CLINICAL PROBLEM SOLVING: RADIOLOGY

91-YEAR-OLD woman presented with a lifelong history of bilateral hearing loss that had resulted in a loss of all residual hearing over the preceding 3 months. The hearing loss became profound during her perimenopausal period. Previously, her residual hearing improved with hearing aids. There were no complaints of tinnitus, vertigo, aural fullness, otalgia, otorrhea, or facial palsy. Her medical history revealed no chronic ear infections, head trauma, meningitis, syphilis, ototoxic drug exposure, or hearing loss due to loud noise exposure. She had undergone no surgical procedures on her ears. There was no hearing loss in her family history. The findings of her physical examination were significant only for an enlarged cranium (Figure 1). Otoscopic examination revealed no abnormalities. Audiometry showed severe bilateral hearing loss that could not be tested. The bone conduction tests were only significant for a vibratory tactile response. A computed tomographic scan of the temporal bone (Figures 2, 3, and 4) demonstrated areas of mixed osteolysis and osteosclerosis, partial thickening of the calvaria, and variable attenuation of the cochlear lumen bilaterally (arrows). What is your diagnosis?