A pilot study of the relationship between Down's syndrome and hearing loss.

OBJECTIVE: Hearing loss is one of the most common disabilities in children with Down's syndrome. The objective of this study is to investigate the incidence and types of hearing loss in Down's syndrome patients. METHODS: Twenty-six subjects with Down's syndrome aged between 2 and 17 year old were evaluated during the year 1998, referred randomly by the Pediatric Department and the Down's Syndrome Centre. Screening audiological procedures were used to evaluate these patients, which include behavioral audiometry, play and pure tone audiometry, tympanometry and ABR, which is the measurement of the activity of the auditory pathway structures from the distal auditory nerve to the midbrain using clicks or tonepips. RESULTS: The study showed that 35% were found to have normal hearing compared to 4% found to have sensorineural hearing loss, while the majority of patients were found to have a conductive hearing loss (50%). CONCLUSION: Hearing evaluation of children with Down's syndrome revealed a high prevalence of conductive hearing loss. This study proposed that this might be attributed to eustachian tube dysfunction and middle ear ciliary malfunction. Further diagnostic studies need to be carried out.

A pilot study of the relationship between Down`s syndrome and hearing loss.

OBJECTIVE: Hearing loss is one of the most common disabilities in children with Down`s syndrome. The objective of this study is to investigate the incidence and types of hearing loss in Down`s syndrome patients. METHODS: Twenty-six subjects with Down`s syndrome aged between 2 and 17 year old were evaluated during the year 1998, referred randomly by the Pediatric Department and the Down`s Syndrome Centre. Screening audiological procedures were used to evaluate these patients, which include behavioral audiometry, play and pure tone audiometry, tympanometry and ABR, which is the measurement of the activity of the auditory pathway structures from the distal auditory nerve to the midbrain using clicks or tonepips. RESULTS: The study showed that 35% were found to have normal hearing compared to 4% found to have sensorineural hearing loss, while the majority of patients were found to have a conductive hearing loss (50%). CONCLUSION: Hearing evaluation of children with Down`s syndrome revealed a high prevalence of conductive hearing loss. This study proposed that this might be attributed to eustachian tube dysfunction and middle ear ciliary malfunction. Further diagnostic studies need to be carried out.

Different faces of non-autoimmune diabetes of infancy.

The aim of this report is to describe four cases of non-autoimmune diabetes that presented in infancy. Three had transient neonatal diabetes mellitus (TNDM) with diabetic ketoacidosis at onset, followed by complete remission after several months of insulin treatment. While the fourth case was initially diagnosed as TNDM, she had renal, hepatic and pancreatic dysplasia. These cases illustrate that diabetes in infants can be difficult to diagnose and that patients with TNDM can have a recurrence of diabetes several years later.

Clinical and serologic responses of Saudi children to Hemophilus influenzae type B capsular polysaccharide diphtheria toxoid conjugate vaccine.

Sixty-eight Saudi children, 17 to 19 months of age, were enrolled in a study to evaluate the safety and immunogenicity of Hemophilus influenzae type B capsular polysaccharide diphtheria toxoid (PRP-D) conjugate vaccine. Adverse reactions to the vaccine were determined through a questionnaire administered to the parents. Local and systemic reactions to the vaccine were mild and resolved within 24 to 48 hours. PRP antibody levels were measured prior to and one to two months following immunization. PRP antibody levels in the pre-immunization sera of 77% of subjects were below the level associated with immediate protection (>/=0.15 microg/ml), and 88% were below the level associated with long-term protection (>/=1 microg/ml) from Hemophilus influenzae type B (HIB) disease. After one dose of PRP-D vaccine, 100% of recipients achieved antibody levels of >/=0.15 microg/ml, and 85% achieved levels of >/=1 microg/ml. The geometric mean level of antibody after immunization (5.66 microg/ml) was significantly higher than that before immunization (0.098 microg/ml). All subjects had a twofold or greater increase in antibody level in response to the vaccine. We conclude that PRP-D is a safe and highly immunogenic vaccine in this age group of Saudi children.