Surgical Treatment in a Case of Cogan's Syndrome Complicated With Proximal Aortic Vasculitis

Kaya, Mehmet; Erkanlı, Korhan; Kılınç, Furkan; Sar, Mehmet; Bakır, İhsan

doi:10.1016/j.athoracsur.2015.01.049

Cited by 7 publications

(7 citation statements)

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“…The association of these symptoms was first described in 1934 [Morgan and Baumgartner, 1934], and Cogan’s syndrome was named after David Cogan, who described an additional four cases [Cogan, 1945]. Systemic vasculitis including aortitis may occur during the course of Cogan’s syndrome [Gluth et al, 2006; Kaya et al, 2015]. Aortitis with aortic insufficiency, which may lead to congestive heart failure has been reported to occur in about 10 % of patients with Cogan’s syndrome [Grasland et al, 2004; Gluth et al, 2006; Kessel et al, 2014].…”

Section: Discussionmentioning

confidence: 99%

Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation

et al. 2017

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The Cogan syndrome is a rare disorder characterized by nonsyphilitic interstitial keratitis and audiovestibular symptoms. Profound sensorineural hearing loss has been reported in approximately half of the patients with the Cogan syndrome resulting in candidacy for cochlear implantation in some patients. The current study is the first histopathologic report on the temporal bones of a patient with the Cogan syndrome who during life underwent bilateral cochlear implantation. Preoperative MRI revealed tissue with high density in the basal turns of both cochleae and both vestibular systems consistent with fibrous tissue due to labyrinthitis. Histopathology demonstrated fibrous tissue and new bone formation within the cochlea and vestibular apparatus, worse on the right. Severe degeneration of the vestibular end organs and new bone formation in the labyrinth were seen more on the right than on the left. Although severe bilateral degeneration of the spiral ganglion neurons was seen, especially on the right, the postoperative word discrimination score was between 50 and 60% bilaterally. Impedance measures were generally higher in the right ear, possibly related to more fibrous tissue and new bone found in the scala tympani on the right side.

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Section: Discussionmentioning

confidence: 99%

Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation

et al. 2017

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“…Systemic vasculitis is seen in 15–21% of the patients [ 6 ]. Aortic root vasculitis, which is reported in 10% of CS patients, can result in life-threatening complications, such as aortic aneurysms, dissection, and insufficiency [ 13 – 15 ]. Mitral insufficiency was also reported [ 16 ].…”

Section: The Clinical Spectrum Of Cogan's Syndromementioning

confidence: 99%

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

Shamriz

Tal

Gross

2018

Journal of Immunology Research

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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.

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“…Vasculitis of small, medium, and large vessels is considered as the underlying pathogenic mechanism for systemic presentation of CS, 8 , 10 which may involve the cardiovascular, neurological, and gastrointestinal systems. 6 The most typical cardiovascular manifestation of CS is aortitis, 9 , 11 leading to kidney and aortic insufficiency, reported in ~10% of patients, and congestive heart failure ( Figure 1 ). 3 , 12 Neurological manifestations may include hemiparesis or hemiplegia following cerebral vascular accidents, and aphasia, due to transient ischemic events.…”

Section: Clinical Featuresmentioning

confidence: 99%

Optimal management of Cogan’s syndrome: a multidisciplinary approach

D’Aguanno¹,

Ralli²,

Vincentiis³

et al. 2017

JMDH

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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.

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Surgical Treatment in a Case of Cogan's Syndrome Complicated With Proximal Aortic Vasculitis

Cited by 7 publications

References 6 publications

Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation

Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation

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

Optimal management of Cogan’s syndrome: a multidisciplinary approach

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Surgical Treatment in a Case of Cogan's Syndrome Complicated With Proximal Aortic Vasculitis

Cited by 7 publications

References 6 publications

Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation

Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation

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

Optimal management of Cogan&rsquo;s syndrome: a multidisciplinary approach

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Optimal management of Cogan’s syndrome: a multidisciplinary approach