From Mondini to the latest inner ear malformations’ classifications: an historical and critical review

More than two hundred years ago, the Italian Carlo Mondini described the first inner ear malformation (IEM). From that moment, a lot of authors from all over the world put their effort on the discovery, description and classification of IEMs. Anatomists, radiologists and surgeons were involved in this amazing field. But the desire for more information on these anomalies should not be considered ephemeral; a deeper understanding of these pathologies has led over the years to the development of new surgical techniques and approaches for auditory rehabilitation of patients affected by congenital hearing loss. The development of new technologies, in particular in radiology and otolaryngology, had a tremendous impact on the evolution of the classification systems proposed over the decades. The evolution of computed tomography, magnetic resonance imaging (MRI) and cochlear implants is still having a great effect in current clinical management of this kind of patients. Even though so much was obtained over the centuries, we cannot really say that the quest is over. The present review aims to sum up the main historical steps that led to the development of the modern classification systems of IEMs