aural atresia regional conference

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CONGENITAL AURAL ATRESIADr Zeeshan AliResident in Department of ENT Head and Neck SurgeryCMH Rawalpindi

Respected teachers and my fellow colleagues AOA.I dr zeeshan resident in ENT head and neck surgery department CMH rwp will be presenting two cases of congenital aural atresia with different surgical scenarios.2

Case 1

Case detail

Displayed with informed consent of patient

Case no 1 is A 6 year old boy brought to our institute by his consanguineous parents for scheduled ear surgery for bilateral hearing loss since birth. on examination altman grade 3 aural atresia with marx grade 3 microtia were seen bilaterally. 4

BERA

BERA revealed the boy having 60 and 70 db hearing threshold in right and left ear respectively. free field hearing test reveal 60 and 80 db in right and left ear respectively.the boy had been wearing soft band bone conducting hearing aid since birth and studying in a special education school .speech therapist suggest adequate receptive and expressive language with good attention span.5

CT Scan

Ct san revealed partially canalised right external auditory canal with aerated middle ear and intact ossicles where as left middle ear showed abnormal configuration with no definitive ossicles.6

Surgery


The boy was graded 9 on Jahrsdoerfer system and planned for surgery of the right ear. Associated risks of total deafness and facial nerve paralysis were explained to the parents.7

Surgery


lateral face and the skin graft donor site were preparedand draped. A 1/2-inch swath of hair is shaved around theexternal ear

8

Surgery


An endaural incision is made and Soft tissue was elevated off the mastoid process in a posterior to anterior direction.

9

Surgery


Temporalis facia garft was harvested.10

Surgery


The temporal root of the zygomatic arch and the glenoid fossa were identified and the cribriform area of the mastoid process was used as a landmark for drilling

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Surgery


Drilling was done by staying superior and anteriorly.Care was taken to hug the tegmen and the glenoid fossa 12

Surgery


Dense atretic bone was found and followed medially. 13

Surgery


fused incus-malleus complex was encountered At a depth of approx 1.5 cm

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Surgery


body of the incus was identified and confirmed by gentlePalpation.15

Surgery


fused malleus head and incus body were found to be mobile on palpation. diamond bur wasUsed to thin the atretic plate to eggshell thickness and gently picked away in small pieces.16

Surgery


facial nerve was seen having a short vertical segment while making a sharp curve anteriorly. Normal mobile stapes suprastructure was confirmed by gentle palpation..17

Surgery


peripheral bone was then drilled to gain room for theplacement of the fascial graft.18

Surgery


fascia graft was placed directly on the ossicularMass so as it was Approximately in center of the new tympanic membrane.

19

Surgery


a U-shaped pedicle flap hinged at the tragal remanent was then positionedinto the new ear canal and sutured to a cuff of periosteum.20

Surgery


SurgeryDisplayed with informed consent of patient

Following proper placement of the meatus the external ear was stabilized with subcutaneous sutures and ear canal was packed with spongiston wicks and BIPP packs.mastoid dressing was applied.22

POSTOPERATIVE CARE


The patient was discharged on the 2nd postop day and sutures were removed on 1 postop week.here the patient is seen in the 2nd week showing granulating wound with healthy pedicle skin.silver nitrate was applied to the granulations and EAC was packed with antibiotic impregnated gelfoam. the boy is scheduled to have his 1st audiogram in 3rd week postoperatively. 23

Case 2

Case detail


Case no 2 is An 18 year-old female patient referred to our institute with painful post-auricular discharging sinus on her left side associated with swelling and redness around it. examination revealed that the patient had aural stenosis dating since birth and grade II microtia with acute mastoiditis that led to sinus formation on the left side,.. the right ear was completely normal. 25

Audiometry

Pure tone audiogram showed conductive hearing loss with an air-bone gap of 50 dB on the left side and near normal hearing of right ear26

CT scan

Ct scan show soft tissue density in the left external auditory meatus with intact ossicles.27

X ray mastoid

X ray mastoid showed a sclerosed left mastoid with an opacity in the attic region while a well pneumatised right mastoid.28

Surgical steps

Patient was prepped for surgery after explaining risks of total deafness and facial nerve paralysis.29

Surgery


lateral face and the skin graft donor site were preparedand draped. A 1/2-inch swath of hair is shaved around theexternal ear30

Surgery


modified pre-auricular incision was made and unhealthy skin around the sinus excised.31


Temporalis fascia graft was harvested and dried on petri dish.32

Surgery


Cortical mastoidectomy was carried out to reveal an automastoidectomy cavity extending into the attic area.

33

Surgery


Mastoid cavity was Filled with a large cholesteatoma sac 34

Surgery


Whole of the cholesteatoma sac was carefully removed.35

Surgery


here is the magnified view of the isolated Cholesteama sac. 36

Surgery


the middle ear was entered via an atticoantrostomy approach And atretic plate found which was carefully drilled away. The ossicular mass in the epitympanum was meticulously dissected free of the atretic plate and left intact after ensuring its mobility.37


The horizontal facial nerve was seen medial to the ossicular mass. 38

Surgery


then temporalis fascia graft was used to cover the ossicular mass and line the mastoid cavity.39


Reconstruction of the external auditory canal was done through removal of the bone lateral to the middle ear space with meatoplasty of the cartilaginous portion40

Post operative


No intra-operative or post-operative complications were seen.41

Post operative


pack was removed on the 10th post-operative day.granulations were seen in the mastod cavity and repacking was done with BIPP. Pt is on periodic visits for the regular cleaning of the mastoid cavity.

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CASE DISCUSSION

Epidemiology1 in 10,000 to 15,000 births10% associated with syndromeUnilateral vs Bilateral30% are bilateralAtresia : Microtia, 7:1

Congenital aural atresia occurs once in every 10,000births. Unilateral atresia is seven times more commonthan bilateral atresia. Aural atresia is associated with arecognizable syndrome in about 10% of cases. In about5% of nonsyndromic cases, the birth defect is inherited.44

EmbryologyTreacher Collins syndromebrachio-otorenal syndromeStickler syndrome

Syndromic conditions associatedwith congenital aural atresia include: Treacher Collins; brachio-otorenal; Sticklers;45

EmbryologyTreacher Collins syndromebrachio-otorenal syndromeStickler syndromeCrouzon syndromeNoonan syndromeFoetal alcohol syndrome

Crouzons; Noonan syndrome; foetal alcohol syndrome;

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EmbryologyTreacher Collins syndromebrachio-otorenal syndromeStickler syndromeCrouzon syndromeNoonan syndromeFoetal alcohol syndromeGoldenhars SyndromeHemifacial Microsomia

CHARGE syndrome

coloboma, heart choanal atresia,developmental and growth retardation, genito-urinarymalformations and ear anomalies (CHARGE)47

Embryology

Inner ear, middle ear, and external eardevelop independently

The inner ear, middle ear, and external eardevelop independently and in such a way that deformityof one does not presuppose deformity of another.Most frequently, abnormalities of the outer and middleear are encountered in combination with a normalinner ear.48

Embryology The better developed the external ear is, the better developed is the middle ear

Some of the literature supports the notion that microtia grade indicate the status of middle ear development. 49

Development of the Auricle

Growth of mesenchymal tissue from the first and second branchial arches forms six hillocks around the primitive meatus that fuse to form the auricle . 50

Embryology (Marx)

Microtia is a result of first and second branchial archAnomalies and is classified by Marx into 4 grades.

51

Embryology

During the second month The first branchial pouch grows outward to formthe middle ear cleft while a solid core of epithelium migrates inward fromthe rudimentary pinna toward the first branchial pouch.52

EmbryologyFirst Branchial Arch-(Meckels)MalleusIncus body Second Arch (Hyoid) (Reicherts)Long process of incusStapes suprastructure Foot plate from the otic capsule and 2nd arch

Meckels cartilage form the malleus and incus body. Reicherts cartilage forms the long process of incus and the stapes superstructure while The footplate has a dual origin from the second arch and the otic capsule.53

Types of aural atresia

Congenital aural atresia can range in severity from athin membranous canal atresia to complete lack of tympanicbone, depending on the time of arrest of intrauterinedevelopment54

Altman classificationGroup 1Minor Aplasia-incomplete recanalization

Of historical significance is a classification by Altmann. In thissystem, atresias are categorized into three groups, In Group 1 Some part of the EAC is hypoplastic. 55

Altman classificationGroup 2Moderate Aplasia- tympanic bone has developed but has failed to recanalize

In Group 2 The EAC is completely absent, thetympanic cavity is small, and its content is deformed,and the atresia plate is partially or completelyosseous56

Altman classificationGroup 3Severe Aplasia-complete absence of the external canal

In Group 3 The EAC is absent, and the tympaniccavity is markedly hypoplastic or missing.57

De la Cruz ClassificationHRCT based

Mastoid PneumatizationInner ear normalityFacial nerveFootplate

The De la Cruz classification includes surgical feasibilityguidelines using HRCT, taking into consideration mastoidpneumatization, inner ear normality, facial nerve andfootplate relationship. 58

De la Cruz ClassificationMinorMalformations

Normal mastoid pneumatizationNormal oval window/footplateGood facial nervefootplate relationshipNormal inner ear

The malformations are divided into minor and major malformations.59

De la Cruz ClassificationMajorMalformations

Poor pneumatizationAbnormal or absent oval window/footplateAbnormal course of facial nerveAbnormalities of inner ear

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Grading System (Jahrsdoerfer)VariablePointsStapes Present2Oval Window Open1Facial Nerve1Middle Ear Space1Mastoid Pneumatization1Malleus/Incus Complex1Incudostapedial Joint1Round Window1External Ear Appearance1 Total Points10

Jahrsdoerfer and colleagues developed a widely usedpoint grading system to guide surgeons in preoperativeassessment of the best candidates for hearing improvement.Point allocation is based primarily on the findings on HRCT.61

Prognostic Rating Scale10 Excellent9 Very good8 Good7 Fair6 Marginal5 Poorde Alarcon A, Jahrsdoerfer RA, Kesser BW. Congenital absence of the oval window: diagnosis, surgery, and audiometric outcomes. Otol Neurotol. 2008;29:2328.

Jahrsdoerfer proposed that the best results areachieved with a score of 8 or better. 62

Schuknechts classification

Type AType BType CType D

Schuknechts system is based on a combination of clinical andsurgical observations. Type A atresia is limitedto the fibrocartilaginous.63



type B there is narrowing of the fibrocartilaginous and bony EAC. 64



Type C is a totally atretic ear canal, but a well-pneumatizedtympanic cavity.65



Type D is a hypopneumatic atresia which is common in dysplasias suchas Treacher Collins syndrome.66

Chiossones classificationType I - the fossa in normal positionType II - moderately displaced

Chiossones classification is based primarily on thelocation of the glenoid fossa. Patients with types Iand II are ideal surgical candidates. 67

Chiossones classificationType I - the fossa in normal positionType II - moderately displacedType III - fossa overlaps the middle ear

Type III cases have a tendency toward graft lateralization. 68

Chiossones classificationType I - the fossa in normal positionType II - moderately displacedType III - fossa overlaps the middle earType IV - lack of mastoid pneumatization

Patients with type IV are not surgical candidates69

Initial evaluation

Where one congenital abnormality is found, others must be sought.After physical examination,evaluation of auditory function should be performed using auditory brainstem response audiometry within the first few days of life.initial evaluation of an older individual is with Audiometry and high-resolution CT scan70

Management

In bilateral cases, a bone-conduction hearing aidshould be applied as soon as possible, ideally in the thirdor fourth week of life.A child with aural atresia and associated cephalicabnormalities surgical correction haspoor results so BAHA is used in such cases.71

Management

Prompt and careful counseling of the parents of achild is necessary to alleviate concerns.The child should be enrolled in special education at an early age to maximize speechand language acquisition, in preparation for mainstreaming at preschool age.72

Prerequisite for surgeryCT scan

Audiometry

There are two requirements for planning surgery incongenital aural atresia: radiographic three-dimensionalevaluation of the temporal bone and audiometric evidenceof cochlear function73

Urgent surgeryCongenital cholesteatomaDraining postoperative atretic earAcute facial palsy

Other conditions mandatingprompt surgical intervention are congenitalcholesteatoma, a draining postoperative atretic ear,and acute facial palsy. The CT scan should always bereviewed for cholesteatoma,which necessitates surgery at any age74

Timing of surgery6 year recommendedExostosis formationLess patient cooperationSufficient costal cartilageMastoid well pneumatised

In bilateral or unilateral atresia, auricular reconstructionand atresiaplasty are recommended at 6 years ofage. Before this age, there may be a tendency to formexostosis-like bony growth and there is less patient cooperation. the costalcartilage has developed sufficiently to allow for reconstructionand the mastoid has become pneumatized. 75

ComplicationsLateralization of TM 3.4% Meatal Stenosis 3.8%High-tone SNHL 7.5%Facial nerve palsy 1%

Complications of surgery include Lateralization of the tympanic membrane ,stenosisof the meatus ,high-tone SNHL andfacial nerve palsy.76

Unilateral atretic earDecreased incidence of major complicationsA rewarding operation Offer a clean & dry ear Binaural hearingMinor unilateral atresiaIn early childhood

Elective surgery in unilateral atresia is a controversial topic .now a day it is rewarding for the surgeonand the patient .offering benefits of a clean, dry ear with binauralHearing. it is done in patient with minor unilateral atresia it maybe offered in childhood with the parents consent.

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Datan= 21Jan 2008-Dec 2014

A total of 21 patients of both sexes with congenital aural atresia were operated from January 2008 to Dec 2014 with a male to female ratio of 2.5:178

DataJan 2008-Dec 2014

26 patients had unilateral atresia while 16 patients had bilateral aural atresia with a ratio of 1.6:1.79

DataJan 2008-Dec 2014

Type I meatal atresia was present in 36 % patients, while 50% patients had type II meatal atresia and 14% patients had type III meatal atresia.

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DataJan 2008-Dec 2014Nishizaki K, Masuda Y, Karita K.Surgical management and its post-operative complications in congenital aural atresia.Acta Otolaryngol Suppl.1999;540:42-4.Oliver ER, Hughley BB, Shonka DC, Kesser BW.Revision aural atresia surgery: indications and outcomes. Otol Neurotol. 2011 Feb;32(2):252-8. doi: 10.1097/MAO.0b013e3182015f27

RestenosisOtorrhea

Facial nerve paralysisSNHL

Our data was compared with regional and international study which show comparable results. our results showed low rate of restenosis in comparison to the regional and international study.

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