Dichotic listening deficits in children with hearing loss.

PURPOSE: This correlational study compared dichotic listening among children with significant hearing loss to typically developing children and children clinically assessed for auditory processing disorder. METHOD: Recorded versions of two dichotic tests were delivered under earphones or in the sound field for children using amplification. Individual ear scores and interaural asymmetry were compared to normative data. Matched deficit patterns from both tests were identified, ranked for severity of deficit, and compared across groups. Relationships between dichotic scores and factors related to amplification were investigated in the children with hearing loss. RESULTS: Dichotic scores were significantly poorer among children with hearing loss without the large interaural asymmetries seen in children assessed clinically for auditory processing problems. Device type and age of implantation had no effect on scores; non-dominant ear scores on the digits test were significantly correlated to age of device use in the left ear and duration of device use in the right ear. Non-dominant ear scores with digits were also significantly correlated with bilateral word recognition. CONCLUSIONS: Poor dichotic perception in children with significant hearing losses may be related to the use of recorded test materials, immature skills in attention and working memory, or other factors that contribute to development of vocabulary and language. These weaknesses may interfere with successful mainstream educational placement in these children. Assessment of dichotic performance in this population could lead to deficit-specific interventions that may improve outcomes and enhance educational opportunities for children with hearing loss.

Development of a Clinical Algorithm for the Early Diagnosis of Mucopolysaccharidosis III

Abstract Mucopolysaccharidosis III (MPS III) is a rare inherited metabolic disease primarily affecting the central nervous system, leading to developmental and/or speech regression. Early diagnosis of the disease is important to introduce appropriate management measures and to optimize therapeutic outcomes. The diagnosis of MPS III is often significantly delayed due to the rarity of the disease, the more attenuated somatic presentation compared to other MPS types, and the symptom overlap with other developmental disorders. To shorten the time to diagnosis, a list of eight early signs and symptoms was identified through an expert system approach by a global, multidisciplinary working group of 13 specialists with expertise in various aspects of MPS and developmental disorders and three parents of MPS III patients. Coarse facial features and persistent hirsutism or prominent, thick eyebrows were identified as the most important MPS III early signs. The list of eight early MPS III signs and symptoms is the first step towards the development of a clinical algorithm aiming to identify neonates and infants with MPS III before the onset of neurocognitive damage, ultimately shortening the diagnostic journey of MPS III patients.