Topic: Cochlear Implantation in Children with High Frequency Sensorineural Hearing Loss
Principal Investegator: Dr. Margaret Kenna, and Dr. Greg Licameli
Summary: Cochlear implantation (CI) is a standard and often successful intervention for young children with bilateral severe to profound hearing loss. The audiologic criteria set by the FDA for children aged 12-24 months are the presence of bilateral profound SNHL, while children older than 24 months can have bilateral severe to profound SNHL. Most children who fit the audiologic criteria have hearing loss in the severe to profound range for all tested frequencies. Children with slight to moderately severe hearing loss for low-frequency sounds, along with profound hearing loss for high-frequency sounds, often do not fit the traditional candidacy criteria. However, this group of children also may not benefit from hearing aids, leaving CI as a viable option even with their residual low-frequency hearing.
In this study we aim to retrospectively investigate those children with profound bilateral high frequency hearing loss who were implanted after consistently scoring poorly on word and sentence recognition assessments. We look to explore the pre and post-implant assessments to determine the effectiveness of the cochlear implant in this pediatric population. The results may also have implications for expanding the current FDA candidacy criteria.
Topic: CHARGE Syndrome in patients with Cochlear implants
Principal Investegator: Dr. Greg Licameli
Summary: CHARGE syndrome is an autosomal dominant disorder characterized by features represented in its acronym: coloboma, heart defect, atretic choanae, retarded growth or development, genital hypoplasia, and ear anomalies. CHARGE syndrome not only is associated with anomalies of the outer ear but also with changes of the inner ear structures and with hearing loss. Given the abnormal anatomy and neurological complexity of these patients, they are not typical candidates for cochlear implantation. However, a few CHARGE patients have been implanted and followed in our program and we would like to learn more about the outcomes of these patients. The aim of this study is to assess the outcome of standard cochlear implantation for children with CHARGE association using retrospective analysis of patient demographics, pure-tone audiograms, sound field measurements post implantation and mode of communication.