Hearing Loss In The Child With Downs Syndrome

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.

Leads to…… OME (middle ear fluid) Retraction (the

eardrum getting sucked in) CholesteatomaA type of skin cyst located in the middle ear and skull bone 

Eustachian tube dysfunction (the tube that runs

from your middle ear to the back of the mouth) & cartilage anomalies

Promotes stillness of fluid in the ear and infection travelling up to the ear from the mouth

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 -Marcell 98 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 Myringotomy is 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.

Tympanostomy tubes/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

Age Recommended hearing evaluations

birth to 2 months

2 to 12 months

1 to 12 years

12 to 18 years

over 18 years

Refer for auditory brainstem response (ABR) test or other objective assessment of hearing to assess hearing by 6 months of age, if not performed at birth.

• Provide brainstem response test (ABR) or other objective assessment of hearing by 6 months of age if not performed previously or if previous results are suspicious. Evaluation by ENT specialist for recurrent otitis media.

• Yearly auditory testing for children 1-3 years old and every two years for children 3-13 years old.

• Hearing evaluations every other year.• Continue auditory testing every two years.

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