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CONGENITAL AURAL ATRESIA Dennis R. Maceri, M.D., F.A.C.S. Associate Professor Otolaryngology- Head and Neck Surgery Keck School of Medicine.

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Presentation on theme: "CONGENITAL AURAL ATRESIA Dennis R. Maceri, M.D., F.A.C.S. Associate Professor Otolaryngology- Head and Neck Surgery Keck School of Medicine."— Presentation transcript:

1 CONGENITAL AURAL ATRESIA Dennis R. Maceri, M.D., F.A.C.S. Associate Professor Otolaryngology- Head and Neck Surgery Keck School of Medicine

2 “ The better developed the auricle the better the middle ear” K ountakis, 1995

3 Atresia Epidemiology 1 in 10,000 to 15,000 births 1 in 10,000 to 15,000 births Up to 50% of the time associated with some craniofacial syndrome Up to 50% of the time associated with some craniofacial syndrome Unilateral : Bilateral, about 3:1 Unilateral : Bilateral, about 3:1 30% are bilateral 30% are bilateral Atresia : Microtia, 7:1 Atresia : Microtia, 7:1 Slightly more common on the right Slightly more common on the right Male : Female, 2:1 Male : Female, 2:1

4 Atresia Aural atresia associated with 22/71 known craniofacial syndromes –Treacher Collins (Mandibulofacial Dysostosis) –Nager Syndrome (Acrofacial Dysostosis) –Cruzoun’s Craniofacial Dysostosis –Goldenhar’s Syndrome –Hemifacial Microsomia

5 Aresia Surgery First attempt to surgically correct aural atresia was by Thomson in 1843 First attempt to surgically correct aural atresia was by Thomson in 1843 Shambaugh, 1967, recommended unilateral surgery only if the cochlear reserve allowed hearing to improve by 25dB Shambaugh, 1967, recommended unilateral surgery only if the cochlear reserve allowed hearing to improve by 25dB Jahrsdoerfer, 1978, first large series using the anterior approach Jahrsdoerfer, 1978, first large series using the anterior approach

6 Embryology 8 Week Stage

7 Embryology

8 Embryology The external ear forms earlier than the middle ear The external ear forms earlier than the middle ear Microtia and Atresia imply an arrest in development at any stage Microtia and Atresia imply an arrest in development at any stage The earlier the arrest, the less developed the middle ear The earlier the arrest, the less developed the middle ear

9 Embryology Inner ear and labyrinth develop at 3 weeks from an invagination of ectoderm called the ottic placode Inner ear and labyrinth develop at 3 weeks from an invagination of ectoderm called the ottic placode Middle ear space, mastoid, eustachian tube and part of the drum develop from the first pharyngeal pouch at 30 weeks Middle ear space, mastoid, eustachian tube and part of the drum develop from the first pharyngeal pouch at 30 weeks Mastoid air cells continue to develop up to age 5 Mastoid air cells continue to develop up to age 5

10 Embryology First Branchial Arch-(Meckel’s) First Branchial Arch-(Meckel’s) –Malleus head, body, tensor tympani (V 3 ) –Short process of the Incus Second Arch (Hyoid) (Reicherts) Second Arch (Hyoid) (Reicherts) –Manubrium of the malleus –Stapes superstructure –VIIth nerve Foot plate from the otic capsule and 2 nd arch Foot plate from the otic capsule and 2 nd arch

11 Embryology External ear (ectoderm) is derived from the 1 st branchial cleft. A solid core of epithelium migrates inward toward the 1 st branchial pouch (endoderm) External ear (ectoderm) is derived from the 1 st branchial cleft. A solid core of epithelium migrates inward toward the 1 st branchial pouch (endoderm)

12 Embryology Solid core of tissue migrates inward External canal re-canalizes by the 6 th month

13 Embryology The external canal starts to hollow out (recanalize) during the 6 th month and progresses from medial to lateral The external canal starts to hollow out (recanalize) during the 6 th month and progresses from medial to lateral Arrest of recanalization process leads to the various deformities seen in atresia Arrest of recanalization process leads to the various deformities seen in atresia Formed tympanic membrane and bony canal with a stenotic membranous canal leads to canal cholesteatoma Formed tympanic membrane and bony canal with a stenotic membranous canal leads to canal cholesteatoma

14 Embryology Arrested growth of the ossicles leaves them in various stages of formation Arrested growth of the ossicles leaves them in various stages of formation –Stapes often malformed but mobile –The incus and malleus are fused –Altered course of facial nerve

15 Atresia Embryology – 7 th Month Canalization complete Canalization complete Mastoid separation from mandible Mastoid separation from mandible Normal posterior-inferior growth No mastoid growth NormalAtresia

16 Atresia Embryology Mastoid growth affects the facial nerve position Mastoid growth affects the facial nerve position Normal 120 o Curve Acute Curve in Atresia

17 Atresia

18 Classification of Deformities After Colman-3 types Minor Aplasia-incomplete recanalization Minor Aplasia-incomplete recanalization Moderate Aplasia- the tympanic bone has developed but has failed to recanalize Moderate Aplasia- the tympanic bone has developed but has failed to recanalize Severe Aplasia-complete absence of the external canal Severe Aplasia-complete absence of the external canal

19 Atresia Classification-Severe Aplasia, no tympanic bone

20 Atresia Complete Atresia

21 Atresia

22 Atresia Moderate Aplasia The most common, solid mass of compact bone that has failed to recanalize The most common, solid mass of compact bone that has failed to recanalize

23 Atresia Minor Aplasia-partial recanalization Middle space constricted, often with severe ossicular abnormalities Middle space constricted, often with severe ossicular abnormalities

24 Microtia Types of Microtia (Based on Marx 1926) Class IMild deformity, auricle smaller, all parts of ear identifiable Class II½ to 2/3 normal size with partially retained structures Class III Severely malformed, peanut shaped

25 Radiological Evaluation High resolution CT in coronal and axial planes High resolution CT in coronal and axial planes Axial to delineate malleus, incus and I-S joint and round window Axial to delineate malleus, incus and I-S joint and round window Coronal to delineate stapes, oval window and vestibule Coronal to delineate stapes, oval window and vestibule 3-D CT of little help 3-D CT of little help Timing indicated by timing of surgery Timing indicated by timing of surgery

26 Grading System Based on high resolution CT scan Based on high resolution CT scan A score of 5 or less denotes a poor candidate A score of 5 or less denotes a poor candidate Microtia indicates an arrest in development and abnormalities of the middle ear Microtia indicates an arrest in development and abnormalities of the middle ear VariablePoints Stapes Present 2 Oval Window Open 1 Facial Nerve 1 Middle Ear Space 1 Mastoid Pneumatization 1 Malleus/Incus Complex 1 Incudostapedial Joint 1 Round Window 1 External Ear Appearance 1 Total Points Total Points10 Kountakis, Helidonis and Jahrsdoerfer Arch. Oto 1995

27 Atresia Grade of Microtia as an Indicator of ME Development Microtia Grade Radiological Score I8.5 II7.2 III5.9 Kountakis Arch Otolaryngol 1995

28 Radiological Evaluation Good middle ear and mastoid aeration Good middle ear and mastoid aeration Prominent ossicular mass with incus and malleus fused Prominent ossicular mass with incus and malleus fused Open oval window Open oval window Score of 8 Score of 8

29 Radiological Evaluation Score of 8 Score of 8 2-stapes & OW 2-I-S joint, Incus 1-Aerated ME space 1-Bony cover on VII 1-Aerated mastoid 1-Pinna

30 Radiological Evaluation Poor middle ear and mastoid development Poor middle ear and mastoid development No definable ossicular mass No definable ossicular mass Closed oval window Closed oval window Not a good candidate Not a good candidate A score of 3 A score of 3

31 Microtia

32 Microtia Repair

33 Audiological Evaluation dB conductive loss dB conductive loss Usually normal sensorineural function Usually normal sensorineural function Unilateral- behavioral audiometry Unilateral- behavioral audiometry ABR for infants ABR for infants Bilateral atresia presents a masking dilemma Bilateral atresia presents a masking dilemma Bone conduction ABR can help resolve the masking problem (Wave I on stimulated ear) Bone conduction ABR can help resolve the masking problem (Wave I on stimulated ear)

34 Surgical Considerations Most consider repair in bilateral atresia Most consider repair in bilateral atresia Many are reluctant to operate on unilateral cases Many are reluctant to operate on unilateral cases –Not simply the hearing loss –Expectations of hearing recovery –Lifetime care of mastoid cavity –Potential risks to facial nerve and labyrinth –55-65% achieve 25 dB speech-hearing level

35 Surgical Considerations Most surgeons choose the anterior approach to avoid the mastoid cavity Most surgeons choose the anterior approach to avoid the mastoid cavity 40% of patients with unilateral atresia are not surgical candidates such as those with severe aplasia as in Treacher Collins syndrome 40% of patients with unilateral atresia are not surgical candidates such as those with severe aplasia as in Treacher Collins syndrome Bilateral atresia- best ear by CT done as child approaches school age Bilateral atresia- best ear by CT done as child approaches school age

36 Surgical Considerations Timing of surgery Usually performed after age 6 or 7 years Usually performed after age 6 or 7 years This allows for microtia repair to be done first (If Medpor used,canalplasty done first) This allows for microtia repair to be done first (If Medpor used,canalplasty done first) Canal cholesteatoma in the stenotic ear usually develops in canals less than 2mm in diameter. If ear unfavorable, canalplasty alone is offered Canal cholesteatoma in the stenotic ear usually develops in canals less than 2mm in diameter. If ear unfavorable, canalplasty alone is offered

37 Canal Cholesteatoma Usually in ears with minor aplasia Usually in ears with minor aplasia Canal <4mm, 50% incidence Canal <4mm, 50% incidence

38 Surgical Technique Minor aplasia- canal widening and middle ear ossicular work with tympanoplasty Minor aplasia- canal widening and middle ear ossicular work with tympanoplasty Moderate Aplasia Moderate Aplasia –Mastoid or posterior approach –Anterior approach

39 Surgical Technique Anterior Approach Middle ear approached through the atretic bone with a limited mastoid opening Middle ear approached through the atretic bone with a limited mastoid opening

40 Surgical Technique The posterior wall of the glenoid fossa becomes the anterior wall of the new ear canal The posterior wall of the glenoid fossa becomes the anterior wall of the new ear canal The epitympanum is the first part of the middle ear encountered The epitympanum is the first part of the middle ear encountered Fused ossicles identified Fused ossicles identified

41 Surgical Technique Atretic bone removed at times with a curette Atretic bone removed at times with a curette Globular mass separated from the stapes to avoid cochlear trauma Globular mass separated from the stapes to avoid cochlear trauma Course of facial nerve determined Course of facial nerve determined Ossiculoplasty performed Ossiculoplasty performed Tympanic membrane grafted Tympanic membrane grafted Meatoplasty Meatoplasty Split thickness skin graft ( inches) lines the canal Split thickness skin graft ( inches) lines the canal

42 Surgical Technique The Meatoplasty must be aligned with the newly created bony canal The Meatoplasty must be aligned with the newly created bony canal

43 Surgical Technique 4cm X 6cm split thickness skin graft 4cm X 6cm split thickness skin graft The graft is positioned in the canal and sewn to the meatal margin The graft is positioned in the canal and sewn to the meatal margin Graft stabilized with Merocel wicks and hydrated with ear drops Graft stabilized with Merocel wicks and hydrated with ear drops

44 Hearing Results Post-op hearing level of 30 dB or better In 50-75% of patients with moderate or severe aplasia Post-op hearing level of 30 dB or better In 50-75% of patients with moderate or severe aplasia 20 dB or better in 15-20% 20 dB or better in 15-20% –Bellucci 20 dB in 2 years –Schuknect similar results at 1.3 years –De La Cruz 56 patients 20 dB at 6 mo. –Lambert early dB, 46% >1 yr.

45 Alternatives to Surgery Bone anchored hearing aid (BAHA) Bone anchored hearing aid (BAHA)

46 BAHA Hearing results better than with BC aids

47 Surgical Complications Persistent or recurrent conductive hearing loss Persistent or recurrent conductive hearing loss –Lateralization of graft –Scar tissue SNHL SNHL VIIth Nerve injury VIIth Nerve injury 30 % revision rate 30 % revision rate –Re-stenosis –Graft migration –Inadequate hearing Chronic cavity infection Chronic cavity infection

48 Summary Choose your patients carefully Choose your patients carefully Realize your potential v. patient expectations Realize your potential v. patient expectations Appropriate radiographic studies Appropriate radiographic studies Accurate audiological analysis Accurate audiological analysis Facial nerve monitoring Facial nerve monitoring Know when NOT to operate Know when NOT to operate


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