Mondini malformations generally affect hearing and are a common type of inner ear malformation.
Mondini malformation is a congenital malformation of incomplete separation type II, usually combined with enlargement of the vestibular aqueduct. The etiology of the disease is mostly related to autosomal dominant inheritance, and may involve one or both ears.
The main manifestation is severe hearing impairment or total deafness in the affected ear, and the auditory brainstem response cannot be elicited. However, when accompanied by vestibular dysfunction, vertigo and other symptoms may also occur. Affected children are born without hearing, or develop no hearing at the age of 1 to 2 years, or even later hearing loss.
Therefore, in the event of mondini malformation, parents need to cooperate with doctors to take treatment measures.