Below is an instruction from Human. Write a response.
    ### Instruction:
    What is (are) Mondini dysplasia ?
    ### Response:
    Mondini dysplasia is a type of inner ear malformation that is present at birth (congenital). Individuals with Mondini dysplasia have one and a half coils of the cochlea instead of the normal two coils. It may occur in one ear (unilateral) or both ears (bilateral) and can cause varying degrees of sensorineural hearing loss, although most individuals have profound hearing loss.
    