Cogan's syndrome: An autoimmune inner ear disease

Greco, Antonio; Gallo, Andrea; Fusconi, Massimo; Magliulo, Giuseppe; Turchetta, Rosaria; Marinelli, C.; Macrì, Giulia; Virgilio, Armando De; Vincentiis, Marco de

doi:10.1016/j.autrev.2012.07.012

Cited by 106 publications

(66 citation statements)

References 48 publications

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“…While defining feature of Behçet's disease is recurrent oral ulceration, Cogan syndrome resembles Meniere's disease [12,13]. Though three fourth of them were familiar about these disease only one fifth had ever suspected and shown confidence in evaluation of these disease.…”

Section: Discussionmentioning

confidence: 99%

Otolaryngologic Manifestations of Various Rheumatic Diseases: Awareness and Practice Among Otolaryngologists

Gera

Kumar

2015

Indian J Otolaryngol Head Neck Surg

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Wegener's granulomatosis, Sarcoidosis, Sjög-ren's Syndrome, Behçet disease, Systemic lupus erythematosus, relapsing polychondritis, Churg-Strauss syndrome and Cogan syndromes are rheumatic diseases commonly encountered by otolaryngologists. The present study was designed to assess the understanding and practice about various ENT problems of rheumatic diseases amongst otolaryngologists. A group of 29 otolaryngologists was asked to fill questionnaire which was based on knowledge and practice of otolaryngologists about various otolaryngologic manifestations of rheumatic diseases. Chronic sinusitis was most frequently seen followed by recurrent oral ulcers while spontaneous septal perforation was most infrequently seen symptom by participants. Otolaryngologists showed high index of suspicion and confidence for Wegener's granulomatosis in comparison to other rheumatic diseases. There was no difference noted in practice of otolaryngologists of tertiary care centers and other practitioners. Eighty two percent of participants felt, they were not adequately trained about these diseases. Most of them (93 %) felt that knowledge of such diseases is also necessary for post graduate training in Otolaryngology. Though otolaryngologists are aware of otolaryngologic manifestations of Rheumatic diseases but their index of suspicion, practical implication of knowledge and confidence for evaluation of such diseases is low. Educational sector should define a better medical curriculum for training in Otorhinolaryngology, head and neck surgery.

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Section: Discussionmentioning

confidence: 99%

Otolaryngologic Manifestations of Various Rheumatic Diseases: Awareness and Practice Among Otolaryngologists

Gera

Kumar

2015

Indian J Otolaryngol Head Neck Surg

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“…Of the 250 cases of CS that have been reported in the literature, about 30% developed an associated inflammatory disease such as aortitis, rheumatoid arthritis, sarcoidosis, or, such as in the case of our patient, Crohn's disease [2][3][4]. Therefore, patients with CS require a complete extensive evaluation at diagnosis and during follow-up in order to identify potential co-occurring disorders and complications, including frequent laboratory studies such as CBC, urinalysis, creatinine, electrolytes,…”

Section: Discussionmentioning

confidence: 68%

“…Most cases evolve to unilateral or, more frequently, bilateral deafness within two years [2]. Recent evidence suggests that CS pathogenesis involves autoantibodies against corneal and inner ear proteins, CD148 and connexin 26, which have been detected in the serum of affected patients [3].…”

Section: Discussionmentioning

confidence: 99%

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Fluctuating Hearing Loss Responding to Corticosteroids: A Case of Cogan’s Syndrome Concomitant with Crohn’s Disease

Desmarais¹,

Laskine²

2016

J Clin Case Rep

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Hearing loss has a broad differential diagnosis. Hearing impairment responding to corticosteroids is suggestive of an underlying autoimmune cause. We report a rare case of Cogan's syndrome in a 28-year-old woman with no previous personal medical history. She had experienced two years of fluctuating hearing loss and bilateral keratitis that would respond to high-dose prednisone prescribed by her otorhinolaryngologist. We performed a complete work-up and ruled out most common causes of autoimmune inner ear diseases. Patient was diagnosed with Cogan's syndrome based on clinical findings and prescribed corticosteroid-sparing immunosuppressive medication. During follow-up, she developed worsening fatigue, new onset anemia, and melena. Endoscopic and pathologic findings were diagnostic of Crohn's disease. This case emphasizes the high prevalence of concomitant autoimmune diseases.

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“…This listening behavior appears to increase neural activity corresponding to the intact ear and, at the same time, reduce neural connections between the affected cochlea and auditory cortex 22 . In order to prevent this maladaptive cortical change induced by 'non-use', it appears to be beneficial for SSHL patients to perform 'constraint-induced sound therapy (CIST) , head trauma, autoimmune inner ear disease 8,9 , Cogan's syndrome 27,28 , genetic diseases 29 , ototoxic drugs 30 , retrocochlear disorders related to vestibular schwannoma 31 , auditory neuropathy 32 , or stroke 33 .…”

Section: Introductionmentioning

confidence: 99%

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

Sekiya

Fukushima

Teismann

et al. 2016

JoVE

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Sudden sensorineural hearing loss (SSHL) is characterized by acute, idiopathic hearing loss. The estimated incidence rate is 5-30 cases per 100,000 people per year. The causes of SSHL and the mechanisms underlying SSHL currently remain unknown. Based on several hypotheses such as a circulatory disturbance to the cochlea, viral infection, and autoimmune disease, pharmaco-therapeutic approaches have been applied to treat SSHL patients; however, the efficacy of the standard treatment, corticosteroid therapy, is still under debate. Exposure to intense sounds has been shown to cause permanent damage to the auditory system; however, exposure to a moderate level enriched acoustic environment after noise trauma may reduce hearing impairments. Several neuroimaging studies recently suggested that the onset of SSHL induced maladaptive cortical reorganization in the human auditory cortex, and that the degree of cortical reorganization in the acute SSHL phase negatively correlated with the recovery rate from hearing loss. This article reports the development of a novel neuro-rehabilitation approach for SSHL, "constraint-induced sound therapy (CIST)". The aim of the CIST protocol is to prevent or reduce maladaptive cortical reorganization by using an enriched acoustic environment. The canal of the intact ear of SSHL patients is plugged in order to motivate them to actively use the affected ear and thereby prevent progress of maladaptive cortical reorganization. The affected ear is also exposed to music via a headphone for 6 hr per day during hospitalization. The CIST protocol appears to be a safe, easy, inexpensive, and effective treatment for SSHL.

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Cogan's syndrome: An autoimmune inner ear disease

Cited by 106 publications

References 48 publications

Otolaryngologic Manifestations of Various Rheumatic Diseases: Awareness and Practice Among Otolaryngologists

Otolaryngologic Manifestations of Various Rheumatic Diseases: Awareness and Practice Among Otolaryngologists

Fluctuating Hearing Loss Responding to Corticosteroids: A Case of Cogan’s Syndrome Concomitant with Crohn’s Disease

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

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