Camurati-Engelmann's disease (CED) is a rare hereditary disorder affecting mainly the diaphysis of long bones but multiple cranial nerve deficits may also develop secondary to bony sclerosis of their foramina, including visual loss, facial palsy, deafness, vestibular disturbances and sensory deficits along the distribution of the trigeminal nerve. Deafness has been reported in about 18 per cent of these cases due to narrowing of the internal auditory canals caused by bony encroachment on nerves and vessels. We report an extremely rare case of a patient with CED who presented with deafness due to gross abnormalities affecting both middle ear and cochlea. The issues relating to the management of these patients with temporal bone involvement are discussed.