The Sensorineural Hearing Loss Related to a Rare Infratentorial Developmental Venous Angioma: A Case Report and Review of Literature

Abstract

Background Developmental venous anomaly (DVA) is a benign venous abnormality draining normal brain parenchyma. It is mostly asymptomatic; however, rare complications such as hemorrhage may lead to symptomatic conditions. Headache and seizure are the most common symptoms. Hearing loss is an extremely rare presentation of DVA. To our knowledge, only five cases of DVA, presenting with hearing loss, had been reported so far. Case Presentation We report the case of a 27-year-old woman who presented with a sensorineural hearing loss followed by facial paresis. Magnetic resonance imaging (MRI) and computed tomography (CT) angiography revealed hematoma with adjacent converging veins showing a typical “caput medusa” sign in the left middle cerebellar peduncle, in favor of DVA. Due to the compression effect of hematoma, she underwent surgery. Hearing loss and facial paresis improved significantly during the postoperative follow-up. Conclusion Although DVA is mostly benign and asymptomatic, complications such as hemorrhage rarely occur. Hearing loss is an extremely rare presentation that can be attributable to the compression effect on the cranial nerve VII to VIII complex. In the case of compression effect or progression of symptoms, surgical intervention is necessary. A good clinical outcome could be expected postoperatively.

Atlas of Nervous System Vascular Malformations: A Systematic Review

“…Specifically, when the location is infratentorial, such as in the middle cerebellar peduncle, neurosensory hearing loss has been reported, followed by facial paresis, due to the anatomical relationship of the lesion’s location with respect to cranial nerves VII to VIII. However, this is an extremely rare manifestation [ 20 ]. It should be underscored that this malformation’s main location is where the superficial and deep supratentorial blood drains converge, usually adjacent to the cortical or ependymal surface; they occur in the cerebellum to a lesser extent [ 17 ].…”

“…The increasing complexity of therapeutic approaches to vascular malformations highlights the need to avoid confusing them with each other through correct characterization. Although clinical data may be useful in this process, such data are not entirely decisive, given that at a clinical level there may be silent courses, as in the cases of CCT [ 58 ] and small CAs [ 31 ] or clinical manifestations that are closely similar, such headaches and seizures that are present in most of the different vascular malformation cases [ 17 , 20 , 32 , 33 , 49 , 50 , 51 , 60 , 62 , 68 ]. An example of a fairly useful clinical feature is port wine stain in the case of SWS [ 63 , 64 , 65 ]; however, this syndrome will only occur in 8% to 33% of the subjects with the skin mark [ 70 ].…”