Inner ear malformations are an important cause of sensorineural hearing loss ranging from severe to profound and they also contribute to Cerebrospinal fluid leakage and recurrent meningitis. The most severe is the complete labyrinthine aplasia (Michel Deformity), then there is cochlear aplasia, cochlear hypoplasia, incomplete partition of the cochlea, enlarged vestibular acqueduct and cochlear aperture abnormality.
© Association of Otolaryngologists of India 2021.