Osteopathia striata-cranial sclerosis: otorhinolaryngologic clinical presentation and radiologic findings.

Background: Osteopathia striata with cranial sclerosis (OS-CS) is a rare bone dysplasia that presents with variable symptoms mainly due to the associated CS.

Methods: The otorhinolaryngologic clinical presentation and temporal bone computed tomography (CT) findings of a new case of OS-CS are presented. Differential diagnosis is also discussed.

Results: Our study confirms the typical appearance of the temporal bone reported in previous CT studies and the utility of CT for evaluating the various etiologies of conductive-mixed hearing loss present in OS-CS. Radiographs of the femur and pelvis revealed the typical striations of OS-CS and allowed us to diagnose this rare form of generalized skeletal dysplasia involving the skull base.

Conclusions: To our knowledge, this is the third reported case of OS-CS in which temporal bone CT evaluation corroborated the smallness of the middle ear cavities, abnormal ossicular fixation, and bone sclerosis of the mastoid cells. Other radiographic studies regarding the whole skeleton may, in combination, be very helpful in the differential diagnosis between the different forms of generalized skeletal diseases with bone sclerosis involving the skull base.