Epitympanic cholesteatoma

Case contributed by Alasdair Grenness , 2 Dec 2019
Diagnosis certain
Changed by Alasdair Grenness, 3 Dec 2019

Updates to Case Attributes

Status changed from draft to pending review.
Body was changed:

Cholesteatomas are epidermal inclusion cysts of either the ear canal, middle ear or mastoid. They contain desquamated debris, mainly keratin, from their keratinising squamous epithelial lining. They do not contain cholesterol, but rather their yellow-white keratin flakes resemble cholesterol crystals.1 

Diagnosis is typically made on clinical examination. However imaging modalities such as high resolution computed tomography (CT) scans and magnetic resonance imaging (MRI) can suggest the presence of cholesteatoma and complement the clinical examination.1 Diffusion weighted MRI has been shown to be a reliable technique for the detection of temporal bone cholesteatoma andis used when clinical surveillance of cholesteatoma is no longer possible (classically after reconstruction surgery post cholesteatoma removal).2 

High resolution CT is useful for operative planning and recommended before both primary and revision surgery. Eradication is currently only possible with surgical resection. Primary treatment goals include removal of all disease. Reconstruction of damaged hearing mechanisms usually happens at a second stage procedure. Surgical approaches range from attitcotomy (transcanal), canal wall up mastoidectomy (where by(whereby the mastoid is resected to gain access to the middle ear but the posterior canal wall is left in tact) or canal wall down mastoidectomy (whereby the posterior canal wall is drilled away to give greater access to the middle ear).1

  • -<p>Cholesteatomas are epidermal inclusion cysts of either the ear canal, middle ear or mastoid. They contain desquamated debris, mainly keratin, from their keratinising squamous epithelial lining. They do not contain cholesterol, but rather their yellow-white keratin flakes resemble cholesterol crystals.<sup>1</sup> </p><p>Diagnosis is typically made on clinical examination. However imaging modalities such as high resolution computed tomography (CT) scans and magnetic resonance imaging (MRI) can suggest the presence of cholesteatoma and complement the clinical examination.<sup>1</sup> Diffusion weighted MRI has been shown to be a reliable technique for the detection of temporal bone cholesteatoma and is used when clinical surveillance of cholesteatoma is no longer possible (classically after reconstruction surgery post cholesteatoma removal).<sup>2</sup> </p><p>High resolution CT is useful for operative planning and recommended before both primary and revision surgery. Eradication is currently only possible with surgical resection. Primary treatment goals include removal of all disease. Reconstruction of damaged hearing mechanisms usually happens at a second stage procedure. Surgical approaches range from attitcotomy (transcanal), canal wall up mastoidectomy (where by mastoid is resected but the posterior canal wall is left in tact) or canal wall down mastoidectomy (whereby the posterior canal wall is drilled away to give greater access to the middle ear).<sup>1</sup></p>
  • +<p>Cholesteatomas are epidermal inclusion cysts of either the ear canal, middle ear or mastoid. They contain desquamated debris, mainly keratin, from their keratinising squamous epithelial lining. They do not contain cholesterol, but rather their yellow-white keratin flakes resemble cholesterol crystals.<sup>1</sup> </p><p>Diagnosis is typically made on clinical examination. However imaging modalities such as high resolution computed tomography (CT) scans and magnetic resonance imaging (MRI) can suggest the presence of cholesteatoma and complement the clinical examination.<sup>1</sup> Diffusion weighted MRI has been shown to be a reliable technique for the detection of temporal bone cholesteatoma and is used when clinical surveillance of cholesteatoma is no longer possible (classically after reconstruction surgery post cholesteatoma removal).<sup>2</sup> </p><p>High resolution CT is useful for operative planning and recommended before both primary and revision surgery. Eradication is currently only possible with surgical resection. Primary treatment goals include removal of all disease. Reconstruction of damaged hearing mechanisms usually happens at a second stage procedure. Surgical approaches range from attitcotomy (transcanal), canal wall up mastoidectomy (whereby the mastoid is resected to gain access to the middle ear but the posterior canal wall is left in tact) or canal wall down mastoidectomy (whereby the posterior canal wall is drilled away to give greater access to the middle ear).<sup>1</sup></p>

References changed:

  • 2.) Ilica AT, Hidir Y, Bulakbasi N, et al. HASTE diffusion weighted MRI for the reliable detection of cholesteatoma. Diagn Interv Radiol 18:153, 2011

Updates to Freetext Attributes

Description was changed:

30 year old female of South Asian descent referred to the emergency department by her general practitioner over concern of fungal otitis externa. She presented with a week of bloody and purulent right otorrhea not improving with topical antibiotics. She reported two previous episodes of otorrhea within the last 6 months. There was no history of previous ear disease or surgery. Upon toileting of a large amount of purulent debris and skin from the ear canal, evaluation of the tympanic membrane could be made and a conductive hearing loss confirmed on tuning fork testing. 

Updates to Study Attributes

Findings was changed:

There is clear erosion of the superior aspect of the ear canal into the attic of the middle ear with what appears to be moist keratin. The head of the malleus appears to be eroded. The pars tensa of the tympanic membrane appears intact and the chorda tympani appears unaffected. It is difficult to establish where the pars flaccida currently is positioned. 

Such appearance of otoscopy is pathognomonic for cholesteatoma (classically epitympanic) with concurrent otitis media(likely chronic supporative otitis media). 

Further management involves first treating the localised infection with topical anitbiotics. Second audiologyan audiogram should be performed to establish the degree of hearing loss associated. Finally a CT PTB is arranged to establish extend of disease and for surgical planning rather than diagnosis.  

Images Changes:

Image Photo (Image 1: Otoscopy of right tympanic membrane with 30 degree endoscope) ( update )

Perspective changed from Image 1: Otoscopy of Right Tympanic Membrane with 30 degree endoscope to Image 1: Otoscopy of right tympanic membrane with 30 degree endoscope.

Image Photo (Image 2: Otoscopy of right tympanic membrane with 30 degree endoscope) ( update )

Perspective changed from Image 2: Otoscopy of Right Tympanic Membrane with 30 degree endoscope to Image 2: Otoscopy of right tympanic membrane with 30 degree endoscope.

Image Photo (Image 3: Otoscopy of right tympanic membrane with 30 degree endoscope with labels) ( update )

Perspective changed from Image 3: Otoscopy of Right Tympanic Membrane with 30 degree endoscope with labels to Image 3: Otoscopy of right tympanic membrane with 30 degree endoscope with labels.

Image Photo (Image 4: Normal tympanic membrane. Modified from: Michael Hawke MD - Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=40796102) ( update )

Perspective changed from Image 4: Normal Tympanic Membrane. Modified from: Michael Hawke MD - Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=40796102 to Image 4: Normal tympanic membrane. Modified from: Michael Hawke MD - Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=40796102.

Updates to Study Attributes

Findings was changed:

The is complete opacification of the right epitympanic space including Prussak's space. The anterior aspect of the right epitympanic space appears expanded and there is erosion of the anterior portion of the scutum (compare with clinical imaging above). There is also erosion of the malleus head and long process of the incus.  The tegmen tympani appears intact. 

The mastoid is poorly pneumatised, sclerotic and fully opacified, an indicator of long standing middle ear disease. The cochlea, vestibule, semicircular canals and facial nerve canal appear intact. 

Such imagingImaging confirms epitympanic cholesteatoma

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