Name the stage that encompasses the first 15 days of life. 

Name the 4 substates

Stage:  Blastogenesis

Substages:  1. Zygote (ovum + sperm)

2. Morula:  day 3, 12-32

3. blastocyst:  day 5 or 6, embeds in uterine wall

4. embryoblast/trophoblast (becomes placenta)

In what stage do the 3 germ layers form?

Name them.

Embryogenesis (days 16-75)

Endoderm, ectoderm, mesoderm

The Mandibular Arch:

Skeleton

Muscles

Innervation

Cleft Derivative

Arch 1

Skeleton:  mandible, maxilla, malleus & incus

Muscles:  masticators, 2 tensors

Innervation:  CN V3

Cleft Derivative:  external auditory meatus

Name the second arch

Skeleton:

Muscles:

Innervation:

Cleft Derivative:

Arch:  Hyoid

Skeleton:  stapes, styloid process, upper part of hyoid

Muscles:  facial expression, stylohyoid, stapedius

Innervation:  CN VII

Cleft Derivative:  cervical sinus

Arch 3

Skeleton:

Muscles:

Innervation:

Cleft derivative:

Skeleton:  greater cornu & lower body of hyoid

Muscles: stylopharyngeus

Innervation: CN IX

Cleft derivative:  obliterated by cervical sinus

Arch 4

Skeleton:

Muscles:

Innervation:

Cleft derivative:

Skeleton: thyroid cartilage

Muscles: pharyngeal muscles

Innervation: CN X

Cleft derivative:  obliterated by cervical sinus

Arch 6

Skeleton:

Muscles:

Innervation:

Skeleton:  cricoid & arytenoids

Muscles: laryngeal muscles

Innervation:  CN X (RLN branch)

When do the 3 "blisters" develop?

Name them

What do they control?

Develop at wk 3

1. prosencephalon

2. mesecephalon

3. rhombencephalon

*all control the growth of head, neck and brain

1. formation of primary palate (fusion of premaxilla & alveolar ridges)

2. formation of secondary palate (fusion at the midline)

At what week gestation are the following structures complete?

Lips

Hard Palate

Soft Palate

Uvula

Lips: wk 6

Hard Palate: wk 9

Soft Palate: wk 10

Uvula: wk 12

Define

Aplasia

Hypoplasia

Hyperplasia

Aplasia:  congenital absence of an organ or tissue

Hypoplasia:  under or incomplete development of an organ or tissue (too small)

Hyperplasia:  gross enlargement

2:1 males to females

*except 2:1 females to males for isolated cleft palate

1. bifid uvula

2. velar midline furrow (zona pellucida) - bluish tinge at midline of soft palate

3. hard palate notch (absent post-nasal spine)

4. insertion of levator muscles into hard palate (instead of soft)

What is the key to watch for an occult submucous cleft palate?

How is it best detected?

Key:  muscular bulk defect of soft palate (classic symptoms absent)

Detection:  endoscopic Ax seen as a midline groove during velar elevation

-plastic surgery

-pediatric medicine

-dentistry & orthodontics

-ENT

-audiology

-psych/social work

-speech therapy

1. ensures continuity of care (treatment can last decades)

2. high patient volume to a centre allows for development of expertise

At what age does discussion of velopharyngoplasty begin (if necessary)?

When does the secondary alveolar bone graft occur?

When would orthagnathic surgery happen?

-Approx 6 yrs old

-approx 10 yrs (as adult teeth begin to erupt)

-approx 18 yrs (once bone growth has stopped)

The use of intraoral moulding plates to guide the growth of alveolar ridges to align (makes later surgery easier)

-adhesive tape bridges the cleft (temporarily completes orbivularis oris & supports the nose)

-helps to pull back a 'runaway maxilla'

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What syndrome is this?

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What syndrome is this?

How can you tell?

Stickler Syndrome

-low-set ears

-pierre-robin sequence

-cleft palate (occult) submucous cleft palate

-many develop/have VPI/resonance issues

-75% w congenital heart disease
(tetralogy of fallot)
-immune features (absent/hypoplastic thymus, some immunodeficiency)
-endocrine features (hypoparathyroidism --> hypocalcemia)
-short stature/growth delay
*some: laryngeal web, GERD, severe feeding probs, brain anomalies, renal anomalies (30%)

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What syndrome is this?

How do you know?

Beckwith-Wiedemann Syndrome

-macroglossia

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What syndrome is this?

Fragile X Syndrome

-long face

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What syndrome is this?

How do you know?

Moebius Syndrome

-flat facies

-ltd facial expression

-lip incompetency

-ocular ptosis (drooping eye)

6th & 7th cranial nerves

6th- ocular ptosis (droopy eye)

7th - ltd facial expression

-lip incompetency

-flat facies/ltd expression

-lip incompetency

-VPI

-ocular ptosis

-ltd tongue mobility

-chronic otitis media

10-15% have mild cognitive impairment

[image]

What syndrome is this?

How do you know?

Goldenhar Syndrome

-ocular anomalies

-microtia (w hearing loss)

-facial asymmetry

-cleft palate

[image]

What syndrome is this?

how do you know?

Treacher-Collins aka Mandibulofacial dysostosis

-coloboma (cleft) of lower eyelid

-hypoplasia of Os zygomaticum

-downslant eyes

-maxillary and mandibular hypoplasia

Treacher-Collins/Mandibulofacial Dystosis, what is involved?

Which Branchial arch?

What is holoprosencephaly?

Why do they not to survive?

*one of most severe craniofacial syndromes, usually die in first year of life
-incomplete differentiation of prosencephalon
-hypotelorism (or cyclopia)
-cleft lip n palate (reduced nasal height)
*poorly developed frontal lobes, low motivation/drive to live

Apert's

Crouzon's

Pfeiffer's

Saethre-Chotzen

Metopic (midline front bone)

Squamosal (of the temoral bone, on either side)

Sagittal

Coronal

Lambdoidal (parietal + occipital)

Skaphocephalus (long skull)

Brachycephalus (short skull)

Trigonocephalus (leaf-shaped skull)

*all can have normal and abnormal versions

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What skull type is this?

Trigonocephaly

[image]

What skull type is this?

Sagittal

-increase in fronto-occipital volume

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What skull type is this?

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What skull shape is this?

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What skull shape is this?

[image]

What skull type is this?

Hypoplastic midface (midface too small)

-usually exophthalmus at birth

What syndrome is this?

How do you know?

[image]

APERT Syndrome

-syndactyly of fingers (fusing of fingers)

-shortened upper arms

-hypoplastic maxilla (class III occlusion + open bite)

-beak-shaped nose w depressed nasal bridge

Dyscraniosynostosis (coronal suture + others)

-syndactyly of fingers

-shortened upper arms

-hydrocephalus

-cognitive impairment

-sometimes cleft palate (30%)

-hypoplastic maxilla

-beak-shaped nose with depressed nasal bridge

-cervical anomalies

Cranial vault reshaping:  reduce intracranial pressure, looks

Airway issues - midface advancement, trache, CPAP

Middle Ear Disease - (crowded nasopharyngeal space) PT tubes

Shunts (reduce intracranial pressure)

Orthodontics/orthognathic surgery

Crouzon Syndrome

-craniosynostosis (multiple sutures)

-exorbitism

-maxillary hypoplasia

-craniosynostosis of multiple sutures

-maxillary hypoplasia

-exobitism

-hearing loss

-sometimes cognitive impairment

-sometimes cleft palate

-accumulation of soft tissue in palatal vault

Clover-leaf shaped skull

maxillary hypoplasia

hypertelorism

exophtalmus

broad thumbs and toes

-coronal suture (brachycephaly or oxycephaly)

-soft tissue syndactyly

-sometimes cleft palate

-maxillary hypoplasia

-low frontal hairline

1. Ventilation of the middle ear

2. Regulation of oral-nasal airflow

3. Prevention of Nasal regurgitation

4. Prevention of pre-deglutitave aspiration

5. contributes to bolus propulsion during swallowing

6. Oral-nasal balance for speech articulation

Origin:  sphenoid & lateral cartilage of eust tube

Insertion: forms palatine aponeurosis (after hooking around pterygoid hamulus)

Origin:  temporal bone and medial cartilage surgace of eust tube

Insertion:  soft palate, inferiorly (levator sling)

1. raises soft palate

2. helps open eust tube

Origin:  oral surface of palatine aponeurosis

Insertion:  lateral post dorsum of tongue

1. lowers the velum (and helps raise dorsum of tongue)

2. forms first faucial arch

origin: palatine aponeurosis

insertion: lateral pharyngeal wall

What does the palatopharyngeus do?

1. pulls pharyngeal walls medially

2. lowers the velum

3. forms second faucial arch

Shortens velum

Forms velar eminence, helping to fill space between elevated velum & post pharyngeal wall

Origin: pterygoid plate & lateral finges of velum

Insertion: pterygomandibular raphe

-male nasopharynx is larger

-males must elevate velum higher & more posteriorly for complete closure

-m. uvulus give extra elevation (may be seen as a 'knee' or 'bump')

1. coronal

2. sagittal

3. circular

4. circular w passavant's ridge

1. hypernasality (func'l vs organic)

2. hyponsality (func'l rare)

3. Mixed nasality

4. alternating nasality (uncoordinated/ out of sync velar movement)

What is mixed nasality?

What can cause it?

Cul-de-sac nasality with little or no resonance or projection

-can be caused by a complete unilateral CLP = hypernasality + blocked nose

Nasal air emission/nasal escape

Nasal turbulence (velum is almost closed, air squeaks through)

Direct visual inspection

Endoscopy (rigid vs flex)

multi-view videofluoroscopy

1. tongue anchor technique

2. mirror test

3. gutzman's l-a-test

4. aerodynamic measurements

5. EMG

6. haskins velotrace

Indirect method to view velum/function

-have client puff cheeks with tongue out.  Should not be able to breathe

An indirect method to check velar function

-subject says nonnasal sentences

-no air should escape from nose

An indirect test of velar function

-theory: hypernasality is heard more on high vowels

-alternate I/a with nose pinched

-should be no different (hypernasal person will have a very different I)

Pressure-flow estimation of VP orifice area

-area in Cm2

-Vn: nasal airflow in litres/s

P: oral & nasal pressure in dynes

D: density of air

k: correction factor

1. nasometer

2. nasalview

3. oralnasal system

Antibiotics

Syringotomy (suck it out)

Myringotomy (tubes)

Palatoplasty (indirect fix)

*those with cleft palate must see an audiologist or ENT on a regular basis

1. VP insufficiency (cleft)

2. VP insufficiency (noncleft)

3. VP incompetency (noncleft)

4. VP mislearning (noncleft)

Unrepaired palatal clefts (overt, submucous or occult)

Postsurgical insufficiency

-post-palatal closure

-post-adenoidectomy

-post-pharyngeal flap

-complicated by fistula

Mechanical interference: tonsils, adenoids, post pillar web

Palatopharyngeal disproportion

Ablative palatal lesions: cancer, traumatic injury

Primary motor control (DYSARTHRIA)

Congenital: CP, myotonia, dystrophies

Acquired: TBI, CVA/brainstem stroke, progressive disease

Motor association/programming (APRAXIA)

speech, oral non-speech

Phoneme specific nasal emission (usually seen in high pressure consonants, ex /s p/

Persisting post-op nasal emission (even with ability to close)

Compensatory misartic (maladaptive VPI)

Deafness/hearing impairment

Front to back (front are more tactile)

/m/ often is first, /b/ is just /m/ with VP closure

Omissions (not always seen)

Distortions

Substitutions within phonological system

Compensatory subs (unviversal for all CLP kids)

-too much air & sound escape through nose

-nonnasal vowels & consonants sound nasal

Organic Etiology: VP insufficiency

Functional etiology: VP incompetency

-too little air & sound travel through nose

-nasal consonants & vowels are denasalized

-Organic Etiology: septum deviation, hypertrophic turbinates

Functional (rare): protectional reaction to autophonia (open eust tubes)

Combo: VP dysfunction with blocked nasal passage

-ant & post form (differ by location of blockage)

-often in unilat CLP (VPI w septal deviation)

-can be seen as anterior mixed

-anterior blocked nasal passage

-sound quality is muffled

-VP mechanism out of sync with other speech articulators

-nasalization & de-nasalization occur randomly

*uncommon diagnostic category

Often caught with mirror test

-often occurs with hypernasality

Audible (non-turb) = large VP gap

Nasal turbulance = close VP approx, counter-indication for secondary VP surgery (may be able to improve with therapy)

Obligatory: structural VPI, oro-nasal fistula, VP incompetence related to neuro damage

Learned:  VP mislearning, phoneme specific nasal emission, persisting post-op emission

-common in hypernasal speakers with CLP

-large nostril flares (tense to try to close VP/reduce nasal escape)

Category 1 (obligatory): VP insufficiency, oronasal fistula

Category 2 (compensatory):  mislearning (may be in response to structural inadequacies)

Glottal stop paired with a stop consonant

-pulse sound heard under a plosive (often with p or b)

-often results from speech therapy

Teach aspiration (cannot close folds for a stop if aspirating)

-exaggerate aspiration & gradually reduce it until left with typical consonant

1. maladaptive placements

2. backed artic (glottal stop, pharyngeal plosives)

3. learned strategies

4. adaptation to insufficient VP closure

-aplasia of nasal septum

-premaxilla aplasia

-hypertelorism

-flat nose

*mid face basically missing, flattened nose

What are the three views for multiview videofluoroscopy?

What do they show?

Sagittal (velar contact with post pharyngeal wall) - velar displacement ant-post

Frontal (height of velum)

Base ie sphinx, from below

What is nasalance?

What is the formula?

Electronic measurement of sound pressure from the nose (as compared to oral sound pressure)

Nasalance = (Nasal SPL/Oral + Nasal SPL)x100

Antibiotics

Syringotomy (suck out the fluid)

Myringotomy (tubes)

Palatoplasty (indirect)

Why worry about otitis media?

Active, purposefully alter the speech sound to produce a substitute sound.  Ex. in a nasal fricative the client blocks the oral flow of air causing all air to exit nose

Passive, client attempts proper production but an insufficiency alters the sound ex. nasal fricative, the articulation is correct and some air flows correctly but air also escapes from the nose