Pier Marco Bianchi, Renato Gaini, and Silvano Vitale
MPS are rare, progressive, and multisystem diseases with insidious signs and symptoms. Various ENT manifestations appear in the early stage of MPS, including otitis media, macroglossia, adenotonsillar hypertrophy, nasal obstruction, OSAS, progressive respiratory disorders, and hearing loss. As the incidence of hearing loss is high in MPS patients, hearing loss should be determined at an early stage. After the diagnosis, the required treatments should be started promptly with the aim of increasing the quality of life. However, it has to be considered that this is only possible as a result of regular and systematic follow-up.