The temporal bones are paired structures located on the lateral aspects of the skull and contribute to the skull base. Trauma is usually the result of blunt head injury and can result in damage to the brain and meninges, the middle and internal ear, and the facial nerve. Complications can include intracranial hemorrhage, cerebral contusion, CSF leak and meningitis, hearing loss, vertigo, and facial paralysis. To prevent these complications, diagnosis followed by appropriate medical and surgical management is critical. Diagnosis relies primarily on physical signs and symptoms as well as radiographic imaging. Emergent intervention is required in situations involving herniation of the brain into the middle ear cavity or hemorrhage of the intratemporal carotid artery. Patients with declining facial nerve function are candidates for early surgical intervention. Conductive hearing loss can be corrected surgically as an elective procedure, while sensorineural hearing loss carries a poor prognosis, regardless of management approach. Children generally recover from temporal bone trauma with fewer complications than adults and experience a markedly lower incidence of facial nerve paralysis.

The Finnish type of familial amyloid polyneuropathy due to variant gelsolin is a rare form of familial amyloidosis. The subtype was first described in 1969 and is characterized by progressive cranial neuropathies, corneal lattice dystrophy and distal sensorimotor dysfunction. It is extremely uncommon, with only two families known to be affected in the UK. We discuss the case of a 70-year-old woman who presented with bilateral facial nerve palsies, bilateral sensorineural hearing loss and Finnish type familial hereditary amyloidosis. A literature search of the Medline database (1966-2005) was performed, using the keywords 'amyloid', 'hearing loss' and 'facial palsy'; however, this association appears to be a novel finding. We review the current literature and discuss otorhinolaryngological presentations of amyloidosis.