Frequency • 3000-5000 a year are born with Downs syndrome • 75-89% of children with Downs have associated hearing Loss !! • 66-89% have a hearing loss greater than 15-20dBHL in at least 1 ear 75% found to have at least a mild hearing problem
Progress in Speech & Language performance is linked to factors which include – hearing status,speech, Non Verbal cognitive skills, and daily experience
Children with downs experience slower development of language relative to other cognitive skills • 1/3 of these children have recurring problems with hearing throughout childhood which if not addressed lead to greater speech and language delay
Physical features affecting listening • Although the skull of a child with Downs grows to nearly the same side as the average adult, it has more of the shape of a newborn.
Eustachian tube dysfunction (the tube that runs from your middle ear to the back of the mouth)& cartilage anomaliesPromotes stillness of fluid in the ear and infection travelling up to the ear from the mouth • Leads to…… • OME (middle ear fluid) • Retraction (the eardrum getting sucked in) • Cholesteatoma A type of skin cyst located in the middle ear and skull bone
Otitis Media with Effusion • The angle in which the Eustachian tube sits in the child with Downs
Other structural differences that can lead to hearing difficulties • EAM stenosis – (narrowing of the ear canal) • Wax build up - impacted • Small nasopharynx(back of the nose & throat) • Impaired swallow • Production of more mucus which can then becomes infected due to a lowered immune system • Changes in the pinna
SO WHAT IS THE OUTCOME? • OME = MILD – MODERATE HEARING LOSS worse in the low frequencies • Congenital SNHL in the high frequencies – bony deposits along the cochlear & Acquired/presbycusis (high frequency hearing loss) in the older years 35-40.
Medical Problems that can occur • 40 –45% congenital heart disease • Intestinal changes • Thyroid dysfunctions • Skeletal problems • Sleep apnoea - 50% • Obesity in adolescence &older • Immunologic concerns • Leukaemia • Alzheimer's • Seizure difficulties • Skin disorders
Other possible symptoms • Average IQ is 30-40 but some children have mild-moderate difficulties -Marcell98 • A narrowed ear canal can make looking at the eardrum difficult • OME = pain, ↓ Attention , Distractibility, Feeling of fullness, bunged up • LEAKY EAR • Inconsistent performance • Low muscle tone
Management • Myringotomyis a surgical procedure in which a small incision is created in the eardrum to relieve pressure caused by build-up of fluid or to drain pus from the middle ear. • Tympanostomytubes/PE tubes This tube is inserted into the eardrum in order to keep the middle ear aerated for a prolonged period of time to prevent re-accumulation of fluid, as without the insertion of a tube, the incision would heal spontaneously in two to three weeks • Grommets a very small tube that is inserted into your child's ear through a small cut in their eardrum. A grommet will drain away fluid in the middle ear and help to maintain the air pressure in the middle ear cavity. • Antibiotics • Hearing aids • Surgical repair or of perforation
So What does a Speech Therapist do? • Observe • Advise • Monitor • Refer to any other relevant professionals • Support • Remind • MDT working • Assess
Focus on the holistic Approach • Strategies that fit into everyday life • Fun • Highlighting great activities that are done anyway