecific symptoms, such as fever and headaches. Therapy with systemic and local corticoids was successful in both cases concerning ocular and systemic symptoms. The first patient had received additional therapy with methotrexate, but had already become deaf before therapy had started. Cochlear implantation was successfully carried out. In the second patient hearing loss was stationary under therapy and the patient could be provided with hearing aids.

Conclusion

Because of obligatory cochleovestibular symptoms the otorhinolaryngologist has a key position in diagnosis and therapy of this rare entity. Early diagnosis is a crucial prognostic factor concerning preservation or restitution of hearing function.     

Atypical Cogan syndrome. Clinical and laboratory spectrum. Report of 2 cases

We show two atypical Cogan's syndrome cases in forty-seven and fifty-six years old individuals. Characteristical symptoms of the syndrome were detected in both (interstitial keratitis, sensorineural deafness and vestibular dysfunction), but they could be considered as unusual because of the association of systematic symptoms and an onset with hearing loss. In both cases there was an increase in erythrosedimentation rate and immunoglobulins levels, as well as high titles for rheumatoid factor and non-specific autoantibodies. By means of a Western-blot technique, several chochlear antibodies with distinct molecular weight were detected, but the 68 KD appeared in both. Metil-prednisolone treatment did not avoid ocular and auditive reactivation in ne of the patients.     

Cogan's syndrome: audiovestibular involvement and prognosis in 18 patients

The experience at the Mayo Clinic with 18 patients who had Cogan's syndrome is reviewed. Typically, the illness began with systemic symptoms. The ocular and audiovestibular symptoms included ocular discomfort and redness, photophobia, and fluctuating sensorineural deafness with imbalance. Each patient had interstitial keratitis; the ocular symptoms periodically recurred but responded to therapy. Thirteen patients who had fluctuating bilateral sensorineural deafness subsequently suffered total bilateral deafness. Two patients who are unilaterally deaf have good residual hearing. Two other patients regained hearing within normal limits after treatment with corticosteroids. No hearing symptoms were observed in one patient who had severe vertigo and whose condition is stable. Seventeen patients had vertigo: 11 have no labyrinthine function, 1 had absent responses on vestibular testing initially but currently demonstrates good responses, and the 5 others have remained stable with diminished vestibular function. If Cogan's syndrome is diagnosed early and treatment with corticosteroids is initiated promptly, hearing can be stabilized; otherwise, the prognosis for auditory function is poor.     

Cogan síndrome. Audio-vestibular findings in a patient and literature review

Cogan Sindrome is described as the association of non-syphilic interstitial keratitis with vestibulo-auditory symptoms in its typical form. We are reporting a case corresponding to a 32 years old male who consulted at first place for ophthalmic clinic and, two months later, affected of bilateral neurosensorial hearing loss with tinnitus and vestibular dysfunction. In spite of the corticoid treatment our patient has not improved. His hearing disorder was even worse after one year-follow up. A bibliographic review has been performed related to this pathology whose etiology is still unknown.     

Das Cogan-Syndrom

Classical Cogan's syndrome is a disease of the inner ear with participation of the eyes, typically involving keratitis. If no objectively assessable optical symptoms are present, a diagnosis is difficult. Additional nonspecific symptoms can, combined with inner ear participation, suggest Cogan's syndrome. Between 2001 and 2003, we documented the course of two patients with Cogan's syndrome. As indicated in the literature, organ related symptoms could be related to a generalized vascular illness. In addition to the usual otological symptoms with cochleovestibular dysfunction and symptoms of typical and atypical ocular manifestations, other non-specific changes were found. Interstitial keratitis was diagnosed in one patient while in the second objective ophthalmological symptoms failed. The symptoms could be improved by systemic and local administration of corticosteroids and immunosuppressive therapy, however, progression of inner ear deafness could not be stopped in one patient and a cochlear implant was necessary.     

Cogan's syndrome: clinical significance of antibodies against the inner ear and cornea.

The aim of this study was to evaluate the pathological significance of antibodies against cornea and inner ear tissue in the development of audiovestibular and ocular symptoms in patients with Cogan's syndrome (CS). We analysed the serum of 5 CS patients for binding of IgM and IgG to fresh cryosections of rat labyrinth (semicircular canals, ampulla, utricle, saccule) and cornea by indirect immunofluorescence (IF). The predominant pattern of anti-corneal IgM was staining of the superficial cell layer of the non-keratinizing squamous epithelium. IgM against cornea was found in 3 patients, all of whom had bilateral inflammatory eye signs at the start of the disease. However, IgM was also detected in the chronic stage of the disease when no clinical signs of eye involvement were apparent. The study includes the first follow-up examination of anti-corneal IgM and IgG antibodies during a complete episode of active CS. During the first episode of CS in 1 patient, anti-corneal IgM became detectable 1 week after the onset of interstitial keratitis and 3 weeks after the onset of audiovestibular symptoms. It increased over several weeks and then fell to very low levels. However, at no time was anti-corneal IgG found. In the course of follow-up examinations, the serum of 4 patients intermittently contained low titre IgG antibodies against inner ear labyrinthine tissue, but without any clear correlation with the active stages of CS. In addition, high-resolution MRI (HR-MRI) of the inner ear was performed in the acute and chronic stages of CS to evaluate the activity of CS. In the acute stage, HR-MRI revealed abnormal MRI signals in the vestibule, semicircular canals, vestibular nerve, or cochlea. In the chronic stage, patients showed narrowing or occlusion of semicircular canals and the cochlea on the 3D-CISS images, but no high signal lesions (T1) and no enhancement. Antibodies against cornea or labyrinthine tissue were not consistently detected in CS and the level of organ-specific antibodies did not correlate with the activity of the disease.     

Cogan's syndrome. Apropos of a case

The syndrome of Cogan associate a non syphilic interstitial keratitis and a bilateral deafness of perception. Referring to a case, the authors review the clinical pathogenic and the different immunological exams.     

Temporal bone pathology in a case of cogan's syndrome

Cogan's syndrome (CS) presents typical and atypical types. Typically, there are episodes of non-syphilitic keratitis and audiovestibular dysfunction. Atypically, there are inflammatory changes in other eye structures and other organ systems, particularly the cardiovascular system. The temporal bone pathology in a case of CS shows changes that are similar to those observed in other autoimmune disorders associated with audiovestibular dysfunction. The following pathologic features characterize autoimmune inner ear disease: 1. acute labyrinthitis resulting in atrophy of inner ear tissues including the sense organs and their supporting structures; 2. endolymphatic hydrops; 3. focal and diffuse proliferation of fibrous tissue and bone; and 4. retrograde neuronal degeneration. These pathologic findings are consistent with an inflammatory (and possibly ischemic) attack on the membranous labyrinth.     

Atypical Cogan's syndrome: report of two cases and revision of literature

Cogan's syndrome is a rare autoimmune disease with systemic involvement. It appears in young people and has two presentations: the typical form with keratitis, sudden deafness with or without vestibular syndrome, and the atypical form with different non keratitic ocular diseases and a great variety of systemic symptoms in relation with the autoimmune etiology of the process. Cogan's syndrome has a bad prognosis and deafness appears in 25% of the cases with the right treatment and in 60% of patients without treatment. The best treatment is systemic and ocular corticotherapy. The second treatment of choice is cyclophosphamide or cyclosporine A. We present two cases of atypical Cogan's syndrome with unilateral deafness in both.     

Cogan syndrome. Case report, review of the literature, therapy

We present a case report of Cogan's syndrome and a review of the literature. Cogan's syndrome is most likely due to autoimmune disease primarily affecting the vestibulocochlear organ and cornea and secondarily resulting in systemic disease. 40% of patients develop bilateral complete deafness and 70% show signs of systemic disease mostly in the form of cardiovascular symptoms. The mortality rate of 10% is mainly due to vasculitis. It is important to consider Cogan's syndrome in the differential diagnosis of sudden hearing loss accompanied by ocular signs in young patients, as high morbidity and mortality rates are effectively lowered by early immunosuppressive treatment.     

Clinical diagnosis of immune inner-ear disease

The purpose of this study was to develop a high-risk clinical profile of patients with autoimmune (immune-mediated) inner-ear disease. The records of 52 patients diagnosed over the past 5 years were reviewed. Age, sex, bilateral versus unilateral involvement, otologic symptoms, concomitant systemic immune disease, and presenting clinical diagnoses were recorded. The presenting diagnoses were Cogan's syndrome, Meniere's syndrome, Dandy's syndrome without hearing loss, or progressive sensorineural hearing loss without dizziness. Because Cogan's and Dandy's syndromes were relatively uncommon, the typical high-risk clinical profile was a middle-aged patient (often female) with bilateral, asymmetric, progressive sensorineural hearing loss, with or without dizziness, and occasional systemic immune disease such as rheumatoid arthritis. When a more common clinical diagnosis cannot be reached in suspicious patients, immune laboratory tests should be obtained and a trial of immunotherapy offered. Positive test results and beneficial response to therapy support a presumptive diagnosis of immune inner-ear disease.     

Cochlear implantation in patients with Cogan's syndrome: a review of four cases

Cogan's syndrome is a rare disorder that is characterized by a rapid onset of bilateral severe sensorineural hearing loss associated with vertigo and tinnitus, ocular involvement (most commonly an interstitial keratitis) and variable systemic symptoms. We review our experience with four cases treated in our department. We describe their symptoms, the evolution of their disease, the diagnosis, and the radiological investigations with computed tomography and/or magnetic resonance imaging. When medical treatment with corticosteroids failed and no benefit could be obtained with hearing aids at each patient's end stage, all patients received a cochlear implant with excellent results. The considerations for implantation in these patients are discussed.     

Mycophenolate mofetil as a treatment of steroid dependent Cogan's syndrome in childhood

Cogan's syndrome is a rare chronic inflammatory disorder which typically associates an ophthalmological and vestibulocochlear involvement, with a risk of systemic symptoms in 50-70% of cases. Autoimmune origin has been consolidated by the recent discoveries of Lunardi as a result of the dysregulation of the response of B and T lymphocytes. We report here a pediatric case of Cogan's syndrome, with long term follow-up and complete vestibulocochlear and ophthalmologic examination. During the acute phase, early steroids treatment (prednisone 1 mg/kg per day) was effective on ocular lesions and hearing loss. The patient required high steroid doses to maintain remission, suggesting the necessity for steroid sparing immunosuppressive agents. We introduced mycophenolate mofetil (MMF), an immunosuppressive agent mainly used in solid organ transplantation. After steroid withdrawal the patient remained in complete remission on MMF. This is the first report of MMF treatment in Cogan's syndrome. MMF produces a targeted inhibition of the proliferation of B and T lymphocytes, particularly interesting in Cogan's syndrome. Low long term toxicity and overall good tolerance make MMF a new treatment option in steroid dependent Cogan's syndrome.