congenital aural atresia

CONGENITAL AURAL ATRESIA
27th Alexandria
Al
d i International
I t
ti
lC
Combined
bi d ORL C
Congress
Alexandria, Egypt
April 8, 2009
Antonio De la Cruz
Cruz, MD
House Ear Institute
Los Angeles, CaliforniaHOUSE
EAR CLINIC
Antonio De la Cruz, MD
g
27th Alexandria International Combined ORL Congress
Alexandria, Egypt April 8, 2009
HOUSE EAR CLINIC
HOUSE EAR CLINIC
Epidemiology
¾1 in 10.000- 20.000 births
¾Unilateral to bilateral 3:1
¾10% associated
d with
h recognizable
bl synd.
d
¾I h it d iin 5% off non-syndromic
¾Inherited
d
i cases
HOUSE EAR CLINIC
Congenital Atresia EAC
18q 22.3-18q23 Region
Long arm Deletion
HOUSE EAR CLINIC
C
Congenital
it l Aural
A
l Atresia
At
i
239 Patients - 302 Ears
141 Males
98 Females
108 Right
61 Left
70 Bilateral
HOUSE EAR CLINIC
Embryology
HOUSE EAR CLINIC
EXTERNAL EAR
• The external ear develops from the
first and second branchial arches and
the interposed
p
first branchial cleft.
HOUSE EAR CLINIC
Auricle
• 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
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
EXTERNAL EAR CANAL
• At 2 months, the 1st branchial cleft migrates
medially to abut the lateral aspect of the 1st
branchial pouch.
• These
Th
2 structures
t
t
are then
th
separated
t d by
b
mesenchyme.
HOUSE EAR CLINIC
EAC
• A solid core of epithelium migrates
from the rudimentary auricle to the
lateral end of the first pharyngeal
pouch and forms the tympanic plate
HOUSE EAR CLINIC
First pharyngeal pouch
HOUSE EAR CLINIC
EAC
• Recanalization starts at the 6th month
• Proceeds
P
d in
i a medial
di l tto llateral
t
l direction
di
ti
• Arrest at any stage leads to different
degrees
g
of atresia
HOUSE EAR CLINIC
Tympanic Ring
• The tympanic ring
develops from four foci of
ossifications.
• The mastoid grows in a
posterior inferior
direction,
d
ect o , ca
carrying
y g with
t itt
the middle ear structures
and the facial nerve
HOUSE EAR CLINIC
Congenital
g
Aural Atresia Surgery
g y
Facial nervre variations
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
Middle Ear
• Ossicles derive from the Meckel
Meckel’s
s and
Reichert’s cartilages
• This
Thi process starts
t t att the
th second
d month
th
and matures by the fourth month
• Lining off the
h middle
ddl ear derives
d
ffrom the
h
1st pharyngeal pouch, a process which is
complete
l t b
by th
the end
d off seventh
th month
th
HOUSE EAR CLINIC
HOUSE EAR CLINIC
INCUS
STAPES
MALLEUS
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
STAPEDIAL ARTERY
•Artery of the 2nd branchial arch
g the 4th-5th week
•Appears during
•It forms the obturator foramen of the stapes
p
during
g the 3rd month
•It atrophies
•Its dorsal branch forms the middle meningeal a.
persistence->absence of the foramen spinosum
p
•Its p
HOUSE EAR CLINIC
HOUSE EAR CLINIC
Atresia
i Evaluation
l
i
Audiology
•
•
•
•
Cochlear function: Pure Tone audiometry
Complete atresia causes 50 to 60 dB CHL
Bone line usually normal
Bilateral cases ->
> masking dilemma
– Wave I of ABR can provide ear specific
i f
information
ti
HOUSE EAR CLINIC
Dr. Jay Hall
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
IMAGING
HOUSE EAR CLINIC
AURAL ATRESIA
IMAGING
• Pneumatization
• Inner Ear
• Facial Nerve – Foot Plate
HOUSE EAR CLINIC
ARE AURAL ATRESIA
CLASSIFICATIONS
USEFULL ?
ALTMAN
JAHRSDOERFER
OMBREDANNE
HOUSE EAR CLINIC
HOUSE EAR CLINIC
Jahrsdoerfer Scale
Stapes
p p
present
2
External ear appearance
1
Middle ear space
1
Mastoid pneumatization
1
Malleus-incus complex
1
Incus stapes connected
1
Facial nerve course
1
Oval window open
1
Round window open
1
HOUSE EAR CLINIC
ATRESIA
Classifications
Th
There
was no relationship
l ti
hi between
b t
hearing
h
i
levels,
l
l
the degree of middle ear aeration, facial nerve
abnormalities or severity of the microtia
IshimotoS et al, Laryngoscope 2007 Mar; 117(3):461-5
HOUSE EAR CLINIC
ATRESIA CLASSIFICATION
Minor
Mi
• Normal pneumatization
• Normal inner ear
• Normal FP/oval window
• Normal facial nerve
HOUSE EAR CLINIC
ATRESIA CLASSIFICATION
M j
Major
• Poor pneumatization
• Inner ear malformation
• Absent oval window
• FP/Facial nerve abnormality
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
INOPERABLE
(At
(Atresiaplasty)
i l t )
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
BAHA
Mixed and Conductive Hearing Loss
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
S
U
R
G
E
R
Y
HOUSE EAR CLINIC
GOALS
• Improvement of hearing
• Long term patency
• Long
g term stability
y of hearing
g
HOUSE EAR CLINIC
CONGENITAL ATRESIA SURGERY
Pre-school age (6 yo)
• Post-op Patient Cooperation
• Audiometry (proper masking)
• Easier Radiological Evaluation
HOUSE EAR CLINIC
ATRESIA - SURGERY
Indications
• Enough Pneumatization
• Bilateral Cases
• Functioning Cochlea
HOUSE EAR CLINIC
ATRESIA - SURGERY
Indications - Unilateral Cases
¾ Cholesteatomas
¾ Minor Malformations
lf
¾ Adults, Children?
HOUSE EAR CLINIC
HOUSE EAR CLINIC
CONGENITAL ATRESIA
First Operation ? Why?
1 Auricular Reconstruction
12- Atresiaplasty / Tympanoplasty
HOUSE EAR CLINIC
Auricular Reconstruction
Reconstructive Surgery
or
Prosthesis?
HOUSE EAR CLINIC
EAR CLINIC
BurtHOUSE
Brent,
MD
HOUSE EAR CLINIC
MEDPOR
®
HOUSE EAR CLINIC
JohnHOUSE
Reinish,
MD
EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
Burt Brent,
MD
HOUSE EAR
CLINIC
MICROTIA RECONSTRUCTION
SURGICAL COMPLICATIONS
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
ATRESIAPLASTY
HOUSE EAR CLINIC
T h i
Technique
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
Congenital
g
Aural Atresia Surgery
g y
Surgical Technique
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
SILASTIC .020
Skin Graft
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
Atresia - Primary
y Cases
Cholesteatomas = 14%
HOUSE EAR CLINIC
ATRESIA
HEARING- LONG TERM FOLLOW-UP
HEARING
FOLLOW UP
Primary
y Operations
p
(
(N = 107)
)
Post-op AB Gap
<10 dB
%
15
<20 dB
53
<30 dB
73
High-tone SNHL
5
HOUSE EAR CLINIC
COMPLICATIONS
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
HOUSE EAR CLINIC
CONGENITAL ATRESIA SURGERY
C
Complications
li ti
Primary (n=87)
Revision (n=29)
Soft tissue EAC stenosis
7 ((8.0%))
1 ((3%))
Bony EAC stenosis
6 (6.9%)
2 (7%)
10 (11.5%)
(11 5%)
2 (7%)
Ossicular chain refixation
SNHL > 30 dB
9 (10.3%)
0
Dead Ears, Facial Paralysis
0
0
HOUSE EAR CLINIC
C
Congenital
it l A
Aurall At
Atresia
i Surgery
S
Present Surgical Technique
• Dissection
Di
ti
off ossicles
i l with
ith laser
l
• Fascia
F
i graft
ft - tabs,
t b G
Gelfim
lfi di
disc
• STSG - 0.008
0 008 inches,
i h
• .020
020 Silastic
Sil ti sheets
h t in
i EAC
• Merocel
M
l wick
i k
HOUSE EAR CLINIC
HOUSE EAR CLINIC
Congenital Atresia
Conclusions
• Early evaluation ( ABR)
• Bone conduction hearing aids
• C.T. Coronal & axial views
• Surgery at age 6
• Split thickness skin graft
• Prevent lateralization
• Prevent
P
t Hi
High
hT
Tone SNHL L
Laser
HOUSE EAR CLINIC
CONGENITAL AURAL ATRESIA
27th Alexandria International Combined ORL Congress
Al
Alexandria,
d i Egypt
E
t
April
A il 8
8, 2009
Antonio De la Cruz, MD
House Ear Institute
Los Angeles, California
HOUSE EAR CLINIC