Pediatrics Audiology

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Embryology- 6th Week

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Utricle and Saccule present, semicircular canals begin development.

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Embryology- 9th Week

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Three layers of tympanic membrane present.

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Embryology- 11th Week

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Two and half cochlear coils present and 8th nerve attaches to cochlea duct.

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Embryology- 12th Week

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Hair cells present.

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Embryology - 15th Week

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Stapes present

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Embryology- 20th Week

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Inner ear matured and adult size. Auricle adult shaped.

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Neonatal Screening

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1-3 per thousand babies born will have hearing loss. 1 in 50 in NICU have hearing loss. 46 states & district of Columbia have newborn hearing screening.

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What is OAE?

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(Otoacoustic Emissions) Testing

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What is ABR?

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Auditory Brain-stem Response

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What is BOA?

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Behavioral Observation Audiometry: used when assessing children up to four months, performed in sound field, looking for behavior response to sound and cannot obtain threshold due to low attention and motivation.

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What is VRA?

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Visual Reinforcement Audiometry: Used when assessing children 5 months to 2 years, child is conditioned to sound using something visually interesting, first present both sound and light and then light alone to reinforce child's behavior and can be in sound field or with headphones.

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What is Play Audiometry?

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Used when assessing children 2-5 years old, child is conditioned to sound by playing a game, may need to do hand over hand training until child play independently, can be in sound field or with headphones.

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What are the 3 components to Speech Audiometry?

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SDT, SRT and WRS(SRS)

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What is SDT?

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Where am I? (Speech Detection Threshold)

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What is SRT?

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Identify body parts (Speech Reception Threshold)

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What is WRS(SRS)?

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WRS- Word Recognition Score, Word Intelligibility by Picture Identification (WIPI)

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ASHA Screening Guidelines

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Birth to 6 months of age: OAE or ABR screening.

7 months to 2 years: VRA (1000, 2000, 4000 Hz at 30dBHL)

3-5 years: 1000, 2000, 4000 at 20 dBHL

5-18 years: 1000, 2000, 4000 HZ at 20 dBHL.

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Aplasia/Dysplasia

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Inner Ear Malformations. 4 types: Michel's, Mondini's, Schebie's, Alexander's.

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What is Michel's Malformation

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No inner ear

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What is Mondini's Malformation?

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Incomplete inner ear

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What is Scheibe's Malformation?

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Cochlea malformation.

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What is Alexander's Malformation?

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Abnormal basal cochlea

Front 23

What is Cleft Palate?

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Both sides of the palate do not fuse correctly. Unilateral, bilateral or in conjunction with cleft lip, caused by both environment and genetic factors.

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What is Down Syndrome?

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Down syndrome is a chromosomal disorder affecting cognition and physical development due to trisomy 21 (a third copy of chromosome 21), it occurs in one in every 700 live births.

More prevalent in children born to women over 35, however 80% are born to women under 35.

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Hearing Loss in Down Syndrome

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Malformations predispose conditions that impair audition. Upper respiratory infections (e.g. sinusitis, otitis media, etc). Varied presentation of HL (unilateral or bilateral/ conducive, sensorineural or mixed).

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Waardenburg Syndrome

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There are four types: The primary symptoms SNHL 35-75% of patients, iris pigmentation abnormalities (e.g. two different colored eyes), hair pigmentation abnormalities, lateral displacement of eye. Secondary symptoms are white skin patches, broad nasal bridge, uni-brow, vestibular symptoms of vertigo or dizziness, premature greying hair, cleft palate or cleft lip.

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Hearing Loss in Waardenburg Syndome

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Unilateral or bilateral, moderate to profound or total or partial SNHL, amplification devices are beneficial, possible temporal bone abnormalities e.g. atrophy of the organ of Corti or reduce number of nerve fibers.

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What is Pendred Syndrome

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An autosomal recessive disorder with three key features such as congential deafness, goiter (enlarged thyroid), and positive perchlorate test caused by a mutation in the SLC26A4 gene.

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Hearing loss in Pendred Syndrome

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Profound SNHL is primary clinical symptom. Worse n high frequencies, HL is pre-lingual but be progressive and appearing in later childhood. Characteristics: enlarged vestibular aqueduct, malformation of cochlea, impaired vestibular function.

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What is Usher's Syndrome?

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Genetic disorder affecting vision and hearing. It is the leading cause of deaf blindness, gene must be inherited from both parents. Symptoms: Retinitis pigmentosa ( bilateral visual dysfunction, loss of vision at night and progressive loss fo peripheral vision, SNHL of cochlear hair cells.)

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Type I Usher's Syndrome

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Severe to profound HL across all frequencies, complete loss of vestibular function from birth, difficulties maintaining balance, retinitis prigmentosa

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Type II Usher's Syndrome

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Moderate to severe hearing loss, vestibular function is normal, onset retintis pigmentosta is typically in third or fourth decade of life.

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Type III Usher's Syndrome

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Progressive HL in childhood or late teens, vestibular function ranges from normal to near normal, problems with balance begin in teens, retinitis pigmentosa varies in severity often begin in teens.

Front 34

Cytomegalovirus (CMV)

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(Not a syndrome) CMV is herpes virus family, transmitted through body fluids, 50-80% will acquire CMV by age 40, most common cause of congenital, nonhereditary SNHL.

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Symptomatic CMV

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Symptoms: jaundice, red purple dots under skin, prematurity, cerebral palsy and vision defects. ( less than 30-40% will develop SNHL)

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Asymptomatic

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90% infants, less than 5-10% will develop SNHL