Cochlear Implant in Cogan's Syndrome

Overview

Journal Eur Arch Otorhinolaryngol

Specialty Otorhinolaryngology

Date 2007 Jul 20

PMID 17639443

Citations 5

Authors

Rajesh Vishwakarma

T Joseph Shawn

Affiliations

Soon will be listed here.

Abstract

Post-lingual deafness is a stressful condition which is rendered even more painful by the sudden emotional isolation that the patient suffers. Cogan's syndrome is a rare autoimmune cause for post-lingual deafness characterized by non-syphilitic interstitial keratitis, bilateral audio vestibular deficiencies and systemic vasculitis. World over very few cases of Cogan's syndrome have been reported. Cochlear implant surgery in such a patient is a challenging but highly satisfactory experience due to the multitude of clinical problems the patient faces. This demands a proper work up, meticulous surgery and stringent post-operative follow-up. Here we present a patient with atypical Cogan's syndrome, diabetes mellitus and hypothyroidism. She went into a major depression with suicidal tendency following the complete loss of hearing. We performed cochlear implant surgery in this patient, but not before facing several clinical obstacles, helped by a dedicated team consisting of a rheumatologist, endocrinologist, neurophysician, psychiatrist, anaesthetists and audiologist. The results are extremely satisfying for the patient and all the people involved. This case underlines the prime importance of hearing in maintaining the psychological well being of a human being.

Citing Articles

Paediatric Cogan´s syndrome - review of literature, case report and practical approach to diagnosis and management.

Rucklova K, von Kalle T, Koitschev A, Gekeler K, Scheltdorf M, Heinkele A Pediatr Rheumatol Online J. 2023; 21(1):54.

PMID: 37291629 PMC: 10251673. DOI: 10.1186/s12969-023-00830-x.

Development of a low grade lymphoma in the mastoid bone in a patient with atypical Cogan's syndrome: A case report.

Kalogeropoulos C, Karachalios D, Pentheroudakis G, Tsikou-Papafrangou A, Abou-Asabeh L, Argyropoulou M J Adv Res. 2015; 6(3):523-7.

PMID: 26257951 PMC: 4522542. DOI: 10.1016/j.jare.2014.05.003.

Cochlear implantation in patients with Cogan syndrome: long-term results.

Bacciu A, Pasanisi E, Di Lella F, Guida M, Bacciu S, Vincenti V Eur Arch Otorhinolaryngol. 2014; 272(11):3201-7.

PMID: 25367706 DOI: 10.1007/s00405-014-3376-9.

Cogan's syndrome: achievement of complete resolution of auditory deficit with steroids.

Singh P, Gupta M, Lehl S, Singh K BMJ Case Rep. 2013; 2013.

PMID: 23704430 PMC: 3669764. DOI: 10.1136/bcr-2013-008951.

Syndromes of hearing loss associated with visual loss.

Abou-Elhamd K, ElToukhy H, Al-Wadaani F Eur Arch Otorhinolaryngol. 2013; 271(4):635-46.

PMID: 23632871 DOI: 10.1007/s00405-013-2514-0.

References

Gaubitz M, Lubben B, Seidel M, Schotte H, Gramley F, Domschke W . Cogan's syndrome: organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature. Clin Exp Rheumatol. 2001; 19(4):463-9. View

Cinamon U, Kronenberg J, Hildesheimer M, Taitelbaum R . Cochlear implantation in patients suffering from Cogan's syndrome. J Laryngol Otol. 1998; 111(10):928-30. DOI: 10.1017/s002221510013899x. View

Majoor M, Albers F, van der Gaag R, Gmelig-Meyling F, Huizing E . Corneal autoimmunity in Cogan's syndrome? Report of two cases. Ann Otol Rhinol Laryngol. 1992; 101(8):679-84. DOI: 10.1177/000348949210100810. View

Morgan Jr G, HOCHMAN R, Weider D . Cogan's syndrome: acute vestibular and auditory dysfunction with interstitial keratitis. Am J Otolaryngol. 1984; 5(4):258-61. DOI: 10.1016/s0196-0709(84)80035-6. View

Minet M, Deggouj N, Gersdorff M . Cochlear implantation in patients with Cogan's syndrome: a review of four cases. Eur Arch Otorhinolaryngol. 1997; 254(9-10):459-62. DOI: 10.1007/BF02439981. View

Lunardi C, Bason C, Leandri M, Navone R, Lestani M, Millo E . Autoantibodies to inner ear and endothelial antigens in Cogan's syndrome. Lancet. 2002; 360(9337):915-21. DOI: 10.1016/S0140-6736(02)11028-2. View

SCHUKNECHT H, Nadol Jr J . Temporal bone pathology in a case of Cogan's syndrome. Laryngoscope. 1994; 104(9):1135-42. DOI: 10.1288/00005537-199409000-00015. View

Garcia Berrocal J, Vargas J, Vaquero M, Ramon Y Cajal S . Cogan's syndrome: an oculo-audiovestibular disease. Postgrad Med J. 1999; 75(883):262-4. PMC: 1741234. DOI: 10.1136/pgmj.75.883.262. View

McDonald T, Vollertsen R, Younge B . Cogan's syndrome: audiovestibular involvement and prognosis in 18 patients. Laryngoscope. 1985; 95(6):650-4. DOI: 10.1288/00005537-198506000-00003. View

10.

Benvenga S, Trimarchi F, Facchiano A . Cogan's syndrome as an autoimmune disease. Lancet. 2003; 361(9356):530-1. DOI: 10.1016/S0140-6736(03)12478-6. View

11.

Cheson B, Bluming A, Alroy J . Cogan's syndrome: a systemic vasculitis. Am J Med. 1976; 60(4):549-55. DOI: 10.1016/0002-9343(76)90722-1. View

12.

Hughes G, Kinney S, Barna B, Tomsak R, Calabrese L . Autoimmune reactivity in Cogan's syndrome: a preliminary report. Otolaryngol Head Neck Surg. 1983; 91(1):24-32. DOI: 10.1177/019459988309100106. View

13.

Helmchen C, Arbusow V, Jager L, Strupp M, Stocker W, Schulz P . Cogan's syndrome: clinical significance of antibodies against the inner ear and cornea. Acta Otolaryngol. 1999; 119(5):528-36. DOI: 10.1080/00016489950180748. View

14.

Eisenstein B, TAUBENHAUS M . Nonsyphilitic interstitial keratitis and bilateral deafness (Cogan's syndrome) associated with cardiovascular disease. N Engl J Med. 1958; 258(22):1074-9. DOI: 10.1056/NEJM195805292582202. View

15.

Aschendorff A, Lohnstein P, Schipper J, Klenzner T . [Obliterated cochlea in Cogan's syndrome -- implications for cochlear implant surgery]. Laryngorhinootologie. 2004; 83(12):836-9. DOI: 10.1055/s-2004-826004. View

16.

Haynes B, Mason P, Fauci A . Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature. Medicine (Baltimore). 1980; 59(6):426-41. View

17.

Del Carpio J, Espinoza L, Osterland C . Cogan's syndrome and HLA BW17. N Engl J Med. 1976; 295(22):1262-3. DOI: 10.1056/nejm197611252952223. View

18.

Allen N, Cox C, Cobo M, Kisslo J, Jacobs M, McCallum R . Use of immunosuppressive agents in the treatment of severe ocular and vascular manifestations of Cogan's syndrome. Am J Med. 1990; 88(3):296-301. DOI: 10.1016/0002-9343(90)90157-9. View