538402
research-article2014
AORXXX10.1177/0003489414538402Annals of Otology, Rhinology & LaryngologyZhu et al
Article
Recurrent Cholesteatoma With Massive Extension to Temporoparietal Scalp: Case Report and Literature Review
Annals of Otology, Rhinology & Laryngology 2014, Vol. 123(11) 754–757 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0003489414538402 aor.sagepub.com
Vivian F. Zhu, BS1, Brandon Isaacson, MD1, Jason Mull, MD2, and Larry L. Myers, MD, FACS1
Abstract Background: Cholesteatomas are locally destructive collections of epithelial debris arising from temporal bone squamous epithelium. Recurrences may occur after removal and are typically located within the temporal bone. Objective: This study aimed to report a case of a massive, recurrent cholesteatoma with extension to temporoparietal scalp in a 37-year-old woman. Methods: Case report with literature review. Results: The patient underwent complete excision of a well-circumscribed left temporal mass, intraoperatively identified to arise from the middle ear and to contain keratin debris. Conclusion: We report a case of recurrent cholesteatoma with massive extension to temporoparietal scalp. Clinical suspicion of recurrent cholesteatoma should remain in the differential diagnosis of temporal mass with prior history of cholesteatoma. Keywords recurrent cholesteatoma, head and neck mass
Introduction Cholesteatomas, classified as either congenital or acquired, are epidermal inclusion cysts of the middle ear or mastoid. They most commonly originate within the temporal bone and often present with hearing loss and purulent otorrhea. The prevalence of cholesteatoma is about 3 in 100 000 children and 12 in 100 000 adults.1 Acquired cholesteatomas are far more common than congenital cholesteatomas, which constitute only 2% to 5% of all cholesteatomas.2 Cholesteatomas can progress through distinct pathways in the temporal bone, such as eroding through the mastoid bone and enlarging in the posterior fossa, penetrating into petrous bone, staying within the middle ear, or extending out to the external auditory canal. If left untreated, cholesteatoma growth can result in severe middle ear destruction—either ossicular destruction or semicircular canal disruptions.3,4 There can be tegmen destruction as well as hearing loss secondary to ossicle erosion.4 The cholesteatoma may develop intratemporally, causing symptoms of an infratemporal lesion, such as acute mastoiditis or lateral sinus thrombosis.5 Other, less likely complications of a cholesteatoma include paralysis of the facial nerve, fistula of the semicircular canal, or perforation of the pars flacida or pars tensa.3
We report the case of acquired, recurrent cholesteatoma extending to the subcutaneous tissues of the temporoparietal region.
Case Report A female patient with Trisomy 21 underwent a canal wall down left mastoidectomy and tympanoplasty for chronic otitis media at a children’s hospital when she was 9 years old. She did not have a second look procedure. She presented 26 years later to another hospital complaining of a progressively enlarging left temporal mass, intermittent left otorrhea, and long-standing history of hearing loss. She denied dysphagia, odynophagia, voice changes, weight loss, radiating otalgia, fever, night sweats, chills, cough and 1
Department of Otolaryngology–Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, Texas, USA 2 Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA Corresponding Author: Larry L. Myers, MD, FACS, Department of Otolaryngology–Head and Neck Surgery, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-9035, USA. Email: [email protected]
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Figure 2. Completely excised recurrent cholesteatoma.
Figure 1. Preoperative coronal T1-weighted magnetic resonance image with contrast demonstrating recurrent cholesteatoma with superior extension to left temporoparietal scalp.
hemoptysis, pain, or vertigo. Physical examination revealed a healthy woman with Trisomy 21 facies. There was a soft, 8 × 6 cm left temporal mass in the subcutaneous tissue. The overlying skin was intact without ulceration or local alopecia. Otoscopy demonstrated a dry left canal wall down cavity without significant epithelial debris and a patent right external auditory canal with a normal tympanic membrane. The remainder of her otolaryngologic examination was within normal limits. Magnetic resonance (MR) T1-weighted imaging with contrast revealed a 8.0 × 6.3 × 2.0 cm (100.8 mL) subcutaneous, fluid intensity, minimally peripherally enhancing lesion with a single internal enhancing septation. There was minimal fluid within the residual left mastoid air cells. There was no definite evidence of connection between the intracranial space and the fluid collection to suggest pseudomeningocele (see Figure 1). The patient was given a provisional diagnosis of lymphocele at the outside hospital and was supported by MR findings. The patient and her family refused fine needle aspiration and incisional biopsy for definitive diagnosis. After several months of deliberation, they agreed to surgery and the patient was taken to the operating room for excisional biopsy via a transtemporal approach. A vertical incision was made in the hear-bearing scalp. The well-encapsulated mass was identified in the subcutaneous tissue. Blunt dissection continued from a superior to inferior direction through the temporalis muscle to the root of the pinna. The capsule was violated near its origin and was
Figure 3. Hematoxylin and eosin stain 100X. Squamous lined cyst with keratinized debris.
found to be filled with squamous debris. The entire temporal mass was removed en bloc (see Figure 2). There were no intraoperative complications. Histopathologic analysis of the mass demonstrated an epithelial lined cyst with squamous debris consistent with cholesteatoma (see Figure 3). An audiogram was performed 2 weeks postoperatively and demonstrated normal hearing through 4000 Hz sloping to a moderate hearing loss at 8000 Hz in the right ear and a moderate rising to mild 500 to 2000 Hz sloping to profound primarily conductive hearing loss in the left ear. Pure tone average/speech reception threshold for right and left ears, respectively, were 8/15 dB SD 100% and 33/45 dB SD 100%. Tympanograms were type A bilaterally. A postoperative temporal bone computed tomography (CT) scan revealed a left tegmen defect and a dehiscent
Downloaded from aor.sagepub.com at UNIV OF MASSACHUSETTS on March 17, 2015
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Annals of Otology, Rhinology & Laryngology 123(11)
superior semicircular canal. A subsequent MR with contrast revealed no enhancing lesions or masses in the brainstem, cerebellopontine angle, internal auditory canal, or inner or middle ear. There was no evidence of restricted diffusion. After 2 years of follow-up, the patient is doing well with no evidence of disease.
Discussion Cholesteatomas are composed of keratinizing squamous epithelial matrix surrounding an adjacent subepithelial connective perimatrix bounded by mucous cuboidal epithelial cells that contain keratin.6 Eustachian tube dysfunction with resultant tympanic membrane retraction from negative middle ear pressure is the most common inciting event in cholesteatoma formation. Cholesteatomas may also arise from trauma or infection that has improperly healed, causing the epithelial cells to invaginated, leading to uncontrolled growth.7 A critical component involved in almost all cases of cholesteatoma is inflammation. Inflammation is heavily involved in all aspects of cholesteatoma onset, development, growth, and recurrence, particularly of the tympanic membrane and the middle ear. Cholesteatomas have also been partially associated with abnormal wound healing due to a related enhanced fibroblast collagen overproduction and augmented levels of TGF-β.8 Disease may progress to involve ossicular erosion that results in conductive hearing loss, but in rare cases, sensorineural hearing loss and vestibular function loss can occur from otic capsule erosion or labyrinthitis. Typically, acquired cholesteatomas present with chronic or recurrent otorrhea and crust formation that can often cover the mouth of the cholesteatoma. Our case was unique for several reasons. Our patient did not present with otorrhea or a retraction pocket in the tympanic membrane. The significance of this case is the rare temporoparietal location and massive size of a recurrent cholesteatoma. Despite its size, a complete resection of the recurrent disease was achieved with no evidence of recurrence or other complications with 2 years of follow-up after surgery. The extratemporal location in this case likely occurred secondary to implantation of epithelial cells producing a cholesteatoma matrix during the initial surgery, performed in 1983. The location within the temporalis muscle supports an etiology of iatrogenic implantation because there are no epithelial rests within mesodermally derived tissue. The precise reason that the patient did not have sustained follow-up after her initial surgery is unknown. The patient’s health care decisions were made by both her parents and herself. Presenting to another hospital more than 2 decades after her initial surgery evidenced a major gap in her continuity of care. The difficulty or inability to obtain her medical records containing a history of cholesteatoma and the
lack of middle ear findings contributed to the misdiagnosis of her enlarging temporal mass. Trisomy 21 is the most common chromosomal abnormality, characterized by mental disability and various other medical problems including otologic diseases.9 One out of 670 to 770 children are born with Down syndrome with a 38% to 78% incidence of hearing loss.10,11 Down syndrome patients are often affected with chronic ear disease such as otitis media, which is a chronic inflammatory infection of the middle ear.10 Recidivism is a well-known feature of cholesteatoma that necessitates long-term follow-up. Staging cholesteatoma surgery has been used for a number of years to improve disease eradication in patients undergoing canal wall up tympanomastoidectomy or middle ear reconstruction in canal wall down procedures. Clinical examination and medical imaging are both used to monitor for recidivistic disease. Temporal bone CT is helpful in identifying recidivistic cholesteatoma but lacks specificity as it is unable to differentiate between scar tissue, fluid, granulation, and cholesteatoma.12 A number of magnetic resonance imaging (MRI) sequences have been analyzed for their ability to detect recidivistic cholesteatoma, including delayed gadolinium imaging, echo planar diffusion weighted imaging (EPI DWI), and non-echo planar diffusion weighted imaging (non-EPI DWI). Diffusion weighted MRI has been shown to be both sensitive and specific in identifying recidivistic cholesteatoma. Non-EPI DWI sequences have a sensitivity, specificity, positive, and negative predictive value of up to 97%. Non-EPI DWI MRI has the ability to detect recidivistic disease as small as 2 mm. Mass like recidivistic disease (keratin pearl) is readily identified with non-EPI DWI MRI, whereas a thin layer of squamous epithelial matrix is difficult to visualize. Diffusion weighted MRI requires expertise in image acquisition and interpretation, as differentiating true restricted diffusion from bone-soft tissue artifact and T2 shine through can be challenging.13
Conclusion We report a case of a massive, recurrent cholesteatoma with superior extension to temporoparietal scalp region. The patient’s presentation after more than 2 decades without follow-up allowed for an observation of a natural history of this disease process. This patient was managed with complete surgical excision. She has no evidence of recurrence after 2 years of follow-up. This case illustrates the need for diligent follow-up after surgery for cholesteatoma to detect recurrence. A complete history and a high index of suspicion are necessary to establish the diagnosis of cholesteatoma in patients presenting with a periauricular or temporal mass with a prior history of cholesteatoma.
Downloaded from aor.sagepub.com at UNIV OF MASSACHUSETTS on March 17, 2015
757
Zhu et al Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Jones RC, Demetroulakos J, Mugge RE. Cholesteatoma. https://www.clinicalkey.com/#!/ContentPlayerCtrl/doPlay Content/21-s2.0-6020108/{%22sectionId%22:%22section ZC18394D_267_2A6%22}. Published June 16, 2013. Accessed May 22, 2014. 2. Janardhan N, Nara J, Peram I, Palukuri S, Chinta A, Satna K. Congenital cholesteatoma of temporal bone with Bezold’s abscess: case report. Indian J Otolaryngol Head Neck Surg. 2012;64(1):97-99. 3. Sadé J, Fuchs C. Cholesteatoma: ossicular destruction in adults and children. J Laryngol Otol. 1994;108(7):541-544. 4. Mills R. Cholesteatoma behind an intact tympanic membrane in adult life: congenital or acquired? J Laryngol Otol. 2009;123(5):488-491.
5. Abdel-Aziz M. Congenital cholesteatoma of the infratemporal fossa with congenital aural atresia and mastoiditis: a case report. BMC Ear Nose Throat Disord. 2012;12:6. 6. Louw L. Acquired cholesteatoma pathogenesis: stepwise explanations. J Laryngol Otol. 2010;124(6):587-593. 7. McHugh TP. Intracranial cholesteatoma: a case report and review. J Emerg Med. 2007;32(4):375-379. 8. Huisman MA, Ten Dijke P, Grote JJ. Transforming growth factor B and wound healing in human cholesteatoma. Laryngoscope. 2008;118(1):94-98. 9. Bacciu A, Pasanisi E, Vincenti V, et al. Surgical treatment of middle ear cholesteatoma in children with Down syndrome. Otol Neurotol. 2005;26(5):1007-1010. 10. Shott SR, Joseph A, Heithaus D. Hearing loss in children with Down syndrome. Int J Pediatr Otorhinolaryngol. 2001;61(3):199-205. 11. Austeng ME, Akre H, Falkenberg ES, Øverland B, Abdelnoor M, Kværner KJ. Hearing level in children with Down syndrome at the age of eight. Res Dev Disabil. 2013;34(7):2251-2256. 12. Austin DF. Staging in cholesteatoma surgery. J Laryngol Otol. 1989;103(2):143-148. 13. Aarts MC, Rovers MM, van der Veen EL, Schilder AG, van der Heijden GJ, Grolman W. The diagnostic value of diffusion-weighted magnetic resonance imaging in detecting a residual cholesteatoma. Otolaryngol Head Neck Surg. 2010;143(1):12-16.
Downloaded from aor.sagepub.com at UNIV OF MASSACHUSETTS on March 17, 2015
research-article2014
AORXXX10.1177/0003489414538402Annals of Otology, Rhinology & LaryngologyZhu et al
Article
Recurrent Cholesteatoma With Massive Extension to Temporoparietal Scalp: Case Report and Literature Review
Annals of Otology, Rhinology & Laryngology 2014, Vol. 123(11) 754–757 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0003489414538402 aor.sagepub.com
Vivian F. Zhu, BS1, Brandon Isaacson, MD1, Jason Mull, MD2, and Larry L. Myers, MD, FACS1
Abstract Background: Cholesteatomas are locally destructive collections of epithelial debris arising from temporal bone squamous epithelium. Recurrences may occur after removal and are typically located within the temporal bone. Objective: This study aimed to report a case of a massive, recurrent cholesteatoma with extension to temporoparietal scalp in a 37-year-old woman. Methods: Case report with literature review. Results: The patient underwent complete excision of a well-circumscribed left temporal mass, intraoperatively identified to arise from the middle ear and to contain keratin debris. Conclusion: We report a case of recurrent cholesteatoma with massive extension to temporoparietal scalp. Clinical suspicion of recurrent cholesteatoma should remain in the differential diagnosis of temporal mass with prior history of cholesteatoma. Keywords recurrent cholesteatoma, head and neck mass
Introduction Cholesteatomas, classified as either congenital or acquired, are epidermal inclusion cysts of the middle ear or mastoid. They most commonly originate within the temporal bone and often present with hearing loss and purulent otorrhea. The prevalence of cholesteatoma is about 3 in 100 000 children and 12 in 100 000 adults.1 Acquired cholesteatomas are far more common than congenital cholesteatomas, which constitute only 2% to 5% of all cholesteatomas.2 Cholesteatomas can progress through distinct pathways in the temporal bone, such as eroding through the mastoid bone and enlarging in the posterior fossa, penetrating into petrous bone, staying within the middle ear, or extending out to the external auditory canal. If left untreated, cholesteatoma growth can result in severe middle ear destruction—either ossicular destruction or semicircular canal disruptions.3,4 There can be tegmen destruction as well as hearing loss secondary to ossicle erosion.4 The cholesteatoma may develop intratemporally, causing symptoms of an infratemporal lesion, such as acute mastoiditis or lateral sinus thrombosis.5 Other, less likely complications of a cholesteatoma include paralysis of the facial nerve, fistula of the semicircular canal, or perforation of the pars flacida or pars tensa.3
We report the case of acquired, recurrent cholesteatoma extending to the subcutaneous tissues of the temporoparietal region.
Case Report A female patient with Trisomy 21 underwent a canal wall down left mastoidectomy and tympanoplasty for chronic otitis media at a children’s hospital when she was 9 years old. She did not have a second look procedure. She presented 26 years later to another hospital complaining of a progressively enlarging left temporal mass, intermittent left otorrhea, and long-standing history of hearing loss. She denied dysphagia, odynophagia, voice changes, weight loss, radiating otalgia, fever, night sweats, chills, cough and 1
Department of Otolaryngology–Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, Texas, USA 2 Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA Corresponding Author: Larry L. Myers, MD, FACS, Department of Otolaryngology–Head and Neck Surgery, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-9035, USA. Email: [email protected]
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Zhu et al
Figure 2. Completely excised recurrent cholesteatoma.
Figure 1. Preoperative coronal T1-weighted magnetic resonance image with contrast demonstrating recurrent cholesteatoma with superior extension to left temporoparietal scalp.
hemoptysis, pain, or vertigo. Physical examination revealed a healthy woman with Trisomy 21 facies. There was a soft, 8 × 6 cm left temporal mass in the subcutaneous tissue. The overlying skin was intact without ulceration or local alopecia. Otoscopy demonstrated a dry left canal wall down cavity without significant epithelial debris and a patent right external auditory canal with a normal tympanic membrane. The remainder of her otolaryngologic examination was within normal limits. Magnetic resonance (MR) T1-weighted imaging with contrast revealed a 8.0 × 6.3 × 2.0 cm (100.8 mL) subcutaneous, fluid intensity, minimally peripherally enhancing lesion with a single internal enhancing septation. There was minimal fluid within the residual left mastoid air cells. There was no definite evidence of connection between the intracranial space and the fluid collection to suggest pseudomeningocele (see Figure 1). The patient was given a provisional diagnosis of lymphocele at the outside hospital and was supported by MR findings. The patient and her family refused fine needle aspiration and incisional biopsy for definitive diagnosis. After several months of deliberation, they agreed to surgery and the patient was taken to the operating room for excisional biopsy via a transtemporal approach. A vertical incision was made in the hear-bearing scalp. The well-encapsulated mass was identified in the subcutaneous tissue. Blunt dissection continued from a superior to inferior direction through the temporalis muscle to the root of the pinna. The capsule was violated near its origin and was
Figure 3. Hematoxylin and eosin stain 100X. Squamous lined cyst with keratinized debris.
found to be filled with squamous debris. The entire temporal mass was removed en bloc (see Figure 2). There were no intraoperative complications. Histopathologic analysis of the mass demonstrated an epithelial lined cyst with squamous debris consistent with cholesteatoma (see Figure 3). An audiogram was performed 2 weeks postoperatively and demonstrated normal hearing through 4000 Hz sloping to a moderate hearing loss at 8000 Hz in the right ear and a moderate rising to mild 500 to 2000 Hz sloping to profound primarily conductive hearing loss in the left ear. Pure tone average/speech reception threshold for right and left ears, respectively, were 8/15 dB SD 100% and 33/45 dB SD 100%. Tympanograms were type A bilaterally. A postoperative temporal bone computed tomography (CT) scan revealed a left tegmen defect and a dehiscent
Downloaded from aor.sagepub.com at UNIV OF MASSACHUSETTS on March 17, 2015
756
Annals of Otology, Rhinology & Laryngology 123(11)
superior semicircular canal. A subsequent MR with contrast revealed no enhancing lesions or masses in the brainstem, cerebellopontine angle, internal auditory canal, or inner or middle ear. There was no evidence of restricted diffusion. After 2 years of follow-up, the patient is doing well with no evidence of disease.
Discussion Cholesteatomas are composed of keratinizing squamous epithelial matrix surrounding an adjacent subepithelial connective perimatrix bounded by mucous cuboidal epithelial cells that contain keratin.6 Eustachian tube dysfunction with resultant tympanic membrane retraction from negative middle ear pressure is the most common inciting event in cholesteatoma formation. Cholesteatomas may also arise from trauma or infection that has improperly healed, causing the epithelial cells to invaginated, leading to uncontrolled growth.7 A critical component involved in almost all cases of cholesteatoma is inflammation. Inflammation is heavily involved in all aspects of cholesteatoma onset, development, growth, and recurrence, particularly of the tympanic membrane and the middle ear. Cholesteatomas have also been partially associated with abnormal wound healing due to a related enhanced fibroblast collagen overproduction and augmented levels of TGF-β.8 Disease may progress to involve ossicular erosion that results in conductive hearing loss, but in rare cases, sensorineural hearing loss and vestibular function loss can occur from otic capsule erosion or labyrinthitis. Typically, acquired cholesteatomas present with chronic or recurrent otorrhea and crust formation that can often cover the mouth of the cholesteatoma. Our case was unique for several reasons. Our patient did not present with otorrhea or a retraction pocket in the tympanic membrane. The significance of this case is the rare temporoparietal location and massive size of a recurrent cholesteatoma. Despite its size, a complete resection of the recurrent disease was achieved with no evidence of recurrence or other complications with 2 years of follow-up after surgery. The extratemporal location in this case likely occurred secondary to implantation of epithelial cells producing a cholesteatoma matrix during the initial surgery, performed in 1983. The location within the temporalis muscle supports an etiology of iatrogenic implantation because there are no epithelial rests within mesodermally derived tissue. The precise reason that the patient did not have sustained follow-up after her initial surgery is unknown. The patient’s health care decisions were made by both her parents and herself. Presenting to another hospital more than 2 decades after her initial surgery evidenced a major gap in her continuity of care. The difficulty or inability to obtain her medical records containing a history of cholesteatoma and the
lack of middle ear findings contributed to the misdiagnosis of her enlarging temporal mass. Trisomy 21 is the most common chromosomal abnormality, characterized by mental disability and various other medical problems including otologic diseases.9 One out of 670 to 770 children are born with Down syndrome with a 38% to 78% incidence of hearing loss.10,11 Down syndrome patients are often affected with chronic ear disease such as otitis media, which is a chronic inflammatory infection of the middle ear.10 Recidivism is a well-known feature of cholesteatoma that necessitates long-term follow-up. Staging cholesteatoma surgery has been used for a number of years to improve disease eradication in patients undergoing canal wall up tympanomastoidectomy or middle ear reconstruction in canal wall down procedures. Clinical examination and medical imaging are both used to monitor for recidivistic disease. Temporal bone CT is helpful in identifying recidivistic cholesteatoma but lacks specificity as it is unable to differentiate between scar tissue, fluid, granulation, and cholesteatoma.12 A number of magnetic resonance imaging (MRI) sequences have been analyzed for their ability to detect recidivistic cholesteatoma, including delayed gadolinium imaging, echo planar diffusion weighted imaging (EPI DWI), and non-echo planar diffusion weighted imaging (non-EPI DWI). Diffusion weighted MRI has been shown to be both sensitive and specific in identifying recidivistic cholesteatoma. Non-EPI DWI sequences have a sensitivity, specificity, positive, and negative predictive value of up to 97%. Non-EPI DWI MRI has the ability to detect recidivistic disease as small as 2 mm. Mass like recidivistic disease (keratin pearl) is readily identified with non-EPI DWI MRI, whereas a thin layer of squamous epithelial matrix is difficult to visualize. Diffusion weighted MRI requires expertise in image acquisition and interpretation, as differentiating true restricted diffusion from bone-soft tissue artifact and T2 shine through can be challenging.13
Conclusion We report a case of a massive, recurrent cholesteatoma with superior extension to temporoparietal scalp region. The patient’s presentation after more than 2 decades without follow-up allowed for an observation of a natural history of this disease process. This patient was managed with complete surgical excision. She has no evidence of recurrence after 2 years of follow-up. This case illustrates the need for diligent follow-up after surgery for cholesteatoma to detect recurrence. A complete history and a high index of suspicion are necessary to establish the diagnosis of cholesteatoma in patients presenting with a periauricular or temporal mass with a prior history of cholesteatoma.
Downloaded from aor.sagepub.com at UNIV OF MASSACHUSETTS on March 17, 2015
757
Zhu et al Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Jones RC, Demetroulakos J, Mugge RE. Cholesteatoma. https://www.clinicalkey.com/#!/ContentPlayerCtrl/doPlay Content/21-s2.0-6020108/{%22sectionId%22:%22section ZC18394D_267_2A6%22}. Published June 16, 2013. Accessed May 22, 2014. 2. Janardhan N, Nara J, Peram I, Palukuri S, Chinta A, Satna K. Congenital cholesteatoma of temporal bone with Bezold’s abscess: case report. Indian J Otolaryngol Head Neck Surg. 2012;64(1):97-99. 3. Sadé J, Fuchs C. Cholesteatoma: ossicular destruction in adults and children. J Laryngol Otol. 1994;108(7):541-544. 4. Mills R. Cholesteatoma behind an intact tympanic membrane in adult life: congenital or acquired? J Laryngol Otol. 2009;123(5):488-491.
5. Abdel-Aziz M. Congenital cholesteatoma of the infratemporal fossa with congenital aural atresia and mastoiditis: a case report. BMC Ear Nose Throat Disord. 2012;12:6. 6. Louw L. Acquired cholesteatoma pathogenesis: stepwise explanations. J Laryngol Otol. 2010;124(6):587-593. 7. McHugh TP. Intracranial cholesteatoma: a case report and review. J Emerg Med. 2007;32(4):375-379. 8. Huisman MA, Ten Dijke P, Grote JJ. Transforming growth factor B and wound healing in human cholesteatoma. Laryngoscope. 2008;118(1):94-98. 9. Bacciu A, Pasanisi E, Vincenti V, et al. Surgical treatment of middle ear cholesteatoma in children with Down syndrome. Otol Neurotol. 2005;26(5):1007-1010. 10. Shott SR, Joseph A, Heithaus D. Hearing loss in children with Down syndrome. Int J Pediatr Otorhinolaryngol. 2001;61(3):199-205. 11. Austeng ME, Akre H, Falkenberg ES, Øverland B, Abdelnoor M, Kværner KJ. Hearing level in children with Down syndrome at the age of eight. Res Dev Disabil. 2013;34(7):2251-2256. 12. Austin DF. Staging in cholesteatoma surgery. J Laryngol Otol. 1989;103(2):143-148. 13. Aarts MC, Rovers MM, van der Veen EL, Schilder AG, van der Heijden GJ, Grolman W. The diagnostic value of diffusion-weighted magnetic resonance imaging in detecting a residual cholesteatoma. Otolaryngol Head Neck Surg. 2010;143(1):12-16.
Downloaded from aor.sagepub.com at UNIV OF MASSACHUSETTS on March 17, 2015
