Standard variant venous dysplasia of the cerebellum in a patient suffering from Muenkeʹs syndrome and tinnitus

Background: Muenkeʹs syndrome is a rarely seen craniosynostosis syndrome. Bilateral or unilateral coronal craniosynostosis, brachydactyly, tarsal and carpal coalitions are the typical clinical findings. Sensorineural hearing loss is reported in more than 30% of the affected patients. In patients with Muenkeʹs syndrome intracranial vascular dysplasia has not been reported yet. Patient and methods: A 13-year-old boy suffering from Muenkeʹs syndrome reported a pulsating left-sided light tinnitus. Clinically a bluish formation was found at the lower part of the left-sided ear drum. In order to clarify the cause of the tinnitus and the bluish formation, extensive diagnostics (e.g. audiometry, magnet resonance tomography, magnet resonance phlebography, angiography) were carried out. Results: Audiometry found a left sensorineural hearing loss with a conductive component. Radiological investigations found a standard variant venous dysplasia of the left petrous bone to be the reason for the tinnitus. Conclusion: Awareness of intracranial vascular dysplasia should be noted in individuals with Muenkeʹs syndrome especially in cases of clinically suggestive symptoms like pulsating tinnitus.