Progressive Loss of Sensitivity to Electrical Stimulation After Cochlear Implantation in X-Linked Incomplete Partition Type III Deafness

Objectives:Patients with X-linked incomplete partition type III (IP3) deafness treated with cochlear implants exhibit higher "Most Comfortable Loudness" (MCL) levels of stimulation and more electrode deactivation than patients with normal morphology. We endeavored to analyze the progression of the MCL levels and electrode deactivation over time and assess those factors that could have led to deactivation. Furthermore, we aimed to assess whether speech perception was affected by a progressive loss of neural contact.Design:All 13 patients with the IP3 malformation in our clinical database were analyzed retrospectively with regard to impedance, stimulation levels, deactivated electrodes, and speech perception. A control group of patients with normal anatomy was included.Results:MCL levels increased over time by 2.5 charge units (qu) per year, which was not seen in the control group. Electrode deactivation was more common in IP3 malformation, and it was estimated that 25% of electrodes would be deactivated by 15 years of age. Impedance was stable but higher in the study population. Speech perception was lower in IP3 malformation generally and was correlated to the number of deactivated electrodes.Conclusions:Patients diagnosed with IP3 malformation deafness may suffer a greater risk of cochlear implant discontinuation compared with those with normal anatomy. A progressive loss of sensitivity to electrical stimulation may indicate a form of neural degradation in the abnormal cochlea. With time, patients in this group, even with cochlear implant technology, may experience gradual deterioration of speech perception. This has clinical implications for the counseling of parents.