The present study describes the characteristics of students in Grades 3-12 with significant cognitive disabilities (SCD) and known hearing loss. The study analyzed results of a survey of teachers of students with SCD (n = 38,367) who were slated to participate in an alternate assessment based on alternate achievement standards in 14 states in the United States. Analysis revealed similar profiles in academic achievement and symbolic language use combined with an increased incidence of additional sensory impairments among students with SCD and known hearing loss compared to their peers without known hearing loss. Results suggest that hearing loss may be underidentified and underserved among students with SCD and point to the need for improved hearing screenings and evaluations combined with services delivered by teams that follow a model of interprofessional practice.