Roles
Genetics
Embryology
Causes of Early Childhood HL
Syndromes
100

The pediatric audiologist serves as:

What is a Diagnostician, Counselor, Audiologic care coordinator/case manager

100

causes of early hearing loss

Genetics, infections during pregnancy, environmental causes, complications after birth, CMV, 1 in 4 children with HL is born with a birth weight less than 2,500 grams

100

Name the 3 layers that develop after gastrulation of the blastula?

Ectoderm, mesoderm, endoderm


100

List the factors associated with increased risk of OM

Age, gender, immune status, other diseases and conditions, familial clustering, feeding practices, childcare settings, passive smoking, season

100

Usually normal hearing, although conductive hearing loss secondary to chronic otitis media with resulting intermittent CHL is common; stenotic ear canals may often cause cerumen impaction and temporary CHL

Down's Syndrome (Trisomy 21)

300

True or false: the Prevalence of hearing loss in children 3-17 years old is 5 per 1000?

TRUE

1.4 per 1000 with at least moderate bilateral HL

  • 5 per 1000 children 3-17 years
  • 14.9% of children ages 6-19 years with low or high frequency hearing loss at least 16 dB in one ear or both
  • 1.4 per 1000 children 8 years old with bilateral HL of 40dB or more
  • Almost 6,000 U.S. infants born in 2019 were identified early with permanent hearing loss
300

Genetic Hearing loss is the cause of what percentage of early childhood hearing loss?

80%


300

The cochlea is fully extended to 2.5 turns at what week? Of development/gestation?

Week 8

300

The most common form of Inner Ear malformation associated with SNHL is?

Enlarged Vestibular Aqueduct syndrome

300

This Autosomal Dominant syndrome has the major features of CHL, SNHL, ossicular anomalies, cochlear anomalies, vestibular anomalies, brachial clefts, pre-auricular pits, malformed auricles, lacrimal duct stenosis, renal anomalies.

BOR (brachio-oto-renal) syndrome

500

Clinical thoughts #1

Think and Act fasrer than with adults

500

The most common form of non-syndromic hearing loss

What is Connexin 26
500

The external auditory canal is formed from what dermal layer?

Ectoderm

500

The leading cause of Unilateral hearing loss in children is?

CMV

500

CHL of varying degree ranging from mild to maximum depending on microtia; fixation of the footplate of the stapes is common; there is occasionally a SNHL

Treacher Collins Syndrome

800

Clinical Thoughts #2: you don't want to start with these

What is, if they don’t want their ears touching; don’t start with otoscopy of tympanometry; try VRA and see what they will do for you. Then try otoscopy, tympanometry and/or DPOAEs after (all ear touching)

800

Examples of Human disorders due to a single pair of recessive genes includes...

  • Albinism
  • Tay-Sachs Disease
  • Cystic Fibrosis
  • Sickle Cell Anemia
  • Phenylketonuria
  • Thalassemia
800

Hillocks 1,2,and 3 come from ...

Hillocks 4,5, and 6 come from...

phayrngeal arch 1

phayrngeal arch 2

800

Examples of causes of Acquired early hearing loss includes:

Herpes, CMV, teratogens, meningitis

800

Usher Syndrome: experience profound deafness in both ears and severe balance problems at birth.  Problems with vision typically begin in early childhood. Type I, II, or III?

Type I

1000

clinical thoughts: What should you start with?

What is with children, begin with obtaining an SRT using the pediatric spondee words; or pointing to body parts

1000

Autosomal Dominant syndromes (5)

Autosomal Recessive syndromes (4)

AD: Waardenburg syndrome, Branchio-oto-renal (BOR) syndrome, Neurofibromatosis Type II (NFII), Stickler syndrome, Treacher-Collins syndrome (FLK- funny looking kid)

AR: Usher syndrome, Alport syndrome, Jervell and Lange-Nielson syndrome, Pendred syndrome

1000

When is the human auditory pathway anatomically formed and how early can an Automated ABR be performed?

26 weeks; 28 weeks

1000

Examples of Aminoglycosides that can cause hearing loss in NICU infants are?

Gentamycin

1000

Major Features: Goiter with SNHL

Hearing: Moderate to profound SNHL, more severe in the HF's with Mondini cochlear anomaly; decreased vestibular function

Pendred Syndrome

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