title: Deterioration of hearing in a cochlear implantee with relapsing polychondritis.
abstract: We report on a rare case of cochlear implantation in a patient affected by relapsing polychondritis (RP), which over time induced cochlear fibrosis/ossification and deterioration of previously excellent hearing performance. The clinical course was determined by CT scan, electrophysiology, and speech perception data. We conclude that RP is a severe autoimmune connective disorder that can cause profound sensorineural hearing loss. Cochlear implantation in these patients can provide excellent performance. Continuation of therapy may improve prognosis, but relapses involving inner ear structures can determine fibrosis/ossification of the modiolus and interfere with cochlear implant use.
authors.affiliation: Department of Sensory Organs, University La Sapienza of Rome, Italy. p.mancini@uniroma1.it
authors.forename: Mancini
authors.initials: M
authors.lastname: Patrizia
authors.forename: Attanasio
authors.initials: A
authors.lastname: Giuseppe
authors.forename: Viccaro
authors.initials: V
authors.lastname: Marika
authors.forename: Filipo
authors.initials: F
authors.lastname: Roberto
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D001309:Auditory Threshold / Q000187:drug effects; D015834:Cochlear Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D019929:Cochlear Implantation; D003520:Cyclophosphamide / Q000627:therapeutic use; D003638:Deafness / Q000209:etiology* / Q000534:rehabilitation*; D004567:Electrodes, Implanted; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D000069285:Infliximab; D009999:Ossification, Heterotopic / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D011081:Polychondritis, Relapsing / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D011183:Postoperative Complications / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D011474:Prosthesis Design; D012008:Recurrence; D013069:Speech Reception Threshold Test; D014057:Tomography, X-Ray Computed
chemical_list: D000911:Antibodies, Monoclonal; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D000069285:Infliximab