[Congenital absence of the stapes suprastructure]. / Agenesia de la supraestructura del estribo.

Congenital absence of the stapes suprastructure is an unusual anomaly, consequence of a developmental aberration involving partial absence of a derivate from the second branchial arch. Sometimes, exploratory tympanotomy may be the only mean of diagnosis when known conductive hearing loss is seen. This anomaly can appear isolated or associated to other major or minor anomalies. The treatment is surgical. We review the literature about the absence of the isolated stapes suprastructure.

[Susac syndrome as a cause of sensorineural hearing loss]. / Síndrome de Susac como causa de hipoacusia neurosensorial.

Susac's syndrome is an extremely rare clinical manifestation characterized by the triad of fluctuating sensorineural hearing loss, sudden visual loss and encephalopathy. Probably underdiagnosed, it affects young women who start the clinical history with headache, visual and hearing disturbances, with neurological findings in MRI. With unknown aetiology, pathogenesis is based on arteriolar microinfarcts in retina, cochlea, and grey and white matter in the brain. Treatment is, as stated in the bibliography and our experience, intravenous high doses of steroids followed by oral steroids together with hyperbaric oxygen to minimize ischaemic lesions. Aspirin associate to nimodipine has been useful to date in the treatment of our patient. We present a case and review the existing literature.