Cochlear implantation in patients with osteogenesis imperfecta.
OBJECTIVE
Hearing loss has been shown to occur in 42% to 58% of patients with osteogenesis imperfecta (OI), with deafness arising in 25% to 60% of the patients. Implantation in patients with OI is relatively rare, with only 4 prior single case reports published in the English-language literature. The goal of this study was to evaluate the feasibility and functional outcome of cochlear implantation in 2 patients with OI tarda type I with profound sensorineural hearing loss.


METHODS
Case series.


METHODS
The implantations were performed in a tertiary academic referral center (Johns Hopkins University).


RESULTS
Though promontory vascularity was encountered, full insertion of a normal cochlear implant array could be achieved in both cases. One-year postimplant scores demonstrated 20 to 40 dB hearing thresholds, Consonant-Nucleus-Consonant Test word scores of 54% and 70%, Consonant-Nucleus-Consonant Test phoneme scores of 75% and 83%, Hearing in Noise Test scores of 76% and 99%, and Central Institute of the Deaf Sentence Score sentence scores of 99% and 100%, for patients 1 and 2, respectively.


CONCLUSIONS
Cochlear implantation in patients with OI is not only technically possible but the results are similar to implant outcomes for patients with sensorineural hearing loss from a variety of other causes.


METHODS
C.
Streubel
Sven-Olrik
SO
Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Lustig
L R
LR
D000328:Adult; D019929:Cochlear Implantation; D005240:Feasibility Studies; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology / Q000601:surgery; D006801:Humans; D008875:Middle Aged; D010013:Osteogenesis Imperfecta / Q000150:complications / Q000601:surgery*; D010040:Otosclerosis / Q000000981:diagnostic imaging / Q000601:surgery; D011859:Radiography; D016896:Treatment Outcome