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Quid Refert, Dummodo non Desinas, Tardius Ire

ANATOMY, PHYSIOLOGY, EMBRYOLOGY & CONGENITAL ANOMALY


ENT Dr. Caluag

ANATOMY Scaphoid fossa or fossa triangularis – pocket-like depression


EAR where anti-helix terminates
2. Tragus & Anti-tragus
 The Organ of Hearing and Balance
Anti-tragus – cartilaginous protrusion above ear lobe
 Attached to Temporal Bone
Anti-trigal notch – depression below the anti-tragus
 5 PARTS OF THE TEMPORAL BONE (part of the facial skeleton)
3. Conchae and Lobule
1) Squamous part
Conchae – big depression inside the pinna; leads into the
2) Mastoid bone
external auditory meatus
 Spongy bone - posterior 4. Meatus and Canal
 Houses the middle ear cavity - Canal is divided into Outer Soft Tissue and Inner Bony
3) Tympanic bone Bony part – encase the bony part of the Part
external canal - Junction is the ISTHMUS → Anatomical Constriction
4) Styloid process – independent part of femoral bone *yung mga naglilinis ng ears beyond the isthmus,
 Stylomastoid foramen → where facial nerve naiiwanyung cotton buds
passes - Anatomical constriction makes theadult ear canal not
5) Petrous – houses the inner ear straight
- Otoscope (how to visualize the canal)
 Adult – upward &backward
 Pediatric – upward & outward
- External ear is important to physician due to lesions
involved

B. MIDDLE EAR (tympanic cavity)


 Wedge-shaped cavity (very narrow side to side) with 6 walls
ANATOMIC SUBDIVISIONS boundaries
1. Superior – tegmen tympani
2. Inferior/floor – jugular bulb
3. Medial –
o oval and round windows;
o lateral semi-circular canal
o footplate of the stapes seals off the oval window
by a structure called annular ligament
4. Lateral – EAR DRUM/tympanic membrane
o 3 layers:
A. EXTERNAL EAR
Outer epithelium
 Outside the Head
Middle Fibrous – give the pearly white pearly gray
 Made up of Pinna and EAC
Inner Mucosal
 Land Marks:
5. Anterior –
1. Helix and Anti Helix
o tensor tympani muscle;
Helix – outermost part of pinna
o internal carotid artery;
Anti helix – inner margin of pinna
o eustachian tube (37 mm Length)

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6. Posterior – facial canal (decending portion turns downward to  ACTION → to draw the handle of the malleus inward
exit into the Stylomastoid foramen) innervated by the motot root of CN V, hence tenses
the ear drum
 2 branch of Facial Nerve in this descending part: o Stapedius muscle – lies in the posterior part of the middle ear
below the aditus (opening to the antrum, which is connected
1. Stapedius Muscle – attached to the stapes (the Horshoe in the
to the mastoid cavity) attached to the head of the stapes
illustration)
 ACTION → to draw the head of the stapes outward and
2. Chorda tympani – attached to the ear drum
downward, innervated by CN VII

 Ear Drum
o a.k.a. Tympanic Membrane;
o 8 mm wide; 9 mm height; 0.1 mm thick
o Normally Pearly white, pearly gray in color (emphasized by
Doc)
o divided into Pars Flaccida (upper) & Pars Tensa (lower) by
Maleolar fold *check illustration below
o PARS FLACCIDA
- retracts when ET is Blocked → inadequate vibration →
conduction deafness
- Bulges if there is Middle Ear Effusion
 e.g., middle ear infection hence also conduction o Ossicular Chain – suspended in the Middle ear cavity forming
deafness a chain for conduction of vibrations from the drum to the oval
 if it bulges, you can’t distinguish the landmarks window
because it is obscured 1. MALLEUS – hammer
o UMBO → silhouette of the handle of malleus - head of the malleus is the large protruding
section, which attaches to the incus.
- The head connects to the neck of malleus, and
the bone continues as the handle of malleus,
which connects to the tympanic membrane
2. INCUS – anvil
- receives vibrations from the malleus, to which
it is connected laterally, and transmits these to
the stapes, medially
3. STAPES – smallest; MOST IMPORTANT because its
footplate seals off the oval window; its movement
causes waves to the perilymph which is important or
hearing functions

 Eustachian Tube
o AKA pharyngotympanic tube
o Joins the middle ear with nasopharynx
o Total length ~ 37 mm long
o Partly bony making up 1/3 of its length (always open) & the
inner 2/3 is cartilagenous (closed at the pharyngeal end
EXCEPT when swallowing or Yawning)
AUTOPHONY – when you hear what you say while yawning
o The ET Lumen is opened by:
 Contraction of TENSOR VELI PALATINI & LEVATOR VELI
PALATINI
 Tensor Veli Palatini – innervated by the mandibular
o Blood and Nerve Supply:
branch of CN V from the Otic Ganglion
a) Deeper Auricular Branches of the Internal Maxillary
 Levator Veli Palatini – innervated by pharyngeal branch
Artery –Blood Supply of the Middle Ear
of CN X; bulk of palate kaya involved sa cleft palate
b) Nerve Supply :
o ET is lined by Pseudostratified Columnar Ciliated Respiratory
Epithelium which are also found on the nose, nasopharynx & - Auriculotemporal branch of CN V
middle ear *kaya nagprproduce ng din ng sipon - Auricular branch of CN X (Arnold’s nerve)
o Tensor Tympani – found at the anterior wall - Tympanic Branch of CN IX (Jacobson’s nerve)
 arises from upper surface of the Cartilagenous end of
ET, and by a rounded tendon over a bony spine-like
projection → COCHLEAFORM PROCESS

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perilymphatic space and the subarachnoid
space
- one of pathway in which infection may reach
the cranial cavity (e.g., otitis media may lead to
meningitis)

C. INNER EAR
 a.k.a. Labyrinth
 With Bony and Membranous Part

 OSSEOUS /BONY LABYRINTH


o Series of cavities filled with perilymph, enclosed by the
vitreous part of the temporal bone
o Hard Bony Shell (one of the hardest bone of the body)
o Midportion between the cochlea & semicircular canal is the
VESTIBULE
o Medially situated in the middle ear space
o 2 parts:
1) COCHLEA – lies in front of the vestibule.
 Resembles a snail shell of 2 ½ turns w/ its Central
Conical Axis (MODIOLUS)
2) SEMICIRCULAR CANAL – Lateral, Superior & posterior
 MEMBRANOUS LABYRINTH
o Osseous spiral lamina
o Series of sacs and ducts
- bony shelf or ledge which projects from the modiolus
o Enclosed within the bony labyrinth
and divided into 2 parts
o Filled with endolymph in the bony labyrinth
- basilar membrane stretches across the outer wall and
separates in another two passages EXCEPT for a small o Consists of 2 Sacs:
communicating opening between them in the apex  UTRICLE & SACCULE containing sensory epithelium
called HELICOTREMA, which the two scalae innervated by the vestibular part of CN VIII
communicate with each other o Spiral Organ of Hearing are found in the Cochlear Duct (scala
- It reaches about half-way toward the outer wall of media/ductus cochlearis)
the tube, and partially divides its cavity into two o COCHLEAR DUCT (scala media) – a spiral tube superiorly
passages or scalae, of which the upper is named the bounded by the Reissner’s membrane(vestibular membrane)
scala vestibuli, while the lower is termed the scala and inferiorly by the Basilar Membrane
tympani
o SCALA VESTIBULI – above the Cochlear Duct (Perilymph)
o Cochlear division of CN VIII enters the malleolus at a space
o SCALA TYMPANI – below the Cochlear duct (Perilymph)
o 3 openings of Cochlear Canal:
o SCALA MEDIA – cochlear duct/ductus cochlearis (Endlolymph)
1) ROUND WINDOW – opens into the cavity of the middle
ear, sealed off by the round window membrane, called
fenestra cochlea PERILYMPH ENDOLYMPH
2) OPENING OF THE VESTIBULE VOLUME 78.3 cu.mm 2.76 cu.mm
3) COCHLEAR AQUEDUCT Na+ Content 310 mg/100ml 15mg/100ml
- smaller than the others; K+ Content 30mg/100ml >600mg/100ml
rd
- open into the basal 3 of the cochlea; CHON 70-100mg/100ml 20-30mg/100ml
- lodges a tubular prolongation of the dura mater pH 7.2 7.5
establishing a communication between the *lumalabas daw to sa BOARD exam, kaya aralin na hehe*

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 The Stapedius and the Tensor tympani contracts to protect the inner ear
against over stimulation by Stiffening the OSSICLES, hence they function
as shock absorbers
 Movement of the Footplate of the stapes results to Perilymphatic motion
disturbing the hair cells which gives rise to an electrical potential that
triggers the nerve impulse

VESTIBULAR SYSTEM
 Controls position & movement of the Body in space
 In conjunction with the visual and proprioceptive system functions to
control positions and movements of the body in space.
 OTOLITH ORGANS – sensitive to linear acceleration and aids in postural
balance/equilibrium and control of muscle tone throughout the body.
- Utricle & saccule
- Linear accelerometers
 SEMICIRCULAR CANAL – responds to angular acceleration and aids in the
coordination of head and eye movements.
Longitudinal section of cochlea - Angular rate sensors
 Receptor Units – hair cells located in the CRISTAE of Semicircular Canal
and Maculae of Otolith Organs
 Due to Inertia, movement of endolymph causes displacement of the hair
cells opposite to the direction of the acceleration stimulus.
 If the constant velocity is maintained for a sufficient period of time, cilia
of the hair cells will return to its normal position and subject will no
longer experience the sensation of movement
 But if the rotation suddenly stops, the cilia of the hair cells will be
deflected toward the opposite direction and subject will still experience
the sensation of movement
 Stimulation of the Vestibular Organs creates a neural excitation that
travels via the vestibular nerve of CN VIII to the 4 vestibular nuclei in the
brainstem
 NYSTAGMUS – is a Reflex Eye Movement elicited upon stimulation of the
semicircular canals via angular acceleration; rapid eye movement; this
movement acts to maintain a constant visual field
 Vestibular Nystagmus – response commonly used by the
otolaryngologist to assess the state of balance system
o INTERNAL AUDITORY ARTERY – blood supply to the Cochlea  UTRICLE – important in orientation to gravity and linear acceleration and
- Branch of basilar artery controls movement needed to maintain an upright posture in the
o ORGAN of CORTI – Epithelial structure on top of the basilar presence of a displacing linear force (e.g., standing in a moving train)
membrane divided by a tunnel composed of 2 rows of Rods  Most ancient part of the ear is the vestibular organ within the
(Rods of CORTI) cartilaginous otic capsule
o RODS of CORTI – sites of attachment of the basilar membrane
to the osseous spinal lamina; single growth of inner hair cells
EMBRYOLOGY
& MOST IMPORTANT sensory cells in the organ of hearing
o TECTORIAL MEMBRANE - forms of the superior surface of the  The ear is a complex sensory organ of triple origin:
Organ of Corti made up of fine, colorless fibers in a a) EXTERNAL EAR
transparent matrix with a hair cells embedded in it  modification of the surface ectoderm
o SPIRAL GANGLION OF CORTI –has an average of 31,000  surface ectoderm by which skin is brought to functional
ganglion cells, each inner hair cell has an innervation from 2 relationship with the ossicles
or more nerve fiber
 Modified for protection, reception and concentration of
sound
PHYSIOLOGY
AUDITORY SYSTEM b) MIDDLE EAR
 Conducts acoustic energy to the inner ear where it is converted to  an air sinus and develops as an outpouching of the pharynx
impulses in the Auditory nerve  outgrowth of pharynx
 Impulses are processed in the higher centers of the auditory chain from  Ossicular chain develops from the outer end of mandibular
the Cochlea to the superior olive; to the lateral lemniscus; to the and hyoid cartilages respectively
Superior Colliculus to the medial geniculate body, to the Auditory
Complex  Cochlear evolution is parallel with that of the middle ear
and auditory ossicles
 Sound waves travels the ear canal causes the ear drum to vibrate which
causes vibration of the ossicles resulting to Perilymphatic Motion
 IMPEDANCE MATCHING – process by which acoustic energy in the c) Cochlea – develops parallel with the middle ear and Ossicles
airborne sound is transformed to Vibratory energy in the Cochlea

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EXTERNAL AND MIDDLE EAR DEVELOPMENT  Vestibular ganglion remains close to the utricle and the saccule and the
peripheral fibers go into the ampulla and into the macula of the utricle
 9 – 10 DAYS AOG (at 4 mm size of the EMBRYO) the Tubotympanic
and the saccule to make a synaptic contact with the sensory receptors.
recess forms an expanded cavity with the lateral extensions of the 1st,
2nd and 3rd branchiall arches with the 1st and 2nd pouches  CRISTAE – derived from the Ampullae of the Semicircular Canals
 The Lateral part of tubo tympanic recess becomes the middle ear and  ORGAN OF CORTI – derived from Cochlear duct
pharyngeal midline structures become the Eustachian Tube  Neurons of vestibular ganglia develops by proliferation of otocyst and its
 The external auditory meatus develops and thickening of the ectoderm subdivision
at the upper end of the 2nd external pharyngeal cleft
 Ectodermal meatal plug and the ectodermal tubotympanic recess TEMPORAL AND MASTOID BONE DEVELOPMENT
becomes the tympanic membrane
 At birth, Temporal Bone is separated into 3 parts:
 Ectodermal meatal plug – where hair follicles and cerumenous glands
 PETROUS BONE
arises
 SQUAMOUS BONE
 Tympanic membrane develops as a circular area of contact between
Ectodermal and endodermal tubotympanic recess  TYMPANIC BONE
 Circular area of contact between the ectodermal meatal plug and the
 It is pneumatized by the extension of the epithelial sac from the middle
ectodermal tubotympanic recess becomes the tympanic membrane
ear to the bone marrow
 The cartilaginous otic capsule conceals the inner ear and later becomes
 3 degrees of Temporal Bone pneumatization:
petrous bone
1) Pneumatic Bone (well-pneumatized)
 Anterior to the tubo tympanic recess – Meckles Cartilage (1st Arch) it’s
upper part forms the Malleus and Incus 2) Diploic Bone (partially pneumatized)
 Posterior to the Tubo tympanic recess – Reichert’s Cartilage (2nd Arch) 3) Sclerotic bone (not pneumatized)
gives rise to the styloid process, the Stylohyoid ligament and the Upper
body of the hyoid  Pneumatization of the temporal bone begins at the first year of life and
by the 6 yrs may actually be complete but can increase throughout
 The bony canal develops as an extension of the tympanic ring by
adolescence
intramembranous ossification.
 Tympanic portion of the temporal bone makes up the ear canal, it is a
 Mastoid process is absent at birth and the facial nerve emerging from
small hole shaped bone that has the inferior margin of the squama
the stylomastoid canal is still superficial can thus be easily injured in
serving also for the attachment of the ear drum.
delivery with forceps.
 Mastoid process - formed by the union of the mastoid and petrous
 3 layers of the Drum:
portion of the temporal bone
a) outer Ectodermal layer continuous with the skin of the ext.
 mastoid antrum is formed by the squamous epithelium portion of the
auditory meatus
temporal bone and as the mastoid develops, the antrum is considered
b) intermediate mesodermal layer containing the handle of the part of the mastoid process. Entrance to the mastoid antrum is via the
malleus and the corda tympani nerve aditus.
c) inner endodermal layer continuous with the mucus membrane of
ear cavity

 nerve supply reflects its origin: CONGENITAL ANOMALIES


a) outer Ectodermal surface – auriculotemporal branch of the EXTERNAL EAR
trigeminal and auricular branch of vagus nerve posteriorly  Usually caused by Maldevelopment of the 1st and 2nd Arches
b) the nerve that supplies the eardrum also supplies the ext. a) Auricular Deformities
auditory meatus
 most prominent
 Irritation of the Auricular branch of CN X may cause reflex coughing,  Most common of these is when the ear protrudes
vomiting or Sneezing because it is the only somatic afferent branch of outward in excessive manner
formerly known as ALDERMAN’S NERVE
 the four elements of the temporal bone are easily distinguished b) Abnormal size – macrotia/microtia
(petrous, squamous, styloid, tympanic … c) Rudimentary ear appendages or total absence of ear – usually
 The mastoid Antrum presents the posterior extension of the Middle Ear associated with partial or complete stenosis of the ear canal
(no mastoid process during birth, and does not present until the end of d) Congenital Pre-auricular Sinus - occurring as a small opening just
the second year of life) a little bit in front of the helix.
 The ossicles are extramucosal and secondarily incorporated to the M.E.  May drain intermittently and may occasionally become
by formation of attic recesses infected
 Can be identified by injection of Methylene Blue and
can be excised under local anesthesia
INNER EAR
*MACROTIA – abnormally large ear
 Develops as an endodermal thickening on the lateral surface of the head
at the level of the 4th ventricle in embryos of 4mm in size (9-10days) *MICROTIA – abnormally small ear
 14 – 15 days (6mm size) –Endolymphatic ducts *Lop Ear – excessive protrusion

 Eventually shifted down by differential growth at the connection


of the utricle and the saccule so that it will be ultimately MIDDLE EAR
attached to the utriosacular duct.  Ossicular chain maybe congenitally deformed, disrupted or fixed
 37 days (15mm size) – Semicircular Canal  Common deformities include a missing part of the Incus and fixation of
the Stapes
 Cochlea develops at 15 mm and grows progressively finally attaining 2
and a half turns at the end of 1st trimester  Stapedectomy and Prosthesis replacement re-establish hearing if the
stapes is fixed

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INNER EAR  RUBELLA
A. GENETIC  in the 1st trimester results in SNHL pathology shows aplasia of
the Saccule and the Organ of Corti
 MICHEL’S DEAFNESS  patients may also suffer from other defects such as cardiac
 Total lack of development of the inner ear, Autosomal anomalies, blindness and retardation
Dominant
 KERNICTERUS – Erythroblastosis Fetalis
 MONDINI’S DEAFNESS  results from Rh blood incompatibility of the blood of the
 partial aplasia of the inner ear resulting in a flattened cochlea parents
with only 1 ½ turns associated with dysgenesis of the organ of  characterized by Bilirubin deposition in the brain, there’s
Corti, Autosomal Dominant jaundice, retardation, cerebral palsy and SNHL
 TX : Post partum exchange transfusion
 UNDERDEVELOPMENT OF ORGAN OF CORTI
 results into sensorineural hearing loss  CRETINISM
 thyroid disease associated with deafness referred to as
 SCHEIBE’S DEAFNESS Endemic Cretinism.
 the bony labyrinth is fully develop but the cochlear duct and  Presents with mixed hearing loss
the saccule is represented by undifferentiated cells, Autosomal
Recessive  HURLER’S SYNDROME
 Residual hearing in low frequencies  results in skeletal deformity, dwarfism, retardation, blindness
and profound SNHL
 ALEXANDER’S DEAFNESS  transmitted as a recessive trait or it may be sex-linked
 aplasia of the Cochlear duct in which the Organ of Corti and
Adjacent ganglion cells are severely affected resulting to High  KLIPPEL-FEIL SYNDROME
Frequency SNHL
 skeletal defect such as fusion of the cervical vertebra, Spina
 Bony and membranous labyrinth appear normal Bifida, Scoliosis, Torticollis
 associated with vestibular dysfunction and profound SNHL.
 WAARDENBURG’S DISEASE  Autosomal Recessive
 there’s lateral displacement of the Lacrimal points and medial
canthus, hyperplasia of eyebrow, flat nasal root and mild to
severe SNHL  ALSTROM’S DISEASE
 features of Obesity, DM, Retinitis pigmentosa and progressive
deafness
 ALBINISM
 Progressive, autosomal recessive
 characterized by severe- profound SNHL maybe Autosomal
dominant, recessive or sex-linked
 Bilateral Severe compound sensorineural hearing loss  CROUZON’S DISEASE - Craniofacial dysostosis
 presented by premature closure of the cranial suture,
 ONYCHODYSTROPHY  short upper lip, protruding lower lip, exophthalmos, hook
nosed.
 affected siblings have small, short fingernails, toenails and
SNHL probably a recessive trait  Atresia of the external meatus and mixed deafness.
 congenital sensorineural hearing loss  Autosomal dominant.
 Normal intelligence
 USHER’S SYNDROME
 presents with progressive retinitis pigmentosa and moderate-  RICHARD’S-RUNDELL SYNDROME
severe SNHL, recessive transmission  features of ataxia, hypogonadism, mental deficiencies & severe
SNHL which is total by 5-6 years of age.
 Autosomal recessive
B. CHROMOSOMAL ABNORMALITIES – not truly hereditary
 ALPORT’S DISEASE
 TRISOMY 13-15 D  Dominantly inherited
 includes low set of ears, undifferentiated pinna, absence of  a progressive glomerulonephritis that usually begins in
external and middle ears, cleft lip and palate, aplasia of the childhood,
optic nerve, microphthalmia and colobama iridis
 progressive S.N.H.L. increases as the renal malfunction worsens
 Infants with this condition expires after a short period of time

END
 TRISOMY 18 E
 includes flexion of the index finger over the 3rd finger, a
prominent occiput, micronathia, low sets of ear and malformed Black – from power point
pinna, Blue – trans from lecturer
 patient dies in infancy Red – from book

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