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Cholesteatoma Cholesteatoma University of Texas Medical University of Texas Medical Branch Branch Department of Otolaryngology Department of Otolaryngology Garrett Hauptman MD Garrett Hauptman MD Tomoko Makishima MD Tomoko Makishima MD January 25, 2006 January 25, 2006
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CholesteatomaCholesteatomaUniversity of Texas Medical University of Texas Medical

BranchBranch

Department of OtolaryngologyDepartment of Otolaryngology

Garrett Hauptman MDGarrett Hauptman MD

Tomoko Makishima MDTomoko Makishima MD

January 25, 2006January 25, 2006

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OverviewOverview

DefinitionDefinition Classification and TheoriesClassification and Theories Anatomic ConsiderationsAnatomic Considerations Patient EvaluationPatient Evaluation ManagementManagement ComplicationsComplications

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DefinitionDefinition

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CholesteatomaCholesteatoma

Named by Johannes Mueller in 1838Named by Johannes Mueller in 1838 Erroneous belief that one of the primary Erroneous belief that one of the primary

components of the tumor was fatcomponents of the tumor was fat ““a pearly tumor of fat…among sheets of a pearly tumor of fat…among sheets of

polyhedral cells”polyhedral cells” More appropriate name has been More appropriate name has been

suggested to be keratoma to suggested to be keratoma to describe tumor compositiondescribe tumor composition

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CholesteatomaCholesteatoma Expanding lesion of the temporal bone composed ofExpanding lesion of the temporal bone composed of

Cystic content: desquamated keratin centerCystic content: desquamated keratin center Matrix: keratinizing stratified squamous epitheliumMatrix: keratinizing stratified squamous epithelium Perimatrix: granulation tissue that secretes Perimatrix: granulation tissue that secretes

multiple proteolytic enzymes capable of bone multiple proteolytic enzymes capable of bone destructiondestruction

May develop anywhere within pneumatized May develop anywhere within pneumatized portions of the temporal boneportions of the temporal bone

Most frequent locations:Middle ear space

Mastoid

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Classification Classification and Theoriesand Theories

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Cholesteatoma Cholesteatoma ClassificationClassification CongenitalCongenital

AcquiredAcquired PrimaryPrimary SecondarySecondary

Cholesteatoma Cholesteatoma FormationFormation

Multiple theories proposed regarding Multiple theories proposed regarding etiology behind tumor formationetiology behind tumor formation

Proposed mechanisms remain theoriesProposed mechanisms remain theories

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Congenital Congenital CholesteatomaCholesteatoma

Definition (Levenson, 1989)Definition (Levenson, 1989) White mass medial to normal tympanic White mass medial to normal tympanic

membranemembrane Normal pars flaccida and pars tensaNormal pars flaccida and pars tensa No prior history of otorrhea or No prior history of otorrhea or

perforationsperforations No prior otologic proceduresNo prior otologic procedures Prior bouts of otitis media were not Prior bouts of otitis media were not

grounds for exclusion as was the case in grounds for exclusion as was the case in original definition original definition

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Congenital Congenital CholesteatomaCholesteatoma

Pathogenesis theoriesPathogenesis theories Failure of involution of ectodermal Failure of involution of ectodermal

epithelial thickening that is present epithelial thickening that is present during fetal development in proximity during fetal development in proximity to geniculate ganglion to geniculate ganglion

Metaplasia of the middle ear mucosaMetaplasia of the middle ear mucosa

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Congenital Congenital CholesteatomaCholesteatoma

Anterosuperior Anterosuperior quadrantquadrant

> Posterosuperior quadrant

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Congenital Congenital CholesteatomaCholesteatoma

Large congenital cholesteatomaLarge congenital cholesteatoma

ossicular erosion

cholesteatoma

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Acquired CholesteatomasAcquired Cholesteatomas

Common factor: keratinizing Common factor: keratinizing squamous epithelium has grown squamous epithelium has grown beyond its normal limits beyond its normal limits

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Primary Acquired Primary Acquired CholesteatomasCholesteatomas

Ultimately form due to underlying Ultimately form due to underlying Eustachian tube dysfunction that causes Eustachian tube dysfunction that causes retraction of pars flaccidaretraction of pars flaccida Results in poor aeration of epitympanic space Results in poor aeration of epitympanic space

which draws pars flaccida medially on top of which draws pars flaccida medially on top of malleus neck, forming retraction pocketmalleus neck, forming retraction pocket

Normal migratory pattern of the tympanic Normal migratory pattern of the tympanic membrane epithelium altered by retraction membrane epithelium altered by retraction pocketpocket

Enhances potential accumulation of keratinEnhances potential accumulation of keratin

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Pars flaccida retractionPars tensa retraction

Primary Acquired Primary Acquired CholesteatomasCholesteatomas

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Secondary Acquired Secondary Acquired CholesteatomasCholesteatomas Implantation theoryImplantation theory

Squamous epithelium implanted in the middle ear as a Squamous epithelium implanted in the middle ear as a result of surgery, foreign body, blast injury, etc.result of surgery, foreign body, blast injury, etc.

Metaplasia theoryMetaplasia theory Desquamated epithelium is transformed to keratinized Desquamated epithelium is transformed to keratinized

stratified squamous epithelium secondary to chronic or stratified squamous epithelium secondary to chronic or recurrent otitis mediarecurrent otitis media

Epithelial invasion theory Epithelial invasion theory Squamous epithelium migrates along perforation edge Squamous epithelium migrates along perforation edge

medially along undersurface of tympanic membrane medially along undersurface of tympanic membrane destroying the columnar epitheliumdestroying the columnar epithelium

Papillary ingrowth theoryPapillary ingrowth theory Inflammatory reaction in Prussack’s space with an intact Inflammatory reaction in Prussack’s space with an intact

pars flaccida (likely secondary to poor ventilation) may pars flaccida (likely secondary to poor ventilation) may cause break in basal membrane allowing cord of epithelial cause break in basal membrane allowing cord of epithelial cells to start inward proliferationcells to start inward proliferation

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AnatomyAnatomy

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Middle Ear RegionsMiddle Ear Regions

Named based on position relative to Named based on position relative to superior and inferior aspect of superior and inferior aspect of external auditory canal (EAC)external auditory canal (EAC) Epitympanum: superior to superior limit Epitympanum: superior to superior limit

of EACof EAC Mesotympanum: bound superiorly by Mesotympanum: bound superiorly by

superior limit of EAC and inferiorly by superior limit of EAC and inferiorly by inferior limit of EACinferior limit of EAC

Hypotympanum: inferior to inferior limit Hypotympanum: inferior to inferior limit of EACof EAC

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Middle Ear RegionsMiddle Ear Regions

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EpitympanumEpitympanum

Lies above the level of the short Lies above the level of the short process of the malleusprocess of the malleus

Contents:Contents: Head of the malleusHead of the malleus Body of the incusBody of the incus Associated ligaments and mucosal foldsAssociated ligaments and mucosal folds

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MesotympanumMesotympanum Contents: Contents:

StapesStapes Long process of the incusLong process of the incus Handle of the malleusHandle of the malleus Oval and round windowsOval and round windows

Eustachian tube exits from the anterior aspectEustachian tube exits from the anterior aspect Two recesses extend posteriorly that are often not Two recesses extend posteriorly that are often not

visible directlyvisible directly Facial recessFacial recess

Lateral to facial nerveLateral to facial nerve Bounded by the fossa incudis superiorly Bounded by the fossa incudis superiorly Bounded by the chorda tympani nerve laterallyBounded by the chorda tympani nerve laterally

Sinus tympaniSinus tympani Lies between the facial nerve and the medial wall of the Lies between the facial nerve and the medial wall of the

mesotympanummesotympanum

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HypotympanumHypotympanum

Lies inferior and medial to the floor Lies inferior and medial to the floor of the bony ear canalof the bony ear canal

Irregular bony groove that is seldom Irregular bony groove that is seldom involved by cholesteatoma involved by cholesteatoma

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Anatomic ConsiderationsAnatomic Considerations

Annular ligament sends fibrous bands from Annular ligament sends fibrous bands from anterior and posterior tympanic spines that anterior and posterior tympanic spines that meet at neck of malleus and make up middle meet at neck of malleus and make up middle layer of pars tensalayer of pars tensa

Dehiscent area in the tympanic bone (Notch of Dehiscent area in the tympanic bone (Notch of Rivinus) lies above fibrous bandsRivinus) lies above fibrous bands

Dense fibers that form middle layer of pars Dense fibers that form middle layer of pars tensa do not extend to pars flaccidatensa do not extend to pars flaccida Lack of structural support predisposes Shrapnell’s Lack of structural support predisposes Shrapnell’s

membrane to retract when negative middle ear membrane to retract when negative middle ear pressure is present secondary to Eustachian tube pressure is present secondary to Eustachian tube dysfunction dysfunction

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Anatomic ConsiderationsAnatomic Considerations

Cholesteatomas of Cholesteatomas of epitympanum start epitympanum start in Prussack’s in Prussack’s space between space between pars flaccida and pars flaccida and neck of malleus neck of malleus with upper with upper boundary being boundary being the lateral mallear the lateral mallear fold fold

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Cholesteatoma SpreadCholesteatoma Spread

Predictable in that they are Predictable in that they are channeled along characteristic channeled along characteristic pathways by:pathways by: LigamentsLigaments FoldsFolds Ossicles Ossicles

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Common Sites of Common Sites of Cholesteatoma OriginCholesteatoma Origin

Posterior Posterior epitympanumepitympanum

Posterior Posterior mesotympanummesotympanum

Anterior Anterior epitympanumepitympanum

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Cholesteatoma SpreadCholesteatoma Spread

Posterior epitympanic cholesteatoma Posterior epitympanic cholesteatoma passing through superior incudal space passing through superior incudal space and aditus ad antrumand aditus ad antrum

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Cholesteatoma SpreadCholesteatoma Spread

Posterior mesotympanic cholesteatoma Posterior mesotympanic cholesteatoma invading the sinus tympani and facial invading the sinus tympani and facial recessrecess

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Cholesteatoma SpreadCholesteatoma Spread

Anterior epitympanic cholesteatoma with Anterior epitympanic cholesteatoma with extension to geniculate ganglionextension to geniculate ganglion

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Patient Patient EvaluationEvaluation

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Patient EvaluationPatient Evaluation HistoryHistory

Detailed otologic history Detailed otologic history Hearing lossHearing loss OtorrheaOtorrhea OtalgiaOtalgia Nasal obstructionNasal obstruction TinnitusTinnitus VertigoVertigo

Previous history of middle ear diseasePrevious history of middle ear disease Chronic otitis media Chronic otitis media Tympanic membrane perforationTympanic membrane perforation Prior surgeryPrior surgery

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Patient EvaluationPatient Evaluation Head and neck examinationHead and neck examination Otologic examination Otologic examination

Otomicroscopy is essential in evaluating the Otomicroscopy is essential in evaluating the extent of diseaseextent of disease

Clean ear thoroughly of otorrhea and debris Clean ear thoroughly of otorrhea and debris with cotton-tipped applicators or suctionwith cotton-tipped applicators or suction

Culture wet, infected ears and treat with topical Culture wet, infected ears and treat with topical and/or oral antibioticsand/or oral antibiotics

Pneumatic otoscopy should be performed in Pneumatic otoscopy should be performed in every patient with cholesteatomaevery patient with cholesteatoma

Positive fistula (pneumatic otoscopy will result in Positive fistula (pneumatic otoscopy will result in nystagmus and vertigo) response suggests erosion of nystagmus and vertigo) response suggests erosion of the semicircular canals or cochleathe semicircular canals or cochlea

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Patient EvaluationPatient Evaluation

Hearing evaluation to assess for conductive Hearing evaluation to assess for conductive hearing losshearing loss Pure tone audiometry with air and bone Pure tone audiometry with air and bone

conductionconduction Speech reception thresholdsSpeech reception thresholds Word recognition Word recognition

512Hz tuning fork exam512Hz tuning fork exam Always correlate with audiometry resultsAlways correlate with audiometry results

TympanometryTympanometry May suggest decreased compliance or TM May suggest decreased compliance or TM

perforationperforation

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Patient EvaluationPatient Evaluation

Degree of conductive loss will vary Degree of conductive loss will vary considerably depending on the extent of considerably depending on the extent of diseasedisease Moderate conductive deficit in excess of 40 Moderate conductive deficit in excess of 40

dB indicates ossicular discontinuitydB indicates ossicular discontinuity Usually from erosion of the long process of the Usually from erosion of the long process of the

incus or capitulum of the stapesincus or capitulum of the stapes Mild conductive deafness may be present Mild conductive deafness may be present

with extensive disease if cholesteatoma sac with extensive disease if cholesteatoma sac transmits sound directly to stapes or transmits sound directly to stapes or footplate footplate

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Patient EvaluationPatient Evaluation

Preoperative imaging with computed Preoperative imaging with computed tomographies (CTs) of temporal tomographies (CTs) of temporal bones (2mm -section without bones (2mm -section without contrast in axial and coronal planes) contrast in axial and coronal planes) Allows for evaluation of anatomyAllows for evaluation of anatomy May reveal evidence of the extent of the May reveal evidence of the extent of the

disease disease Screen for asymptomatic complicationsScreen for asymptomatic complications

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Patient EvaluationPatient Evaluation

CT is not essential for preoperative CT is not essential for preoperative evaluationevaluation

Should be obtained for:Should be obtained for: Revision cases due to altered landmarks from Revision cases due to altered landmarks from

previous surgeryprevious surgery Chronic suppurative otitis mediaChronic suppurative otitis media Suspected congenital abnormalitiesSuspected congenital abnormalities Cases of cholesteatoma in which sensorineural Cases of cholesteatoma in which sensorineural

hearing loss, vestibular symptoms, or other hearing loss, vestibular symptoms, or other complication evidence exists complication evidence exists

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Patient EvaluationPatient Evaluation Preoperative counseling is an absolute Preoperative counseling is an absolute

necessity prior to surgerynecessity prior to surgery Primary objective of surgery is a safe dry Primary objective of surgery is a safe dry

ear which is accomplished by:ear which is accomplished by: Treating all supervening complicationsTreating all supervening complications Removing diseased bone, mucosa, Removing diseased bone, mucosa,

granulation polyps, and cholesteatomagranulation polyps, and cholesteatoma Preserving as much normal anatomy as Preserving as much normal anatomy as

possiblepossible Improvement of hearing is a secondary Improvement of hearing is a secondary

goalgoal

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Patient EvaluationPatient Evaluation

Possible adverse outcomes must be Possible adverse outcomes must be discusseddiscussed Facial paralysisFacial paralysis VertigoVertigo Further hearing lossFurther hearing loss TinnitusTinnitus

Patient should understand that long-Patient should understand that long-term follow-up will be necessary and term follow-up will be necessary and that they may need additional surgeries that they may need additional surgeries

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Cholesteatoma Cholesteatoma ManagementManagement

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Preventative Preventative ManagementManagement

Tympanostomy tube for early retraction Tympanostomy tube for early retraction pocketspockets

Surgical exploration for retraction Surgical exploration for retraction persistencepersistence

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Cholesteatoma Cholesteatoma ManagementManagement

Treated surgically with primary goal Treated surgically with primary goal of total eradication of cholesteatoma of total eradication of cholesteatoma to obtain a safe and dry earto obtain a safe and dry ear

Patients with unacceptable risk of Patients with unacceptable risk of anesthesia need local careanesthesia need local care

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Surgical ManagementSurgical Management

Canal-wall-down procedures (CWD)Canal-wall-down procedures (CWD) Canal-wall-up procedure (CWU)Canal-wall-up procedure (CWU) Transcanal anterior atticotomyTranscanal anterior atticotomy Bondy modified radical procedureBondy modified radical procedure

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Canal-Wall-DownCanal-Wall-Down

Prior to the advent of the tympanoplasty, Prior to the advent of the tympanoplasty, all cholesteatoma surgery was performed all cholesteatoma surgery was performed using CWD approachusing CWD approach

Procedure involves:Procedure involves: Taking down posterior canal wall to level of Taking down posterior canal wall to level of

vertical facial nerve vertical facial nerve Exteriorizing the mastoid into external Exteriorizing the mastoid into external

auditory canalauditory canal Epitympanum is obliterated with removal Epitympanum is obliterated with removal

of scutum, head of malleus and incusof scutum, head of malleus and incus

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Canal-Wall-DownCanal-Wall-Down

Classic CWD operation is the modified radical Classic CWD operation is the modified radical mastoidectomy in which middle ear space is mastoidectomy in which middle ear space is preservedpreserved

Radical mastoidectomy is CWD operation in Radical mastoidectomy is CWD operation in which:which: Middle ear space is eliminatedMiddle ear space is eliminated Eustachian tube is plugged Eustachian tube is plugged

Meatoplasty should be large enough to allow Meatoplasty should be large enough to allow good aeration of mastoid cavity and permit good aeration of mastoid cavity and permit easy visualization to facilitate postoperative easy visualization to facilitate postoperative care and self cleaning care and self cleaning

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Canal-Wall-DownCanal-Wall-Down

Indications for CWD approach:Indications for CWD approach: Cholesteatoma in an only hearing earCholesteatoma in an only hearing ear Significant erosion of the posterior bony canal Significant erosion of the posterior bony canal

wallwall History of vertigo suggesting a labyrinthine History of vertigo suggesting a labyrinthine

fistulafistula Recurrent cholesteatoma after canal-wall-up Recurrent cholesteatoma after canal-wall-up

surgerysurgery Poor eustachian tube functionPoor eustachian tube function Sclerotic mastoid with limited access to Sclerotic mastoid with limited access to

epitympanumepitympanum

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Canal-Wall-DownCanal-Wall-Down

Advantages:Advantages: Residual disease is easily detectedResidual disease is easily detected Recurrent disease is rareRecurrent disease is rare Facial recess is exteriorizedFacial recess is exteriorized

Disadvantages:Disadvantages: Open cavity created Open cavity created

Takes longer to healTakes longer to heal Mastoid bowl maintenance can be a lifelong Mastoid bowl maintenance can be a lifelong

problemproblem Shallow middle ear space makes OCR difficultShallow middle ear space makes OCR difficult Dry ear precautions are essential Dry ear precautions are essential

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Canal-Wall-DownCanal-Wall-Down

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Canal-Wall-DownCanal-Wall-Down

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Canal-Wall-DownCanal-Wall-Down

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Canal-Wall-DownCanal-Wall-Down

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Canal-Wall-DownCanal-Wall-Down

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Canal-Wall-UpCanal-Wall-Up CWU procedure developed to avoid problems CWU procedure developed to avoid problems

and maintenance necessary with CWD and maintenance necessary with CWD proceduresprocedures

CWU consists of preservation of posterior bony CWU consists of preservation of posterior bony external auditory canal wall during simple external auditory canal wall during simple mastoidectomy with or without a posterior mastoidectomy with or without a posterior tympanotomytympanotomy

Staged procedure often necessary with a Staged procedure often necessary with a scheduled second look operation at 6 to 18 scheduled second look operation at 6 to 18 months for:months for: Removal of residual cholesteatomaRemoval of residual cholesteatoma Ossicular chain reconstruction if necessaryOssicular chain reconstruction if necessary

Procedure should be adapted to extent of Procedure should be adapted to extent of disease as well as skill of otologistdisease as well as skill of otologist

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Canal-Wall-UpCanal-Wall-Up

CWU may be indicated in patients CWU may be indicated in patients with large pneumatized mastoid and with large pneumatized mastoid and well aerated middle ear spacewell aerated middle ear space Suggests good eustachian tube functionSuggests good eustachian tube function

CWU procedures are contraindicated CWU procedures are contraindicated in:in: Only hearing earOnly hearing ear Patients with labyrinthine fistulaPatients with labyrinthine fistula Long-standing ear diseaseLong-standing ear disease Poor eustachian tube functionPoor eustachian tube function

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Canal-Wall-UpCanal-Wall-Up Advantages:Advantages:

Rapid healing timeRapid healing time Easier long-term careEasier long-term care Hearing aids easier to fitHearing aids easier to fit No water precautionsNo water precautions

Disadvantages:Disadvantages: Technically more difficultTechnically more difficult Staged operation often necessaryStaged operation often necessary Recurrent disease possibleRecurrent disease possible Residual disease harder to detectResidual disease harder to detect

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Canal-Wall-UpCanal-Wall-Up

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Canal-Wall-UpCanal-Wall-Up

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Transcanal Anterior Transcanal Anterior AtticotomyAtticotomy

Indicated for limited cholesteatoma Indicated for limited cholesteatoma involving middle ear, ossicular involving middle ear, ossicular chain, and epitympanumchain, and epitympanum

If extent of the cholesteatoma is If extent of the cholesteatoma is unknown approach can be combined unknown approach can be combined with CWU mastoidectomy or with CWU mastoidectomy or extended to CWD procedureextended to CWD procedure

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Transcanal Anterior Transcanal Anterior AtticotomyAtticotomy

Procedure involves:Procedure involves: Elevation of tympanomeatal flap via Elevation of tympanomeatal flap via

endaural incision with removal of scutum endaural incision with removal of scutum to limits of the cholesteatomato limits of the cholesteatoma

Aditus obliteration with muscle, fascia, Aditus obliteration with muscle, fascia, cartilage or bone prior to reconstruction of cartilage or bone prior to reconstruction of the middle ear spacethe middle ear space

Reconstruction of lateral attic wall with Reconstruction of lateral attic wall with bone or cartilage is optionalbone or cartilage is optional

May lead to retraction disease and possible May lead to retraction disease and possible recurrence in patients with poor eustachian recurrence in patients with poor eustachian tube function tube function

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Transcanal Anterior Transcanal Anterior AtticotomyAtticotomy

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Bondy Modified Radical Bondy Modified Radical ProcedureProcedure

Useful for attic and mastoid cholesteatoma that Useful for attic and mastoid cholesteatoma that does not involve middle ear space and lateral to does not involve middle ear space and lateral to ossiclesossicles

Like modern modified radical mastoidectomy with Like modern modified radical mastoidectomy with exception that middle ear space is not entered exception that middle ear space is not entered

Mastoid should be poorly developed for creation Mastoid should be poorly developed for creation of a small cavityof a small cavity

Eustachian tube function should be adequate with Eustachian tube function should be adequate with intact pars tensa and aerated middle ear space.intact pars tensa and aerated middle ear space.

Rarely used todayRarely used today

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Canal Wall StatusCanal Wall Status

In 2002 Shohet et. al. reports In 2002 Shohet et. al. reports recidivism rates with CWU as high as recidivism rates with CWU as high as Adults = 36%Adults = 36% Children = 67%Children = 67%

Approximately 30% of cases for Approximately 30% of cases for cholesteatoma will be CWDcholesteatoma will be CWD House Ear Institute 1982House Ear Institute 1982 St. Joseph’s Hospital in Phoenix, AZ 2003St. Joseph’s Hospital in Phoenix, AZ 2003

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Canal Wall StatusCanal Wall Status

Syms et. al. examined surgical Syms et. al. examined surgical approach to manage cholesteatoma- approach to manage cholesteatoma- retrospective review of 486 cases in retrospective review of 486 cases in 20032003 68.5% CWU: 31.5% CWD68.5% CWU: 31.5% CWD CWU had “second look operation”CWU had “second look operation”

Residual cholesteatoma found in 26.9% Residual cholesteatoma found in 26.9% second proceduressecond procedures

Residual cholesteatoma found in 2.7% third Residual cholesteatoma found in 2.7% third proceduresprocedures

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Canal Wall StatusCanal Wall Status

Kos et. al. examined long-term Kos et. al. examined long-term implications of CWD- retrospective implications of CWD- retrospective review in 2004review in 2004 259 cases with follow-up range of 1 to 259 cases with follow-up range of 1 to

24 years (mean = 7 years)24 years (mean = 7 years) Recurrent cholesteatoma in 6.1%Recurrent cholesteatoma in 6.1% TM perforation in 7.3%TM perforation in 7.3% Dry, self-cleaning ears in 95%Dry, self-cleaning ears in 95% Otorrhea in 5%Otorrhea in 5%

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Canal Wall StatusCanal Wall Status

Kos et. al. (continued)Kos et. al. (continued) Hearing thresholdHearing threshold

Improved in 30.7%Improved in 30.7% Unchanged in 41.3%Unchanged in 41.3% Decreased in 28%Decreased in 28%

Sensorineural hearing loss greater than Sensorineural hearing loss greater than 60dB in 2 patients60dB in 2 patients

Facial paralysis in 1 patientFacial paralysis in 1 patient Vertigo in 4 patientsVertigo in 4 patients

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Novel TechniquesNovel Techniques

In 2005 Gantz et. al. reported 130 In 2005 Gantz et. al. reported 130 cases of canal wall reconstruction cases of canal wall reconstruction tympanomastoidectomy with mastoid tympanomastoidectomy with mastoid obliterationobliteration No evidence of recurrence = 98.5%No evidence of recurrence = 98.5% Recurrence treated with CWD (1.5%)Recurrence treated with CWD (1.5%) Second look ossiculoplasty in 78%Second look ossiculoplasty in 78% Post-operative wound infection was Post-operative wound infection was

14.3% for first 42 patients14.3% for first 42 patients Decreased rate to 4.5% in last 88 patients Decreased rate to 4.5% in last 88 patients

with 2 days of post-operative IV antibioticswith 2 days of post-operative IV antibiotics

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Novel TechniquesNovel Techniques Canal Wall Reconstruction techniqueCanal Wall Reconstruction technique

Complete cortical mastoidectomy with opening Complete cortical mastoidectomy with opening of facial recess and removal of incus and malleus of facial recess and removal of incus and malleus headhead

Posterior canal wall skin elevated, annulus Posterior canal wall skin elevated, annulus elevatedelevated

Microsagittal saw used to cut posterior canal Microsagittal saw used to cut posterior canal wallwall

Cholesteatoma removedCholesteatoma removed Posterior canal wall bone replacedPosterior canal wall bone replaced

Cortical bone chips used to block attic and mastoid Cortical bone chips used to block attic and mastoid from tympanumfrom tympanum

Bone pate’ holds bone chips in placeBone pate’ holds bone chips in place

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Novel TechniquesNovel Techniques

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Novel TechniquesNovel Techniques

In 2005, Godinho et. al. reported 42 In 2005, Godinho et. al. reported 42 cases of Canal Wall Window (CWW) cases of Canal Wall Window (CWW) performed for pediatric cholesteatomaperformed for pediatric cholesteatoma CWW compared to CWU and CWDCWW compared to CWU and CWD

CWW converted to CWD in 14%CWW converted to CWD in 14% Dry ear resultsDry ear results

CWW = 94%, CWD = 92%, CWU = 90%CWW = 94%, CWD = 92%, CWU = 90% Recidivation rate at 1 yearRecidivation rate at 1 year

CWW = 19.5%, CWD = 0%, CWU = 7.7%CWW = 19.5%, CWD = 0%, CWU = 7.7% CWW provided similar post-operative hearing CWW provided similar post-operative hearing

to CWU, rather than increased air-bone gap to CWU, rather than increased air-bone gap seen with CWDseen with CWD

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Novel TechniquesNovel Techniques

CWW effective for posterior superior CWW effective for posterior superior cholesteatomacholesteatoma Increases visibilityIncreases visibility Conduit for instrument manipulationConduit for instrument manipulation

CWW techniqueCWW technique CWU mastoidectomy performedCWU mastoidectomy performed Lateral epitympanotomyLateral epitympanotomy Slit drilled from lateral cortex to medial Slit drilled from lateral cortex to medial

aspect of canal wall at annulusaspect of canal wall at annulus

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Novel TechniquesNovel Techniques

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Novel TechniquesNovel Techniques

CWW reconstructionCWW reconstruction Tragal cartilage placed in defect after Tragal cartilage placed in defect after

scoring proximal perichondrium and scoring proximal perichondrium and cartilagecartilage

Cortical bone graft may be used as an Cortical bone graft may be used as an alternativealternative

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Novel TechniquesNovel Techniques

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ComplicationsComplications

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Cholesteatoma SequelaeCholesteatoma Sequelae InfectionInfection OtorrheaOtorrhea Bone destructionBone destruction Hearing lossHearing loss Facial nerve paresis or paralysisFacial nerve paresis or paralysis Labyrinthine fistulaLabyrinthine fistula Intracranial complications Intracranial complications

Complications are caused by expansion and infection

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Hearing LossHearing Loss Conductive hearing loss: ossicular chain erosion Conductive hearing loss: ossicular chain erosion

(30%)(30%) Erosion of lenticular process and/or stapes Erosion of lenticular process and/or stapes

superstructure may produce 50dB conductive hearing superstructure may produce 50dB conductive hearing loss loss

Hearing loss varies despite disease extent (natural Hearing loss varies despite disease extent (natural myringostapediopexy, transmission of sound through myringostapediopexy, transmission of sound through cholesteatoma sac)cholesteatoma sac)

Sensorineural hearing loss: involvement of Sensorineural hearing loss: involvement of labyrinthlabyrinth

Following surgery, 30% have further impairment Following surgery, 30% have further impairment due to:due to: Extent of disease present Extent of disease present Complications in healing processComplications in healing process

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Labyrinthine FistulaLabyrinthine Fistula

Incidence: as high as 10% Incidence: as high as 10% Symptom: Sensorineural hearing loss and/or Symptom: Sensorineural hearing loss and/or

vertigo induced by noise or pressure changevertigo induced by noise or pressure change Common site: horizontal semicircular canal, Common site: horizontal semicircular canal,

basal turn of cochleabasal turn of cochlea Diagnosis: Fine cut temporal bone CT Diagnosis: Fine cut temporal bone CT

(1mm)(1mm) Management: modified radical Management: modified radical

mastoidectomy with management of matrix mastoidectomy with management of matrix overlying fistulaoverlying fistula

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Facial ParalysisFacial Paralysis May develop:May develop:

Acutely secondary to infection Acutely secondary to infection Slowly from chronic expansion of cholesteatomaSlowly from chronic expansion of cholesteatoma

Temporal bone CT: localize the nerve Temporal bone CT: localize the nerve involvementinvolvement

Most common site: geniculate ganglion due to Most common site: geniculate ganglion due to disease in the anterior epitympanumdisease in the anterior epitympanum

Management: Needs immediate surgeryManagement: Needs immediate surgery Removal of cholesteatoma and infected material with Removal of cholesteatoma and infected material with

decompression of the nerve (mastoidectomy, middle decompression of the nerve (mastoidectomy, middle fossa approach)fossa approach)

Administration of intravenous antibiotics and high-Administration of intravenous antibiotics and high-dose steroidsdose steroids

Iatrogenic injury to the nerve during surgery should Iatrogenic injury to the nerve during surgery should be immediately repaired with decompression of nerve be immediately repaired with decompression of nerve proximal and distal to site of injuryproximal and distal to site of injury

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Intracranial Intracranial ComplicationsComplications

Potentially life-threateningPotentially life-threatening Incidence: as high as 1%Incidence: as high as 1% Complications Complications

Periosteal abscessPeriosteal abscess Lateral sinus thrombosisLateral sinus thrombosis Intracranial abscessIntracranial abscess MeningitisMeningitis

Symptom: Symptom: Suppurative malodorous otorrheaSuppurative malodorous otorrhea Chronic headacheChronic headache FeverFever OtalgiaOtalgia

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Intracranial Intracranial ComplicationsComplications

Management:Management: Presence of mental status changes with Presence of mental status changes with

nuchal rigidity or cranial neuropathies nuchal rigidity or cranial neuropathies warrant neurosurgical consultation with warrant neurosurgical consultation with urgent interventionurgent intervention

Epidural abscess, subdural empyema, Epidural abscess, subdural empyema, meningitis and cerebral abscesses meningitis and cerebral abscesses should be treated immediately prior to should be treated immediately prior to definitive otologic management of ear definitive otologic management of ear diseasedisease

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ConclusionsConclusions Pathogenesis of cholesteatoma remains uncertainPathogenesis of cholesteatoma remains uncertain Essential to possess basic knowledge of the Essential to possess basic knowledge of the

important anatomic and functional characteristics important anatomic and functional characteristics of the middle ear for successful management of of the middle ear for successful management of cholesteatomascholesteatomas

Careful and thorough evaluations are the key to Careful and thorough evaluations are the key to early diagnosis and treatment early diagnosis and treatment

Treatment is surgical with primary goal to Treatment is surgical with primary goal to eradicate disease and provide a safe and dry eareradicate disease and provide a safe and dry ear

Surgical approaches must be customized to each Surgical approaches must be customized to each patient depending on extent of diseasepatient depending on extent of disease

Surgeon must be aware of serious and potentially Surgeon must be aware of serious and potentially life-threatening complications of cholesteatomaslife-threatening complications of cholesteatomas

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BibliographyBibliography Portions of this paper and presentation were taken directly form the June 18, 2003 Portions of this paper and presentation were taken directly form the June 18, 2003

Grand Rounds presentation by Michael Underbrink and Arun Gadre entitled Grand Rounds presentation by Michael Underbrink and Arun Gadre entitled Cholesteatoma.Cholesteatoma.

Bailey BJ. Head and Neck Surgery – Otolaryngology 3rd Edition. 2001: 1787-1797.Bailey BJ. Head and Neck Surgery – Otolaryngology 3rd Edition. 2001: 1787-1797. Godinho RA, Kamil SH, Lubianca JN, Keogh IJ, Eavey RD. Pediatric cholesteatoma: Godinho RA, Kamil SH, Lubianca JN, Keogh IJ, Eavey RD. Pediatric cholesteatoma:

canal wall window alternative to canal wall down mastoidectomy. Otology and canal wall window alternative to canal wall down mastoidectomy. Otology and Neurotology. 2005. 26:466-471.Neurotology. 2005. 26:466-471.

Gantz BJ, Wilkinson EP, Hansen MR. Canal wall reconstruction Gantz BJ, Wilkinson EP, Hansen MR. Canal wall reconstruction tympanomastoidectomy with mastoid obliteration. The Laryngoscope. 2005. 115: tympanomastoidectomy with mastoid obliteration. The Laryngoscope. 2005. 115: 1734-1740.1734-1740.

Syms MJ, Luxford WM. Management of cholesteatoma: status of the canal wall. Syms MJ, Luxford WM. Management of cholesteatoma: status of the canal wall. The Laryngoscope. 2003. 113: 443-448.The Laryngoscope. 2003. 113: 443-448.

Kos MI, Castrillon R, Montandon P, Guyot JP. Anatomic and functional long-term Kos MI, Castrillon R, Montandon P, Guyot JP. Anatomic and functional long-term results of canal wall-down mastoidectomy. Annals of Otology, Rhinology and results of canal wall-down mastoidectomy. Annals of Otology, Rhinology and Laryngology. 2004. 113: 872-876.Laryngology. 2004. 113: 872-876.

Karmarkar S, Bhatia S, Saleh E, et al. Cholesteatoma surgery: the individualized Karmarkar S, Bhatia S, Saleh E, et al. Cholesteatoma surgery: the individualized technique. Annals of Otology, Rhinology and Laryngology. 1995. 104: 591–595.technique. Annals of Otology, Rhinology and Laryngology. 1995. 104: 591–595.

Roden D, Honrubia V, Wiet R. Outcome of residual cholesteatoma and hearing in Roden D, Honrubia V, Wiet R. Outcome of residual cholesteatoma and hearing in mastoid surgery. Journal of Otolaryngology. 1996. 25: 178–181.mastoid surgery. Journal of Otolaryngology. 1996. 25: 178–181.

Chang C, Chen M. Canal-wall-down tympanoplasty with mastoidectomy for Chang C, Chen M. Canal-wall-down tympanoplasty with mastoidectomy for advances cholesteatoma. Journal of Otolaryngology. 2000. 29: 270–273.advances cholesteatoma. Journal of Otolaryngology. 2000. 29: 270–273.

Shohet JA, de Jong AL. The management of pediatric cholesteatoma. Shohet JA, de Jong AL. The management of pediatric cholesteatoma. Otolaryngology Clinics of North America. 2002. 35:841–851.Otolaryngology Clinics of North America. 2002. 35:841–851.

McElveen JT Jr, Chung AT. Reversible canal wall down mastoidectomy for acquired McElveen JT Jr, Chung AT. Reversible canal wall down mastoidectomy for acquired cholesteatomas: preliminary results. The Laryngoscope. 2003. 113:1027–1033.cholesteatomas: preliminary results. The Laryngoscope. 2003. 113:1027–1033.

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What To DoWhat To Do Treatment should be tailored to caseTreatment should be tailored to case Absolute CWD indicationsAbsolute CWD indications

Cholesteatoma in an only hearing earCholesteatoma in an only hearing ear Significant erosion of the posterior bony canal wallSignificant erosion of the posterior bony canal wall History of vertigo suggesting a labyrinthine fistulaHistory of vertigo suggesting a labyrinthine fistula Recurrent cholesteatoma after canal-wall-up Recurrent cholesteatoma after canal-wall-up

surgerysurgery Poor eustachian tube functionPoor eustachian tube function Sclerotic mastoid with limited access to Sclerotic mastoid with limited access to

epitympanumepitympanum No harm in starting CWU and converting to No harm in starting CWU and converting to

CWD as necessaryCWD as necessary Know your surgical limitationsKnow your surgical limitations

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QuizQuiz

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QuizQuiz

1. What space lies 1. What space lies between the pars between the pars flaccida and #13?flaccida and #13? A. Prussak’s spaceA. Prussak’s space B. Anterior pouch B. Anterior pouch

of Von of Von TroeltschTroeltsch

C. Posterior pouch C. Posterior pouch of of Von Von TroeltschTroeltsch

D. The final frontierD. The final frontier

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QuizQuiz

2. The primary component of 2. The primary component of cholesteatoma is fat, as discovered cholesteatoma is fat, as discovered by Johannes Mueller in 1838.by Johannes Mueller in 1838. A. TrueA. True B. FalseB. False

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QuizQuiz

3. What 2 major groups are 3. What 2 major groups are cholesteatomas classified into (pick cholesteatomas classified into (pick 2 letters)?2 letters)? A. AcquiredA. Acquired B. InfectedB. Infected C. ExpansiveC. Expansive D. CongenitalD. Congenital

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QuizQuiz

4. Possible adverse outcomes of 4. Possible adverse outcomes of surgical treatment of cholesteatomas surgical treatment of cholesteatomas areare A. Facial paralysisA. Facial paralysis B. VertigoB. Vertigo C. Further hearing lossC. Further hearing loss D. TinnitusD. Tinnitus E. All of the aboveE. All of the above

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QuizQuiz

5. CT Scans must be obtained prior 5. CT Scans must be obtained prior to any surgery for cholesteatomato any surgery for cholesteatoma A. TrueA. True B. FalseB. False C. It depends on who your faculty isC. It depends on who your faculty is

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QuizQuiz

6. CWU procedures are 6. CWU procedures are contraindicated incontraindicated in A. Only hearing earsA. Only hearing ears B. Patients with a labyrinthine fistulaB. Patients with a labyrinthine fistula C. Long-standing ear diseaseC. Long-standing ear disease D. Poor eustachian tube functionD. Poor eustachian tube function E. All of the aboveE. All of the above

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QuizQuiz

7. Surgical treatment of 7. Surgical treatment of cholesteatoma has a primary goal of cholesteatoma has a primary goal of hearing restorationhearing restoration A. TrueA. True B. FalseB. False

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QuizQuiz

8. Surgical approaches must be 8. Surgical approaches must be customized to each patient customized to each patient depending on extent of diseasedepending on extent of disease A. TrueA. True B. FalseB. False

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Quiz BonusQuiz Bonus

What is this a What is this a picture of?picture of? A. Cat eyeA. Cat eye B. Snake skinB. Snake skin C. Tympanic C. Tympanic

membrane stained membrane stained with osmium to with osmium to show keratin show keratin patchespatches