To report a case of a patient with Hemoglobin SC disease who presented with spontaneous labyrinthine hemorrhage.

Data obtained through the review of clinical records.

A 27-year-old man with a diagnosis of Hemoglobin SC disease, with no transfusion history, and under erratic treatment with phlebotomy for 9 years, seldom achieving the hemoglobin target of less than 11 g/dL, reported sudden oscillopsia, nausea, vomiting, mild hearing loss, and tinnitus in the left ear, with a worsening evolution over three days. He received flunarizine and meclizine for 10 days in another healthcare service, with no improvement, and came to our service for further evaluation. As background history, the patient had bilateral grade IV proliferative retinopathy since 2012, treated with photocoagulation, and had previously experienced sudden hearing loss in the right ear in 2010, accompanied by prolonged vertigo, constant tinnitus and permanent deafness in that ear since then. During the investigation of the new complaint of hypoacusis, the brain and temporal bone magnetic resonance imaging showed filling of labyrinthine structures on the left side with probable hematic content, suggesting labyrinthine hemorrhage, and the audiometry confirmed a new neurosensory loss on the left. At that time, the level of hemoglobin was 13.5 g/dL. The patient was treated with Prednisone and Vitamin A for 1 month. Hydroxyurea and phlebotomy were prescribed, considering the severity of the complications; and vestibular rehabilitation exercises were advised. Audiometry repeated 1 month after the onset of the condition showed mild neurosensory loss in the left ear from 2kHz, stable compared to the previous study; and anacusis in the right ear. After 1 month, the patient persisted with intermittent sensation of left ear fullness, mild otalgia, and occasional oscillopsia.

Sensorineural otological disorders in patients with Hemoglobin SC disease have been described in only a few studies, with a reported prevalence ranging from 9.7 to 29%. Labyrinthine hemorrhage is a cause of sensorineural hearing loss that has been associated with proliferative retinopathy; appears to be more frequent in Hemoglobin SC disease than in other types of sickle cell disease; and has a pathophysiology that possibly involves altered capillary hemodynamics, vaso-occlusion, and reperfusion injury in the labyrinthine vessels. In this particular case, such manifestation had a significant impact due to the pre-existing contralateral deafness. Hyperviscosity is an important cause of proliferative retinopathy and it is probably also implicated in the sensorineural disorders.

The early identification of labyrinthine hemorrhage as the cause of sudden hearing loss in patients with Hemoglobin SC disease is essential for the appropriate management of this clinical condition, aiming to minimize sequelae. Considering the importance of hyperviscosity in patients with Hemoglobin SC disease, the early institution of phlebotomy in those with higher levels of hemoglobin is crucial.