CASE STUDY
USE OF A COCHLEAR IMPLANT IN A PATIENT WITH COGAN’S SYNDROME AND PROFOUND SENSORINEURAL HEARING LOSS: A CASE STUDY
 
More details
Hide details
1
World Hearing Center, Institute of Physiology and Pathology of Hearing, Warsaw/Kajetany, Poland
2
Rehabilitation Clinic, World Hearing Center, Institute of Physiology and Pathology of Hearing, Warsaw/Kajetany, Poland
3
Department of Genetics, World Hearing Center, Institute of Physiology and Pathology of Hearing, Warsaw/Kajetany, Poland
4
Heart Failure and Cardiac Rehabilitation Department, Second Faculty, Medical University of Warsaw, Warsaw, Poland
5
Institute of Sensory Organs, Warsaw/Kajetany, Poland
CORRESPONDING AUTHOR
Andrzej Pastuszak   

Andrzej Pastuszak, World Hearing Center, Institute of Physiology and Pathology of Hearing, Mokra 17 Str., Kajetany, 05-830, Nadarzyn, Poland, e-mail: a.pastuszak@ifps.org.pl
Publication date: 2020-04-10
 
J Hear Sci 2017;7(3):55–61
KEYWORDS
ABSTRACT
Background:
Cogan’s syndrome is a very rare autoimmune disease characterized by the coexistence of inflammatory lesions in the eyeball and inner ear dysfunction. The symptoms of Cogan’s syndrome within the inner ear appear suddenly and resemble Meniere’s disease symptoms: severe vertigo, nausea, vomiting, and usually bilateral, fluctuating sensorineural hearing loss with concomitant tinnitus.

Case report:
A 59-year-old woman was admitted to the Otolaryngology Head and Neck Surgery Clinic to undergo cochlear implantation. At 57 years of age, the patient had been diagnosed with scleritis. About a year later, vertigo and nausea arose, followed by rapidly progressing hearing loss – at first in the left ear and subsequently in the right, accompanied by tinnitus. At the time of implantation, the patient’s speech discrimination score was 0% for both ears. Taking into consideration the audiometric tests results and imaging scans, promontorial cochleostomy was carried out during the cochlear implantation procedure. In the postoperative period, no complications were observed. After 24 months of using the speech processor, the subjective assessment of speech intelligibility given by the patient on a 0–10 scale was 8.5 in quiet and 5.0 in a noisy environment. The patient could identify 95% of monosyllabic words in silence and 35% in noise (in conditions of SNR +10 dB, speech level at 70 dB HL, and noise level at 60 dB HL). The benefits obtained were confirmed by results of an Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire. The patient is presently eligible for cochlear implantation in the other ear.

Conclusions:
Cochlear implantation was effective in improving hearing in a patient with Cogan’s syndrome.

 
REFERENCES (30)
1.
Ying Y-LM, Hirsch BE. Atypical Cogan’s syndrome: A case report. Am J Otolaryngol, 2010;31: 279–82.
 
2.
Cogan DG. Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol, 1945; 33: 144–49.
 
3.
Haynes BF, Kaiser-Kupfer MI, Mason P, Fauci AS. Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature. Medicine (Baltimore), 1980; 59: 426–41.
 
4.
Cundiff J, Kansal S, Kumar A, Goldstein DA, Tessler HH. Cogan’s syndrome: A cause of progressive hearing deafness. Am J Otolaryngol, 2006; 27: 68–70.
 
5.
Grasland A, Pouchot J, Hachulla E, Blétry O, Papo T, Vinceneux P et al. Typical and atypical Cogan’s syndrome: 32 cases and review of the literature. Rheumatology, 2004; 43: 1007–15.
 
6.
Jančatová D, Zeleník K, Komínek P, Matoušek P. Atypical Cogan’s syndrome: A case report and summary of current treatment options. Int J Pediatr Otorhinolaryngol, 2015; 79: 428–31.
 
7.
Allen NB, Cox CC, Cobo M, Kisslo J, Jacobs MR, McCallum RM et al. Use of immunosuppressive agents in the treatment of severe ocular and vascular manifestations of Cogan’s syndrome. Am J Med, 1990; 88: 296–301.
 
8.
Vaiopoulos G, Sfikakis PP, Skoumas B, Kavouklis E, Doukas E, Kaklamanis P. Lack of response to corticosteroids and pulse cyclophosphamide therapy in Cogan’s syndrome. Clin Rheumatol, 1994; 13: 110–12.
 
9.
Baumann A, Helbling A, Oertle S, Häusler R, Vibert D. Cogan’s syndrome: clinical evolution of deafness and vertigo in three patients. Eur Arch Oto-Rhino-Laryngol Head Neck 2005; 262: 45–49.
 
10.
Bovo R, Ciorba A, Trevisi P, Aimoni C, Cappiello L, Castiglione A et al. Cochlear implant in Cogan syndrome. Acta Otolaryngol (Stockh.), 2011; 131: 494–97.
 
11.
Pasanisi E, Vincenti V, Bacciu A, Guida M, Berghenti T, Barbot A et al. Cochlear implantation and Cogan syndrome. Otol Neurotol 2003; 24: 601–4.
 
12.
Cinamon U, Kronenberg J, Hildesheimer M, Taitelbaum R. Cochlear implantation in patients suffering from Cogan’s syndrome. J Laryngol Otol, 1997; 111: 928–30.
 
13.
Minet M, Deggouj N, Gersdorff M. Cochlear implantation in patients with Cogan’s syndrome: A review of four cases. Eur Arch Otorhinolaryngol, 1997; 254: 459–62.
 
14.
Vishwakarma R, Shawn TJ. Cochlear implant in Cogan’s syndrome. Eur Arch Otorhinolaryngol, 2007; 264: 1121–24.
 
15.
Low WK, Burgess R, Teoh CK. Cochlear implantation in a patient with Cogan’s syndrome, chronic ear disease and on steroid therapy. In: Kim CS, Chang SO, Lim D (eds.), Updates in Cochlear Implantation, Adv Otorhinolaryngol, 2000; 57: 157–59.
 
16.
Kontorinis G, Giourgas A, Neuburger J, Lesinski-Schiedat A, Lenarz T. Long-term evaluation of cochlear implantation in Cogan syndrome. J Otorhinolaryngol Relat Spec, 2010; 72: 275–79.
 
17.
Wang JR, Yuen HW, Shipp DB, Stewart S, Lin VY, Chen JM et al. Cochlear implantation in patients with autoimmune inner ear disease including Cogan syndrome: A comparison with ageand sex-matched controls. Laryngoscope, 2010; 120: 2478–83.
 
18.
Kawamura S1, Sakamoto T, Kashio A, Kakigi A, Ito K, Suzuki M et al. Cochlear implantation in a patient with atypical Cogan’s syndrome complicated with hypertrophic cranial pachymeningitis. Auris Nasus Larynx, 2010; 37: 737–41.
 
19.
Bacciu A, Pasanisi E, Lella FD, Guida M, Bacciu S, Vincenti V. Cochlear implantation in patients with Cogan syndrome: longterm results. Eur Arch Otorhinolaryngol, 2015; 272: 3201–7.
 
20.
Schuknecht HF, Nadol JB. Temporal bone pathology in a case of Cogan’s syndrome. Laryngoscope, 1994; 104: 1135–42.
 
21.
Wysocki J, Skarzynski H. Cochleostomy during the intracochlear implantation. Anatomical conditions in children and adults. Otolaryngol Pol, 1998; 52: 689–94.
 
22.
Skarzynski H, Matusiak M, Furmanek M, Skarzynski PH. Deep insertion: Round window approach by using SRA electrode. Cochlear Implants Int, 2014; 15(Suppl 1): S4–7.
 
23.
Skarzynski H, Lorens A, Matusiak M, Porowski M, Skarzynski PH, James CJ. Cochlear implantation with the Nucleus slim straight electrode in subjects with residual low-frequency hearing. Ear Hear, 2014; 35: 33–43.
 
24.
Van de Heyning P, Adunka O, Arauz SL, Atlas M, Baumgartner WD, Brill S et al. Standards of practice in the field of hearing implants. Cochlear Implants Int, 2013; 14(Suppl 2): S1–5.
 
25.
Brown KD, Balkany TJ. Benefits of bilateral cochlear implantation: A review. Curr Opin Otolaryngol Head Neck Surg, 2007; 15: 315–18.
 
26.
Touma Z, Nawwar R, Hadi U, Hourani M, Arayssi T. The use of TNF-alpha blockers in Cogan’s syndrome. Rheumatol Int, 2007; 27: 995–96.
 
27.
Richardson B. Methotrexate therapy for hearing loss in Cogan’s syndrome. Arthritis Rheum, 1994; 37: 1559–61.
 
28.
McDonald TJ, Vollertsen RS, Younge BR. Cogan’s syndrome: Audiovestibular involvement and prognosis in 18 patients. Laryngoscope, 1985; 95: 650–54.
 
29.
St Clair EW, McCallum RM. Cogan’s syndrome. Curr Opin Rheumatol, 1999; 11: 47–52.
 
30.
Chynn EW, Jakobiec FA. Cogan’s Syndrome: Ophthalmic, audiovestibular, and systemic manifestations and therapy. Int Ophthalmol Clin, 1996; 36: 61–72.
 
eISSN:2084-3127
ISSN:2083-389X