Cholesteatoma

Contents

Foreword: Cholesteatoma: Skin Growing Where It Does Not Belong xv

Sujana S. Chandrasekhar

Preface: Cholesteatoma xix

Samantha Anne and Michael Hoa

The Science of Cholesteatoma 1

Tirth R. Patel and Christopher M. Welch
Cholesteatoma is a potential end-stage outcome of chronic ear infections
that can result in the destruction of temporal bone structures with potential
resultant hearing loss, vertigo, and intracranial infectious complications.
There is currently no treatment apart from surgery for this condition, and
despite years of study, the histopathogenesis of this disease remains
poorly understood. This review is intended to summarize our accumulated
knowledge of the mechanisms of cholesteatoma development and the
underlying molecular biology. Attention will be directed particularly to re-
cent developments, covering many potential pharmacologic targets that
could be used to treat this disease in the future.

Evaluation of Cholesteatoma 29
Anne K. Maxwell and Stephen R. Hoff
Evaluation of cholesteatoma depends on clinical history and examination,
with microscope and/or endoscope. A history of hearing loss with a
chronic draining ear, refractory to ototopical medication, raises suspicion
for cholesteatoma. Symptoms of Eustachian tube dysfunction or prior ear
surgery including ear tubes should be elicited. Inflammation can be severe
and should be suppressed if possible. Once cholesteatoma is diagnosed
or strongly suspected, further workup includes audiometry prior to surgical
excision. Imaging may supplement the workup and is especially helpful if
there are concerning features including vertigo, third window symptoms,
asymmetric bone line, facial nerve weakness, or for anticipatory guidance.

Principles of Cholesteatoma Management 41

Simon I. Angeli and Kay W. Chang
Surgery for cholesteatoma should be tailored to individual patients, con-
sidering demographic and disease factors, to obtain a dry, safe, and func-
tional ear. The EAONO/JOS classification and staging system provide a
valuable framework for data collection and outcome assessment. Canal
wall-up and canal wall-down surgical approaches each have their advan-
tages and disadvantages, though it is not definitive that one approach is
clearly more advantageous than the other. Mastoid obliteration techniques
show promise in reducing recidivistic disease rates but require further re-
search and standardization. Endoscopic ear surgery further augments our
surgical capabilities to visualize and eradicate cholesteatoma.
x Contents

Complications of Cholesteatoma 51
Nanki Hura, Daniel Choo, and Peter Luke Santa Maria
Complications of cholesteatoma result from characteristic inflammatory
and resorptive processes that erode the structures of the middle and inner
ear with potential to spread locally. Common intratemporal complications
include hearing loss, facial nerve palsy, labyrinthine fistula, and dysgeusia.
Extratemporal complications, though less common, may be life-threaten-
ing, and include cerebrospinal fluid leak and encephalocele, meningitis,
epidural and intraparenchymal abscesses, subdural empyema, and otic
hydrocephalus.

Congenital Cholesteatoma 65
Kristan P. Alfonso
Congenital cholesteatoma is a cyst of keratinizing squamous cell epithe-
lium in the setting of an intact tympanic membrane, in a patient without
a history of otorrhea, tympanic membrane perforation, or otologic surgery.
The most common presentation of a congenital cholesteatoma is that of an
asymptomatic pearly white mass in the anterosuperior quadrant of the
tympanic cavity. The etiology of congenital cholesteatoma has been de-
bated at length, with the leading theory being the epithelial rest theory.
Treatment for congenital cholesteatoma is surgical, with advances in en-
doscopic ear surgery allowing for improved intraoperative visualization
and postoperative lowered recidivism rates.

Management of Cholesteatoma: Surgical Options for Disease Limited to Middle Ear/

Mastoid 75
Keelin Fallon, David Chi, and Aaron Remenschneider
Surgery remains the mainstay of cholesteatoma management. Through
advancement in technique and technology, the available surgical ap-
proaches have expanded to include not only the traditional procedures,
but also endoscopic procedures, canal wall reconstruction procedures,
mastoid obliteration, and retrograde mastoidotomy. Selection of manage-
ment technique will depend on disease characteristics, patient factors,
and surgeon preference.

Management of Cholesteatoma: Extension Beyond Middle Ear/Mastoid 89

Jason K. Adams, Robert J. Macielak, Oliver F. Adunka, and Maura K. Cosetti
Petrous bone cholesteatoma, or cholesteatoma that extends beyond the
middle ear and mastoid, represents a rare but destructive pathology. Di-
agnosis can be difficult before substantial morbidity is incurred, and pa-
tients can present with life-threatening complications. Determination of
disease extent and the functional status of the facial nerve and cochleo-
vestibular system are critical in surgical planning. Typically, surgery in-
volves ablative procedures with the goal of complete disease resection
given the low likelihood of preserved inner ear function. In experienced
hands, disease control and facial nerve outcomes are favorable; how-
ever, disease recidivism is not uncommon and, thus, these patients re-
quire lifelong surveillance.
 Contents xi

Techniques in Management of Cholesteatoma: Endoscopic Approaches 99

Adrian Lewis James and Manuela Fina
Video content accompanies this article at [Link]
com.

Outcomes from cholesteatoma surgery are improved by using endo-

scopes to guide dissection as the wide-angled view facilitates more com-
plete removal of cholesteatoma matrix, reducing the risk of residual
disease. Furthermore, surgery can often be completed transcanal, reduc-
ing postoperative morbidity. The decision to complete cholesteatoma re-
moval endoscopically transcanal is made from a combination of
preoperative imaging and intraoperative findings. A one-handed approach
to operating is required as the other hand is used to optimize endoscope
positioning. Aspects of technique and instrumentation particular to endo-
scopic cholesteatoma surgery are presented.
Techniques in Management of Cholesteatoma: Modified Radical and Retrograde
Mastoidectomy 113
Alexandra M. Arambula, Lacey Magee, Edward J. Doyle, and Sarah Mowry
Video content accompanies this article at [Link]
com.

When surgically managing cholesteatoma, the Bondy modified radical mas-

toidectomy (MRM) and retrograde mastoidectomy can enhance visualiza-
tion while minimizing the extent of surgery required for disease removal.
The Bondy MRM can be used for disease limited to the atticoantral space,
when the ossicles and middle ear space are otherwise healthy, and for canal
cholesteatomas. The retrograde mastoidectomy offers a safe alternative for
patients with unfavorable tegmen and sigmoid sinus positions and poorly
pneumatized mastoids. Both approaches achieve favorable recidivism rates
and postoperative hearing outcomes. This article serves as a reference re-
garding indications, techniques, and complications for these approaches.
Techniques in Management of Cholesteatoma: Radical and Canal Wall down
Mastoidectomy 123
Emily K. Gjini, Olivia A. Kalmanson, Kathryn Noonan, and Ashley M. Nassiri
Video content accompanies this article at [Link]
com.

Canal wall down mastoidectomy is performed when demanded by the ex-

tent of disease: presence of a large labyrinthine fistula, extensive erosion of
posterior auditory canal wall, prior failed intact canal wall surgery, a con-
tracted, sclerotic mastoid with extensive disease, or need for exterioriza-
tion due to inadequate exposure or inability to remove disease safely
with a canal wall up approach.

Techniques in Management of Cholesteatoma: Subtotal Petrosectomy; Blind Sac

Closure 133
Kevin Wong, Samuel P. Gubbels, and Jason A. Brant
Subtotal petrosectomy plays a critical role in the management of late-
stage cholesteatoma. This procedure is indicated either for recurrent
xii Contents

cholesteatoma that has failed prior surgical therapies or when life-threat-

ening infectious complications are present. By exenterating all diseased
areas of the temporal bone and closing off the ear canal, subtotal petro-
sectomy has a much greater chance of removing cholesteatoma and pre-
venting recidivism.

Outcomes with Management of Cholesteatoma 141

Donald Tan, Natalie Schauwecker, Elizabeth L. Perkins, and Kenneth Lee
This article provides an expert summary of the immense amount of out-
comes research in cholesteatoma surgery. Specific topics discussed in-
clude canal wall-up versus canal wall-down surgery, ossicular chain
reconstruction, endoscopic ear surgery, disease-specific quality-of-life
outcomes, and congenital cholesteatoma.

Management of Cholesteatoma: Hearing Rehabilitation 153

Corinne Pittman, Samantha Anne, Sujana S. Chandrasekhar, and Michael Hoa
Surgical treatment of cholesteatoma requires a highly individualized ap-
proach, balancing disease eradication with hearing preservation, and
necessitates thorough patient counseling on expected auditory out-
comes and options for rehabilitation. The choice between canal wall
up and canal wall down mastoidectomy can influence hearing out-
comes. Hearing rehabilitation options following cholesteatoma surgery
include air- and bone-conduction devices and are individualized for
the patient. Assistive listening devices such as frequency modulation
systems, hearing loops, and Bluetooth technology are critical for en-
hancing speech understanding in noisy environments for patients who
employ various hearing rehabilitation methods and require further ad-
vanced solutions.

Complications of Surgery: Recognition and Management 165

Nadine I. Ibrahim, Christine Settoon, Ken Kazahaya, and Emily Z. Stucken
The middle ear and mastoid spaces contain delicate and functionally
important structures. Safe cholesteatoma surgery requires mastery of
otologic anatomy as well as thorough preoperative investigation. Cho-
lesteatoma can damage otologic structures on its own; however, there
is also an opportunity for an iatrogenic complication given the distortion
of anatomy by the disease process. This article explores complications
that can occur during cholesteatoma surgery, guidance on how to avoid
intraoperative complications, and treatment of such complications.

Recidivism and Recurrence 177

Shayna Portanova Cooperman, Kevin Wong, Tiffany Peng Hwa, and
Jennifer Alyono
Recidivistic cholesteatoma encompasses residual as well as recurrent dis-
ease, and can occur in up to 61% of cases. Pediatric disease may have a
higher propensity for recidivism. Serial physical examination and MRI in-
cluding non-EPI DWI sequences are useful in surveillance. Canal wall
down approaches with mastoid obliteration may be an approach to reduce
 Contents xiii

recidivism while minimizing the need for mastoid cavity maintenance.

Modern techniques of Eustachian tube dilation and endoscopic ear sur-
gery may yet prove particularly helpful in reducing re-retraction and resid-
ual disease in the retrotympanum, respectively; however, they require
further study.