title: Speech recognition and communication outcomes with cochlear implantation in Usher syndrome type 3.
abstract: BACKGROUND
  Usher syndrome Type 3 (USH3) is an autosomal recessive disorder characterized by variable type and degree of progressive sensorineural hearing loss and retinitis pigmentosa. Cochlear implants are widely used among these patients.
  
  
  OBJECTIVE
  To evaluate the results and benefits of cochlear implantation in patients with USH3.
  
  
  METHODS
  A nationwide multicenter retrospective review.
  
  
  METHODS
  During the years 1995-2005, in 5 Finnish university hospitals, 19 patients with USH3 received a cochlear implant. Saliva samples were collected to verify the USH3 genotype. Patients answered to 3 questionnaires: Glasgow Benefit Inventory, Glasgow Health Status Inventory, and a self-made questionnaire. Audiological data were collected from patient records.
  
  
  RESULTS
  All the patients with USH3 in the study were homozygous for the Finnish major mutation (p.Y176X). Either they had severe sensorineural hearing loss or they were profoundly deaf. The mean preoperative hearing level (pure-tone average, 0.5-4 kHz) was 110 ± 8 dB hearing loss (HL) and the mean aided hearing level was 58 ± 11 dB HL. The postoperative hearing level (34 ± 9 dB HL) and word recognition scores were significantly better than before surgery. According to the Glasgow Benefit Inventory scores and Glasgow Health Status Inventory data related to hearing, the cochlear implantation was beneficial to patients with USH3.
  
  
  CONCLUSIONS
  Cochlear implantation is beneficial to patients with USH3, and patients learn to use the implant without assistance.
authors.affiliation: Department of Otorhinolaryngology, University of Helsinki, Helsinki, Finland. laura.pietola@helsinki.fi
authors.forename: Laura
authors.initials: L
authors.lastname: Pietola
authors.forename: Antti A
authors.initials: AA
authors.lastname: Aarnisalo
authors.forename: Akram
authors.initials: A
authors.lastname: Abdel-Rahman
authors.forename: Hanna
authors.initials: H
authors.lastname: Västinsalo
authors.forename: Juha
authors.initials: J
authors.lastname: Isosomppi
authors.forename: Heikki
authors.initials: H
authors.lastname: Löppönen
authors.forename: Erna
authors.initials: E
authors.lastname: Kentala
authors.forename: Reijo
authors.initials: R
authors.lastname: Johansson
authors.forename: Hannu
authors.initials: H
authors.lastname: Valtonen
authors.forename: Juha-Pekka
authors.initials: JP
authors.lastname: Vasama
authors.forename: Eeva-Marja
authors.initials: EM
authors.lastname: Sankila
authors.forename: Jussi
authors.initials: J
authors.lastname: Jero
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001301:Audiometry, Pure-Tone; D002648:Child; D019929:Cochlear Implantation / Q000009:adverse effects; D003054:Cochlear Implants / Q000009:adverse effects; D003147:Communication Disorders / Q000534:rehabilitation / Q000628:therapy*; D004252:DNA Mutational Analysis; D005260:Female; D005387:Finland; D006309:Hearing / Q000502:physiology; D006310:Hearing Aids; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D012189:Retrospective Studies; D012463:Saliva / Q000737:chemistry; D013067:Speech Perception / Q000502:physiology*; D011795:Surveys and Questionnaires; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D052245:Usher Syndromes / Q000235:genetics / Q000534:rehabilitation / Q000628:therapy*; D014785:Vision, Ocular / Q000502:physiology; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult