Korean J Otorhinolaryngol-Head Neck Surg.  2023 Oct;66(10):686-691. 10.3342/kjorl-hns.2023.00514.

A Case of Bilateral Sequential Sudden Sensorineural Hearing Loss in Protein S Deficiency and Successful Recovery of Hearing After Cochlear Implantation

• Lee D
• Park E
• Jung HH
• Im GJ

• KMID: 2547167
• DOI: http://doi.org/10.3342/kjorl-hns.2023.00514

Abstract

Bilateral sequential sudden sensorineural hearing loss (SSHL) is a rare and challenging clinical condition, but its association with protein S deficiency has received limited attention. This paper reports a case of bilateral sequential SSHL in a patient with protein S deficiency who underwent cochlear implantation and achieved significant improvement in hearing. The patient received high-dose systemic corticosteroids and anticoagulation therapy before the implantation, which had no significant effect. Cochlear implantation was then performed in the left ear, and the patient showed significant improvement in speech understanding and overall hearing ability. A year later, the patient underwent surgery for the opposite ear with favorable outcome. This case highlights the potential benefits of cochlear implantation in patients with bilateral sequential SSHL and protein S deficiency and emphasizes the importance of considering cochlear implantation as a treatment option in such patients.

Keyword

Blood coagulation; Brain infarction; Cochlear implantation; Protein S deficiency; Sudden hearing loss