Since 1832 many families have been described showing hearing loss and branchial arch abnormalities transmitted in an autosomal dominant mode (Branchiooto Syndrome). In 1976 one family was described showing renal malformations associated with hearing loss and branchial arch anomalies also with an autosomal dominant mode of transmission. This was called Branchio-otorenal Syndrome. Today it is widely accepted that branchiooto and branchio-otorenal syndrome are variants of the same genetic mutation in a single gene mapped to chromosome 8q. Hearing loss in BOR is most often mixed, sometimes conductive and less frequently sensorineural. Surgical findings on the middle ear and ossicles have not been well described and documented before. We present and document our surgical findings in the middle ear and ossicles on three patients from two different families. Some of these findings have not been described before and the amazing differences between different families illustrate the phenotypic variability in BOR.