title: Favorable outcome of cochlear implant in VIIIth nerve deficiency.
abstract: OBJECTIVE
  To report on the outcomes of cochlear implantation (CI) in a child with cochleovestibular nerves (CVN) hypoplasia.
  
  
  METHODS
  Retrospective case review.
  
  
  METHODS
  Tertiary referral center, University hospital.
  
  
  METHODS
  An 18-month-old child with profound bilateral congenital hearing loss and bilateral hypoplasia of the CVN at imaging.
  
  
  METHODS
  Left CI at age 29 months with a Nucleus Contour device (Cochlear Ltd., Lane Cove, New South Wales, Australia) after unsatisfactory results of hearing aid use for 10 months.
  
  
  METHODS
  Speech perception tests, behavioral observation, electrophysiologic tests, and cognitive evaluation.
  
  
  RESULTS
  Although the child scores poorly in every perceptive category with the CI alone, the device greatly enhances his speech understanding with the hearing aid in the opposite ear. In the bimodal condition, his words and sentences identification, recognition, and comprehension far exceed the monaural figures. The Meaningful Auditory Integration Scale (MAIS) tests reaches a score of 26/40, and the MacArthur's questionnaires confirm the improvement of language production and comprehension. These results became noticeable after 5 to 6 months and continued to improve up to the 10th month. The child's cognitive scores and overall performance competences greatly benefit from the CI, with the mental age overcoming the chronological age.
  
  
  CONCLUSIONS
  We can confirm the chance of achieving satisfactory results by CI even when the imaging of CVN is doubtful and the electrophysiological tests are disappointing. In our experience, a CI in Type IIb dysplasia of the CVN is a feasible option, provided that the candidate shows some responses at aided audiogram and at least minimal signs of language development. Adequate counseling is necessary for these children because the expected outcome is somewhat lower than that of their deaf peers with normal appearance of the nerves.
authors.affiliation: Otorhinolaryngologic Department, University of Brescia, Brescia, Italy. diego_zanetti@iol.it
authors.forename: Diego
authors.initials: D
authors.lastname: Zanetti
authors.forename: Maurizio
authors.initials: M
authors.lastname: Guida
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Barezzani
authors.forename: Chiara
authors.initials: C
authors.lastname: Campovecchi
authors.forename: Nader
authors.initials: N
authors.lastname: Nassif
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Pinelli
authors.forename: Lucio
authors.initials: L
authors.lastname: Giordano
authors.forename: Giovanna
authors.initials: G
authors.lastname: Olioso
mesh_terms: D001301:Audiometry, Pure-Tone; D001309:Auditory Threshold; D019929:Cochlear Implantation / Q000379:methods; D003054:Cochlear Implants; D005260:Female; D006310:Hearing Aids; D034381:Hearing Loss / Q000209:etiology / Q000601:surgery / Q000628:therapy*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D013063:Speech Discrimination Tests; D013067:Speech Perception; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D000159:Vestibulocochlear Nerve / Q000002:abnormalities*