Cogan syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Synonyms and keywords: Oculovestibuloauditory syndrome

Overview

Cogan syndrome is a rare disorder characterized by recurrent inflammation of the front of the eye (the cornea) and often fever, fatigue, and weight loss, episodes of dizziness, and hearing loss. It can lead to deafness or blindness if untreated. The classic form of the disease was first described by D.G. Cogan in 1945.

Historical Perspective

In 1945 D.G. Cogan first described the "nonsyphilitic interstitial keratitis and vestibuloauditory symptoms" that would later bear his name (Cogan, 1945). In 1963, the atypical form of Cogan syndrome was first described (Bennett, 1963)

Classification

Pathophysiology

Causes

Differentiating Cogan Syndrome from other Diseases

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging
Acute onset Recurrency Nystagmus Hearing problems
Peripheral
BPPV
[1][2][3] 		+ + +/− − − −
Vestibular neuritis
[4] 		+ +/− + /−

(unilateral)

−
  • + Head thrust test
 − −
HSV oticus
[5][6][7][8] 		+ +/− − +/− + VZV antibody titres
Meniere disease
[9][10] 		+/− + +/− + (Progressive) −
Labyrinthine concussion
[11][12] 		+ − − + −
Perilymphatic fistula
[13][14][15] 		+/− + − + −
  • CT scan may show fluid around the round window recess
Semicircular canal

dehiscence syndrome
[16][17]

+/− + − +

(air-bone gaps on audiometry)

−
Vestibular paroxysmia
[18][19][20] 		+ + +/−

(Induced by hyperventilation)

− −
Cogan syndrome
[21][22][23] 		− + +/− + Increased ESR and cryoglobulins
  • In CT scan we may see calcification or soft tissue attenuation obliterating the intralabyrinthine fluid spaces
Vestibular schwannoma
[24][25] 		− + +/− + −
Otitis media
[26][27] 		+ − − +/− Increased acute phase reactants
Aminoglycoside toxicity
[28] 		+ − − + − −
Recurrent vestibulopathy
[29][30] 		+ − − − − − −
  • It may happen infrequently, every one to two years
  • It may be associated with nausea and vomiting
  • It may overlap with vestibular migraine
Central
Vestibular migrain
[31][32] 		– + +/− +/− −
  • ICHD-3 criteria
Epileptic vertigo
[33] 		− + +/− − − −
  • They response well to anti-seizure drugs
Multiple sclerosis
[34][35][36] 		− + +/− − Elevated concentration of CSF oligoclonal bands
  • MS is at least two times more common among women than men
  • The onset of symptoms is mostly between the age of fifteen to forty years, rarely before age fifteen or after age sixty
Brain tumors
[37] 		+/− + + + Cerebral spinal fluid (CSF) may show cancerous cells
  • On CT scan most of the brain tumors appears as a hypodense mass lesions
  • On MRI most of the brain tumors appears as a hypointense or isointense on T1-weighted scans, or hyperintense on T2-weighted MRI.
Cerebellar infarction/hemorrhage + − ++/− − −
  • Based on the time interval between stroke and imaging we may have different presentations
Brain stem ischemia + − +/− − −
  • Based on the time interval between stroke and imaging we may have different presentations
  • For more information click here
Chiari malformation
[38][39] 		− + + − −
  • Patient may experience ringing in the ears
Parkinson
[40][41][42] 		− + − − −

ABBREVIATIONS

VZV= Varicella zoster virus, MRI= Magnetic resonance imaging, ESR= Erythrocyte sedimentation rate, EEG= Electroencephalogram, CSF= Cerebrospinal fluid, GPe= Globus pallidus externa, ICHD= International Classification of Headache Disorders

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

Related disorders

References

  1. ↑ Lee SH, Kim JS (June 2010). "Benign paroxysmal positional vertigo". J Clin Neurol. 6 (2): 51–63. doi:10.3988/jcn.2010.6.2.51. PMC 2895225. PMID 20607044.
  2. ↑ Chang MB, Bath AP, Rutka JA (October 2001). "Are all atypical positional nystagmus patterns reflective of central pathology?". J Otolaryngol. 30 (5): 280–2. PMID 11771020.
  3. ↑ Dorresteijn PM, Ipenburg NA, Murphy KJ, Smit M, van Vulpen JK, Wegner I, Stegeman I, Grolman W (June 2014). "Rapid Systematic Review of Normal Audiometry Results as a Predictor for Benign Paroxysmal Positional Vertigo". Otolaryngol Head Neck Surg. 150 (6): 919–24. doi:10.1177/0194599814527233. PMID 24642523.
  4. ↑ Mandalà M, Nuti D, Broman AT, Zee DS (February 2008). "Effectiveness of careful bedside examination in assessment, diagnosis, and prognosis of vestibular neuritis". Arch. Otolaryngol. Head Neck Surg. 134 (2): 164–9. doi:10.1001/archoto.2007.35. PMID 18283159.
  5. ↑ Wackym, Phillip A. (1997). "Molecular Temporal Bone Pathology: II. Ramsay Hunt Syndrome (Herpes Zoster Oticus)". The Laryngoscope. 107 (9): 1165–1175. doi:10.1097/00005537-199709000-00003. ISSN 0023-852X.
  6. ↑ Zhu, S.; Pyatkevich, Y. (2014). "Ramsay Hunt syndrome type II". Neurology. 82 (18): 1664–1664. doi:10.1212/WNL.0000000000000388. ISSN 0028-3878.
  7. ↑ Mishell JH, Applebaum EL (February 1990). "Ramsay-Hunt syndrome in a patient with HIV infection". Otolaryngol Head Neck Surg. 102 (2): 177–9. doi:10.1177/019459989010200215. PMID 2113244.
  8. ↑ Tada, Yuichiro; Aoyagi, Masaru; Tojima, Hitoshi; Inamura, Hiroo; Saito, Osamu; Maeyama, Hiroyuki; Kohsyu, Hidehiro; Koike, Yoshio (2009). "Gd-DTPA Enhanced MRI in Ramsay Hunt Syndrome". Acta Oto-Laryngologica. 114 (sup511): 170–174. doi:10.3109/00016489409128326. ISSN 0001-6489.
  9. ↑ Watanabe, Isamu (1980). "Ménière's Disease". ORL. 42 (1–2): 20–45. doi:10.1159/000275477. ISSN 1423-0275.
  10. ↑ Saeed SR (January 1998). "Fortnightly review. Diagnosis and treatment of Ménière's disease". BMJ. 316 (7128): 368–72. PMC 2665527. PMID 9487176.
  11. ↑ Dürrer, J.; Poláčková, J. (1971). "Labyrinthine Concussion". ORL. 33 (3): 185–190. doi:10.1159/000274994. ISSN 1423-0275.
  12. ↑ Choi MS, Shin SO, Yeon JY, Choi YS, Kim J, Park SK (April 2013). "Clinical characteristics of labyrinthine concussion". Korean J Audiol. 17 (1): 13–7. doi:10.7874/kja.2013.17.1.13. PMC 3936518. PMID 24653897.
  13. ↑ Fox, Eileen J.; Balkany, Thomas J.; Arenberg, Kaufman (1988). "The Tullio Phenomenon and Perilymph Fistula". Otolaryngology–Head and Neck Surgery. 98 (1): 88–89. doi:10.1177/019459988809800115. ISSN 0194-5998.
  14. ↑ Casselman JW (February 2002). "Diagnostic imaging in clinical neuro-otology". Curr. Opin. Neurol. 15 (1): 23–30. PMID 11796947.
  15. ↑ Seltzer S, McCabe BF (January 1986). "Perilymph fistula: the Iowa experience". Laryngoscope. 96 (1): 37–49. PMID 3941579.
  16. ↑ Lempert T, von Brevern M (February 2005). "Episodic vertigo". Curr. Opin. Neurol. 18 (1): 5–9. PMID 15655395.
  17. ↑ Watson SR, Halmagyi GM, Colebatch JG (February 2000). "Vestibular hypersensitivity to sound (Tullio phenomenon): structural and functional assessment". Neurology. 54 (3): 722–8. PMID 10680810.
  18. ↑ Hufner, K.; Barresi, D.; Glaser, M.; Linn, J.; Adrion, C.; Mansmann, U.; Brandt, T.; Strupp, M. (2008). "Vestibular paroxysmia: Diagnostic features and medical treatment". Neurology. 71 (13): 1006–1014. doi:10.1212/01.wnl.0000326594.91291.f8. ISSN 0028-3878.
  19. ↑ Strupp M, von Stuckrad-Barre S, Brandt T, Tonn JC (February 2013). "Teaching neuroimages: Compression of the eighth cranial nerve causes vestibular paroxysmia". Neurology. 80 (7): e77. doi:10.1212/WNL.0b013e318281cc2c. PMID 23400324.
  20. ↑ Hüfner K, Barresi D, Glaser M, Linn J, Adrion C, Mansmann U, Brandt T, Strupp M (September 2008). "Vestibular paroxysmia: diagnostic features and medical treatment". Neurology. 71 (13): 1006–14. doi:10.1212/01.wnl.0000326594.91291.f8. PMID 18809837.
  21. ↑ Vollertsen RS (May 1990). "Vasculitis and Cogan's syndrome". Rheum. Dis. Clin. North Am. 16 (2): 433–9. PMID 2189159.
  22. ↑ Hughes, Gordon B.; Kinney, Sam E.; Barna, Barbara P.; Tomsak, Robert L.; Calabrese, Leonard H. (1983). "Autoimmune reactivity in Cogan's syndrome: A preliminary report". Otolaryngology–Head and Neck Surgery. 91 (1): 24–32. doi:10.1177/019459988309100106. ISSN 0194-5998.
  23. ↑ Majoor, M. H. J. M.; Albers, F. W. J.; Casselman, J. W. (2009). "Clinical Relevance of Magnetic Resonance Imaging and Computed Tomography in Cogan's Syndrome". Acta Oto-Laryngologica. 113 (5): 625–631. doi:10.3109/00016489309135875. ISSN 0001-6489.
  24. ↑ Robert W. Foley, Shahram Shirazi, Robert M. Maweni, Kay Walsh, Rory McConn Walsh, Mohsen Javadpour & Daniel Rawluk (2017). "Signs and Symptoms of Acoustic Neuroma at Initial Presentation: An Exploratory Analysis". Cureus. 9 (11): e1846. doi:10.7759/cureus.1846. PMID 29348989. Unknown parameter |month= ignored (help)
  25. ↑ E. P. Lin & B. T. Crane (2017). "The Management and Imaging of Vestibular Schwannomas". AJNR. American journal of neuroradiology. 38 (11): 2034–2043. doi:10.3174/ajnr.A5213. PMID 28546250. Unknown parameter |month= ignored (help)
  26. ↑ "Ear infection - acute: MedlinePlus Medical Encyclopedia".
  27. ↑ Rettig E, Tunkel DE (2014). "Contemporary concepts in management of acute otitis media in children". Otolaryngol. Clin. North Am. 47 (5): 651–72. doi:10.1016/j.otc.2014.06.006. PMC 4393005. PMID 25213276.
  28. ↑ Ernfors P, Duan ML, ElShamy WM, Canlon B (April 1996). "Protection of auditory neurons from aminoglycoside toxicity by neurotrophin-3". Nat. Med. 2 (4): 463–7. PMID 8597959.
  29. ↑ Oh AK, Lee H, Jen JC, Corona S, Jacobson KM, Baloh RW (May 2001). "Familial benign recurrent vertigo". Am. J. Med. Genet. 100 (4): 287–91. PMID 11343320.
  30. ↑ Rutka JA, Barber HO (April 1986). "Recurrent vestibulopathy: third review". J Otolaryngol. 15 (2): 105–7. PMID 3712538.
  31. ↑ "The International Classification of Headache Disorders: 2nd edition". Cephalalgia. 24 Suppl 1: 9–160. 2004. PMID 14979299.
  32. ↑ Absinta M, Rocca MA, Colombo B, Copetti M, De Feo D, Falini A, Comi G, Filippi M (December 2012). "Patients with migraine do not have MRI-visible cortical lesions". J. Neurol. 259 (12): 2695–8. doi:10.1007/s00415-012-6571-x. PMID 22714135.
  33. ↑ Tarnutzer AA, Lee SH, Robinson KA, Kaplan PW, Newman-Toker DE (April 2015). "Clinical and electrographic findings in epileptic vertigo and dizziness: a systematic review". Neurology. 84 (15): 1595–604. doi:10.1212/WNL.0000000000001474. PMC 4408281. PMID 25795644.
  34. ↑ McDonald WI, Compston A, Edan G, Goodkin D, Hartung HP, Lublin FD, McFarland HF, Paty DW, Polman CH, Reingold SC, Sandberg-Wollheim M, Sibley W, Thompson A, van den Noort S, Weinshenker BY, Wolinsky JS (July 2001). "Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis". Ann. Neurol. 50 (1): 121–7. PMID 11456302.
  35. ↑ Barrett L, Drayer B, Shin C (January 1985). "High-resolution computed tomography in multiple sclerosis". Ann. Neurol. 17 (1): 33–8. doi:10.1002/ana.410170109. PMID 3985583.
  36. ↑ Fazekas F, Barkhof F, Filippi M, Grossman RI, Li DK, McDonald WI, McFarland HF, Paty DW, Simon JH, Wolinsky JS, Miller DH (August 1999). "The contribution of magnetic resonance imaging to the diagnosis of multiple sclerosis". Neurology. 53 (3): 448–56. PMID 10449103.
  37. ↑ Dunniway, Heidi M.; Welling, D. Bradley (2016). "Intracranial Tumors Mimicking Benign Paroxysmal Positional Vertigo". Otolaryngology–Head and Neck Surgery. 118 (4): 429–436. doi:10.1177/019459989811800401. ISSN 0194-5998.
  38. ↑ Caldarelli M, Di Rocco C (May 2004). "Diagnosis of Chiari I malformation and related syringomyelia: radiological and neurophysiological studies". Childs Nerv Syst. 20 (5): 332–5. doi:10.1007/s00381-003-0880-4. PMID 15034729.
  39. ↑ Sarnat HB (2008). "Disorders of segmentation of the neural tube: Chiari malformations". Handb Clin Neurol. 87: 89–103. doi:10.1016/S0072-9752(07)87006-0. PMID 18809020.
  40. ↑ van Wensen, E.; van Leeuwen, R.B.; van der Zaag-Loonen, H.J.; Masius-Olthof, S.; Bloem, B.R. (2013). "Benign paroxysmal positional vertigo in Parkinson's disease". Parkinsonism & Related Disorders. 19 (12): 1110–1112. doi:10.1016/j.parkreldis.2013.07.024. ISSN 1353-8020.
  41. ↑ Steiner I, Gomori JM, Melamed E (1985). "Features of brain atrophy in Parkinson's disease. A CT scan study". Neuroradiology. 27 (2): 158–60. PMID 3990948.
  42. ↑ Kosta P, Argyropoulou MI, Markoula S, Konitsiotis S (January 2006). "MRI evaluation of the basal ganglia size and iron content in patients with Parkinson's disease". J. Neurol. 253 (1): 26–32. doi:10.1007/s00415-005-0914-9. PMID 15981079.
  • Cogan DG (1945). "Syndrome of nonsyphilitic interstitial keratitis and vestiboloauditory symptoms". Arch Ophthalmol. 33: 144–9.
  • Bennett FM (1963). "Bilateral recurrent episcleritis associated with posterior corneal changes, vestibulo-auditory symptoms and rheumatoid arthritis". Am J Ophthalmol. 55: 815–8. PMID 13967466
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