title: Cochlear implant in Cogan syndrome.
abstract: CONCLUSIONS
  Despite the need for special fitting strategies, improvements in speech discrimination tests support the use of cochlear implantation (CI) for patients with Cogan syndrome. Adequate preimplant counselling is mandatory, to prevent high expectations and to stress the necessity for bilateral implantation.
  
  
  OBJECTIVE
  In 60% of patients with Cogan syndrome, CI remains the only treatment option. Literature data agree that once the electrode array is properly inserted, functional outcomes are very good. Nevertheless, results may deteriorate due to progressive cochlear ossification. A few studies have documented the outcomes of CI in these patients, but none have reported the long-term results.
  
  
  METHODS
  This was a retrospective study describing the outcomes of 3 implanted patients with Cogan syndrome--among 300 adult patients who received a cochlear implant, 3 had become deaf due to Cogan syndrome.
  
  
  RESULTS
  In one patient the cochlear ossification advanced and the speech perception abilities worsened from the highest category to identification of words in closed set. The second patient complained of an abrupt reduction of loudness at 18 months post-implant, which required an increased electrical stimulation. The third patient reached the identification category probably due to auditory dyssynchrony, as an atypical consequence of the syndrome.
authors.affiliation: Audiology Department, University of Ferrara, Ospedale S.Anna, Corso Giovecca 203, Ferrara, Italy. roberto.bovo@unife.it
authors.forename: Roberto
authors.initials: R
authors.lastname: Bovo
authors.forename: Andrea
authors.initials: A
authors.lastname: Ciorba
authors.forename: Patrizia
authors.initials: P
authors.lastname: Trevisi
authors.forename: Claudia
authors.initials: C
authors.lastname: Aimoni
authors.forename: Lucia
authors.initials: L
authors.lastname: Cappiello
authors.forename: Alessandro
authors.initials: A
authors.lastname: Castiglione
authors.forename: Marcello
authors.initials: M
authors.lastname: Govoni
authors.forename: Alessandro
authors.initials: A
authors.lastname: Martini
mesh_terms: D000293:Adolescent; D000328:Adult; D001302:Audiometry, Speech; D019929:Cochlear Implantation; D055952:Cogan Syndrome / Q000150:complications*; D005260:Female; D006312:Hearing Loss, Bilateral / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008875:Middle Aged; D012189:Retrospective Studies; D013067:Speech Perception; D016896:Treatment Outcome