Standard variant venous dysplasia of the cerebellum in a patient suffering from Muenke's syndrome and tinnitus.

BACKGROUND

Muenke's 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 Muenke's syndrome intracranial vascular dysplasia has not been reported yet.

PATIENT AND

METHODS

A 13-year-old boy suffering from Muenke's 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 Muenke's syndrome especially in cases of clinically suggestive symptoms like pulsating tinnitus.