Recurrence of the pyramid of the temporal bone cholesteatoma: systematic review of literature

To evaluate whether a combined translabyrinthine-transsphenoidal approach can be used to achieve adequate surgical resection of an extensive petrous bone cholesteatoma and create a debris drainage route for the residual cholesteatoma that is maintained long-term. A 71-year-old man with residual petrous temporal bone cholesteatoma that had spread extensively to the internal carotid artery and posterior cranial fossa. Surgical resection of the cholesteatoma via a translabyrinthine approach and creation of a debris drainage route into the nasopharynx via a transsphenoidal approach. Control of unresectable petrous temporal bone cholesteatoma and occurrence of cholesteatoma- or surgery-related complications. Although complete removal of the cholesteatoma was attempted via a translabyrinthine approach, this was not possible because the epithelium of the cholesteatoma was strongly attached to the internal carotid artery and posterior cranial fossa. A debris drainage route leading to the nasopharynx was created by drilling the clivus on the side of the lesion via a transsphenoidal approach. The patient has had no complications since surgery. The drainage route remains open, and the cholesteatoma has been controlled for 57 months. A markedly advanced petrous temporal bone cholesteatoma can be managed safety and reliably by combining a translabyrinthine approach with a transsphenoidal approach. Creation of a debris drainage route into the nasopharynx can prevent isolation of the cholesteatoma and mastoid cavity problems after surgery. This one-stage surgery may be a suitable method for keeping cholesteatoma under control in patients with unresectable petrous bone cholesteatoma.

A literature review on the infralabyrinthine petrous bone cholesteatoma (PBC) was presented in this article. Attention is paid to etiology and clinical symptoms of the disease, classifications. All modern classifications divide cholesteatoma with localization under the labyrinth into two big groups: infralabyrinthine and infralabyrinthine apical. This is not enough to determine the algorithm of surgical tactic of these patients. The most used approaches to the infrlabyrinyhine area and lateral skull base were analyzed (infralabyrinthine and infracochlear, subtotal petrosectomy, transotic approach, group of transcochlear approaches, infratemporal and translabyrinthine approaches). Possible variants of the surgery ending are described (tympanoplasty or “cul-de-sac” closure). Comparison of the results of different authors, starting from 1990, in which the infralabyrinthine cholesteatoma was distinguished as a separate class was carried out. 16 publications were analyzed based on the type of used classification, the total number of cases with infralabyrinthine cholesteatoma, the type of surgical approach, the complications and recurrence rate. The total number of patients was 141, 84 with infralabyrinthine (59,6%), 57 (40,4%) with infralabyrinthine apical PBC. The most common type of surgery were subtotal petrosectomy, transcochlear approach in different variations and transotic approach. The recurrence rate ranged from 0 to 29%. This paper identifies unresolved issues, the necessity of new classification and algorithm of surgical management based on it.

Introduction. Petrous temporal bone (PTB) cholesteatoma is an epidermal cyst, which is the result of uncontrolled growth of keratinizing squamous epithelium in the petrous part of the temporal bone. Cholesteatoma is classified into congenital, acquired, and iatrogenic.Objective. To discuss the classification of infralabyrinthine petrous bone cholesteatoma (PBC), add modified classificationand to propose adequate differential surgical management.Methods. The setting was a National Medical Scientific Center of Otorhinolaryngology FMBA (Russia). The data of 14 patients who underwent surgery for different variations of infralabyrinthine PBC from 2017 till 2020 were analyzed and included into the study (with respect to localization type of the approach used, complications, recurrences and outcome). The follow-up period ranged from 6 to 34 months with a median of 18 months.Results. Based on preoperative CT scans and intraoperative findings a Scale of Cholesteatoma extension CLIF(APO) and Modified classification of infralabyrinthine cholesteatoma (in relation to mastoid segment of the facial nerve) are proposed. The scale includes the main anatomical structures of the temporal bone and the adjacent parts of the occipital and sphenoid bones, which may be involved in the cholesteatoma process: cochlea, vestibule and semicircular canals, internal auditory canal, jugular foramen, bony chanal of the internal carotid artery, petrous apex, occipital condyle. Based on the modified classification and scale we present an algorithm for decision making and surgical approach choosing.Conclusion. The implementation of the Scale of Cholesteatoma Extension in Otology and Radiology practice will allow to preoperatively diagnose the extension of PBC, unify the data of the localization of cholesteatoma; allows standardization in reporting and continuity at all stages of treatment. The modified classification proposed by us in this article facilitate the algorithm for selecting the type of surgical approach and determine whether to perform less aggressive combined microscopic approaches with endoscopic control.

Objective: To discuss the therapeutic scheme of petrous bone cholesteatoma(PBC) and the technique of facial nerve reconstruction. Method: The data of 28 patients who underwent surgery for PBC in our center were analyzed retrospectively. All patients were diagnosed radiologically with PBCs and reconfirmed pathologically after surgery. The surgical approach was discussed basing Sanna's classification of PBCs, and the facial nerve outcomes were analyzed moreover. Result: PBC cases 11 were supralabyrinthine, 4 infralabyrinthine, 3 infralabyrinthine-apical, 10 massive and none apical. The facial nerve was involved in 50% of the cases. The translabyrinthine approach were used in 3 cases. The transotic approach was used in 7 cases in this series.The transcochlear approach type was applied in 12 cases. The Infratemporal fossa type B approach and subtotal petrosectomy were employed in 2 cases and 4 cases respectively. Active management of the nerve(rerouting, anastomosis, or grafting) was required in 14 cases, postoperative facial nerve function were inproved in 10 cases(71.43%). Conclusion: The appropriate surgery approach was vitally important to radical disease clearance in PBCs. The facial nerve preservation was preceded hearing preservation. Active facial nerve management were beneficial to facial nerve recovery..

Decision-making in Petrous Bone Cholesteatoma (PBC): Surgical options according to PBC classification.

The aim of this study is to assess the clinical characteristics, classification, surgical approaches, interventional strategies, and treatment outcomes of patients with petrous bone cholesteatoma (PBC). Observational retrospective study of patients with PBC managed by the senior author of this paper between 1995 and 2024 in a tertiary referral center. A literature review was made, identifying 16 articles. Clinical presentations, Sanna's classification, pre- and post-surgical facial function and audiometry, surgical approach and intraoperative endoscopic assistance, postoperative complications, and recurrence rates were reported. We report on a large series of 94 cases managed by the senior author. The most common type was supralabyrinthine PBCs (40.43%; 38 cases), followed by massive (18.09%; 17). Hearing loss was present in almost all the patients (98.91%). The most frequent approaches were the modified translabyrinthine approach (24.51%; 25), the subtotal petrosectomy (22.55%; 23) and the transotic approach (18.63%; 19). In 11 patients the approach was assisted by the use of an endoscope. Recurrence of PBC was identified in 8 cases (8.51%) that were surgically resolved. Additionally, we report a review of the literature (16 articles). We focus on the introduction of endoscopic assisted techniques that may help in reducing the rate of residual and avoid rerouting of the facial nerve. At the same time, we encourage techniques that include elimination of the middle ear through a subtotal petrosectomy, as they are essential to protect vital structures that have been exposed by the disease or by the surgical technique.

To evaluate the effectiveness of surgical treatment of patients with petrous bone cholesteatoma (PBC) depending on the localization of the pathological process. The analysis of surgical treatment using various surgical approaches and its results in 32 patients with PBC, depending on the type, localization in petrous bone and intraoperative findings, is presented. Patients with supralabirint PBC underwent extended atticoantromastoidotomy with tympanoplasty and mastoidoplasty with automaterials (n=19), labyrinthectomy (n=4), subtotal petrozectomy with labyrinthectomy and suturing of the external auditory meatus (EAM) (n=2). In infralabirint and infralabirint-apical PBC, a transotic approach was used with Rambo suturing of EAM (n=9). The pre-sigmoid approach was performed in 1 patient. With an extradural subtemporal approach, PBC of apical localization was removed in 1 case. After surgical treatment, hearing remained at the same level in 15 (47%) patients, 14 of them had deafness. In the early postoperative period, a temporary increase in bone conduction hearing thresholds by 10-20 dB was detected in 14 (44%) patients with their gradual recovery over 3 months. Deafness in the postoperative period developed in 3 (9%) patients after removal of supralabirint cholesteatoma. In the early postoperative period, 3 (9%) patients developed systemic dizziness, which was stopped after 3 months. In 25 (78%) patients, the function of the facial nerve in the early postoperative period remained at the same level, of which 14 (44%) were normal, and 11 (34%) had the same degree according to the House-Brackmann (HB) classification. Improvement of function by one degree of HB classification was observed in 4 (12.5%) patients on average 5 months after surgery. An adequate personalized choice of surgical treatment methods allowed mainly to preserve the function of hearing and facial nerve. In cases of deterioration of facial nerve function in the postoperative period, gradual improvement was observed for 3-10.5 months with further positive dynamics.

The term petrous bone cholesteatoma (PBC) refers to lesions extending deep to the bony labyrinth via superior, inferior, and posterior cell tracts. PBC is a rare incidence accounting for only 4-9% of petrous bone lesions. Lesions of petrous bone represent a real surgical challenge due to its complex relationship with critical neurovascular structures. To demonstrate our 40-plus years' experience in the management of PBC, depict the clinical features of PBC according to Sanna's Classification, evaluate the postoperative follow-up of surgically treated PBC patients, and determine the recurrence rate. Retrospective medical record review. Medical records of 298 PBC cases operated from the year 1983 to 2024 were thoroughly evaluated. A total of 298 PBC cases were surgically treated at our center. The average age at presentation in this series was 47 years. Males are more affected than females with a male-to-female ratio of 2.2:1. The most common presenting symptoms were hearing loss (84%), tinnitus (48%), and facial nerve paralysis (45%). Mixed hearing loss (41%) was the commonest audiometric pattern of hearing loss followed by conductive hearing loss (26%) and profound sensorineural hearing loss (4%) and a total of 86 (29%) had anacusis at presentation. On preoperative facial nerve function examination, 133(45%) of patients had various degrees of paresis and complete paralysis whereas 55% had normal HB-I function. The commonest degree of paresis noted was HB-III (18%) followed by HB-VI (5%). A total of 150 (50%) patients had previous otologic surgery and two-thirds of these cases had two or more prior otologic surgeries. According to Sanna's PBC Classification system, we identified that the supralabyrinthine class (44%) is the commonest of all classes followed by massive (33%), infralabyrinthine-apical (9%), infralabyrinthine(8%), and apical (5%) classes in that order. However, only ten patients had congenital type of PBC. Extension to clivus, sphenoid, nasopharynx, intradural space, and occipital condyle was found in 8, 2, 1, and 2 cases respectively. The most commonly used surgical approaches at our center were TO, MTCA with rerouting of the facial nerve, and TLAB with external auditory canal (EAC) closure. Postoperative complications were minimal and the duration of follow-up ranged from one to 458 months with a mean duration of 65 months. Residual lesions were evident in 11 cases (3.7%), with the surgical cavity, middle and posterior fossa dura, and jugular bulb being the commonest sites. Petrous bone cholesteatoma represents diagnostically and surgically challenging lesions of temporal bone which are usually frustrating to the treating surgeon. A high index of clinical suspicion, thorough clinical evaluation examination, and preoperative radiologic evaluation make the diagnosis easier. Preoperative anatomic classification of the lesion enables the physician to choose the appropriate surgical approach. Sanna's classification is widely used to classify PBC in relation to the labyrinthine block. Radical disease removal should always come before hearing preservation. Cavity obliteration is the solution to the problems related to a large cavity. Finally, advancements in lateral skull base approaches create adequate surgical access for the complete removal of the lesion with excellent control of critical neurovascular structures.

Objective: To analyze the clinical features and surgical outcomes of petrous bone cholesteatomas (PBCs). Methods: Data from 39 PBCs patients treated in the Department of Otorhinolaryngology, Xijing Hospital from September 2011 to December 2017 were reviewed retrospectively, including 23 males, 16 femals, aged 12-71 years old, with the median age of 37. Clinical classifications, surgical methods, facial and hearing function, and intraoperative and postoperative complications were made summary analysis. Results: In this study, five patients were congenital PBCs and 34 patients were acquired PBCs. The common clinical symptoms were hearing loss (100%, 39/39), ear discharge/pus (89.7%, 35/39) and facial paralysis (46.2%, 18/39). According to Sanna's classification, 14 cases were supralabyrinthine, including three cases underwent transcochlear (TC) approach, six cases underwent transotic (TO) approach and five underwent translabyrinthine (TL) approach. 10 cases were infralabyrinthine, including eight cases underwent subtotal petrosectomy, one case underwent TO approach and one underwent TL approach.10 cases were massive, including seven cases underwent TC approach, three cases underwent TO approach. Five cases were infralabyrinthine-apical, including two cases underwent TC approach, two cases underwent TO approach, and one case underwent endoscope assisted infratemporal fossa type B. The degree of facial nerve (FN) dysfunction from high to low was massive (6/10), supralabyrinthine (8/14), infralabyrinthine-apical (2/5) and infralabyrinthine (2/10). 19 cases involved in facial nerve operation, three cases underwent FN decompression, four cases underwent FN rerouting, four cases underwent nerve grafting, and one case underwent facial-hypoglossal anastomosis. Preoperative FN involvement in 18 cases, and the FN function was improved in 14 cases after surgery. The improved rate of postoperative FN function was 77.8%. The bone conducted hearing retained 50.0% (14/28) postoperatively. Five cases with cerebrospinal fluid leak were managed by inserting free muscle plugs and cavity obliteration. Two cases with the cholesteatomas matrix involved the sigmoid sinus and the jugular bulb, and occlusion of the sigmoid sinus was performed. Postoperatively, two patients presented with synkinesis. The patients were followed up for 40 to 115 months, and there was no recurrence. Conclusions: There are no specific clinical manifestations for PBCs, thus, it is difficult in early diagnosis and treatment. According to Sanna's classification, preoperative FN and hearing function, the best surgical approach should be selected with minimal recurrences and perioperative morbidity.

The term petrous bone cholesteatoma (PBC) represents a slow-growing epidermal lesion arising from the petrous part of the temporal bone. It is a rare incidence accounting for only 4-9% of all petrous bone lesions. PBC represents a real surgical challenge due to its complex relationship with critical neurovascular structures. To demonstrate our experience in using various options of facial nerve (FN) management during surgical treatment of PBC and analyze the long-term facial function outcomes. Retrospective medical record review in a quaternary skull-base center. Medical records of 298 PBC cases operated between the years 1983 and 2024 were thoroughly evaluated. Sanna's classification scheme was used to classify cases into the appropriate class and the House-Brackmann (HB) grading system of the FN was used to assess the facial function pre- and postoperatively. A total of 298 PBC cases were surgically treated at our center. Males constitute 68% (n = 203) of total operated PBC patients while the rest 32% (95) were females, making the male-to-female ratio 2.2:1. The age in this series ranged from 9 to 85. According to updated Sanna's PBC classification, 44% were supralabyrinthine, 33% were massive, 9% were infralabyrinthine-apical, 8% were infralabyrinthine, and 5% were apical. On preoperative FN function examination, 45% (n = 133) of patients had various degrees of paresis and complete paralysis whereas 55% (n = 165) had normal FN function. The commonest degree of paresis observed was HB-III (18.5%) followed by HB-IV (5.7%). A total of 40(13.4%) patients, however, had complete facial paralysis at the initial presentation. The facial function was compromised more frequently in supralabyrinthine and massive PBCs. Among the total patients who had an abnormal facial function at presentation, 52% had paresis/paralysis for less than a year whereas 48% had FN paresis/paralysis for greater than one year. The most commonly used surgical approaches at our center were transotic (TO), modified transcochlear type A (MTCA) with rerouting of the FN, and translabyrinthine (TLAB) with external auditory canal (EAC) closure. Intraoperatively an intact fallopian canal was found only in 33% (n = 99) cases whereas the rest 67% (n = 199) had erosion of the fallopian canal either with intact (n = 24) or infiltrated FN (n = 175). The FN was maintained in the bony fallopian canal in 35% (n = 104) cases. An active form of FN management, however, was required in 65% (n = 194) of cases either intraoperatively or as a second-stage procedure. Factors affecting postoperative FN function include age, preoperative FN function (HB), duration of paralysis, class of PBC, surgical approach, and method of active FN management. PBCs represent diagnostically and surgically challenging lesions of temporal bone which are usually frustrating to the treating surgeon. Preoperative anatomic classification of the lesion enables the physician to choose the appropriate surgical approach. Sanna's classification is the most widely accepted system used to classify PBC. This classification is based on the relation of the lesion to the labyrinthine block. Active FN management, including rerouting, end-to-end anastomosis, cable nerve grafting, and masseteric-to-FN anastomosis routinely come into play in the surgical management of PBCs, and the postoperative FN results after such interventions can be satisfactory in most cases.

Objective: To investigate the choice of surgical approach of petrous bone cholesteatoma (PBC)and surgical outcomes. Methods: A retrospective study was performed on 90 patients diagnosed and treated for PBC from January 2000 to December 2014 by the Chinese People's Liberation Army General Hospital otolaryngologists. According to Sanna's classification, 40 out of the 90 cases were supralabyrinthine, five infralabyrinthine, four infralabyrinthine-apical, 25 massive and 16 apical. Five cases underwent transmastoid and retrolabyrinthine approach, translabyrinthine approach was performed on six patients, 19 cases underwent subtotal petrosectomy, seven cases underwent transotic approach, 41 cases underwent middle fossa approach, combined transmastoid/middle fossa approach was performed on 11 cases, translabyrinthine and sphenoid sinus approach were performed on one case. Supralabyrinthine cases mainly applied middle fossa approach (77.5%, 31/40) and combined transmastoid and middle-fossa approach(20.0%, 8/40). Combined transmastoid-retrolabyrinthine approach were applied for all the infralabyrinthine cases (100.0%, 5/5). Infralabyrinthine-apical cases mainly applied subtotal petrosectomy (75.0%, 3/4). Massive cases mainly applied subtotal petrosectomy (60.0%, 15/25), transcochlear approach (20.0%, 5/25), and translabyrinthine approach (16.0%, 4/25). Apical cases mainly applied middle fossa approach (62.5%, 10/16). Results: Ninty percent (18/20) of the patients who had preoperative grade Ⅰ facial nerve function maintained in the postoperative period. Out of 90 cases, only 11 cases received open cavity, and the rest cases received cavityobliteration. There were three cases of recurrence, four cases of cavity infection, three cases of cerebrospinal fluid leakage, and one case of epidural hematoma, who all received surgeries. Conclusions: Sanna's classification should be used to classify different kinds of PBC cases, choose the best surgical approach for different cases, and preserve or repair facial function during removal of PBC, and thus reduce recurrence and complications.

Objective: To review the classification and management of petrous bone cholesteatomas (PBCs) at our center and the outcomes of facial nerve (FN) management in these lesions. Methods: This was a retrospective study. The setting was a quaternary referral center for skull base pathology in Italy. A total of 200 patients with 201 PBCs were included in the study. All patients diagnosed radiologically with PBCs were classified according to the Sanna classification. All patients were surgically treated and followed up with radiology. The main outcome measures - classification of PBCs, the surgical approach used, disease control, and FN outcomes - were analyzed. Results: Supralabyrinthine PBCs were the most common type with 92 cases (45.8%) followed by the massive PBCs with 72 cases (35.8%). Preservation of preoperative FN function was highest in the infralabyrinthine (72.2%) and infralabyrinthine-apical (73.3%) types. The transotic approach was used in 66 cases (32.8%) in this series. The modified transcochlear approach type A was applied in 55 cases (27.3%). Active management of the nerve (rerouting, anastomosis, or grafting) was required in 53 cases (26.4%). Postoperatively, of the 116 cases with FN House-Brackmann grade I and II, 107 cases (92.2%) retained the same grade or improved. Recurrence was seen in 7 cases (3.5%). The mean duration of follow-up was 6.3 years. Conclusions: Radical disease clearance must take precedence over hearing and FN preservation in PBCs. Active FN management, including rerouting, end-to-end anastomosis, and cable nerve grafting, routinely come to play in the surgical management of PBCs, and the postoperative FN results after such interventions can be satisfactory in most cases.

This paper describes a series of patients with a petrous temporal bone cholesteatoma paying particular attention to the complications and their management. Sixteen patients who underwent surgery in our department were reviewed. Topographically, the petrous bone cholesteatomas were grouped into five categories according to the classification proposed by Sanna et al. There were five massive labyrinthine; five infralabyrinthine; one apical; four supralabyrinthine; and one infralabyrinthine-apical. Clinically, the presenting symptom of these lesions were facial nerve paralysis (10 patients) and unilateral deafness (13 patients). Total removal of the cholesteatomas was achieved in all patients using different surgical approaches according to their site and extent. Recurrences were observed in two patients after 8 months and 24 months, respectively. The facial nerve was infiltrated and compressed by the cholesteatoma in eight patients. Seven were managed with cable grafts using sural nerve. One of these patients was treated using a facial-hypoglossal anastomosis because of the failure of the graft. In the remaining patient, a baby-sitter procedure was employed. In the other two patients, the preoperative facial paralysis was due to compression by the cholesteatoma, and its removal allowed partial recovery of facial function. The rationale of the surgical management of petrous bone cholesteatoma is its radical and total removal. Our present policy is to prefer approaches which result in a closed cavity obliterating the eustachian tube and closing the auditory canal as a blind sac. Facial nerve function is the main complication of these lesions, Facial nerve involvement requires rapid management because the duration of the paralysis is directly related to poor recovery of facial function.

To demonstrate our experience in the treatment of petrous bone cholesteatoma (PBC). Data of PBC patients in our hospital from January 2000 to December 2019 were collected. Surgical approaches and facial function were mainly discussed and compared with the literature. The management of 2 giant PBC cases affecting rhinopharynx has been demonstrated. The supralabyrinthine type was the most frequent type followed by the massive type. There were 5 cases with cholesteatoma extending into the clivus (2 cases), sphenoid (1 case) and rhinopharynx (2 cases). The translabyrinthine approach (40%) was our most frequently used approach followed by the middle fossa approach (36%) and the transmastoid approach (11%). There were 10 cases managed with the assistance of endoscope, including 3 cases with cholesteatoma extending into clivus, sphenoid and rhinopharynx separately. Obliteration of the cavity was performed in 70.3% (135/192) cases; 3 of them recurred. For the 2 giant PBC cases affecting rhinopharynx, traditional microscopic surgery assisted with transnasal endoscope was performed. The reduced exposure was beneficial for postoperative recovery, and the approach in the nasal cavity provided a permanent drainage for postoperative examination. Otologic endoscope combined with traditional microscopic surgery could reduce the exposure in surgery. For extremely extended cases of PBC, supplementary transnasal endoscopic approach deserves to be considered for the traditional temporal bone approach.

We report a case of successful cochlear implantation in a patient with petrous bone cholesteatoma in the only hearing ear. A 63-year-old man presented with a four-year history of right-sided, progressive hearing loss in his only hearing ear. Computed tomography and magnetic resonance imaging revealed a right supralabyrinthine petrous bone cholesteatoma, with erosion of the superior semicircular canal and the roof of the internal auditory canal. Due to the high risk of post-operative right-sided deafness, we decided first to perform left cochlear implantation. Five months later, the patient had a 40 per cent score for open-set two-syllable word recognition and an 85 per cent score for sentence recognition. Given these good performances, we decided to eradicate the cholesteatoma via a translabyrinthine approach, with insertion of a second cochlear implant, as a single-stage procedure. A successful outcome was achieved. Cochlear implantation can be an effective method of hearing rehabilitation in patients with petrous bone cholesteatoma, following total eradication of disease, if the cochlea remains intact. To our best knowledge, this is the first English language report of cochlear implantation in a patient with petrous bone cholesteatoma.