Rare Diseases of the Middle Ear and Lateral Skull Base

Otalgia, otorrhea and hearing loss are the most common ear-related symptoms that lead to the consultation of an otolaryngologist. Furthermore, balance disorders and affections of the cranial nerve function may play a role in the consultation. In large academic centres, but also in primary care, the identification of rare diseases of the middle ear and the lateral skull base is essential, as these diseases often require interdisciplinary approaches to establish the correct diagnosis and to initiate safe and adequate treatments. This review provides an overview of rare bone, neoplastic, haematological, autoimmunological and infectious disorders as well as malformations that may manifest in the middle ear and the lateral skull base. Knowledge of rare disorders is an essential factor ensuring the quality of patient care, in particular surgical procedures. Notably, in untypical, complicated, and prolonged disease courses, rare differential diagnoses need to be considered.