A 41-year-old man complained of a full right ear, periauricular pain, and right-sided neck pain since approximately 25 years for which he had consulted several ENT surgeons. He tried to alleviate his discomfort by Valsalva maneuvers that were performed frequently. About 10 years ago he started to notice recurrent visual symptoms in his left visual field, usually after ‘‘clearing’’ his ear. The auras consisted of slowly moving crystal-like images that started either on the left side of the visual field or centrally, moving to the lateral left hemifield. Sometimes they were colored (orange, white, never black). They started as small, half-moon-like figures that gradually enlarged. These aura images lasted approximately 20–30 min. This was followed by visual field disturbance on the left side that sometimes lasted for several days. The patient was aware he lacked his left hemifield. Besides the visual field disturbance he reported vague dizziness that also lasted several days. Nystagmus had not been observed and neither could we illicit a history of oscillopsia. Auras sometimes started without preceding Valsalva maneuver, but most of the approximately hundred auras that he had experienced were after Valsalva and Valsalvalike (such as traveling and riding a bike in the hills) maneuvers, usually consisting of a forceful exhalation against a closed airway. He often performed the maneuvers aggressively. Following the Valsalva maneuver he experienced a dull sensation on the right posterior part of his head, but never suffered from headache. Because of long-standing complaints of the patient a computed tomography (CT) scan of the head was performed, which showed a hyperpneumatization of the temporal, parietal, and occipital bone on the right side. The air collection was located epidurally with a very thin layer of tabula interna still intact (epidural pneumocele, Fig. 1a). Subsequently an ear drum grommet was inserted into his right ear drum in March 2008. During the procedure, an air flow through the grommet was noticed. For 4 months, Valsalva maneuvers were no longer needed and he did not experience any auras. However, on the day of his marriage, he carried his new bride over the threshold of their home. Shortly thereafter he noticed blood running from his right ear and that night his pillow was covered with blood. The following day extraordinary long auras came and went, prompting him to visit the ER department. A new CT scan of the head was performed, which showed no changes with respect to the known hyperpneumatization of the right temporal and occipital bone. During the following year up to the present there have been only a few auras. Follow-up ENT examinations did not show any signs of infection of the bony cavity (various negative swabs and negative beta-2 transferrin-tests for liquorrhea) even though a chronic otorrhea persisted N. J. Weerkamp (&) P. J. Koehler Department of Neurology, Atrium Medical Center, Henri Dunantstraat 5, 6401 CX Heerlen, The Netherlands e-mail: nicoweerkamp@hotmail.com