USE OF A COCHLEAR IMPLANT IN A PATIENT WITH COGAN’S SYNDROME AND PROFOUND SENSORINEURAL HEARING LOSS: A CASE STUDY

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 cochle- ostomy 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 Cogans syndrome.