CONGENITAL ANOMALIES OF

EXTERNAL AND MIDDLE EAR

 EMBRYOLOGY
Pinna :
• 4th week of gestation - 5 pharyngeal arches develops

• Each arch - outer ectodermal cleft, inner endodermal pouch and

middle mesodermal core

• 5th and 6th week - 6 mesodermal proliferation ( HILLOCKS OF HIS )

develops around 1st ectodermal cleft - three on each side of cleft

• These fuses by 3rd month and form pinna

• 1st hillock - Tragus

• 2nd hillock - Crus of helix

• 3rd hillock - Helix

• 4th hillock - Antihelix

• 5th hillock - Antitragus

• 6th hillock - Lobule

• Concha - from 1st ectodermal cleft

• Initially, developing pinna is located in neck region caudal to

mandibular area

• 20th week of gestation - with development of mandibular process,

pinna migrates to the adult location

• 4 to 5 years old child - pinna is approx 80% of adult size

• 9 year old child - pinna attains complete adult size

External auditory canal

• EAC develops from 1st ectodermal cleft

• At 8th week of gestation - cavum concha

deepens and forms primary meatus - later
develops into cartilaginous part of EAC

• At 9th week - solid epidermal plug forms

extending from primary meatus to
primitive tympanic cavity - Meatal plate

• Between 24 and 28 weeks of gestation -

Meatal plate canalises and forms bony
part of EAC

• Innermost portion of Meatal plate forms

outer layer of TM

 Middle ear
• Third week of gestation - 1st pharyngeal pouch expands and form
tubotympanic recess

• At 7th week - mid portion of recess is constricted by 2nd branchial

arch - forms Eustachian tube (medially) and tympanic cavity
(laterally)

• During 4th-6th week of gestation- mesenchymal tissue differentiates

and forms ossicles

• 1st arch - Head of malleus, body and short process of incus

• 2nd arch - Handle of malleus

Long process of incus

Stapes suprastructure

Tympanic surface of stapes footplate

• Medial surface of stapes footplate, annular ligament - Otic capsule

 PREAURICULAR SINUS
• Theories :

1) Incomplete fusion of Hillocks of His

2) Isolated folding of ectoderm

• Sporadic or inherited

• Sporadic - unilateral ; Inherited - bilateral

• Opening lies in front of helix leading into tract lined

by squamous epithelium

• Tract lies in subcutaneous tissue between temporalis fascia and

parotid fascia

• Terminal portion - adherent to cartilage of helix

• MC associated with Branchio-oto-renal syndrome

• Presentation :

Opening will be present from birth

Asymptomatic

Sebaceous discharge

Recurrent infection - abscess formation

Congdon’s classi cation :

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Treatment :

• If free of infection - left alone

• Acute infection - IV antibiotics

• Recurrent infection - Excision of tract

Elliptical incision around sinus

Dissecting tract using methylene blue

Tract was excised en bloc along with the attached portion of

the ear cartilage

 Preauricular appendages / Skin tags

• Very common

• Unilateral or bilateral

• Solitary or multiple

• Usually contains skin, subcutaneous tissue and cartilage

• Found along the line drawn from tragus to angle of

mandible - re ects origin from 1st pharyngeal arch

• Treatment - Excision of tags along with cartilage

Liga clips - tag drops off

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 Microtia
• Incomplete development and growth of pinna

• Unilateral > bilateral

• Right side > left side

• Male > female

• Associated with congenital canal atresia

• Presents with total conductive hearing loss

• Other deformities - facial asymmetry, facial nerve weakness, cleft lip

or palate, urogenital defects, cardiovascular malformations and
macrostomia

 Etiology :
• In-utero tissue ischemia secondary to obliteration of the stapedial
artery

• Increasing maternal age

• Teratogens exposure - thalidomide, isotretinoin, vincristine,

colchicine and cadmium

• 18q chromosomal deletion

Grading system:
A) Marx grading

Based on clinical appearance

Grade 1 - Smaller pinna, but all features of a normal pinna are

recognizable

Grade 2 - Some features of a normal pinna are recognizable

Grade 3 - Rudiment of soft tissue and cartilage ( Peanut shaped )

Grade 4 - Absent pinna or Anostia

B) Weerda grading

Based on surgical management

1st degree - Reconstruction does not require the use of additional

skin or cartilage

2nd degree - Partial reconstruction requires the use of some

additional skin and cartilage

3rd degree - Total reconstruction

4th degree - Anotia

Pre operative management:

• Ideal age to perform reconstruction is at age 10

• Because at age 10 - contralateral ear will reaches adult size and the
rib cartilages will be large enough to create an ear framework.

• Upto age 10 - BAHA ( Bone anchored hearing aid ) is used for

development of speech and language

Management of microtia :
• No intervention

• Bone anchored auricular prosthesis

• Ear reconstruction - Autologous rib cartilage

Medpor

Methods of ear reconstruction :

1) Brent technique :
• 4 stage procedure

• Age of initiation- 6 yrs

• 1st stage - Cartilage implantation

• 2nd stage - Lobule formation

• 3rd stage - Elevation of auricle

• 4th stage - Tragus reconstruction

Stage of cartilage implantation :

• Contralateral 6th,7th,8th ribs are harvested

• Synchondrosis of the sixth and seventh ribs

harvested and used for body of auricular
reconstruction

• Portion of eighth rib harvested and sculpted

into helix, antihelix and crura

• Perichondrium also removed

Stage of Lobule formation :

• After 2 - 3 months

• Lobule associated with a microtic auricle is

usually positioned anterosuperiorly

• Lobule is rotated into a more posterior and

inferior position by Z plasty manner

Stage of auricle elevation :

• Postauricular incision is made
approximately 3–4 mm posterior and
superior to the cartilage graft

• Auricle is elevated

• Raw area on medial surface is closed by

split thickness or full thickness skin graft

 Stage of tragal reconstruction :

• Involves creation of a tragus and the appearance
of an external auditory meatus

• J-shaped incision is made in the anticipated

conchal region

• Posterior skin ap elevated, soft tissue is excised

deep to the elevated skin - To create the
appearance of a conchal bowl

• Anterior ap elevated - graft for tragus is placed

• Unilateral microtia - Chondrocutaneous composite

ap from normal ear

• Bilateral microtia - Rib cartilage

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2) Nagata technique :
• Variant of Brent technique

• 2 stage procedure

• Timing of surgery - 10 years of age

Chest circumference at xiphoid > 60cm

• 1st stage - Cartilage implantation, Lobule formation and tragus

reconstruction

Ipsilateral 6th to 9th costal cartilage harvested

Perichondrium is left behind

• 2nd stage ( After 6 months ) - Auricle elevation

After elevation of auricle - wedge of rib cartilage is harvested from

the fth rib and placed under the ear to prevent repositioning of the framework

Temporoparietal fascial ap to cover posterior surface of ear and

cartilage graft
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3) Firmin technique :
• 2 stage procedure

• Two projection pieces are added to the

framework to increase the depth of the
framework

• P1 links the tragus and helical crus and provides

stability to the anterior frame

• P2 - placed under the base plate deep to the

antihelix

 Brent Nagata Firmin

Age of initiation 6 years 10 years 10 years

No of stages 4 2 2

Interval between
3 months 6 months 6 months
stages
Contralateral Ipsilateral 6th - 9th Ipsilateral 6th - 9th
Cartilage harvested
6th,7th,8th rib rib rib
Harvested along Ant. perichondrium
Perichondrium Left behind
with graft harvested

Tragus creation 4th stage 1st stage 1st stage

 Medpor implant reconstruction :
• Synthetic biocompatible porous polyethylene
implant

• Can be done between 3 and 6 years of age

• Single stage procedure

• Can be performed at the same time as canal

atresia surgery

• Post auricular incision or Y shaped or zigzag incision

• Remnant cartilage is removed

• Implant is placed and wrapped with temporoparietal fascial ap

• Lateral surface of implant is covered with anteriorly based skin ap

• Post auricular surface is covered with full thickness skin graft ( lower abdomen
/ supraclavicular area )

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 Bone anchored auricular prosthesis :

• Alternative for ear reconstruction

• Principle - Osseointegration

• Osseointegration - A process whereby clinically asymptomatic rigid

xation of alloplastic materials is achieved and maintained in bone

• Single stage or 2 stage procedure

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• Titanium implants is placed into temporal bone

• After 3 months abutments are placed

• Then ear prosthesis can be tted

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 CONGENITAL AURAL ATRESIA

• Complete absence of EAC

• Often associated with Microtia and maldevelopment of middle ear

ossicles

• Rarely CAA can be seen in patients with normal pinna

• First branchial arch anomaly with the temporo-mandibular joint

displaced posteriorly

Grading systems :
A) Weerda grading :

Based on clinical appearance

Type A - Marked narrowing of EAC with an intact skin layer

Type B - Partial patency of the lateral EAC with an atretic medial

meatal plate

Type C - Complete atresia of the EAC

B) Jahrsdoerfer grading

Based on radiological appearance

Best candidates for hearing improvement

Score : >= 8 — good candidate

7 — fair candidate

6 — marginal candidate

<= 5 — poor candidate

 Timing of surgery :
• Atresia repair is recommended at 5-6 years of age

• Reason - Form exostosis like bony growths that may occlude the
EAC

Mastoid and middle ear are better pneumatized in older

children

Maturation of Eustachian tube function

 Bilateral CAA

1) No Microtia - Atresia repair at 5-6 years of age

Until then BAHA soft band used

2) Grade 1 or Grade 2 ( no cartilage reconstruction ) Microtia -

Atresia repair done at 5-6 years rst followed by Microtia repair

3) Grade 2 ( cartilage reconstruction ) or Grade 3 Microtia -

i) Autologous rib cartilage - Microtia reconstruction followed by atresia

repair

ii) Medpor - Atresia repair followed by Microtia reconstruction

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Surgical technique :
• Post auricular incision

• Temporalis fascia graft harvested

• Periosteum incised - starting at the zygomatic

root and continuing along the linea temporalis

second incision starting at the anterior

aspect of the linea temporalis incision down to the
mastoid tip

• Periosteum elevated Posteriorly

Canalplasty :

• Drilling begins at the junction of the linea temporalis and

superior aspect of the posterior rim of the glenoid fossa

• Tegmen is identi ed superiorly

• Middle ear is entered in epitympanum so that fused

malleus-incus complex can be identi ed

• Bone must now be carefully removed 360 degrees

around the chain to mobilize it completely

• 5-10% cases - incus will not be connected to the stapes

• In such cases lateral chain is removed and PORP is

placed over stapes

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• Split thickness skin graft harvested

• Meatoplasty :

In patients with autologous rib graft microtia

repair - U shaped incision is made in superior
aspect of tragus

Anteriorly based conchal skin ap is raised

Underlying soft tissue is removed to align

meatus with bony canal

Then anteriorly based skin ap is delivered to

newly created meatus - it forms anterolateral canal
wall

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• Temporalis fascia graft in placed over

ossicles

• New ear canal in lined with Split thickness

skin graft all around and sutured to conchal
skin

• Reconstructed neo-canal is centered on the

epitympanum with the tympanic membrane
draped over the fused malleus-incus
complex.

Bone anchored hearing aid ( BAHA )

• Alternative for aural atresia repair

• Children with unfavourable anatomy for atresia repair / poor surgical

candidates

• Implantation site- 5.5cm posterior and superior to EAC

Patients planned for future auricular reconstruction - 7cm

posterior to planned site of EAC

• Scalp ap is raised in the postauricular area

• Hole is created using drill for placement of the

porous titanium osseointegrated implant

• Surrounding soft tissue of the scalp is excised

• Hole is cut through the skin ap to pass implant

transcutaneously

• Abutment is placed

• After 3 months of healing period - sound processor

placed
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 PERSISTENT STAPEDIAL ARTERY

• During development 6 arches with
corresponding arteries develops which connects
truncus arteriosus with dorsal aorta

• 1st arch - Mandibular artery which regresses

later

• 2nd arch - Hyoid artery - persist as

caroticotympanic branch of ICA

• 3rd arch - CCA and proximal ICA

• When 1st and 2nd arches involute - hyoid artery becomes branch of 3rd
arch

• 4-5 weeks of fetal life - Stapedial artery arises from the hyoid artery near its
origin from the proximal ICA

• Then it extends cranially from the hyoid artery and passes through the
primordium of the stapes forming the obturator foramen of the stapes

• After entering cranial cavity it divides into 2 divisions

1) Upper division ( Supraorbital division ) - Middle meningeal artery

2) Lower division ( Maxillomandibular division )

i) Infraorbital branch - Infraorbital artery

ii) Mandibular branch - Inferior alveolar artery

• Lower division leaves cranial cavity through foramen spinosum and

anastomoses with ventral pharyngeal arteries ( Future ECA )

• During 10th week - Stapedial artery degenerates

• Middle meningeal artery, inferior alveolar artery and Infraorbital

artery then become branch of ECA

• Hyoid artery decreases in size and persists as caroticotympanic

branch of ICA

Persistent Stapedial artery arises from the petrous ICA

|
Enters hypotympanum
|
Traverses Jacobsen’s canal
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stapes obturator foramen
|
Enters Fallopian canal close to the cochleariform process
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Exits the canal at the geniculate ganglion
|
Enters middle cranial fossa

• Foramen spinosum will be usually absent

• Mostly Asymptomatic

• Pulsatile tinnitus

• Presents as vascular mass within middle ear

CONGENITAL PERIPLYMPH FISTULA

• Abnormal communication between the perilymphatic space of the

inner ear and the middle ear space

• Perilymphatic uid pass into the middle ear

• Due to - i) Micro ssures around oval and round window

ii) Labyrinthine or IAM dysplasia

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 Clinical features :
• Sensorineural hearing loss - uctuating, progressive or sudden
onset

• Tinnitus

• Vertigo

• Aural fullness

• Recurrent meningitis

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• No reliable Pre operative test for this condition

• Weber proposed intra operative diagnosis - based on identi cation

of clear uid which re-accumulates with Valsalva or Trendelenburg
manoeuvre

• Management - Placing Temporalis fascia or tragal perichondrium

over oval and round window
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 HIGH JUGULAR BULB
• If it reaches superiorly to the level of the oor of the external auditory canal

• Often covered by thin bone or is dehiscent

• Usually asymptomatic

• Associated with hearing loss and pulsatile tinnitus

Mostly conductive hearing loss

If it impinges on cochlear or vestibular aqueduct - sensorineural

hearing loss

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Management :

• Asymptomatic - No intervention

• Symptomatic - bulb is displaced inferior to the round window by a

cartilage or cortical bone graft.

Thank you