A New Treatment Option in Incomplete Partition Type III: The Varese Bone-Air Stimulation (B.A.S.)

The incomplete partition type III is a severe cochlear malformation present in X-linked deafness. It is a rare, non-syndromic cause of severe to profound mixed hearing loss, often progressive. The complete absence of bony modiolus and the wide communication between the cochlea and the internal auditory canal make cochlear implantation challenging, with still no consensus on the management of these patients. To the best of our knowledge, no results have ever been published in the literature on the treatment of these patients with hybrid stimulation (bone and air). We present three cases in which this hybrid stimulation gave better audiological results then air stimulation alone. A literature review on audiological results of the current treatment options in children affected by IPIII malformation was conducted independently by two researchers. Ethical considerations on the treatment of these patients were conducted by the Bioethics department of the University of Insubria. In two of the patients, the bone-air stimulation, associated with prosthetic-cognitive rehabilitation, meant that surgery was avoided, obtaining similar communication performances of those present in the literature. We believe that, when the bone threshold appears partially preserved, a stimulation through the bone or hybrid modality, such as the Varese B.A.S. stimulation, should be attempted.

Automated summary

Incomplete partition type III (IPIII) is a severe cochlear malformation associated with X-linked deafness. It causes severe to profound mixed hearing loss, and there is currently no consensus on the treatment approach. This study presents three cases in which hybrid stimulation (bone and air) resulted in better audiological outcomes than air stimulation alone. The findings suggest that hybrid stimulation should be considered when the bone threshold appears partially preserved.