Sudden hearing loss as the presenting symptom of systemic sclerosis

Sudden sensorineural hearing loss (SSHNL) is an emergency in otolaryngology. In most cases, the exact cause cannot be identified, but different immunologic disorders and microvascular events have been suggested to play a role in its pathogenesis. Sudden sensorineural hearing loss can be caused by collagen vascular disorders, but it has rarely been reported as the presenting symptom of these diseases. This case presented with bilateral hearing loss and was finally diagnosed as a systemic sclerosis (SSc) patient. This is the first case of SSc that presented with SSNHL as an initial symptom.

A 65-year-old man presented with bilateral hearing loss. He also complained of generalized fatigue and intermittent paresthesia. Brain imaging and blood tests were negative except for antinuclear antibodies. Administration of high-dose prednisone did not make any improvement. After extensive workup, follow-up, and referral to rheumatologist, the diagnosis of SSc was made. The patient's hearing improved after receiving intravenous immunoglobulin along with other symptoms of the disease.

This case illustrates the importance of follow-up and appropriate of SSNHL patients with other systemic symptoms.