Metabolic bone disease refers to a spectrum of disorders characterized by abnormal bone formation or absorption. These diseases tend to present with generalized effects on the skeleton system. Direct involvement of the temporal bone can occur in these diseases, with resulting symptoms such as hearing loss, dysequilibrium, tinnitus, otorrhea, headaches, cosmetic deformities and cranial nerve deficits. These can often mimic other Otological diseases and a diagnosis requires an awareness of the key clinical features and radiological findings. A systematic review is presented in this two-part series. In the first part of this series, we discussed fibrous dysplasia, osteitis deformans (Paget’s disease), osteoporosis and osteomalacia. In part two of this review, the topics of otosclerosis, osteogenesis imperfecta, osteopetrosis and other rarer metabolic bone diseases are presented.