Congenital malformations of the inner ear

Summary Congenital malformations of the inner ear consist of many different anomalies of the labyrinth. They often cause hearing loss, mostly of the sensorineural type. Eighty percent of hearing loss is caused by an anomaly of the membranous labyrinth, and 20% by an anomaly of the bone labyrinth. The role in pathogenesis is played by hereditary factors and influence of the environment. The treatment depends on the severity of the hearing loss, abnormalities of the external and middle ear, associated defects, and presence and function of the auditory nerve. We have modern hearing aids or implantable systems. Another options include a sign language and mouth-reading. The article includes a retrospective analysis of patients with congenital inner ear malformations at our tertiary center in 2010–2020. In conclusion, our patients clearly prove that even children with profound hearing loss are successfully implanted and restored hearing can be achieved in most of them. Key words congenital ear malformation – inner ear – hearing loss – anomalies of bony labyrinth – anomalies of membranous labyrinth – rehabilitation