scholarly journals Rituximab Not Effective for Hearing Loss in Cogan’s Syndrome

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Daniel R. Bunker ◽  
Leslie Dubin Kerr
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Refractory Disease ◽  
Disease Course ◽  
Cogan’S Syndrome ◽  
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.

scholarly journals Sudden sensorineural hearing loss in atypical Cogan's syndrome: A case report

2018 ◽  
Vol 30 ◽  
pp. 50-53
Author(s):  
José Luis Treviño González ◽  
German A. Soto-Galindo ◽  
Rafael Moreno Sales ◽  
Josefina A. Morales Del Ángel
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome

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

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.

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

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

Hypertrophic Pachymeningitis of the Internal Auditory Canal: A Rare Case of Unilateral Sudden Sensorineural Hearing Loss

2018 ◽  
Vol 127 (9) ◽  
pp. 649-652 ◽  
Author(s):  
Thomas Muelleman ◽  
Hannah Kavookjian ◽  
James Lin ◽  
Hinrich Staecker
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Internal Auditory Canal ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Hypertrophic Pachymeningitis ◽  
Eighth Cranial Nerve ◽  
Mri Scans ◽  
Magnetic Resonance Imaging Mri

Objectives: To describe and increase awareness of a rare cause of unilateral sudden sensorineural hearing loss. Methods: Case report and literature review. Results: We present a 66-year-old female who suffered left-sided sudden sensorineural hearing loss and dizziness. Diagnostic magnetic resonance imaging (MRI) did not reveal masses or lesions along the eighth cranial nerve or in the inner ear. Upon eventual referral to neurotology clinic, hypertrophic pachymeningitis of her left internal auditory canal and adjacent middle and posterior fossa dura were identified. The ensuing laboratory workup for autoimmune and infectious etiology revealed mild elevation of ACE 93 (9-67) but otherwise normal results. Conclusions: Idiopathic hypertrophic pachymeningitis is a diagnosis of exclusion. Neoplastic, infectious, and autoimmune causes must be ruled out. The prevailing treatment for this condition is high-dose corticosteroids. This entity should be considered when evaluating MRI scans obtained in the setting of sudden sensorineural hearing loss.

scholarly journals Bilateral Sudden Sensorineural Hearing Loss Associated with Sepsis: A Case Report and Literature Review

2021 ◽  
Vol 64 (9) ◽  
pp. 674-679
Author(s):  
Hyun-Jin Lee ◽  
Seong Ki Ahn ◽  
Chae Dong Yim ◽  
Dong Gu Hur
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Systemic Disease ◽  
Vital Signs ◽  
Detailed Examination ◽  
Acute Onset ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Electrolyte Imbalance ◽  
High Dose

Bilateral sudden sensorineural hearing loss (SSNHL) is rare and usually indicates a serious systemic pathology. We describe an unusual case of bilateral SSNHL caused by sepsis. A 28-year-old female complained of acute-onset bilateral hearing impairment; in addition to otological symptoms, she had a systemic condition that met the criteria for sepsis. We performed a physical examination and laboratory tests to diagnose sepsis. Pure tone audiogram and videonystagmography were performed to evaluate the otological symptoms. Intravenous antibiotics and high-dose methylprednisolone were prescribed for treatment, and audiogram was repeated during that period. The fever subsided and the vital signs were stabilized. The electrolyte imbalance and abnormal urine parameters became normal. Hearing gradually recovered to a normal level on day 7 of hospitalization. In conclusion, sepsis should be considered as a cause of SSNHL. When conducting a detailed examination of patients with bilateral SSNHL, the clinician should consider systemic disease.

Sign in / Sign up

Export Citation Format

Share Document