The purpose of this study is to evaluate and document longitudinal efficacy in young children implanted bilaterally with MED-EL COMBI 40+ / PULSARCI100/SONATATI100 cochlear implant systems.

Inclusion Criteria:

• Between 12 and 36 months of age at time of implantation
• Profound bilateral sensorineural hearing loss
• English as the primary language in the home
• Realistic expectations of guardians
• Child must be enrolled in a post-operative rehabilitative/educational program that supports the use of cochlear implants and the development of auditory-based skills
• Willing and available to comply with all scheduled procedures as defined in the protocol

Audiological:

• Profound bilateral sensorineural hearing loss with average thresholds between ears of 90 dB HL or greater at 1000 Hz and above, and must demonstrate minimal functional benefit from conventional amplification
• Behavioral test measures must include unaided threshold measures for the right and left ears using insert earphones; left-alone and right-alone aided sound field threshold measurements; and aided and unaided speech-awareness / detection thresholds (for each ear individually)
• Children accepted into the study must be able to demonstrate a consistent response to sound. If a child has no measurable hearing, he/she must be able to demonstrate a consistent response to vibrotactile stimuli.
• All children must have completed an appropriate trial period with optimally fit amplification prior to inclusion in the study.

Medical:

• Good general health status, as judged by Primary Investigator
• Patent cochleae bilaterally, as indicated by radiological evaluation
• No contraindications for surgery, in general, or cochlear implant surgery in particular

Exclusion Criteria:

• Prior experience with any cochlear implant system
• Younger than 12 months or older than 36 months at time of implantation
• Audiological: Presence of otoacoustic emissions and/or cochlear microphonic, indicating possible condition of auditory neuropathy

Medical:

• Evidence of ossification that would prevent full insertion of the standard C40+ / PULSARCI100 /SONATATI100 electrode array in the cochlea(e) to be implanted, as indicated by radiological evaluation
• Abnormal or malformed cochlea(e) to be implanted
• Severed or non-functional auditory nerve in the ear(s) to be implanted
• Central auditory lesion
• Cognitive and/or neurological dysfunction
• Auditory neuropathy

Adverse events were collected from the time of enrollment through the study endpoint ( 5 years postoperatively) for each subject. However, due to early termination of the study the endpoint was not reached, and participants were followed for an average of 3 years postoperatively.

Definitions do not differ

Protocol was terminated due to inability to find unilateral matches.