Otitis media in children with congenital immunodeficiencies.

Otitis media represents one of the most common infections in childhood. Within the first 3 years of life, up to 80% of children experience at least one episode of otitis media. It is often resolved with supportive therapies and consequently not considered a worrisome problem. However, it may be an early manifestation of a severe underlying disease. Primary immunodeficiencies are rare congenital defects of the immune system that often remain unrecognized, or diagnosis can be delayed, sometimes resulting in fatal consequences for the child. Patients suffer from recurrent, prolonged, and/or unusual infections leading to local sequelae, failure to thrive, developmental delays, and systemic infections with severe courses. This review provides a brief insight into primary immunodeficiencies and an overview of leading findings that should result in further evaluation of the immune system in cases of otitis media. A stepwise diagnostic approach is proposed to facilitate early and accurate diagnosis and, consequently, effective and timely therapy to improve the patient's outcome and quality of life.