Fever of unknown origin, bilateral sensorineural hearing loss with canal paresis and uveitis with iridocyclitis and episcleritis: a case of Cogan’s syndrome

Importance. Rituximab was not effective in ameliorating the hearing loss in a patient with atypical Cogan’s syndrome.Observations. We report the case of a patient who developed acute bilateral uveitis and sensorineural hearing loss. A diagnosis of atypical Cogan’s syndrome was made. The patient’s hearing loss did not improve despite high dose steroids and azathioprine. Rituximab was administered given a recent report of its efficacy in a patient with refractory disease; however, our patient’s hearing loss did not improve.Conclusion. Hearing loss in Cogan’s syndrome is difficult to treat. Though rituximab was ineffective in our case, earlier administration in the disease course could be effective for future patients.

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Sensorineural Hearing Loss of Suspected Autoimmune Etiology: Two Cases of Cogan’s Syndrome

Korean Journal of Otorhinolaryngology - Head and Neck Surgery ◽

10.3342/kjorl-hns.2021.00437 ◽

2021 ◽

Vol 64 (12) ◽

pp. 943-948

Author(s):

Jungmin Ahn ◽

Brian Kim ◽

Kyoung Rai Cho ◽

Young-Soo Chang

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Immunosuppressive Drugs ◽

Sensorineural Hearing ◽

Systemic Steroid ◽

Ocular Involvement ◽

Cogan’S Syndrome ◽

Steroid Administration ◽

Cogan's Syndrome ◽

Sparing Effect

Cogan’s syndrome is a rare inflammatory disease characterized by non-syphilitic keratitis and vestibulo-auditory symptoms including hearing loss, tinnitus, and vertigo. Although its precise pathogenesis is not known, Cogan’s syndrome is generally considered an autoimmune disease. This hypothesis is supported by the frequently successful remission of hearing loss after steroid administration and the association with other autoimmune disorders such as rheumatoid arthritis. Medical treatment of Cogan’s syndrome depends on disease severity and on how extensive the disease is. The involvement of inner ear pathology requires systemic corticosteroid therapy. In cases of treatment failure or the need for a corticosteroid-sparing effect, other immunosuppressive drugs can be used. We experienced two patients with typical Cogan’s syndrome, presenting bilateral progressive sensorineural hearing loss and dizziness with ocular involvement, which we have successfully treated with systemic steroid administration and immunosuppressive therapy.

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Atypical Cogan's Syndrome with Steroid-Responsive Sensorineural Hearing Loss

AUDIOLOGY JAPAN ◽

10.4295/audiology.47.175 ◽

2004 ◽

Vol 47 (3) ◽

pp. 175-180

Author(s):

Tsukasa Ito ◽

Tomoo Watanabe ◽

Masaru Aoyagi

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Cogan’S Syndrome ◽

Cogan's Syndrome

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Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss

Russian Journal of Occupational Health and Industrial Ecology ◽

10.31089/1026-9428-2019-59-9-624-625 ◽

2020 ◽

pp. 624-624

Author(s):

S. I. Ereniev ◽

O. V. Plotnikova

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Biological Age ◽

Biological Aging ◽

Vibration Disease ◽

Bilateral Sensorineural Hearing Loss

Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss were studied. The biological age of patients exceeded the calendar age by an average of 7.36±0.36 years and the proper biological age by 10.79±0.72 years. The rate of biological aging of the examined patients was 1.14±0.08 times higher than the rate of aging of their healthy peers.

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Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

The Journal of Laryngology & Otology ◽

10.1017/s0022215113001047 ◽

2013 ◽

Vol 127 (7) ◽

pp. 708-711 ◽

Cited By ~ 3

Author(s):

A C Hall ◽

A C Leong ◽

D Jiang ◽

A Fitzgerald-O'Connor

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Cochlear Implantation ◽

Skin Lesions ◽

Sudden Hearing Loss ◽

Sudden Onset ◽

Sensorineural Hearing ◽

Urticarial Vasculitis ◽

Wells Syndrome ◽

Bilateral Sensorineural Hearing Loss

AbstractBackground:Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.Method:To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.Results:The patient underwent a cochlear implantation with a modest outcome.Conclusion:Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

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Comprehensive medical evaluation of pediatric bilateral sensorineural hearing loss

Laryngoscope Investigative Otolaryngology ◽

10.1002/lio2.657 ◽

2021 ◽

Author(s):

Suat Kılıç ◽

Malek H. Bouzaher ◽

Michael S. Cohen ◽

Judith E. C. Lieu ◽

Margaret Kenna ◽

...

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Medical Evaluation ◽

Bilateral Sensorineural Hearing Loss

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Is simultaneous bilateral mastoidectomy ever advisable?

The Journal of Laryngology & Otology ◽

10.1017/s0022215107006330 ◽

2007 ◽

Vol 121 (11) ◽

pp. 1041-1047 ◽

Cited By ~ 5

Author(s):

J J Klemens ◽

E Mhoon ◽

M Redleaf

Keyword(s):

Hearing Loss ◽

Decision Tree ◽

Sensorineural Hearing Loss ◽

Chart Review ◽

Historical Data ◽

Sensorineural Hearing ◽

Opinion Survey ◽

Potential Patient ◽

Simultaneous Procedures ◽

Bilateral Sensorineural Hearing Loss

AbstractIntroduction:We report our experience with bilateral, simultaneous tympanomastoidectomies and the results of an opinion survey of otologists.Methods:A chart review of 116 tympanomastoidectomies revealed 12 patients who underwent bilateral, simultaneous tympanomastoidectomies. An opinion survey generated 121 responses.Results:Of the 12 patients, none suffered any outcome which would have been avoided by staging the procedures. Twenty-three of 24 operated ears had the same or better hearing post-operatively. Of the survey respondents, 74 felt that performing bilateral, simultaneous tympanomastoidectomies was unsafe, largely because of the risk of bilateral sensorineural hearing loss.Discussion:Although bilateral, simultaneous tympanomastoidectomies carry double the risk of unilateral sensorineural hearing loss, compared with the unilateral procedure, the risk of bilateral sensorineural hearing loss is only 0.006–0.2 per cent, as derived mathematically from historical data. Respondents to the survey were mostly opposed to bilateral, simultaneous tympanomastoidectomies, but even those opposed gave indications for simultaneous procedures. This finding probably reflects an ambivalence about the theoretical risks of the operation versus the potential patient benefits. A decision tree for proceeding to the second case is presented.

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Cochlear nerve anomalies in paediatric single-sided deafness – prevalence and implications for cochlear implantation strategies

The Journal of Laryngology & Otology ◽

10.1017/s002221512000225x ◽

2020 ◽

pp. 1-4

Author(s):

K Pollaers ◽

A Thompson ◽

J Kuthubutheen

Keyword(s):

Magnetic Resonance Imaging ◽

Hearing Loss ◽

Magnetic Resonance ◽

Sensorineural Hearing Loss ◽

Cochlear Nerve ◽

Sensorineural Hearing ◽

Resonance Imaging ◽

Bilateral Sensorineural Hearing Loss ◽

Single Sided Deafness ◽

Cochlear Nerve Hypoplasia

Abstract Objective To determine the prevalence of cochlear nerve anomalies on magnetic resonance imaging in patients with unilateral or bilateral sensorineural hearing loss. Methods A retrospective case series was conducted at a tertiary referral centre. The inclusion criteria were paediatric patients with bilateral or unilateral sensorineural hearing loss, investigated with magnetic resonance imaging. The primary outcome measure was the rate of cochlear nerve hypoplasia or aplasia. Results Of the 72 patients with unilateral sensorineural hearing loss, 39 per cent (28 cases) had absent or hypoplastic cochlear nerves on the affected side. Fifteen per cent (11 cases) had other abnormal findings on magnetic resonance imaging. Eighty-four patients had bilateral sensorineural hearing loss, of which cochlear nerve hypoplasia or aplasia was identified only in 5 per cent (four cases). Other abnormal findings were identified in 14 per cent (12 cases). Conclusion Paediatric patients with unilateral sensorineural hearing loss are more likely to have cochlear nerve anomalies than those patients with bilateral sensorineural hearing loss. This has important implications regarding cochlear implantation for patients with single-sided deafness.

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