Hearing in school-aged children with trisomy 21 - results of a longitudinal cohort study in children identified at birth.

OBJECTIVES: To report the prevalence of hearing problems and the hearing sequelae in school-aged children with trisomy 21 in a longitudinal study.

DESIGN: All children with trisomy 21 were identified via schools, community-based child development centres, general practitioners, or the universal newborn hearing screen. Audiological data and otorhinolaryngological problems were prospectively entered in to the Audiological Surveillance Programme database from each visit.

SETTING: Retrospective review of the Audiological Surveillance Programme database in the Glasgow area (United Kingdom) of all children reviewed between 2004 and 2012.

PARTICIPANTS: All pre-teenaged children with trisomy 21 of school age (aged 5-12 years old).

MAIN OUTCOME MEASURES: Hearing thresholds, aetiology of hearing loss and management of hearing loss was determined for the cohort of children.

RESULTS: A total of 102 children were included. Fifty-four had normal hearing. Twenty-six had fluctuating otitis media with effusion; five had hearing in normal limits, six were managed with hearing aids, fourteen were managed conservatively, and one had ventilation tube insertion. Fifteen had persistent otitis media with effusion; four had ventilation tube insertion; and nine were managed with hearing aids. Seven had mixed hearing loss with four required hearing aids.

CONCLUSIONS: Otitis media with effusion was the commonest cause of hearing impairment; effusions may fluctuate through the pre-teenaged years, and thus, hearing aids are beneficial. Ventilation tube insertion and bone-conducting hearing aids were useful when ear-level hearing aids were not tolerated. Mixed hearing loss occurred in later years as sensorineural hearing loss developed on a background of otitis media with effusion.