Below is an instruction from Human. Write a response.
    ### Instruction:
    What are the symptoms of Mondini dysplasia ?
    ### Response:
    What are the signs and symptoms of Mondini dysplasia? Mondini dysplasia is a congenital malformation (present at birth). It may occur either unilaterally (in one ear) or bilaterally (in both ears). Most affected individuals have profound sensorineural hearing loss, but some individuals do have residual hearing.
    