Chronic Otitis Media and Cholesteatoma

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Chronic Otitis Media and Cholesteatoma

Otology/Audiology
Author Credentials 

Written By:
Noga Lipschitz, MD
International Advanced Otology Fellow
Department of Otolaryngology – Head and Neck Surgery
University of Cincinnati College of Medicine
Cincinnati, OH
E-mail: lipschna@ucmail.uc.edu

 

Edited By:
Ravi N. Samy, MD, FACS
Chief, Division of Otology/Neurotology
Program Director, Neurotology and International Advanced Otology Fellowships
Department of Otolaryngology – Head and Neck Surgery
University of Cincinnati College of Medicine
Cincinnati Children’s Hospital Medical Center
Neurosensory Disorders Center at University of Cincinnati Gardner Neuroscience Institute
Cincinnati, OH
E-mail: Ravi.Samy@UC.edu

 

 

 

Module Summary

Cholesteatoma is a non-neoplastic expansile and erosive epithelial-lined cyst filled with keratin and desquamated debris. Chronic otitis media without cholesteatoma is characterized by a chronic and multifactorial inflammatory response. Complications of untreated disease may include chronic otorrhea, hearing loss, vertigo, facial nerve paralysis, pain, headache, and intracranial complications. Diagnosis is primarily clinical, and imaging studies are used to assess the disease extent before surgery or to evaluate for complications. While chronic otitis media without cholesteatoma may be managed conservatively, treatment of cholesteatoma is surgical with the goal of achieving a safe, dry ear. Hearing improvement is a secondary goal and may be achieved with a planned second stage procedure. A variety of surgical approaches exists for treatment of chronic otitis media with and without cholesteatoma, and treatment is chosen based on disease extent as well as surgeon’s and patient’s preference. 

Module Learning Objectives 
  1. Describe the diagnosis of chronic otitis media with and without cholesteatoma. 
  2. Discuss the classification and pathogenesis of cholesteatoma.
  3. Review the management of chronic otitis media with and without cholesteatoma.
  4. List the surgical approaches used in the treatment of chronic otitis media and cholesteatoma.

 

Embryology

Learning Objectives 
  1. Be familiar with the current evidence on the pathogenesis of congenital cholesteatoma, as an embryonic remnant of epithelial tissue in the middle ear.
References 
  1. Michaels L. An epidermoid formation in the developing middle ear: possible source of cholesteatoma. J Otolaryngol. 1986;15(3):169-174.
  2. Meyer TA, Strunk Jr. CL, Lembert PR. Cholesteatoma. In: Johnson JT, Rosen CA, Bailey BJ, eds. Bailey's Head and Neck Surgery - Otolaryngology. Vol 2. Fifth edition. ed. Philadelphia, Pa.: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2014:2433-2446.
  3. Gopen Q. Pathology and Clinical Course of the Inflammatory Diseases of the Middle Ear. In: Gulya AJ, Minor LB, Poe D, eds. Glasscock-Shambaugh Surgery of the Ear. 6th ed. Shelton, Ct.: People's Medical Publishing House-USA; 2010:425-436.

 

Anatomy

Learning Objectives 
  1. Identify the scutum and explain its significance in patients with pars flaccida cholesteatoma.
  2. Define the boundaries of Prussak’s space. 
  3. Recognize the anatomic locations associated with high recidivism of cholesteatoma – oval window, sinus tympani, and anterior epitympanum

 

References 
  1. Meyer TA, Strunk Jr. CL, Lembert PR. Cholesteatoma. In: Johnson JT, Rosen CA, Bailey BJ, eds. Bailey's Head and Neck Surgery - Otolaryngology. Vol 2. Fifth edition. ed. Philadelphia, Pa.: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2014:2433-2446.

 

Pathogenesis

Learning Objectives 
  1. Explain the pathogenesis of congenital vs. acquired cholesteatoma.
  2. Describe the theories of the pathogenesis of acquired cholesteatoma.
  3. Describe the pathogenesis of external ear canal cholesteatoma.
  4. Discuss the pathogenesis of chronic otitis media without cholesteatoma.
  5. Recognize the role of secondary bacterial infection and biofilm formation in chronic otitis media with and without cholesteatoma. 
  6. Explain how eustachian tube dysfunction may contribute to the development of chronic otitis media and cholesteatoma.
References 
  1. Persaud R, Hajioff D, Trinidade A, et al. Evidence-based review of aetiopathogenic theories of congenital and acquired cholesteatoma. J Laryngol Otol 2007;121(11):1013-1019.
  2. Galli J, Calo L, Giuliani M, et al. Biofilm's Role in Chronic Cholesteatomatous Otitis Media: A Pilot Study. Otolaryngol Head Neck Surg 2016;154(5):914-916.
  3. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.
  4. Meyer TA, Strunk Jr. CL, Lembert PR. Cholesteatoma. In: Johnson JT, Rosen CA, Bailey BJ, eds. Bailey's Head and Neck Surgery - Otolaryngology. Vol 2. Fifth edition. ed. Philadelphia, Pa.: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2014:2433-2446.
  5. Gopen Q. Pathology and Clinical Course of the Inflammatory Diseases of the Middle Ear. In: Gulya AJ, Minor LB, Poe D, eds. Glasscock-Shambaugh Surgery of the Ear. 6th ed. Shelton, Ct.: People's Medical Publishing House-USA; 2010:425-436.
  6. Sharon JD, Chole RA. Microbiology of Chronic Ear Disease. In: Dornhoffer JL, Gluth Mb, eds. The Chronic Ear. New York, NY: Thieme; 2016:26-31.
  7. Kalcioglu MT, Guldemir D, Unaldi O, et al. Metagenomics Analysis of Bacterial Population of Tympanosclerotic Plaques and Cholesteatomas. Otolaryngol Head Neck Surg 2018;159(4):724-732.
  8. Vila PM, Thomas T, Liu C, Poe D, Shin JJ. The Burden and Epidemiology of Eustachian Tube Dysfunction in Adults. Otolaryngol Head Neck Surg 2017;156(2):278-284.

 

Basic Science

Learning Objectives 
  1. Describe the role of proteolytic enzymes, bacterial endotoxins, and osteoclasts within the cholesteatoma matrix in bony erosion and middle ear and mastoid damage.
  2. Recognize the migratory properties of tympanic membrane and EAC epithelium.

 

References 
  1. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.
  2. Meyer TA, Strunk Jr. CL, Lembert PR. Cholesteatoma. In: Johnson JT, Rosen CA, Bailey BJ, eds. Bailey's Head and Neck Surgery - Otolaryngology. Vol 2. Fifth edition. ed. Philadelphia, Pa.: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2014:2433-2446.

 

Incidence

Learning Objectives 
  1. Note that the incidence and prevalence of cholesteatoma are not well studied.
  2. Recognize that the incidence of chronic otitis media is higher in children compared with adults, whereas the incidence of cholesteatoma is higher in adults.
  3. Review the differences in the incidence and prevalence of chronic otitis media among different areas and ethnic groups around the world.

 

 

References 
  1. Buzi A, Gluth Mb, Black B. Chronic Ear disease in the Modern Era: Evolution of Treatment, Epidemiology, and Classification. In: Dornhoffer JL, Gluth Mb, eds. The Chronic Ear. New York, NY: Thieme; 2016:2-7.
  2. Gopen Q. Pathology and Clinical Course of the Inflammatory Diseases of the Middle Ear. In: Gulya AJ, Minor LB, Poe D, eds. Glasscock-Shambaugh Surgery of the Ear. 6th ed. Shelton, Ct.: People's Medical Publishing House-USA; 2010:425-436.
  3. Homøe P, Kværner K, Casey JR, et al. Panel 1: Epidemiology and Diagnosis. Otolaryngol Head Neck Surg. 2017;156(4_suppl): S1-S21.

 

Genetics

Learning Objectives 
  1. Understand there is limited evidence on the role of genetics in the development of cholesteatoma.
  2. Recognize there is growing evidence on the role of genetic predisposition to chronic otitis media. 

 

References 
  1. Jennings BA, Prinsley P, Philpott C, Willis G, Bhutta MF. The genetics of cholesteatoma. A systematic review using narrative synthesis. Clin Otolaryngol. 2018;43(1):55-67.
  2. Lin J, Hafren L, Kerschner J, et al. Panel 3: Genetics and Precision Medicine of Otitis Media. Otolaryngol Head Neck Surg. 2017;156(4_suppl): S41-S50.
  3. MacArthur CJ, Wilmot B, Wang L, Schuller M, Lighthall J, Trune D. Genetic susceptibility to chronic otitis media with effusion: candidate gene single nucleotide polymorphisms. Laryngoscope. 2014;124(5):1229-1235.

 

Patient Evaluation

Learning Objectives 
  1. Review components of the patient’s history that may identify risk factors for development of chronic otitis media and cholesteatoma and their complications.
  2. Discuss the relevant physical examination, including microscopic and / or endoscopic examination of the ear, tuning fork examination, facial nerve and other cranial nerve assessment and basic vestibular examination.
  3. Recognize that audiometric assessment should be a part of the evaluation of every patient with chronic otitis media and cholesteatoma.
  4. Review the diagnosis of chronic otitis media and cholesteatoma, emphasizing this is a clinical diagnosis primarily based on the history and physical examination.
  5. Discuss the role of imaging studies in the evaluation of chronic otitis media and cholesteatoma

 

References 
  1. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.
  2. Meyer TA, Strunk Jr. CL, Lembert PR. Cholesteatoma. In: Johnson JT, Rosen CA, Bailey BJ, eds. Bailey's Head and Neck Surgery - Otolaryngology. Vol 2. Fifth edition. ed. Philadelphia, Pa.: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2014:2433-2446.
  3. Lin J, Staecker H. Evaluation and Nonsurgical Management of Chronic Suppurative Otitis Media. In: Dornhoffer JL, Gluth Mb, eds. The Chronic Ear. New York, NY: Thieme; 2016:118-124.
  4. van Egmond SL, Stegeman I, Grolman W, Aarts MC. A Systematic Review of Non-Echo Planar Diffusion-Weighted Magnetic Resonance Imaging for Detection of Primary and Postoperative Cholesteatoma. Otolaryngol Head Neck Surg 2016;154(2):233-240.

 

Imaging

Learning Objectives 
  1. Describe the role of imaging studies in the evaluation of chronic otitis media and cholesteatoma.
  2. Recognize that high resolution temporal bone computed tomography (CT) without contrast provides anatomic information on middle ear and mastoid involvement, bony erosion, labyrinthine fistula, and tegmen dehiscence.
  3. Discuss the role of magnetic resonance imaging (MRI) and specifically the non echo-planar (non-EPI) diffusion weighted imaging (DWI) in the diagnosis of residual and recurrent cholesteatoma. 

 

References 
  1. van Egmond SL, Stegeman I, Grolman W, Aarts MC. A Systematic Review of Non-Echo Planar Diffusion-Weighted Magnetic Resonance Imaging for Detection of Primary and Postoperative Cholesteatoma. Otolaryngol Head Neck Surg 2016;154(2):233-240.
  2. Gurgel RK, Wiggins RH, Clough. S. Radiographic Evaluation of Chronic Ear Disease. In: Dornhoffer JL, Gluth Mb, eds. The Chronic Ear. New York, NY: Thieme; 2016:88-96.
  3. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.

 

Pathology

Learning Objectives 
  1. Recognize that cholesteatoma is a non-neoplastic lesion lined by stratified squamous epithelium forming the cholesteatoma matrix and containing keratin and desquamated debris. 
References 
  1. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.
  2. Meyer TA, Strunk Jr. CL, Lembert PR. Cholesteatoma. In: Johnson JT, Rosen CA, Bailey BJ, eds. Bailey's Head and Neck Surgery - Otolaryngology. Vol 2. Fifth edition. ed. Philadelphia, Pa.: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2014:2433-2446.

 

Treatment

Learning Objectives 
  1. Recognize that treatment of cholesteatoma is primarily surgical.
  2. Understand that treatment of chronic otitis media without cholesteatoma may be managed conservatively or may necessitate surgical intervention.  

 

References 
  1. Trinidade A, Page JC, Dornhoffer JL. Therapeutic Mastoidectomy in the Management of Noncholesteatomatous Chronic Otitis Media: Literature Review and Cost Analysis. Otolaryngol Head Neck Surg 2016;155(6):914-922.
  2. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.

Medical Therapies

Learning Objectives 
  1. Recognize that dry ear precautions should be used in chronic otitis media with and without cholesteatoma.
  2. Note that chronic otitis media without cholesteatoma may be managed conservatively with topical antibiotic preparations with or without steroids.
  3. Cite that topical antibiotic preparations may reduce otorrhea in cholesteatoma.

 

References 
  1. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.

Pharmacology

Learning Objectives 
  1. Be familiar with the available topical agents used in the treatment of chronic otitis media with and without cholesteatoma:
  • Quinolone containing otic or ophthalmic suspensions
  • Powder preparations containing antibiotic, antifungal, steroid and boric acid
  • Topical acetic acid 
  • Use of preparations containing aminoglycosides should be limited due to ototoxicity

 

References 
  1. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.
  2. Lin J, Staecker H. Evaluation and Nonsurgical Management of Chronic Suppurative Otitis Media. In: Dornhoffer JL, Gluth Mb, eds. The Chronic Ear. New York, NY: Thieme; 2016:118-124.
  3. Kutz Jr. JW, Roland PS, Isaacson B. Office Management of Tympanic Membrane Perforation and the Draining Ear. In: Gulya AJ, Minor LB, Poe D, eds. Glasscock-Shambaugh Surgery of the Ear. 4th ed. Shelton, Ct.: People's Medical Publishing House-USA; 2010:86-97.
  4. Ozagar A, Koc A, Ciprut A, et al. Effects of topical otic preparations on hearing in chronic otitis media. Otolaryngol Head Neck Surg 1997;117(4):405-408. 

 

Surgical Therapies

Learning Objectives 
  1. Recognize that the primary surgical goal of chronic otitis media with and without cholesteatoma is achieving a safe and dry ear. Hearing improvement is a secondary goal that is often addressed in a staged procedure. 
  2. Discuss surgical approaches:
  • Microscopic, endoscopic, or combined approaches
  • Transcanal, endaural, and transmastoid approaches
  1. Recognize the role of tympanoplasty in eliminating cholesteatoma limited to the middle ear:
  • Discuss how the use of endoscopes may improve surgical results and reduce residual disease (i.e. through improved exposure with angled endoscopes). 
  • Describe the use of atticotomy for epitympanic disease
  1. Review the differences between canal wall up (CWU) and canal wall down (CWD) mastoidectomies.
  2. Discuss the different CWU approaches: 
  • Complete mastoidectomy
  • Facial recess approach (mastoidectomy with posterior tympanotomy) and extended facial recess approach. 
  1. Explain the differences between the different CWD approaches
  • Radical mastoidectomy
  • Modified radical mastoidectomy 
  • Recognize that the Bondy procedure is a specific case of modified radical mastoidectomy
  1. Describe the canal wall reconstruction (CWR) with or without mastoid obliteration.
  2. Discuss the follow-up options of CWU or CWR approaches, including observation, a 2nd look surgery, or non-Epi DWI MRI.

 

References 
  1. Trinidade A, Page JC, Dornhoffer JL. Therapeutic Mastoidectomy in the Management of Noncholesteatomatous Chronic Otitis Media: Literature Review and Cost Analysis. Otolaryngol Head Neck Surg 2016;155(6):914-922. 
  2. Wilson KF, Hoggan RN, Shelton C. Tympanoplasty with intact canal wall mastoidectomy for cholesteatoma: long-term surgical outcomes. Otolaryngol Head Neck Surg 2013;149(2):292-295.
  3. Stevens SM, Walters ZA, Babo K, et al. Canal reconstruction mastoidectomy: Outcomes comparison following primary versus secondary surgery. Laryngoscope 2019 (published ahead of print).
  4. Hunter JB, Zuniga MG, Sweeney AD, et al. Pediatric Endoscopic Cholesteatoma Surgery. Otolaryngol Head Neck Surg 2016;154(6):1121-1127.
  5. Cohen MS, Basonbul RA, Kozin ED, Lee DJ. Residual Cholesteatoma during Second-Look Procedures following Primary Pediatric Endoscopic Ear Surgery. Otolaryngol Head Neck Surg 2017;157(6):1034-1040.
  6. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.
  7. Meyer TA, Strunk Jr. CL, Lembert PR. Cholesteatoma. In: Johnson JT, Rosen CA, Bailey BJ, eds. Bailey's Head and Neck Surgery - Otolaryngology. Vol 2. Fifth edition. ed. Philadelphia, Pa.: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2014:2433-2446.
  8. Gantz BJ, Eppsteiner RW, Gubbels SP, Wilkinson EP. Canal Wall Reconstruction Tympanomastoidectomy. In: Gulya AJ, Minor LB, Poe D, eds. Glasscock-Shambaugh Surgery of the Ear. 4th ed. Shelton, Ct.: People's Medical Publishing House-USA; 2010:144-153.
  9. Syms III CA, Syms MJ, Sheehy JL. Mastoidectomy: Intact Canal Wall Procedure. In: Gulya AJ, Minor LB, Poe D, eds. Glasscock-Shambaugh Surgery of the Ear. 4th ed. Shelton, Ct.: People's Medical Publishing House-USA; 2010:163-173.
  10. Arriaga MA. Mastoidectomy: Canal Wall Down Procedure. In: Gulya AJ, Minor LB, Poe D, eds. Glasscock-Shambaugh Surgery of the Ear. 4th ed. Shelton, Ct.: People's Medical Publishing House-USA; 2010:174-184.

 

Staging

Learning Objectives 
  1. Be familiar with the classification systems for middle ear atelectasis:
  • Sadé classification for pars tensa retraction
  • Tos classification for pars flaccida retraction

 

References 
  1. Sadé J, Berco E. Atelectasis and secretory otitis media. Ann Otol Rhinol Laryngol. 1976;85(2 Suppl 25 Pt 2):66-72.
  2. Tos M, Poulsen G. Attic retractions following secretory otitis. Acta Otolaryngol. 1980;89(5-6):479-486.

 

Case Studies

  1. A 35 year-old female with a history of chronic ear infections in childhood presents with a 6 month history of right hearing loss, otalgia and foul smelling otorrhea. What diagnosis do you suspect? What ancillary tests would you order? Which management options would you discuss with the patient?
  2. A 25 year-old male with a history of recurrent acute otitis media and prior pressure equalizing tube insertion many years ago, presents with a left sided otorrhea for 6 months. You diagnose a tympanic membrane perforation, place mastoid powder and instruct on dry ear precaution. He returns after 3 weeks with continued otorrhea. What is your diagnosis? What would be your next step in the management of this patient? Discuss the role of imaging in the evaluation of this patient

 

Complications

Learning Objectives 
  1. Recognize that the complications of cholesteatoma are similar to the complications of surgery to eradicate cholesteatoma.
  2. List the complications and emergencies of cholesteatoma:
  • Hearing loss
  • Vertigo
  • Nerve associated complications (facial nerve and chorda tympani nerve)
  • Labyrinthine and cochlear fistula
  • Tegmen dehiscence with associated meningoencephalocele, cerebrospinal fluid (CSF) leak 
  • Intracranial infection (meningitis, epidural abscess, brain abscess)
  1. Describe the management of these complications.

 

References 
  1. Prasad SC, Shin SH, Russo A, Di Trapani G, Sanna M. Current trends in the management of the complications of chronic otitis media with cholesteatoma. Curr Opin Otolaryngol Head Neck Surg 2013;21(5):446-454.
  2. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.
  3. Meyer TA, Strunk Jr. CL, Lembert PR. Cholesteatoma. In: Johnson JT, Rosen CA, Bailey BJ, eds. Bailey's Head and Neck Surgery - Otolaryngology. Vol 2. Fifth edition. ed. Philadelphia, Pa.: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2014:2433-2446.
  4. Carlson ML, Haynes DS, Wanna GB. Intratemporal and intracranial Complications of Otitis Media. In: Dornhoffer JL, Gluth Mb, eds. The Chronic Ear. New York, NY: Thieme; 2016:125-132.
  5. Wiet RJ, Harvey SA, Littlefield PD. Complication of Surgery for Chronic Otitis Media. In: Gulya AJ, Minor LB, Poe D, eds. Glasscock-Shambaugh Surgery of the Ear. 4th ed. Shelton, Ct.: People's Medical Publishing House-USA; 2010:190-205.

 

Review

Review Questions 
  1. What is cholesteatoma?
  2. How is the diagnosis of chronic otitis media with and without cholesteatoma made?
  3. What is the role of imaging studies in the evaluation of chronic otitis media with and without cholesteatoma?
  4. What is the treatment of cholesteatoma?
  5. What surgical approaches are used for the treatment of chronic otitis media with and without cholesteatoma?

 

References 
  1. Chole RA. Chronic Otitis Media, Mastoiditis, and Petrositis. In: Flint PW, Cummings CW, eds. Cummings Otolaryngology Head & Neck Surgery. Vol 2. 6th ed. Philadelphia, PA: Mosby/Elsevier; 2014:2139-2155.
  2. Meyer TA, Strunk Jr. CL, Lembert PR. Cholesteatoma. In: Johnson JT, Rosen CA, Bailey BJ, eds. Bailey's Head and Neck Surgery - Otolaryngology. Vol 2. Fifth edition. ed. Philadelphia, Pa.: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2014:2433-2446.

 

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