Prompted by a recurring skull base multidisciplinary team debate on the necessity of securing a definitive tissue diagnosis before initiating treatment for lesions of the orbital apex, a review of anterior skull base procedures over an 11-year period was undertaken.

Data collected prospectively on cases from 2006 to 2017 were analysed. Presenting symptoms, imaging and histology findings, outcomes, complications, and impact on treatment were evaluated. All surgery was carried out endoscopically with the aid of image guidance.

Twenty-one patients undergoing endoscopic orbital apex and/or optic canal biopsy were included. The mean patient age was 49 years. Five malignant tumours were identified, five benign tumours, seven infective cases (two tuberculosis and five fungal) and two cases of immunoglobulin G4 related disease. Two patients had non-diagnostic biopsies (one lesional) and were treated successfully as Tolosa–Hunt syndrome cases.

A successful diagnosis was achieved in nearly all cases without adverse impact, other than one cerebrospinal fluid leakage case. Management was directly influenced by the outcome in all cases.

To report a case of multifocal fibrosclerosis with a nine-year follow up, and to discuss this disease's radiological appearance and management. The disease is a rare systemic disorder of unknown cause characterised by fibrous proliferation involving multiple anatomical sites.

A 50-year-old woman presented with histological findings characterised by similar inflammatory processes involving the meninges, pituitary gland, peritoneum, retroperitoneum and orbits, prompting a search for a common pathophysiology. A diagnosis of multifocal fibrosclerosis was postulated. Symptom improvement was noted after treatment with prednisone and azathioprine.

This is the first documented case of involvement of the cochleovestibular nerve in a patient with multifocal fibrosclerosis. The rare association between fibrotic diseases and masses showing various clinical patterns should be kept in mind by otolaryngologists, and imaging performed to investigate for multifocal fibrosclerosis. However, diagnosis can only be confirmed with tissue biopsy and histopathological examination.