Hearing Disorders

Recurrent and chronic ear infections in noncommunicative adults often go undiagnosed and can lead to mastoiditis and hearing loss. Almost half of the DS adults in our study have hearing concerns, most frequently secondary to recurrent ear infections. 18% have a history of mastoiditis, usually resulting in mastoidectomy.

Significant hearing loss is present in at least 25% of our elderly group. Difficulties in testing and inconsistent reporting in yearly medical reviews underestimates the presence of less severe hearing losses. Balkany et al. [1979] report on significant hearing loss in 78% of 107 individuals with DS, age 2 months to 60 years, tested with otoscopy, audiometry, and impedance tympanometry. The most complete study available on hearing concerns among DS adults living in residential centres is reported by Evenhuis et al. [1991]. Thirty-five adults, age 35–62 years, are evaluated with otoscopy, pure tone audiometry, impedance audiometry, and auditory evoked responses.

A loss of hearing greater than 20 dB is present in 59 ears tested. The documented hearing losses are conductive, presumably resulting from middle ear disease, and sensorineural. Cardiovascular Diseases Congenital heart anomalies (CHA) are a common structural anomaly in DS, occurring in 42% of newborn infants evaluated by echocardiography [Tubman et al., 1991]. The incidence of CHA in surviving adults is unknown. In the Woodlands population, 15.8% of DS adults have untreated CHA including three with ventricular septal defects, two with atrioventricular canal defects, and one with a patent ductus arteriosus.

An additional 10 of 38 DS adults in our study have significant cardiac murmurs without cardiac symptoms. Hamada et al. [1998] report a high incidence of valvular heart abnormalities in 30 institutionalised DS adults with no history of congenital heart anomalies and no cardiac symptoms. Echocardiograms on these adults show that 26.7% have mitral valve prolapse, with an increase in 20% of echo brightness of the mitral valve; 16.75% have mitral valve regurgitation; and 13.3% have aortic valve regurgitation.