CONGENITAL AURAL ATRESIA27th Al d i I t ti l C bi d ORL C27th Alexandria International Combined ORL Congress

Alexandria, Egypt April 8, 2009

Antonio De la Cruz MD

HOUSE EAR CLINIC

Antonio De la Cruz, MDHouse Ear Institute Los Angeles, California

Antonio De la Cruz, MD27th Alexandria International Combined ORL Congress

HOUSE EAR CLINIC

g

Alexandria, Egypt April 8, 2009

HOUSE EAR CLINIC

EpidemiologyEpidemiology1 in 10.000- 20.000 births

Unilateral to bilateral 3:1

d h bl d10% associated with recognizable synd.

I h it d i 5% f d i Inherited in 5% of non-syndromic cases

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Congenital Atresia EAC

18q 22.3-18q23 Region

Long arm Deletion

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C it l A l At iCongenital Aural Atresia239 Patients - 302 Ears239 Patients - 302 Ears

141 Males

98 Females

108 Right108 Right

61 Left61 Left

70 Bilateral

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EmbryologyEmbryology

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EXTERNAL EAREXTERNAL EAR

• The external ear develops from the • The external ear develops from the

first and second branchial arches and first and second branchial arches and

the interposed first branchial cleft.p

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AuricleAuricle• 4 weeks → condensation from 1st and • 4 weeks → condensation from 1st and

2nd arches form the hillocks of Hiss

• Hillocks fuse to form the auricle

• Adult configuration reached by 20 weeks

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EXTERNAL EAR CANAL

• At 2 months, the 1st branchial cleft migrates medially to abut the lateral aspect of the 1st branchial pouch.Th 2 t t th t d b

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• These 2 structures are then separated by mesenchyme.

EAC• A solid core of epithelium migrates • A solid core of epithelium migrates

from the rudimentary auricle to the from the rudimentary auricle to the

lateral end of the first pharyngeal

pouch and forms the tympanic plate

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First pharyngeal pouch

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First pharyngeal pouch

EAC

• Recanalization starts at the 6th month

P d i di l t l t l di ti• Proceeds in a medial to lateral direction

• Arrest at any stage leads to different • Arrest at any stage leads to different

degrees of atresiag

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Tympanic Ring

• The tympanic ring

develops from four foci of

ossifications.

• The mastoid grows in a

posterior inferior

direction, carrying with it d ect o , ca y g t t

the middle ear structures

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and the facial nerve

Congenital Aural Atresia Surgeryg g yFacial nervre variations

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Middle Ear• Ossicles derive from the Meckel’s and • Ossicles derive from the Meckel s and

Reichert’s cartilagesThi t t t th d th • This process starts at the second month and matures by the fourth month

f h ddl d f h• Lining of the middle ear derives from the 1st pharyngeal pouch, a process which is

l t b th d f th thcomplete by the end of seventh month

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INCUSINCUS

STAPES

MALLEUSMALLEUS

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STAPEDIAL ARTERYSTAPEDIAL ARTERY

•Artery of the 2nd branchial arch•Appears during the 4th-5th weekg•It forms the obturator foramen of the stapes •It atrophies during the 3rd month p g•Its dorsal branch forms the middle meningeal a. •Its persistence->absence of the foramen spinosump p

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i l iAtresia Evaluation AudiologyAudiology

• Cochlear function: Pure Tone audiometry• Complete atresia causes 50 to 60 dB CHLComplete atresia causes 50 to 60 dB CHL• Bone line usually normal• Bilateral cases > masking dilemma• Bilateral cases -> masking dilemma

–Wave I of ABR can provide ear specific i f tiinformation

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HOUSE EAR CLINICDr. Jay Hall

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IMAGINGIMAGING

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AURAL ATRESIAAURAL ATRESIAIMAGINGIMAGING

• Pneumatization

• Inner Ear

• Facial Nerve – Foot PlateHOUSE EAR CLINIC

ARE AURAL ATRESIA CLASSIFICATIONSCLASSIFICATIONS

USEFULL ?USEFULL ?

ALTMANJAHRSDOERFEROOMBREDANNE

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Jahrsdoerfer ScaleJahrsdoerfer ScaleStapes present 2p p

External ear appearance 1

Middle ear space 1

Mastoid pneumatization 1Mastoid pneumatization

Malleus-incus complex 1

Incus stapes connected 1

Facial nerve course 1Facial nerve course

Oval window open 1

HOUSE EAR CLINICRound window open 1

ATRESIAClassifications

Th l ti hi b t h i l l There was no relationship between hearing levels,

the degree of middle ear aeration, facial nerve the degree of middle ear aeration, facial nerve

abnormalities or severity of the microtia

IshimotoS et al, Laryngoscope 2007 Mar; 117(3):461-5

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ATRESIA CLASSIFICATIONATRESIA CLASSIFICATION

MiMinor• Normal pneumatization

• Normal inner ear

• Normal FP/oval window Normal FP/oval window

• Normal facial nerveHOUSE EAR CLINIC

• Normal facial nerve

ATRESIA CLASSIFICATIONATRESIA CLASSIFICATION

M jMajor• Poor pneumatization

• Inner ear malformation

• Absent oval window

• FP/Facial nerve abnormality

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INOPERABLEINOPERABLE(At i l t )(Atresiaplasty)

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BAHA Mixed and Conductive Hearing Loss

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SSUURRGGERY

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GOALSGOALS

• Improvement of hearing

•Long term patency

•Long term stability of hearingg y g

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CONGENITAL ATRESIA SURGERYCONGENITAL ATRESIA SURGERY

Pre-school age (6 yo)

• Post-op Patient Cooperation

• Audiometry (proper masking)

• Easier Radiological Evaluation

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ATRESIA - SURGERY

Indications• Enough Pneumatization

Indications• Enough Pneumatization

Bilateral Cases• Bilateral Cases

• Functioning Cochlea

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ATRESIA - SURGERY

Indications - Unilateral Cases

Cholesteatomas

Indications Unilateral Cases

Cholesteatomas

lfMinor Malformations

Adults, Children?HOUSE EAR CLINIC

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CONGENITAL ATRESIA

First Operation ? Why?

1 Auricular Reconstruction1- Auricular Reconstruction

2- Atresiaplasty / Tympanoplasty

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Auricular Reconstruction

Reconstructive Surgery

or

Prosthesis?Prosthesis?

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HOUSE EAR CLINICBurt Brent, MD

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MEDPOR®MEDPOR

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HOUSE EAR CLINICJohn Reinish, MD

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HOUSE EAR CLINICBurt Brent, MD

MICROTIA RECONSTRUCTIONSURGICAL COMPLICATIONS

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ATRESIAPLASTY

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T h iTechnique

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Congenital Aural Atresia Surgeryg g ySurgical Technique

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SILASTIC .020

Skin Graft

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Atresia - Primary Casesy

Cholesteatomas = 14%

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ATRESIAATRESIAHEARING LONG TERM FOLLOW UPHEARING- LONG TERM FOLLOW-UPPrimary Operations (N = 107)y p ( )

%Post-op AB Gap<10 dB

<20 dB

15

53<20 dB

<30 dB

53

73

High-tone SNHL 5

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COMPLICATIONSCOMPLICATIONS

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CONGENITAL ATRESIA SURGERYCONGENITAL ATRESIA SURGERY

C li tiComplicationsPrimary (n=87) Revision (n=29)

Soft tissue EAC stenosis 7 (8.0%) 1 (3%)( ) ( )

Bony EAC stenosis 6 (6.9%) 2 (7%)

Ossicular chain refixation 10 (11 5%) 2 (7%)Ossicular chain refixation 10 (11.5%) 2 (7%)

SNHL > 30 dB 9 (10.3%) 0

Dead Ears, Facial Paralysis 0 0

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C it l A l At i SCongenital Aural Atresia Surgery

Present Surgical Technique

Di ti f i l ith l

Present Surgical Technique

• Dissection of ossicles with laser

F i ft t b G lfi di• Fascia graft - tabs, Gelfim disc

STSG 0 008 i h• STSG - 0.008 inches,

020 Sil ti h t i EAC • .020 Silastic sheets in EAC

M l i kHOUSE EAR CLINIC

• Merocel wick

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Congenital AtresiaCongenital AtresiaConclusions

• Early evaluation ( ABR)

ConclusionsEarly evaluation ( ABR)

• Bone conduction hearing aids• C.T. Coronal & axial views• Surgery at age 6• Surgery at age 6• Split thickness skin graft• Prevent lateralization

P t Hi h T SNHL LHOUSE EAR CLINIC

• Prevent High Tone SNHL Laser

CONGENITAL AURAL ATRESIACONGENITAL AURAL ATRESIA27th Alexandria International Combined ORL Congress

Al d i E t A il 8 2009Alexandria, Egypt April 8, 2009

Antonio De la Cruz, MD

HOUSE EAR CLINIC

House Ear Institute Los Angeles, California