Permanent hearing loss affects 1 to 3 per 1000 children and interferes with typical communication development. Early detection through newborn hearing screening and hearing technology provide most children with the option of spoken language acquisition. However, no consensus exists on optimal interventions for spoken language development.


To conduct a systematic review of the effectiveness of early sign and oral language intervention compared with oral language intervention only for children with permanent hearing loss.


An a priori protocol was developed. Electronic databases (eg, Medline, Embase, CINAHL) from 1995 to June 2013 and gray literature sources were searched. Studies in English and French were included.


Two reviewers screened potentially relevant articles.


Outcomes of interest were measures of auditory, vocabulary, language, and speech production skills. All data collection and risk of bias assessments were completed and then verified by a second person. Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) was used to judge the strength of evidence.


Eleven cohort studies metinclusion criteria, of which 8 included only children with severe to profound hearing loss with cochlear implants. Language development was the most frequently reported outcome. Other reported outcomes included speech and speech perception.


Several measures and metrics were reported across studies, and descriptions of interventions were sometimes unclear.


Very limited, and hence insufficient, high-quality evidence exists to determine whethersign language in combination with oral language is more effective than oral language therapy alone. More research is needed to supplement the evidence base.

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