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Case Report Translabyrinthine Petrous Apex Cholesteatoma Surgery with Hearing Preservation

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Case Reports in Otolaryngology
Authors:
  • Kopfzentrum Bielefeld

Abstract and Figures

Objective: To introduce a novel surgical approach to petrous apex lesion (PA) with superior semicircular canal plugging for hearing preservation. Patient. A 63-year-old patient presented with a recurrent cholesteatoma of the left petrous apex. The patient had a long-term history of cholesteatoma and MRI with diffusion-weighted imaging (DWI) detected a suspicious lesion in the left petrous apex on follow-up. Intervention. The cholesteatoma could be completely removed from the petrous apex with partial superior semicircular canal plugging and removal with hearing preservation. Outcomes. Cholesteatomas of the temporal bone are managed by surgery with complete excision of the lesion. Results: The translabyrinthine approach, generally useful in nonhearing ears, could be utilized with the additional technique of superior semicircular canal plugging to preserve hearing in this patient. Conclusions: This case highlights the possibility of a hearing preservation strategy for PA cholesteatomas using a translabyrithine approach.
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Case Report
Translabyrinthine Petrous Apex Cholesteatoma Surgery with
Hearing Preservation
Holger Sudhoff ,
1
Randolf Klingebiel,
2
Lars-Uwe Scholtz,
1
and Ingo Todt
1
1
Department of Otorhinolaryngology, Head and Neck Surgery, Medical Faculty OWL, Bielefeld University,
Campus Klinikum Bielefeld, Bielefeld, Germany
2
Department of Diagnostic and Interventional Neuroradiology, Protestant Hospital Bethel, Bielefeld, Germany
Correspondence should be addressed to Holger Sudhoff; holger.sudhoff@rub.de
Received 28 February 2021; Accepted 2 June 2021; Published 14 June 2021
Academic Editor: Vikrant Borse
Copyright ©2021 Holger Sudhoff et al. is is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Objective. To introduce a novel surgical approach to petrous apex lesion (PA) with superior semicircular canal plugging for
hearing preservation. Patient. A 63-year-old patient presented with a recurrent cholesteatoma of the left petrous apex. e patient
had a long-term history of cholesteatoma and MRI with diffusion-weighted imaging (DWI) detected a suspicious lesion in the left
petrous apex on follow-up. Intervention. e cholesteatoma could be completely removed from the petrous apex with partial
superior semicircular canal plugging and removal with hearing preservation. Outcomes. Cholesteatomas of the temporal bone are
managed by surgery with complete excision of the lesion. Results. e translabyrinthine approach, generally useful in nonhearing
ears, could be utilized with the additional technique of superior semicircular canal plugging to preserve hearing in this patient.
Conclusions. is case highlights the possibility of a hearing preservation strategy for PA cholesteatomas using a
translabyrithine approach.
1. Introduction
e petrous apex (PA) is embedded in the medial portion of
the temporal bone, bordered by several vital structures [1]. A
variety of different petrous apex pathologies such as cho-
lesterol granulomas, cholesteatomas, meningiomas, and
schwannomas have been described [2]. Cholesterol granu-
lomas are significantly more common than cholesteatomas
of the petrous apex, comprising the most frequent de-
structive lesion of the petrous apex. e surgical options for
petrous apex cholesteatoma use open, endoscopic, or
combined techniques. e specific approach is determined
by the pathology and its anatomical localization and ex-
tension related to vital structures. Individual anatomical
variations in cranial morphology may interfere with dif-
ferent surgical approaches. Removal of the lesion can be
achieved through the transcanal infracochlear, transmastoid
infralabyrinthine, middle fossa, translabyrinthine, transotic
approach, or occasionally by a transsphenoidal approach
[3–5]. However, there is a need for a novel surgical strategy
to petrous apex lesion (PA) with hearing preservation as a
regular approach usually linked to a loss of hearing and
decrease of quality of life [6, 7]. e recently introduced
superior semicircular canal plugging for hearing pre-
servation seems as a rational for this translabyrinthine ap-
proach [6].
PA pathologies are usually found incidentally on im-
aging for unrelated symptoms. If present, they are hetero-
geneous. e most common complaint is hearing loss, but
aural fullness and vertigo secondary to compression of the
8th cranial nerve are also common. ese symptoms are
present in the majority of patients with petrous apex pa-
thology. Additional symptoms include headaches due to the
involvement of the trigeminal nerve, diplopia from com-
pression of the sixth cranial nerve, and seventh-nerve-re-
lated facial spasm or weakness. Extended cholesteatoma may
also result in Gradenigo’s syndrome including purulent
otorrhea, abducens palsy, and otalgia [6]. Evaluation of PA
lesions involves a comprehensive evaluation of cranial nerve
function and audiometry. Computed tomography (CT) is
Hindawi
Case Reports in Otolaryngology
Volume 2021, Article ID 5541703, 4 pages
https://doi.org/10.1155/2021/5541703
routinely used to determine the optimal surgical approach if
surgical intervention is necessary (Figure 1). Magnetic
resonance imaging (MRI) can narrow the differential di-
agnosis. In our case, a 63-year-old patient presented with a
cholesteatoma of the left petrous apex six years after ex-
tended middle ear surgery on follow-up images. MRI with
diffusion-weighted imaging (DWI) detected a suspicious
lesion in the left petrous apex (Figure 1).
2. Case
e basic goal of cholesteatoma surgery is the complete re-
moval of the squamous epithelium to minimize the risk of
recurrence. Cholesteatoma is a life-threatening disease due to
its intracranial complications [7]. Due to an expected inner ear
trauma during surgery, 1 g of prednisolone was administered in
addition to the antibiotic treatment preoperatively. Facial nerve
monitoring was constantly applied. A retroauricular incision
was used in this patient. It allowed extensive exposure of the
mastoid. e mastoid was partially obliterated with hydrox-
yapapatite granules but did not reveal the cholesteatoma
matrix. A titanium net had been implanted to stabilize the
middle and posterior cranial fossae in a revision surgery six
years earlier (Figure 1). An extended mastoidectomy was
performed. e sinus, media, and posterior fossa were iden-
tified as well as the temporarily removed total ossicular chain
prosthesis. e semicircular canals were detected and exposed.
e common crus of the posterior and superior canals was
identified. Finally, the bone between the superior canal and
tegmen was carefully drilled away layer by layer with a small
diamond burr until the blue lines of the superior canal were
visible. e dura and the titanium plate were slightly elevated to
expose the superior canal. e endosteal islands of the superior
semicircular were elevated and the membranous labyrinth
visualized. e plug was a mix of dry cortex bone dust and
fibrinogen sealant. is was carefully packed into both fen-
estrations of the superior semicircular canal to occlude the
superior, lateral, and medial portion. is created a partition
from the rest of the inner ear. Temporalis fascia was placed over
the occlusions and covered with a fibrinogen sealant to protect
against perilymph leakage. After the occlusion and removal of
the superior aspect of the semicircular canal, sufficient access
was granted to the petrous apex. Hopkin’s wide-angle endo-
scope was used to control the extension and removal of the
cholesteatoma of the petrous apex that could not be assessed
with the microscope (Figures 2 and 3). All cholesteatoma
matrixes were eliminated from the petrous apex (Figure 4). e
preoperative hearing threshold could be preserved by superior
semicircular canal plugging (Figure 5). Histopathology con-
firmed a cholesteatoma of the petrous apex. Postoperatively,
the patient was stable with preserved hearing thresholds on the
left ear and declining dizziness with a loss of vestibular function
on one-year follow-up.
3. Discussion
Semicircular canal plugging was originally introduced to
treat patients with intractable benign paroxysmal positional
vertigo and was subsequently applied to other hydropic ear
diseases such as Meniere’s disease [8, 9]. Semicircular canal
plugging can reduce vertiginous symptoms in those patients
representing an effective therapy for this disorder. erefore,
it seems possible to utilize the additional technique of su-
perior semicircular canal plugging, to preserve hearing in
partial translabyrinthine surgery. Previously, a modified
traditional translabyrinthine approach for the removal of an
intracanalicular acoustic schwannoma by sealing the vesti-
bule with bone wax allowed the hearing function to be
preserved in one patient [10, 11]. e described trans-
labyrithine approach for hearing preservation is currently
limited to selected cases with constrained pathologies, such
as petrous apex cholesteatomas [6, 12, 13]. Due to the very
limited experience with a partial removal of the superior
semicircular canal, patients have to be consented about
deafness, significant hearing loss, and vertigo. e use of
Hopkin’s wide-angle endoscope was necessary in the petrous
apex that could not be completely assessed with the mi-
croscope. Computed tomography of the temporal bone is
helpful in revealing a possible residual cholesteatoma and to
assess the extension, exact location, and possible compli-
cations. Nonecho planar diffusion-weighted magnetic res-
onance imaging (non-EPI DW MRI) is a powerful tool to
detect of localization and extension of cholesteatoma [14].
Due to a possible inner ear trauma during surgery, steroids
and antibiotic prophylaxis should be administered. Facial
nerve monitoring is recommendable during the entire
procedure.
e translabyrinthine approach provides the straightest
route to the petrous apex [1, 6, 13]. However, it has pre-
viously been considered unsuited for hearing preservation.
erefore, it was limited to cases where hearing and ves-
tibular function were absent. Our case highlights the pos-
sibility to grant access to the petrous apex region and to
preserve hearing in partial labyrinthectomy. e additional
2Case Reports in Otolaryngology
Figure 2: Intraoperative image showing the petrous apex with the cholesteatoma matrix prior to removal. e superior semicircular canal
has been plugged, sealed, and partly amputated.
(a) (b)
Figure 3: 3D reconstruction of CTs. (a) e preoperative scan reconstruction. (b) e postoperative scan exhibiting the defect of the petrous
apex after the partial translabyrinthine approach.
(a) (b)
(c) (d)
Figure 1: (a, b) Preoperative axial CT showing (asterixs) the petrous apex with bone erosion and a titanium plate covering the partially
covering anterior limitation of the temporal bone. (c, d) Non-EPI DWI with coronal and axial planes. Note the diffusion restriction in the
petrous apex of the left temporal bone.
Case Reports in Otolaryngology 3
application of superior semicircular canal plugging, sealing,
and partial removal provides the possibility to preserve
hearing in selected patients [15]. e case underlines this
surgical option in selected skull base pathologies requiring
limited access to the petrous apex.
4. Conclusions
A limited number of studies appear to demonstrate the
possibility of hearing preservation in patients after trans-
labyrinthine cholesteatoma removal. erefore, this case
report further highlights the potential mechanisms for
hearing preservation after labyrinthectomy. However, fur-
ther studies are required to implement this approach to
preserve hearing for extended cholesteatomas of the petrous
apex region.
Conflicts of Interest
e authors declare that they have no conflicts of interest.
Acknowledgments
e authors would also like to express their gratitude and
dedicate this paper to Mr. David Andrew Moffat, BSc, MA,
MBBS, PhD, FRCS (1947–2020).
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Figure 4: Coronal CTof the amputated superior semicircular canal
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0
20
40
60
80
100
120
Hearing level (dBHL)
0.125 0.25 0.5 1248
Frequency (kHz)
Figure 5: Left ear, pre- ( air conduction and bone conduction)
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tone audiogram. Hearing thresholds remained on the operated left
ear one year after surgery.
4Case Reports in Otolaryngology
... Each of the surgical approaches offers a unique set of benefits, and some can predispose to complications more than others [ Table 2]. [1,2,5,6,15,20,31,32,36] In certain instances, mastoidectomy is indicated in order to achieve maximal resection. For example, the canal-wall down (CWD) open mastoidectomy approach involves removal of the posterior semicircular canal which can afford superior visualization of the diseased middle ear anatomy. ...
... Most direct route to petrous apex, [15,36] approach of choice in patients with a high jugular bulb or anterior sigmoid sinus, wider exposure of anterior, medial, and median cerebellopontine angle [6,20] Unsuited for hearing preservation in most cases, high risk of damage to vestibular and cochlear organs [6] Infralabyrinthine Approach of choice in patients with severe hearing loss or deafness [2] May result in conductive hearing loss [2] Infracochlear (meatal) Preserves hearing function in most cases, may be accessed via endoscopic techniques [20] May result in conductive hearing loss [20] Middle cranial fossa Recommended approach for isolated congenital petrous apex cholesteatomas and hearing preservation [1,5] CSF leak (in up to 13% of patients), facial nerve palsy, meningitis, hydrocephalus, ataxia, more limited access, limited exposure to petrous apex and may lead to a secondary surgery [1] ...
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Background: Cholesteatomas are growths of squamous epithelium that can form inside the middle ear and mastoid cavity and damage nearby structures causing hearing loss when located at the petrous apex. The primary goal of petrous apex cholesteatoma resection is gross total removal with tympanoplasty and canal-wall up or canal-wall down tympanomastoidectomy. At present, there is no definitive surgical approach supported by greater than level 4 evidence in the literature to date. Methods: A systematic review was conducted utilizing PubMed, Embase, and Scopus databases. Articles were screened and selected to be reviewed in full text. The articles that met inclusion criteria were reviewed for relevant data. Data analysis, means, and standard deviations were calculated using Microsoft Excel. Results: After screening, five articles were included in the systematic review. There were a total of eight pediatric patients with nine total cholesteatomas removed. Conductive hearing loss was the most common (77%) presenting symptom. Perforations were noted in seven ears (86%). Recurrence was noted in 50% of patients with an average recurrence rate of 3.5 years (SD = 1.73). Average length of follow-up was 32.6 months (SD = 21.7). Canal-wall up was the most utilized technique (60%) and there were zero noted surgical complications. Five of the seven (71%) patients that experienced hearing loss from perforation noted improved hearing. Conclusion: Due to its rarity, diagnostic evaluation and treatment can vary. Further, multi-institutional investigation is necessary to develop population-level management protocols for pediatric patients affected by petrous apex cholesteatomas.
... Cholesterol granulomas encompass the most frequent destructive lesion of the petrous apex. 1 Cholesterol granulomas form when the aeration of the petrous apex is hindered, thus creating a vacuum into which blood is drawn, causing foreign body giant cell reaction to cholesterol deposits, with fibrosis. 2 They are ten times more common than cholesteatomas and forty times more common than petrous apex mucoceles. ...
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