Hearing Loss and Autism Spectrum Disorder

Abstract

CASE: Gretta is a 3.5-year-old girl with a history of congenital cytomegalovirus, congenital bilateral profound sensorineural hearing loss (SNHL), and bilateral vestibular dysfunction, resulting in frequent falls. She underwent cochlear implantation at 12 months of age and was diagnosed with autism spectrum disorder at 2.5 years of age. On presentation for follow-up in the developmental-behavioral pediatrics (DBP) clinic, Gretta's mother reports that Gretta has refused to wear her cochlear implants for the past 5 months. Before that, she seemed to enjoy having access to sound and like dancing to music, and her receptive and expressive language skills, including speech, were progressing. Initially, the rejection of her devices occurred only at preschool. When frustrated or overwhelmed, she would close her eyes and remove her devices for up to 5 minutes before allowing them to be reapplied. Over time, this progressed to a complete refusal to wear her devices at school and then at home, rendering her without access to sound and spoken language. Gretta's mood has become sullen, and she is now having tantrums at school. She physically startles when attempts are made to reintroduce her devices. Her ability to participate in classroom learning or interact with her classmates is limited, as she attends a spoken-language–focused preschool program. A board-certified behavioral analyst, hired by the family, recommended that Gretta not be allowed to participate in classroom activities unless she wears her devices. She now becomes visibly anxious even when in the same room as her devices and repetitively states “no implant, no implant.” Her mother is worried about her inability to communicate and has “no idea” what may have changed or sparked her initial refusal to wear the devices. What factors would you consider when determining the cause and function of Gretta's refusal to wear the cochlear implants? How would you guide her parents, teachers, and clinicians to ensure the best developmental and behavioral outcomes for her?