Abstract
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.
Key words
Differential diagnosis - otorrhoea - otalgia - rare entity - manifestation - otitis
media
