One of the conditions causing hearing loss that I often see parents asking about, is Mondini syndrome, also known as Mondini dysplasia or a Mondini malformation. With this condition, the cochlea is incomplete, with only one and a half turns instead of the normal two and a half turns. This results in gradual or even sudden hearing loss that may be profound in nature. Pictures are available on the web of Mondini malformations. Quite a bit of research has been done into Mondini. In addition, many parents opt to use cochlear implants to correct the hearing loss associated with Mondini. It is not difficult to find other parents of children with Mondini malformations in order to exchange information.
Research
- National Temporaral Bone Registry which enrolls and donates temporaral bones for research purposes. A search of the registry's website turns up some mentions of Mondini.
