Prions in otolaryngology: literature review

Abstract

Prion diseases are rare, transmissible and rapidly progressive neurodegenerative diseases caused by the misfolding of the cellular prion protein to its pathological form. The most prevalent is Creutzfeldt-Jakob disease (CJD), which has a higher proportion of cases in Chile compared to other countries. Although they are not otorhinolaryngological diseases, they have important implications in this specialty. Initial manifestations of the disease have been described as hearing loss, vertigo, dysphonia, dysphagia, anosmia, chordal paralysis, among others, which can be confused with peripheral pathologies, delaying the diagnosis. Additionally, the presence of prions in nasal mucosa, middle ear and tonsils implies a risk of iatrogenic transmission, since they are resistant to conventional sterilization methods. Knowledge of these pathologies is essential for early clinical suspicion, implementation of infection control measures, and the multidisciplinary management of affected patients.