15th ANNUAL EARLY HEARING DETECTION & INTERVENTION MEETING
March 13-15, 2016 • San Diego, CA

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Enlarged Vestibular Aqueduct and Cochlear Implants:

Introduction: Enlarged vestibular aqueduct (EVA) is the most frequently identified anatomical abnormality in children with sensorineural hearing loss (Lin et al., 2005). The age at onset of hearing loss may range from birth to adolescence (Berrettini et al., 2005), but typically fluctuates or progresses to a profound degree by early childhood (Valvassori & Clemis, 1978). Many patients with EVA eventually will be referred for consideration for cochlear implantation (CI). Research has shown that this route can prove successful for these patients (Lai et al., 2012) if no other developmental delays are present. One previous study found that the average length of time between hearing sensitivity documented at a severe/profound hearing level and implantation was 6.3 months (Lee et al., 2010) during which period children would have limited access to language. Method: Studying a sample of 42 children who received CIs with a diagnosis of EVA, we investigated their status on newborn hearing screening, their progression of hearing sensitivity from identification of sensorineural hearing loss of any degree to severe/profound degree (candidacy criteria for CI), and the length of time between identification of severe/profound degree of hearing loss and surgical cochlear implantation. Results: Prior to progression in degree of hearing loss and subsequent surgical implantation, subjects of this current study were largely functionally hard of hearing and predominantly aural/oral communicators. Some children used signing in support of spoken English. When spoken language became no longer accessible auditorily, efforts to limit the amount of time between these children reaching audiological candidacy criteria and implantation was imperative to maintain access to spoken language. Discussion: Efforts to limit the amount of time between reaching audiological candidacy criteria and scheduling surgery may benefit patients. These findings contribute to influencing the anticipatory process of guidance for children and families by providing comprehensive management, supports, and recommendations for follow-along care.

  • Describe the status of newborn hearing screening referral in this population.
  • Describe the length of time between hearing deteriorating to CI candidacy and implantation.

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Presenters/Authors

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Terrell Clark (), Children's Hospital Boston, Terrell.Clark@childrens.harvard.edu;
Terrell A. Clark, PhD, a contributing author for this resource, is director of the Deaf and Hard of Hearing Program at Boston Children’s Hospital. Her endeavors through this program include teaching, supervising, conducting research, and providing clinical assessments with babies, school-aged children, and teens. As a pediatric psychologist at Boston Children’s, she is appointed as a senior associate in the Department of Psychiatry (Psychology) while at Harvard Medical School, she is an assistant professor of psychology. She has also taught at Tufts University in the Department of Child Development for many years, given numerous national and international presentations, served as an advisor to agencies and organizations on matters affecting deaf and hard of hearing children, and received regional and national recognition for her work.

ASHA DISCLOSURE:

Financial - Receives Salary,Intellectual property rights for Employment from Boston Children's Hospital.  

Nonfinancial - No relevant nonfinancial relationship exist.


Katlyn Bostic (Primary Presenter), Boston Children's Hospital, katlyn.bostic@childrens.harvard.edu;
Originally from West Virginia, and having completed a Bachelor’s of Science degree in Speech Pathology and Audiology at West Virginia University, Katlyn Bostic is currently an AuD student at the University of Maryland in College Park. She is currently a LEND fellow in audiology pursuing a graduate externship at Boston Children's Hospital. She has had a strong interest in pediatric audiology since beginning her training.

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Brianna Chai (Co-Presenter), Boston Children's Hospital, Brianna.Chai@childrens.harvard.edu;
After graduating with a Bachelor's degree in Deaf Studies, Brianna Chai decided she wanted to work in audiology where she might better help children with hearing loss and their families navigate their journey and learn about all of the options available. She is originally from the San Francisco Bay Area, attends the University of Colorado Boulder where she is pursuing a doctorate of audiology, and currently a LEND fellow graduate student extern at Boston Children's Hospital.

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Rebecca Lewis (Co-Presenter), Boston Children's Hospital, Rebecca.Lewis@childrens.harvard.edu;
Having completed her undergraduate degrees in Communicative Disorders and Spanish at the University of Wisconsin, Rebecca Lewis remains on the Stevens Point campus as part of the University of Wisconsin Audiology Consortium program. She is a graduate student extern and LEND fellow in audiology at Boston Children's Hospital.

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