Hearing Loss In Children

Question 1

Minimal hearing loss in children is not inconsequential

Answer 1

37% fail a grade
8% don’t have skills at grade level
41% receive educational assistance

Question 2

Hearing loss symptoms in babies

Answer 2

Do not startle at loud noise
Do not turn to source of sound by 6 months
Do not say single words by 1 year
Turn head when see you but do not respond to name
Hears some sounds over others

Question 3

Hearing loss symptoms in children

Answer 3

Speech delay
Speech not clear
Do not follow instructions
Often say “huh”
TV volume too high

Question 4

Screening babies/children

Answer 4

Screen by 1 month, diagnosis by 3, treat by 6
If fail get full evaluation by 6 month
Screen kids before school

Question 5

What does congenital hearing loss imply

Answer 5

Present at birth

Question 6

What percentage of hearing loss cases in babies is due to genetic causes?

Answer 6

~1 out of 2 cases of HL in babies is due to genetic causes.

Question 7

What is a common factor among babies with genetic hearing loss?

Answer 7

~1 out of 3 babies with genetic HL have a ‘syndrome’ or other conditions in addition to the HL.

Question 8

What percentage of hearing loss cases in babies is attributed to maternal infections during pregnancy or complications after birth?

Answer 8

~1 out of 4 cases of HL in babies.

Question 9

What percentage of babies born with hearing loss have an unknown cause?

Answer 9

~1 out of 4 babies.

Question 10

Causes associated with birth

Answer 10

Infection - CMV very common
5 days or more in NICU
Blood transfusion from severe jaundice
Head, face or ears shaped or form in unusual way
Neurological disorder that may be associated with hearing loss
Infection around brain and spinal cord - meningitis
Injury to the head that required hospital stay

Question 11

What are the genetic factors related to congenital hearing loss in babies

Answer 11

Autosomal dominant
Autosomal recessive
X-linked

Question 12

Autosomal dominant hearing loss

Answer 12

One parent carries dominant gene
50% probability to pass to child
Probability is higher if both parents have dominant gene or both grandparents

Question 13

Autosomal recessive hearing loss

Answer 13

Both parents have normal hearing but carry a recessive gene
Probability child has loss 25%

Question 14

X linked hearing loss

Answer 14

Mother carries the recessive trait on sex chromosome
Passes it on to males

Question 15

Genetic syndromes where hearing loss is one of the known characterisitcs

Answer 15

Down syndrome
Crouzon (autosomal dominant)
Treacher Collin’s (autosomal dominant)
Usher syndrome (autosomal recessive)
Alport (x-linked)

Question 16

What gene is important in functioning of the cochlea?

Answer 16

GJB2

Question 17

What type of hearing loss almost always occurs with otitis media

Answer 17

Conductive hearing loss

Question 18

How many children get otitis media?

Answer 18

75% have 1 episode by 3rd bday
1/2 of those will have 3 or more

Question 19

If otitis media keeps occurring what will happen

Answer 19

Permanent, sensorineural hearing loss

Question 20

Case history red flags indicating further hearing testing

Answer 20

1. Caregivers concern
2. Family history of childhood hearing loss
3. NICU sta over 5 days
4. In-utero infections
5. Craniofacial anomalies
6. Physical findings
7. Syndromes associated with hearing loss or progressive or late-onset
8. Neurodegenerative disorders
9. Head trauma
10. Chemotherapy