The aim of the study is to identify the most common minor malformations encountered in the inner ear and cochlear nerve in patients with profound bilateral sensorineural hearing loss (SNHL) of unspecified cause. Material and method Images obtained by CT scan were compared in a control group and a group of patients with bilateral profound SNHL of unspecified cause, to detect the most common anatomical changes in the inner ear and cochlear nerve. Results and discussions. Inner ear malformations come in a variety of forms from complete aplasia to subtle hypoplasia of some inner ear elements and adjacent nervous structures contained in the internal auditory canal. Statistics descriptive parameters calculation were obtained for the two groups for each item measured for each ear separately. The existence of anatomical malformations of the inner ear was one proposed factor to be influencing the results after cochlear implantation, but an exact prediction is difficult as there are different other variables involved. Conclusions. Simple visual inspection of CT images proves to be insufficient in delivering the best individualized treatment in the pathology of inner ear.