Inner ear malformations present primarily with auditory symptoms while vestibular malformations, though clinically significant, are under-recognized .The seminar aims to explore the correlation between functional assessments—specifically audiological and vestibular evaluations—and radiological findings in patients with inner ear malformations. Particular attention is given to discrepancies that may arise between clinical and imaging data, and how these mismatches can influence therapeutic decisions. Incomplete cochlear partitions, 8th cranial nerve dysplasia and enlarged vestibular aqueduct are some examples. Third window syndromes blur the line between congenital and acquired pathology. It highlights the complex interplay between hearing and balance dysfunction in the context of inner ear anomalies.