Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically determined

Published evidence and clinical experience support early diagnosis and treatment. Wherever feasible, hearing aid provision before or at the end of the first year of life is recommended for children with UHL. What is Known: • Almost 30 years ago, poor academic performance was reported in children with unilateral hearing loss (UHL). • Despite improvements in treatment options, it is traditionally held that unilateral hearing is the minimum requirement for adequate speech development and hearing aid provision is unnecessary. What is New: • Academic and behavioral deficits in children with UHL may be mediated by deficiencies in the default mode network. • Published evidence supports the recommendation for hearing aid provision before or at the end of the first year of life in children with UHL.

From the actual point of view, the "sensitive period" for the effects of hearing impairment on speech and language development is within the first year of life. Early exposure to acoustic or electric stimulation can compensate for the acoustic deficit. A regional-based, specifically designed concept of a universal newborn hearing screening (UNHS) was started in Hamburg in the year 2002. For the first time in Germany, a comprehensive protocol including screening measurement, follow-up procedures, tracking, and early intervention was implemented. An interdisciplinary approach from the very beginning could be realized. Sixty-three thousand, four hundred fifty-nine out of 65,466 births were registered during the period August 2002 to July 2006, 93% were primarily screened. 3.3% failed the test and 31.3% were lost to follow-up. A total of 118 children were diagnosed with hearing loss in the follow-up. The median age at time of diagnosis was 3.5 months. Seventy-four children received hearing aids. Out of these 74 children, 6 were subsequently supplied with cochlear implants. The high lost-to-follow-up rate is the biggest challenge for the tracking. Our results will be discussed in part II.