A Mother with a Child with Mondini Dysplasia


A Chinese colleague wrote to me about a young mother in Shanghai who has a baby girl born with Mondini Dysplasia.

Mondini dysplasia is a type of inner ear malformation that is present at birth, in which affected individuals have one and one half coils of the cochlea instead of the normal two coils. It may be unilateral or bilateral and can cause sensorineural hearing loss and a predisposition to recurrent meningitis. Mondini dysplasia may be associated with other ear malformations or other syndromes. Treatment options include surgical repair of the defect in an attempt to prevent meningitis, hearing aids, and cochlear implants.

My colleague in Shanghai asked how this mother could help her little girl during the years before undergoing a surgical operation to “build” a new ear (she was told that this operation can only be done around 14 years old).  She worries that this defect may result in “disorders of sensory integration”. My colleague describes the mother as a strong and intelligent woman who is willing to learn about the problem and do everything she can to support her daughter’s healthy development. 

I responded to my colleague that I would like to know more about her friend’s child’s problem – the degree of severity, whether it is unilateral or bilateral, what her particular deficits are, and what her strengths are – so that when I write something about it I am sure to address her particular questions. Actually, what would be best is if she could ask the mother what questions she would like answered. 

Before hearing from the mother about her specific questions, I will inquire of my colleagues who might treat children with Mondini dysplasia, and also those who work with children who have more general sensori-perceptual problems. In the meantime, the most important recommendation I have for her friend is to build a loving relationship with her child and to gather around her people to support her while she does that.

My colleague, Debbie Bausch, an O.T. who specializes in sensori-perceptual disorders, responded: “When I looked up Mondini Dysplasia, the site mentioned that there could be absence not only of the cochlea but possibly the inner ear- semi circular canals, etc. If this were the case, I would suspect a greater possible degree of SPD (sensori-perceptual disorder). The major issue would be balance deficits- having difficulty knowing where your head was in relation to gravity. In order to answer this we’d have to know more about the specifics of how Mondini dysplagia affected this child including whether it is unilateral, bilateral, etc.”

I am hoping that other readers with greater familiarity with this disorder than I can send me a comment that will help this mother. 

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