The aim of the study is to present the severe, extensive lesions in the temporal bone appearing in the mildest forms of osteogenesis imperfecta, correlate these with audiometric results and discuss the possible surgical treatments available. We present three patients suffering hearing loss due to osteogenesis imperfecta type 1 to various functional degrees. All patients underwent an audiological, medical and radiological evaluation and were then treated with different surgical procedures. The patients presented mild, severe and profound mixed hearing losses and the radiological images showed extensive areas of demineralization affecting the ossicular chain and removal of protection for the vital structures inside the temporal bone. Also, the cochlea showed otic capsule demineralization, dehiscence, distortions and even destructions. The various surgical treatments, indicated under current international criteria, obtained limited functional results. This study reviews the need to evaluate the current surgical criteria for this specific condition.