Successful partial cochlear implantation in a patient with relapsing polychondritis

Abstract

The author reports on a case of relapsing polychondritis in which partial insertion of a cochlear implant has been successful. A woman with known relapsing polychondritis gave a history of increasingly severe auditory loss of sensorineural type occurring bilaterally. Previous repeated treatments with corticosteroids and immunosuppressive medications had been attempted. A right-sided cochlear implantation with partial electrode array insertion was carried out due to the patient's cochlea being obliterated. The outcome was excellent and the improvement in hearing was maintained. Relapsing polychondritis (RP) is a rarely-seen disorder, the aetiology of which is obscure, but which results in inflammation within cartilage throughout the entire body. The cochlea may become obliterated by this process. Cochlear implantation may be needed in such circumstances. The successful outcome in this case shows a potential role for cochlear implantation in individuals with inner ear disorders produced by immune disorders or relapsing polychondritis.

Keywords

• Polychondritis,relapsing,sensorineural hearing loss,cochlear implantation

Kaynakça

1. 1. Jaksch-Wartenhorst R. Polychondropathia. Wien Arch Inn Med 1923;6:93-100.
2. 2. McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore) 1976;55:193-215.
3. 3. Zeuner M, Straub RH, Rauh G, Albert ED, Scholmerich J, Lang B. Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol 1997;24:96-101.
4. 4. Pearson CM, Kline HM, Newcomer VD. Relapsing polychondritis. N Eng J Med 1960;263:51-8.
5. 5. Trentham DE, Le CH. Relapsing polychondritis. Ann Int Med 1998;129:114-22.
6. 6. Cody DT, Sones DA. Relapsing polychondritis: audiovestibular manifestations. Laryngoscope 1971;81:1208-22.
7. 7. Hoshino T, Kato I, Kodama A, Suzuki H. sudden deafness in relapsing polychondritis: a scanning electron microscopy study. Acta Otolaryngol 1978;86:418-27.
8. 8. Miyazawa T, Lino Y, Murakami Y, Ishii T. Immunohistochemical study on the temporal bones from patients with relapsing polychondritis. Jibimeneki Arerugi 2000;18:66-7.

9. 9. Tomoda K, Yamashita T, Kumazawa T, Yoo TG. Type II collagen distribution in the middle and inner ear: immunohistochemical studies. Ear Res Jpn 1984;15:199-202.
10. 10. Issing WJ, Selover D, Schulz P. Anti-labyrinthine antibodies in a patient with relapsing polychondritis. Eur Arch Otorhinolaryngol 1999;256:163-6.
11. 11. Schuknecht H. Ear pathology in autoimmune disease. Adv Oto Rhino Larynol 1991;46:50-7.
12. 12. Michet C. Vasculitis and relapsing polychondritis. Rheum Dis Clin North Am 1990;16:441-4.
13. 13. Bachor E , Blevins NH, Karmody C, Kuhnel T. Otologic manifestations of relapsing polychondritis. Review of literature and report of nine cases. Auris Nasus Larynx 2006;33:135-41.
14. 14. McCaffrey TV, McDonald TG, McCaffrey LA. Head and neck manifestations of relapsing polychondritis: review of 29 cases. Otolaryngology 1978;86:473-8.
15. 15. Quaranta N, Bartoli R, Giagnotti F, Di Cuonzo F, Quaranta A. Cochlear implants in systemic autoimmune vasculitis syndromes. Acta Otolaryngol Suppl 2002;548:44-8.