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

2. Overview Definition Definition Classification and Theories Classification and Theories Anatomic Considerations Anatomic Considerations Patient Evaluation Patient Evaluation Management Management Complications Complications

3. Definition

4. Cholesteatoma Named by Johannes Mueller in 1838 Named by Johannes Mueller in 1838 Erroneous belief that one of the primary components of the tumor was fat Erroneous belief that one of the primary components of the tumor was fat “a pearly tumor of fat…among sheets of polyhedral cells” “a pearly tumor of fat…among sheets of polyhedral cells” More appropriate name has been suggested to be keratoma to describe tumor composition More appropriate name has been suggested to be keratoma to describe tumor composition

5. Cholesteatoma Expanding lesion of the temporal bone composed of Expanding lesion of the temporal bone composed of Cystic content: desquamated keratin center Cystic content: desquamated keratin center Matrix: keratinizing stratified squamous epithelium Matrix: keratinizing stratified squamous epithelium Perimatrix: granulation tissue that secretes multiple proteolytic enzymes capable of bone destruction Perimatrix: granulation tissue that secretes multiple proteolytic enzymes capable of bone destruction May develop anywhere within pneumatized portions of the temporal bone May develop anywhere within pneumatized portions of the temporal bone Most frequent locations: Middle ear space Mastoid

6. Classification and Theories

7. Cholesteatoma Classification Congenital Congenital Acquired Acquired Primary Primary Secondary Secondary Cholesteatoma Formation Multiple theories proposed regarding etiology behind tumor formation Multiple theories proposed regarding etiology behind tumor formation Proposed mechanisms remain theories Proposed mechanisms remain theories

8. Congenital Cholesteatoma Definition (Levenson, 1989) Definition (Levenson, 1989) White mass medial to normal tympanic membrane White mass medial to normal tympanic membrane Normal pars flaccida and pars tensa Normal pars flaccida and pars tensa No prior history of otorrhea or perforations No prior history of otorrhea or perforations No prior otologic procedures No prior otologic procedures Prior bouts of otitis media were not grounds for exclusion as was the case in original definition Prior bouts of otitis media were not grounds for exclusion as was the case in original definition

9. Congenital Cholesteatoma Pathogenesis theories Pathogenesis theories Failure of involution of ectodermal epithelial thickening that is present during fetal development in proximity to geniculate ganglion Failure of involution of ectodermal epithelial thickening that is present during fetal development in proximity to geniculate ganglion Metaplasia of the middle ear mucosa Metaplasia of the middle ear mucosa

10. Congenital Cholesteatoma Anterosuperior quadrant quadrant > Posterosuperior quadrant

11. Congenital Cholesteatoma Large congenital cholesteatoma ossicular erosion cholesteatoma

12. Acquired Cholesteatomas Common factor: keratinizing squamous epithelium has grown beyond its normal limits Common factor: keratinizing squamous epithelium has grown beyond its normal limits

13. Primary Acquired Cholesteatomas Ultimately form due to underlying Eustachian tube dysfunction that causes retraction of pars flaccida Ultimately form due to underlying Eustachian tube dysfunction that causes retraction of pars flaccida Results in poor aeration of epitympanic space which draws pars flaccida medially on top of malleus neck, forming retraction pocket Results in poor aeration of epitympanic space which draws pars flaccida medially on top of malleus neck, forming retraction pocket Normal migratory pattern of the tympanic membrane epithelium altered by retraction pocket Normal migratory pattern of the tympanic membrane epithelium altered by retraction pocket Enhances potential accumulation of keratin Enhances potential accumulation of keratin

14. Pars flaccida retraction Pars tensa retraction Primary Acquired Cholesteatomas

15. Secondary Acquired Cholesteatomas Implantation theory Implantation theory Squamous epithelium implanted in the middle ear as a result of surgery, foreign body, blast injury, etc. Squamous epithelium implanted in the middle ear as a result of surgery, foreign body, blast injury, etc. Metaplasia theory Metaplasia theory Desquamated epithelium is transformed to keratinized stratified squamous epithelium secondary to chronic or recurrent otitis media Desquamated epithelium is transformed to keratinized stratified squamous epithelium secondary to chronic or recurrent otitis media Epithelial invasion theory Epithelial invasion theory Squamous epithelium migrates along perforation edge medially along undersurface of tympanic membrane destroying the columnar epithelium Squamous epithelium migrates along perforation edge medially along undersurface of tympanic membrane destroying the columnar epithelium Papillary ingrowth theory Papillary ingrowth theory Inflammatory reaction in Prussack’s space with an intact pars flaccida (likely secondary to poor ventilation) may cause break in basal membrane allowing cord of epithelial cells to start inward proliferation Inflammatory reaction in Prussack’s space with an intact pars flaccida (likely secondary to poor ventilation) may cause break in basal membrane allowing cord of epithelial cells to start inward proliferation

16. Anatomy

17. Middle Ear Regions Named based on position relative to superior and inferior aspect of external auditory canal (EAC) Named based on position relative to superior and inferior aspect of external auditory canal (EAC) Epitympanum: superior to superior limit of EAC Epitympanum: superior to superior limit of EAC Mesotympanum: bound superiorly by superior limit of EAC and inferiorly by inferior limit of EAC Mesotympanum: bound superiorly by superior limit of EAC and inferiorly by inferior limit of EAC Hypotympanum: inferior to inferior limit of EAC Hypotympanum: inferior to inferior limit of EAC

18. Middle Ear Regions

19. Epitympanum Lies above the level of the short process of the malleus Lies above the level of the short process of the malleus Contents: Contents: Head of the malleus Head of the malleus Body of the incus Body of the incus Associated ligaments and mucosal folds Associated ligaments and mucosal folds

20. Mesotympanum Contents: Contents: Stapes Stapes Long process of the incus Long process of the incus Handle of the malleus Handle of the malleus Oval and round windows Oval and round windows Eustachian tube exits from the anterior aspect Eustachian tube exits from the anterior aspect Two recesses extend posteriorly that are often not visible directly Two recesses extend posteriorly that are often not visible directly Facial recess Facial recess Lateral to facial nerve Lateral to facial nerve Bounded by the fossa incudis superiorly Bounded by the fossa incudis superiorly Bounded by the chorda tympani nerve laterally Bounded by the chorda tympani nerve laterally Sinus tympani Sinus tympani Lies between the facial nerve and the medial wall of the mesotympanum Lies between the facial nerve and the medial wall of the mesotympanum

21. Hypotympanum Lies inferior and medial to the floor of the bony ear canal Lies inferior and medial to the floor of the bony ear canal Irregular bony groove that is seldom involved by cholesteatoma Irregular bony groove that is seldom involved by cholesteatoma

22. Anatomic Considerations Annular ligament sends fibrous bands from anterior and posterior tympanic spines that meet at neck of malleus and make up middle layer of pars tensa Annular ligament sends fibrous bands from anterior and posterior tympanic spines that meet at neck of malleus and make up middle layer of pars tensa Dehiscent area in the tympanic bone (Notch of Rivinus) lies above fibrous bands Dehiscent area in the tympanic bone (Notch of Rivinus) lies above fibrous bands Dense fibers that form middle layer of pars tensa do not extend to pars flaccida Dense fibers that form middle layer of pars tensa do not extend to pars flaccida Lack of structural support predisposes Shrapnell’s membrane to retract when negative middle ear pressure is present secondary to Eustachian tube dysfunction Lack of structural support predisposes Shrapnell’s membrane to retract when negative middle ear pressure is present secondary to Eustachian tube dysfunction

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

24. Cholesteatoma Spread Predictable in that they are channeled along characteristic pathways by: Predictable in that they are channeled along characteristic pathways by: Ligaments Ligaments Folds Folds Ossicles Ossicles

25. Common Sites of Cholesteatoma Origin Posterior epitympanum Posterior epitympanum Posterior mesotympanum Posterior mesotympanum Anterior epitympanum Anterior epitympanum

26. Cholesteatoma Spread Posterior epitympanic cholesteatoma passing through superior incudal space and aditus ad antrum Posterior epitympanic cholesteatoma passing through superior incudal space and aditus ad antrum

27. Cholesteatoma Spread Posterior mesotympanic cholesteatoma invading the sinus tympani and facial recess Posterior mesotympanic cholesteatoma invading the sinus tympani and facial recess

28. Cholesteatoma Spread Anterior epitympanic cholesteatoma with extension to geniculate ganglion Anterior epitympanic cholesteatoma with extension to geniculate ganglion

29. Patient Evaluation

30. History History Detailed otologic history Detailed otologic history Hearing loss Hearing loss Otorrhea Otorrhea Otalgia Otalgia Nasal obstruction Nasal obstruction Tinnitus Tinnitus Vertigo Vertigo Previous history of middle ear disease Previous history of middle ear disease Chronic otitis media Chronic otitis media Tympanic membrane perforation Tympanic membrane perforation Prior surgery Prior surgery

31. Patient Evaluation Head and neck examination Head and neck examination Otologic examination Otologic examination Otomicroscopy is essential in evaluating the extent of disease Otomicroscopy is essential in evaluating the extent of disease Clean ear thoroughly of otorrhea and debris with cotton- tipped applicators or suction Clean ear thoroughly of otorrhea and debris with cotton- tipped applicators or suction Culture wet, infected ears and treat with topical and/or oral antibiotics Culture wet, infected ears and treat with topical and/or oral antibiotics Pneumatic otoscopy should be performed in every patient with cholesteatoma Pneumatic otoscopy should be performed in every patient with cholesteatoma Positive fistula (pneumatic otoscopy will result in nystagmus and vertigo) response suggests erosion of the semicircular canals or cochlea Positive fistula (pneumatic otoscopy will result in nystagmus and vertigo) response suggests erosion of the semicircular canals or cochlea

32. Patient Evaluation Hearing evaluation to assess for conductive hearing loss Hearing evaluation to assess for conductive hearing loss Pure tone audiometry with air and bone conduction Pure tone audiometry with air and bone conduction Speech reception thresholds Speech reception thresholds Word recognition Word recognition 512Hz tuning fork exam 512Hz tuning fork exam Always correlate with audiometry results Always correlate with audiometry results Tympanometry Tympanometry May suggest decreased compliance or TM perforation May suggest decreased compliance or TM perforation

33. Patient Evaluation Degree of conductive loss will vary considerably depending on the extent of disease Degree of conductive loss will vary considerably depending on the extent of disease Moderate conductive deficit in excess of 40 dB indicates ossicular discontinuity Moderate conductive deficit in excess of 40 dB indicates ossicular discontinuity Usually from erosion of the long process of the incus or capitulum of the stapes Usually from erosion of the long process of the incus or capitulum of the stapes Mild conductive deafness may be present with extensive disease if cholesteatoma sac transmits sound directly to stapes or footplate Mild conductive deafness may be present with extensive disease if cholesteatoma sac transmits sound directly to stapes or footplate

34. Patient Evaluation Preoperative imaging with computed tomographies (CTs) of temporal bones (2mm - section without contrast in axial and coronal planes) Preoperative imaging with computed tomographies (CTs) of temporal bones (2mm - section without contrast in axial and coronal planes) Allows for evaluation of anatomy Allows for evaluation of anatomy May reveal evidence of the extent of the disease May reveal evidence of the extent of the disease Screen for asymptomatic complications Screen for asymptomatic complications

35. Patient Evaluation CT is not essential for preoperative evaluation CT is not essential for preoperative evaluation Should be obtained for: Should be obtained for: Revision cases due to altered landmarks from previous surgery Revision cases due to altered landmarks from previous surgery Chronic suppurative otitis media Chronic suppurative otitis media Suspected congenital abnormalities Suspected congenital abnormalities Cases of cholesteatoma in which sensorineural hearing loss, vestibular symptoms, or other complication evidence exists Cases of cholesteatoma in which sensorineural hearing loss, vestibular symptoms, or other complication evidence exists

36. Patient Evaluation Preoperative counseling is an absolute necessity prior to surgery Preoperative counseling is an absolute necessity prior to surgery Primary objective of surgery is a safe dry ear which is accomplished by: Primary objective of surgery is a safe dry ear which is accomplished by: Treating all supervening complications Treating all supervening complications Removing diseased bone, mucosa, granulation polyps, and cholesteatoma Removing diseased bone, mucosa, granulation polyps, and cholesteatoma Preserving as much normal anatomy as possible Preserving as much normal anatomy as possible Improvement of hearing is a secondary goal Improvement of hearing is a secondary goal

37. Patient Evaluation Possible adverse outcomes must be discussed Possible adverse outcomes must be discussed Facial paralysis Facial paralysis Vertigo Vertigo Further hearing loss Further hearing loss Tinnitus Tinnitus Patient should understand that long-term follow-up will be necessary and that they may need additional surgeries Patient should understand that long-term follow-up will be necessary and that they may need additional surgeries

38. Cholesteatoma Management

39. Preventative Management Tympanostomy tube for early retraction pockets Tympanostomy tube for early retraction pockets Surgical exploration for retraction persistence Surgical exploration for retraction persistence

40. Cholesteatoma Management Treated surgically with primary goal of total eradication of cholesteatoma to obtain a safe and dry ear Treated surgically with primary goal of total eradication of cholesteatoma to obtain a safe and dry ear Patients with unacceptable risk of anesthesia need local care Patients with unacceptable risk of anesthesia need local care

41. Surgical Management Canal-wall-down procedures (CWD) Canal-wall-down procedures (CWD) Canal-wall-up procedure (CWU) Canal-wall-up procedure (CWU) Transcanal anterior atticotomy Transcanal anterior atticotomy Bondy modified radical procedure Bondy modified radical procedure

42. Canal-Wall-Down Prior to the advent of the tympanoplasty, all cholesteatoma surgery was performed using CWD approach Prior to the advent of the tympanoplasty, all cholesteatoma surgery was performed using CWD approach Procedure involves: Procedure involves: Taking down posterior canal wall to level of vertical facial nerve Taking down posterior canal wall to level of vertical facial nerve Exteriorizing the mastoid into external auditory canal Exteriorizing the mastoid into external auditory canal Epitympanum is obliterated with removal of scutum, head of malleus and incus Epitympanum is obliterated with removal of scutum, head of malleus and incus

43. Canal-Wall-Down Classic CWD operation is the modified radical mastoidectomy in which middle ear space is preserved Classic CWD operation is the modified radical mastoidectomy in which middle ear space is preserved Radical mastoidectomy is CWD operation in which: Radical mastoidectomy is CWD operation in which: Middle ear space is eliminated Middle ear space is eliminated Eustachian tube is plugged Eustachian tube is plugged Meatoplasty should be large enough to allow good aeration of mastoid cavity and permit easy visualization to facilitate postoperative care and self cleaning Meatoplasty should be large enough to allow good aeration of mastoid cavity and permit easy visualization to facilitate postoperative care and self cleaning

44. Canal-Wall-Down Indications for CWD approach: Indications for CWD approach: Cholesteatoma in an only hearing ear Cholesteatoma in an only hearing ear Significant erosion of the posterior bony canal wall Significant erosion of the posterior bony canal wall History of vertigo suggesting a labyrinthine fistula History of vertigo suggesting a labyrinthine fistula Recurrent cholesteatoma after canal-wall-up surgery Recurrent cholesteatoma after canal-wall-up surgery Poor eustachian tube function Poor eustachian tube function Sclerotic mastoid with limited access to epitympanum Sclerotic mastoid with limited access to epitympanum

45. Canal-Wall-Down Advantages: Advantages: Residual disease is easily detected Residual disease is easily detected Recurrent disease is rare Recurrent disease is rare Facial recess is exteriorized Facial recess is exteriorized Disadvantages: Disadvantages: Open cavity created Open cavity created Takes longer to heal Takes longer to heal Mastoid bowl maintenance can be a lifelong problem Mastoid bowl maintenance can be a lifelong problem Shallow middle ear space makes OCR difficult Shallow middle ear space makes OCR difficult Dry ear precautions are essential Dry ear precautions are essential

46. Canal-Wall-Down

47. Canal-Wall-Down

48. Canal-Wall-Down

49. Canal-Wall-Down

50. Canal-Wall-Down

51. Canal-Wall-Up CWU procedure developed to avoid problems and maintenance necessary with CWD procedures CWU procedure developed to avoid problems and maintenance necessary with CWD procedures CWU consists of preservation of posterior bony external auditory canal wall during simple mastoidectomy with or without a posterior tympanotomy CWU consists of preservation of posterior bony external auditory canal wall during simple mastoidectomy with or without a posterior tympanotomy Staged procedure often necessary with a scheduled second look operation at 6 to 18 months for: Staged procedure often necessary with a scheduled second look operation at 6 to 18 months for: Removal of residual cholesteatoma Removal of residual cholesteatoma Ossicular chain reconstruction if necessary Ossicular chain reconstruction if necessary Procedure should be adapted to extent of disease as well as skill of otologist Procedure should be adapted to extent of disease as well as skill of otologist

52. Canal-Wall-Up CWU may be indicated in patients with large pneumatized mastoid and well aerated middle ear space CWU may be indicated in patients with large pneumatized mastoid and well aerated middle ear space Suggests good eustachian tube function Suggests good eustachian tube function CWU procedures are contraindicated in: CWU procedures are contraindicated in: Only hearing ear Only hearing ear Patients with labyrinthine fistula Patients with labyrinthine fistula Long-standing ear disease Long-standing ear disease Poor eustachian tube function Poor eustachian tube function

53. Canal-Wall-Up Advantages: Advantages: Rapid healing time Rapid healing time Easier long-term care Easier long-term care Hearing aids easier to fit Hearing aids easier to fit No water precautions No water precautions Disadvantages: Disadvantages: Technically more difficult Technically more difficult Staged operation often necessary Staged operation often necessary Recurrent disease possible Recurrent disease possible Residual disease harder to detect Residual disease harder to detect

54. Canal-Wall-Up

55. Canal-Wall-Up

56. Transcanal Anterior Atticotomy Indicated for limited cholesteatoma involving middle ear, ossicular chain, and epitympanum Indicated for limited cholesteatoma involving middle ear, ossicular chain, and epitympanum If extent of the cholesteatoma is unknown approach can be combined with CWU mastoidectomy or extended to CWD procedure If extent of the cholesteatoma is unknown approach can be combined with CWU mastoidectomy or extended to CWD procedure

57. Transcanal Anterior Atticotomy Procedure involves: Procedure involves: Elevation of tympanomeatal flap via endaural incision with removal of scutum to limits of the cholesteatoma Elevation of tympanomeatal flap via endaural incision with removal of scutum to limits of the cholesteatoma Aditus obliteration with muscle, fascia, cartilage or bone prior to reconstruction of the middle ear space Aditus obliteration with muscle, fascia, cartilage or bone prior to reconstruction of the middle ear space Reconstruction of lateral attic wall with bone or cartilage is optional Reconstruction of lateral attic wall with bone or cartilage is optional May lead to retraction disease and possible recurrence in patients with poor eustachian tube function May lead to retraction disease and possible recurrence in patients with poor eustachian tube function

58. Transcanal Anterior Atticotomy

59. Bondy Modified Radical Procedure Useful for attic and mastoid cholesteatoma that does not involve middle ear space and lateral to ossicles Useful for attic and mastoid cholesteatoma that does not involve middle ear space and lateral to ossicles Like modern modified radical mastoidectomy with exception that middle ear space is not entered Like modern modified radical mastoidectomy with exception that middle ear space is not entered Mastoid should be poorly developed for creation of a small cavity Mastoid should be poorly developed for creation of a small cavity Eustachian tube function should be adequate with intact pars tensa and aerated middle ear space. Eustachian tube function should be adequate with intact pars tensa and aerated middle ear space. Rarely used today Rarely used today

60. Canal Wall Status In 2002 Shohet et. al. reports recidivism rates with CWU as high as In 2002 Shohet et. al. reports recidivism rates with CWU as high as Adults = 36% Adults = 36% Children = 67% Children = 67% Approximately 30% of cases for cholesteatoma will be CWD Approximately 30% of cases for cholesteatoma will be CWD House Ear Institute 1982 House Ear Institute 1982 St. Joseph’s Hospital in Phoenix, AZ 2003 St. Joseph’s Hospital in Phoenix, AZ 2003

61. Canal Wall Status Syms et. al. examined surgical approach to manage cholesteatoma- retrospective review of 486 cases in 2003 Syms et. al. examined surgical approach to manage cholesteatoma- retrospective review of 486 cases in 2003 68.5% CWU: 31.5% CWD 68.5% CWU: 31.5% CWD CWU had “second look operation” CWU had “second look operation” Residual cholesteatoma found in 26.9% second procedures Residual cholesteatoma found in 26.9% second procedures Residual cholesteatoma found in 2.7% third procedures Residual cholesteatoma found in 2.7% third procedures

62. Canal Wall Status Kos et. al. examined long-term implications of CWD- retrospective review in 2004 Kos et. al. examined long-term implications of CWD- retrospective review in 2004 259 cases with follow-up range of 1 to 24 years (mean = 7 years) 259 cases with follow-up range of 1 to 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%

63. Canal Wall Status Kos et. al. (continued) Kos et. al. (continued) Hearing threshold Hearing 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 60dB in 2 patients Sensorineural hearing loss greater than 60dB in 2 patients Facial paralysis in 1 patient Facial paralysis in 1 patient Vertigo in 4 patients Vertigo in 4 patients

64. Novel Techniques In 2005 Gantz et. al. reported 130 cases of canal wall reconstruction tympanomastoidectomy with mastoid obliteration In 2005 Gantz et. al. reported 130 cases of canal wall reconstruction tympanomastoidectomy with mastoid obliteration 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 14.3% for first 42 patients Post-operative wound infection was 14.3% for first 42 patients Decreased rate to 4.5% in last 88 patients with 2 days of post-operative IV antibiotics Decreased rate to 4.5% in last 88 patients with 2 days of post-operative IV antibiotics

65. Novel Techniques Canal Wall Reconstruction technique Canal Wall Reconstruction technique Complete cortical mastoidectomy with opening of facial recess and removal of incus and malleus head Complete cortical mastoidectomy with opening of facial recess and removal of incus and malleus head Posterior canal wall skin elevated, annulus elevated Posterior canal wall skin elevated, annulus elevated Microsagittal saw used to cut posterior canal wall Microsagittal saw used to cut posterior canal wall Cholesteatoma removed Cholesteatoma removed Posterior canal wall bone replaced Posterior canal wall bone replaced Cortical bone chips used to block attic and mastoid from tympanum Cortical bone chips used to block attic and mastoid from tympanum Bone pate’ holds bone chips in place Bone pate’ holds bone chips in place

66. Novel Techniques

67. In 2005, Godinho et. al. reported 42 cases of Canal Wall Window (CWW) performed for pediatric cholesteatoma In 2005, Godinho et. al. reported 42 cases of Canal Wall Window (CWW) performed for pediatric cholesteatoma CWW compared to CWU and CWD CWW compared to CWU and CWD CWW converted to CWD in 14% CWW converted to CWD in 14% Dry ear results Dry ear results CWW = 94%, CWD = 92%, CWU = 90% CWW = 94%, CWD = 92%, CWU = 90% Recidivation rate at 1 year Recidivation 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 to CWU, rather than increased air-bone gap seen with CWD CWW provided similar post-operative hearing to CWU, rather than increased air-bone gap seen with CWD

68. Novel Techniques CWW effective for posterior superior cholesteatoma CWW effective for posterior superior cholesteatoma Increases visibility Increases visibility Conduit for instrument manipulation Conduit for instrument manipulation CWW technique CWW technique CWU mastoidectomy performed CWU mastoidectomy performed Lateral epitympanotomy Lateral epitympanotomy Slit drilled from lateral cortex to medial aspect of canal wall at annulus Slit drilled from lateral cortex to medial aspect of canal wall at annulus

69. Novel Techniques

70. CWW reconstruction CWW reconstruction Tragal cartilage placed in defect after scoring proximal perichondrium and cartilage Tragal cartilage placed in defect after scoring proximal perichondrium and cartilage Cortical bone graft may be used as an alternative Cortical bone graft may be used as an alternative

71. Novel Techniques

72. Complications

73. Cholesteatoma Sequelae Infection Infection Otorrhea Otorrhea Bone destruction Bone destruction Hearing loss Hearing loss Facial nerve paresis or paralysis Facial nerve paresis or paralysis Labyrinthine fistula Labyrinthine fistula Intracranial complications Intracranial complications Complications are caused by expansion and infection

74. Hearing Loss Conductive hearing loss: ossicular chain erosion (30%) Conductive hearing loss: ossicular chain erosion (30%) Erosion of lenticular process and/or stapes superstructure may produce 50dB conductive hearing loss Erosion of lenticular process and/or stapes superstructure may produce 50dB conductive hearing loss Hearing loss varies despite disease extent (natural myringostapediopexy, transmission of sound through cholesteatoma sac) Hearing loss varies despite disease extent (natural myringostapediopexy, transmission of sound through cholesteatoma sac) Sensorineural hearing loss: involvement of labyrinth Sensorineural hearing loss: involvement of labyrinth Following surgery, 30% have further impairment due to: Following surgery, 30% have further impairment due to: Extent of disease present Extent of disease present Complications in healing process Complications in healing process

75. Labyrinthine Fistula Incidence: as high as 10% Incidence: as high as 10% Symptom: Sensorineural hearing loss and/or vertigo induced by noise or pressure change Symptom: Sensorineural hearing loss and/or vertigo induced by noise or pressure change Common site: horizontal semicircular canal, basal turn of cochlea Common site: horizontal semicircular canal, basal turn of cochlea Diagnosis: Fine cut temporal bone CT (1mm) Diagnosis: Fine cut temporal bone CT (1mm) Management: modified radical mastoidectomy with management of matrix overlying fistula Management: modified radical mastoidectomy with management of matrix overlying fistula

76. Facial Paralysis May develop: May develop: Acutely secondary to infection Acutely secondary to infection Slowly from chronic expansion of cholesteatoma Slowly from chronic expansion of cholesteatoma Temporal bone CT: localize the nerve involvement Temporal bone CT: localize the nerve involvement Most common site: geniculate ganglion due to disease in the anterior epitympanum Most common site: geniculate ganglion due to disease in the anterior epitympanum Management: Needs immediate surgery Management: Needs immediate surgery Removal of cholesteatoma and infected material with decompression of the nerve (mastoidectomy, middle fossa approach) Removal of cholesteatoma and infected material with decompression of the nerve (mastoidectomy, middle fossa approach) Administration of intravenous antibiotics and high-dose steroids Administration of intravenous antibiotics and high-dose steroids Iatrogenic injury to the nerve during surgery should be immediately repaired with decompression of nerve proximal and distal to site of injury Iatrogenic injury to the nerve during surgery should be immediately repaired with decompression of nerve proximal and distal to site of injury

77. Intracranial Complications Potentially life-threatening Potentially life-threatening Incidence: as high as 1% Incidence: as high as 1% Complications Complications Periosteal abscess Periosteal abscess Lateral sinus thrombosis Lateral sinus thrombosis Intracranial abscess Intracranial abscess Meningitis Meningitis Symptom: Symptom: Suppurative malodorous otorrhea Suppurative malodorous otorrhea Chronic headache Chronic headache Fever Fever Otalgia Otalgia

78. Intracranial Complications Management: Management: Presence of mental status changes with nuchal rigidity or cranial neuropathies warrant neurosurgical consultation with urgent intervention Presence of mental status changes with nuchal rigidity or cranial neuropathies warrant neurosurgical consultation with urgent intervention Epidural abscess, subdural empyema, meningitis and cerebral abscesses should be treated immediately prior to definitive otologic management of ear disease Epidural abscess, subdural empyema, meningitis and cerebral abscesses should be treated immediately prior to definitive otologic management of ear disease

79. Conclusions Pathogenesis of cholesteatoma remains uncertain Pathogenesis of cholesteatoma remains uncertain Essential to possess basic knowledge of the important anatomic and functional characteristics of the middle ear for successful management of cholesteatomas Essential to possess basic knowledge of the important anatomic and functional characteristics of the middle ear for successful management of cholesteatomas Careful and thorough evaluations are the key to early diagnosis and treatment Careful and thorough evaluations are the key to early diagnosis and treatment Treatment is surgical with primary goal to eradicate disease and provide a safe and dry ear Treatment is surgical with primary goal to eradicate disease and provide a safe and dry ear Surgical approaches must be customized to each patient depending on extent of disease Surgical approaches must be customized to each patient depending on extent of disease Surgeon must be aware of serious and potentially life-threatening complications of cholesteatomas Surgeon must be aware of serious and potentially life-threatening complications of cholesteatomas

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81. What To Do Treatment should be tailored to case Treatment should be tailored to case Absolute CWD indications Absolute CWD indications Cholesteatoma in an only hearing ear Cholesteatoma in an only hearing ear Significant erosion of the posterior bony canal wall Significant erosion of the posterior bony canal wall History of vertigo suggesting a labyrinthine fistula History of vertigo suggesting a labyrinthine fistula Recurrent cholesteatoma after canal-wall-up surgery Recurrent cholesteatoma after canal-wall-up surgery Poor eustachian tube function Poor eustachian tube function Sclerotic mastoid with limited access to epitympanum Sclerotic mastoid with limited access to epitympanum No harm in starting CWU and converting to CWD as necessary No harm in starting CWU and converting to CWD as necessary Know your surgical limitations Know your surgical limitations

82. Quiz

83. Quiz 1. What space lies between the pars flaccida and #13? 1. What space lies between the pars flaccida and #13? A. Prussak’s space A. Prussak’s space B. Anterior pouch of Von Troeltsch B. Anterior pouch of Von Troeltsch C. Posterior pouch of Von Troeltsch C. Posterior pouch of Von Troeltsch D. The final frontier D. The final frontier

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

85. Quiz 3. What 2 major groups are cholesteatomas classified into (pick 2 letters)? 3. What 2 major groups are cholesteatomas classified into (pick 2 letters)? A. Acquired A. Acquired B. Infected B. Infected C. Expansive C. Expansive D. Congenital D. Congenital

86. Quiz 4. Possible adverse outcomes of surgical treatment of cholesteatomas are 4. Possible adverse outcomes of surgical treatment of cholesteatomas are A. Facial paralysis A. Facial paralysis B. Vertigo B. Vertigo C. Further hearing loss C. Further hearing loss D. Tinnitus D. Tinnitus E. All of the above E. All of the above

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

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

89. Quiz 7. Surgical treatment of cholesteatoma has a primary goal of hearing restoration 7. Surgical treatment of cholesteatoma has a primary goal of hearing restoration A. True A. True B. False B. False

90. Quiz 8. Surgical approaches must be customized to each patient depending on extent of disease 8. Surgical approaches must be customized to each patient depending on extent of disease A. True A. True B. False B. False

91. Quiz Bonus What is this a picture of? What is this a picture of? A. Cat eye A. Cat eye B. Snake skin B. Snake skin C. Tympanic membrane stained with osmium to show keratin patches C. Tympanic membrane stained with osmium to show keratin patches