Post-CI Mastoiditis with Otoliquorrhea in Mondini Dysplasia: A family case

The classification of inner ear dysplasias is based on Sennaroglu's nomenclature. In Mondini dysplasia, the turns of the bony cochlea are incomplete, and the membranous cochlea is only rudimentary. Cochlear implantation is an option for these patients. In children with such dysplasia, there is a risk of a gusher phenomenon both intra- and postoperatively. We report on a pair of twins with Mondini dysplasia who experienced an acute mastoiditis with otoliquorrhea within a span of 3 weeks. The five-year-old sister, who underwent bilateral cochlear implantation due to bilateral deafness, presented urgently due to retroauricular swelling on the right side. The parents reported a progressively worsening infection over several weeks. Clinically, there was a pronounced mastoiditis on the right side. A complete opacification of the middle ear and right mastoid was observed in the DVT. An emergency mastoid revision with explantation of the cochlear implant and immediate reimplantation was performed due to the otoliquorrhea. Three weeks later, the twin brother experienced a similar scenario with the clinical picture of acute mastoiditis on the right side. He also had a history of bilateral cochlear implantation due to Mondini malformation. An emergency re-mastoidectomy on the right side was performed to close the perilymph fistula. However, the cochlear implant could be preserved in this case. The twins are doing well and have no symptoms four months after the operation. This case report highlights the importance of close follow-up of children with Mondini dysplasia who have CIs. Parents should be particularly aware and prompt in seeking the treating ENT specialist in case of acute inflammatory ear conditions.