Anterior Skull Base Abnormalities in Congenital Anosmia

Introduction: The structures of the skull and the brain are related to each other. Prior work in individuals with isolated congenital anosmia (ICA) showed that these individuals were characterized by olfactory bulb (OB) defects. The aim of this study was to compare the morphological pattern of the anterior skull base surrounding the OB between individuals with ICA and normosmic controls. We meant to investigate whether these features can help distinguish abnormalities from normal variation.

Methods: We conducted a retrospective study to acquire T2-weighted magnetic resonance images from individuals diagnosed with ICA (n = 31) and healthy, normosmic controls matched for age and gender (n = 62). Between both groups, we compared the depth and width of the olfactory fossa, the angle of the ethmoidal fovea, as well as the angle of the lateral lamella of the cribriform plate. Within the ICA group, we further performed subgroup analyses based on the presence or absence of the OB, to investigate whether the morphology of the anterior skull base relates to the presence of OBs. The diagnostic performance of these parameters was evaluated using receiver operating characteristic analysis.

Results: Individuals with ICA exhibited a flattened ethmoid roof and shallower olfactory fossa when compared to controls. Further, the absence of the OB was found to be associated with a higher degree of flattening of the ethmoid roof and a shallow olfactory fossa. We reached the results in the following areas under the receiver operating characteristic curves: 0.80 - angle of fovea ethmoidalis, 0.76 - depth of olfactory fossa, 0.70 - angle of lateral lamella of the cribriform plate for significant differentiation between individuals with ICA and normosmic controls.

Conclusion: Individuals with ICA exhibited an unusual anterior skull base surrounding the OB. This study supports the idea of an integrated development of OB and anterior skull base. Hence, the morphological pattern of the anterior skull base surrounding the OB helps distinguish individuals with ICA from normosmic controls and may therefore be useful for the diagnosis of ICA, although it is certainly not an invariable sign of congenital anosmia.