Objective: To evaluate outcomes and issues pertaining to cochlear implantation in a group of subjects affected by Cogan syndrome. Study Design: Prospective cohort. Setting: Department of Ophthalmology and Otorhinolaryngology, University of Parma. Patients: Five postlingually deafened adults suffering from a typical form of Cogan syndrome who underwent cochlear implantation. Main Outcome Measures: Benefit from cochlear implantation as measured by word and everyday sentence recognition tests. Surgical issues and postoperative complications were also evaluated. Results: In two cases, intracochlear electrodes were inserted into the scala vestibuli because of the ossification of the scala tympani. Two patients experienced a recurrence of keratitis the day after surgery. To date, with a follow-up of 1 to 4 years, no patient has experienced flap complications or other local or systemic complications. At the 12-month postoperative evaluation, all patients had gained useful open-set speech perception, achieving a mean score of 91% and 95% on word and everyday sentence recognition tests, respectively. Conclusions: Patients deafened by Cogan syndrome demonstrated high levels of speech understanding after undergoing cochlear implantation. Obliteration of the cochlea may complicate electrode implantation, requiring modifications of the surgical technique. Stress consequent to the surgical procedure may instigate an acute phase of the basic illness
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