PROBLEM/OBJECTIVES: Unilateral hearing loss (UHL) in children is a relatively common problem often identified by newborn screening or school screening. This review summarizes the outcomes of a large study of children with UHL which identified risk factors for educational problems, describes the results of pilot studies of intervention, and proposes guidelines for interventions and future studies. METHODS Cohort of children age 6-12 years with UHL was compared to their siblings with normal hearing on the primary outcomes of cognition, achievement, and language. Secondary outcomes examined included behavioral, speech, and educational problems. A subgroup of children with UHL was followed longitudinally to examine change over time. RESULTS Children with UHL had lower language and verbal IQ scores than their siblings with normal hearing. They required individualized education plans (a measure of educational difficulty) at three times the rate and speech therapy at twice the rate of their siblings. The additional educational concerns did not ameliorate over time. Low maternal education levels and lower baseline cognitive skills were independent risk factors for school problems. Pilot studies suggest that children with UHL might benefit from amplification, but may need attention to cognitive or executive function skills as well. CONCLUSIONS Children with UHL are at risk for delays in speech-language development, cognition, and behavioral problems that can affect functioning at school. Controlled studies are urgently needed to determine whether amplification, auditory rehabilitation, school-provided educational assistance, or other interventions are able to reduce or eliminate this risk.