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Patients with petrous bone cholesteatoma most commonly present with hearing loss and facial paralysis. Other clinical presentations can include otorrhea, tinnitus, aural fullness, otalgia, and dizziness. Here, we report a patient with petrous bone cholesteatoma who presented with cerebrospinal fluid rhinorrhea. This patient was initially misdiagnosed with rhinogenic cerebrospinal fluid leakage, and was finally diagnosed with the supralabyrinthine type of petrous bone cholesteatoma. During the surgical repair through the transmastoid trans-superior semicircular canal approach, the fistula was found in the superior wall of the internal auditory canal. He had satisfactory outcomes after the surgery, with no recurrent cerebrospinal fluid rhinorrhea., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Rhabdomyosarcoma is a common soft tissue tumor, but isolated involvement of anterior portion of petrous bone is exceedingly rare. Here, we present a case of embryonal rhabdomyosarcoma involving the anterior petrous without involvement of the mastoid and middle ear., Patient: A 6-year-old boy presented with a progressive right side lower motor neuron facial paresis for 1-month duration along with headache and recurrent vomiting episodes. Radiology showed a contrast-enhancing lesion involving the right petrous apex. He underwent craniotomy and excision of the lesion. Based on the frozen section, a diagnosis of rhabdomyosarcoma was rendered, and gross total resection could be achieved. Postoperative course was uneventful., Conclusion: Isolated petrous bone involvement of embryonal rhabdomyosarcoma is a rare presentation. Intra-operative frozen section plays a key role in decision making regarding the extent of excision. Hence, a prompt and accurate diagnosis is essential in managing these cases.

Background and Objectives: Triangular corridors have been used as reliable surgical entry points for open transcranial approaches to the petrous apex (PA) and petroclival region (PCR). The endoscopic endonasal approaches have grown rapidly in the last decade, and the indications have advanced. The knowledge of accurate and reliable anatomic landmarks through endoscopic endonasal route is essential and remain to be established. The purpose of this study was to describe the feasibility and surgical exposure of the anteromedial petrous (Gardner's) triangle as a novel corridor to the PA and PCR., Methods: Five anatomic specimens were dissected. The PA and PCR were accessed through endoscopic endonasal approaches and contralateral transmaxillary approach. The limits of the anteromedial petrous (Gardner's) triangle were identified and dissected and associated measurements performed., Results: The dissection was divided into 6 steps. The limits of the anteromedial petrous (Gardner's) triangle were identified and defined by the paraclival internal carotid artery anterolaterally, the abducens nerve posteromedially, and the petroclival synchondrosis inferiorly. Three lines were established following the limits of the triangle. The mean distance of the anterolateral limit was 10.03 mm (SD = 0.94), of the posteromedial limit was 20.06 mm (SD = 2.90), and of the inferior limit was 17.99 mm (SD = 2.99). The mean area was 87.56 mm 2 (SD = 20.06). The 3 anatomic landmarks with a critical role to safely define the triangle were the pterygosphenoidal fissure, the petrosal process of the sphenoid bone, and the petroclival synchondrosis., Conclusion: The anteromedial (Gardner's) triangle is a well-defined bone corridor which provides access to the entire petrous bone and petroclival junction through endoscopic endonasal route. Regardless of the anatomic variations or tumor location, the landmarks of the abducens nerve, paraclival internal carotid artery, and petroclival synchondrosis are key for understanding lateral access to tumors extending from the clivus., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Significance Statement: Petrous cholesteatoma is rare but frequently leads to complications. A thorough radiological evaluation helps in identifying the pathology and the extent of disease. Sound anatomical knowledge is vital for the planning of surgical approach to get adequate access without damaging important structures. Presentations in congenital lesions are varied due to the uneven growth model of the temporal bone. To the best of our knowledge, the pattern of involvement in this case has never been reported earlier., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background and Objective: The combined petrosectomy is one of the workhorse skull base approaches to the petroclival region. Traditionally, this approach starts with a temporosuboccipital craniotomy, followed by the mastoidectomy/anterior petrosectomy, and completed with the dural opening/tumor resection. This sequence of events (neurosurgery-neuro-otology-neurosurgery) involves at least 2 handoffs and change of surgical teams and instrumentation. This report describes a resequencing of events and a modification of the technique used to craft the temporosuboccipital craniotomy, with aims to reducing handoffs between surgical teams and improving operating room workflow., Methods: Adhering to PROCESS guidelines, a case series is provided in addition to the surgical technique and surgical images., Results: The technique for performing a combined petrosectomy is described with illustrations. This description shows that the temporal bone drilling may be performed before the craniotomy to allow for direct visualization of the dura and sinuses before completing the craniotomy. In doing so, only 1 transition between the otolaryngologist and neurosurgeon is necessary, thereby improving operating room workflow and time management. A series of 10 patients is presented, showing the feasibility of this procedure and providing operative details that were previously absent in the peer-reviewed literature., Conclusions: Combined petrosectomy, although often performed in a 3-step manner with the neurosurgeon starting the craniotomy, can be performed as described here in a 2-step manner, with similar outcomes and reasonable operating time., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Aneurysmal bone cysts are expansile benign lesions associated with compressive destruction and obscure pathogenesis. The most common sites of temporal bone involvement are the petrous apex, squamous portions and mastoid., Case Report: This paper reports a right temporal aneurysmal bone cyst in a 51-year-old man who presented clinically with facial palsy, and hearing loss and impaired vestibular function. Magnetic resonance imaging and computed tomography findings were consistent with a diagnosis of aneurysmal bone cyst. Inter-operative findings showed that the lesion had caused compressive damage to the internal auditory canal. Following surgical excision, the patient experienced vertigo, indicating recovery of vestibular function. Follow-up imaging revealed complete resection without clinical recurrence., Conclusion: To our knowledge, this is the first report of aneurysmal bone cyst invasion of the inner auditory canal. Our clinical experience indicates that vestibular nerve damage recovery is relatively uncommon. This case report will hopefully inform future studies.

Competing Interests: The authors disclose no conflicts of interest.

Purpose: Report our experience in the management of posterior petrous surface meningiomas (PPSMs), and identify features that affect hearing, facial nerve (FN) function, and control of the disease., Methods: Retrospective case series of 131 patients surgically managed for PPSMs. FN status, hearing and tumour radicality were assessed and compared between patients with tumours of different locations (Desgeorges classification) and internal auditory canal involvement (IAC)., Results: At the time of surgery 74.8% of patients had a hearing loss. Hearing was mostly unserviceable in tumors attached to the meatus. Pure tone audiometry did not correlate to IAC extension, while speech discrimination scores were statistically worse when the tumor occupied the IAC (unpaired t test, p = 0.0152). Similarly, extrameatal tumors undergoing removal by otic preserving techniques maintained postoperative hearing, whereas hearing worsened significantly in tumors involving the IAC (paired t test, p = 0.048). The FN was affected preoperatively in 11.4% of cases. Postoperative FN palsy was significantly correlated to the IAC involvement (Fisher's exact test, p = 0.0013), while it was not correlated to tumor size. According to the Desgeorges classification, a postoperative FN palsy complicated the majority of anteriorly extending tumors and, two-fifths of meatus centred tumors. 75% of posterior located tumors had a postoperative FN grade I HB., Conclusions: Since the involvement of the IAC by the tumor affects both hearing and FN function, the IAC is of primary importance in PPSMs and should be studied and addressed as much as the tumor location in the CPA., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The petrous apex (PA) is involved in a myriad of pathological conditions, some of which are exclusive in children. Diagnosis may be difficult due to vague clinical presentation, and local examination is challenging owing to its inaccessible location. This is further complicated by multiple unfused sutures and ongoing PA pneumatization in children. Cross-sectional imaging is vital for the evaluation of the PA lesions, due to their precarious location and proximity to the major neurovascular structures. Several classification systems have been proposed for these lesions based on their site of origin, solid or cystic appearance, surgical or non-surgical (no touch lesions) management, and benign or malignant nature. In this article, we emphasize the distinctive role of different cross-sectional imaging modalities in the diagnosis of pediatric PA lesions, with special attention to normal variants that should not be mistaken for pathology. We also propose a radiological classification and algorithmic approach to aid in the precise diagnosis and facilitate appropriate management of the various PA lesions in children.

Background: The choice of surgical approach for a petrous apex lesion depends on its relationship with the internal carotid artery, degree of medial expansion and pathology. The correct identification of patients who will benefit from this approach is necessary., Case Reports: Two adult patients presented with a lesion in the left petrous apex. Computed tomography showed a homogeneous mass extending anteromedially, and abutting the internal carotid artery and the sphenoid sinus in both patients. Using magnetic resonance imaging, a third recurrence of cholesterol granuloma in case one and cholesteatoma in case two were diagnosed. Both patients underwent trans-sphenoid excision, as the sphenoid sinus was well pneumatised and the lesion was medial to the internal carotid artery., Conclusion: Nasal endoscopic access to the petrous apex via the trans-sphenoid corridor should be preferred for benign lesions extending anteromedially in cases where lateral access is impeded by the internal carotid artery, the labyrinth and the facial nerve, and anterior trans-sphenoidal access offers a low-morbidity alternative.

Introduction: Internal auditory canal (IAC) diverticula, also known as IAC cavitary lesions or anterior cupping of the IAC, observed in otopathologic specimens and high-resolution computed tomography (CT) scans of the temporal bone are thought to be related to otosclerosis. Herein, we examined the usefulness of CT scans in identifying diverticula and determined whether IAC diverticula are associated with otosclerosis on otopathology., Methods: One hundred five consecutive specimens were identified from the National Temporal Bone Hearing and Balance Pathology Resource Registry. Inclusion criteria included the availability of histologic slides and postmortem specimen CT scans. Exclusion criteria included cases with severe postmortem changes or lesions causing bony destruction of the IAC., Results: Ninety-seven specimens met criteria for study. Of these, 42% of the specimens were from male patients, and the average age of death was 77 years (SD = 18 yr). IAC diverticula were found in 48 specimens, of which 46% were identified in the CT scans. The mean area of the IAC diverticula was 0.34 mm 2 . The sensitivity and specificity of detecting IAC diverticula based on CT were 77% and 63%, respectively. Overall, 27% of specimens had otosclerosis. Histologic IAC diverticula were more common in specimens with otosclerosis than those without (37.5% versus 16%; p = 0.019). Cases with otosclerosis had a greater mean histologic diverticula area compared with nonotosclerosis cases (0.69 mm 2 versus 0.14 mm 2 ; p = 0.001)., Conclusion: IAC diverticula are commonly found in otopathologic specimens with varied etiologies, but larger diverticula are more likely to be associated with otosclerosis. The sensitivity and specificity of CT scans to detect IAC diverticula are limited., Competing Interests: J.C. has a patent pending at Grace Medical. E.K. is a scientific advisor at Desktop Metal and has a patent pending at Grace Medical. H.S. is in the surgical advisory board of MedEl and a shareholder at Rescue Hearing. M.J.McK. is the co-founder and CMO of Akouos Inc. A.Q. has a sponsored research agreement with Frequency Therapeutics, sponsored research agreement, patent pending at Grace Medical, and consulting for Alcon. The remaining authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Cholesterol granulomas are the most common primary lesions of the petrous apex. Although their benign character, impingement of critical neurovascular structures can cause significant symptomatology such as hearing impairment. We present unique results after treatment of a cholesterol granuloma located in the petrous apex causing sensorineural hearing impairment. A transmastoidal approach was performed using an intraoperative computed tomography-guided navigation system. The video, which is included for reference, illustrates clear drainage of the cyst and drain positioning. The hearing improved completely in the lower frequencies at 500 and 1000 Hz and with a 19 dB improvement in overall bone conduction in the affected ear.

Objective: Concerns about the approach-related morbidity of the extradural anterior petrosal approach (EAPA) have been raised, especially regarding temporal lobe and venous injuries, hearing impairment, facial nerve palsy, cerebrospinal fluid fistula, and seizures. There is lack in the literature of studies with detailed analysis of surgical complications. The authors have presented a large series of patients who were treated with EAPA, focusing on complications and their avoidance., Methods: The authors carried out a retrospective review of patients who underwent EAPA at their institution between 2012 and 2021. They collected preoperative clinical characteristics, operative reports, operative videos, findings on neuroimaging, histological diagnosis, postoperative course, and clinical status at last follow-up. For pathologies without petrous bone invasion, the amount of petrous apex drilling was calculated and classified as low (< 70% of the volume) or high (≥ 70%). Complications were dichotomized as approach related and resection related., Results: This study included 49 patients: 26 with meningiomas, 10 brainstem cavernomas, 4 chondrosarcomas, 4 chordomas, 2 schwannomas, 1 epidermoid cyst, 1 cholesterol granuloma, and 1 osteoblastoma. The most common approach-related complications were temporal lobe injury (6.1% of patients), seizures (6.1%), pseudomeningocele (6.1%), hearing impairment (4.1%), and dry eye (4.1%). Approach-related complications occurred most commonly in patients with a meningioma (p = 0.02) and Meckel's cave invasion (p = 0.02). Gross-total or near-total resection was correlated with a higher rate of tumor resection-related complications (p = 0.02) but not approach-related complications (p = 0.76). Inferior, lateral, and superior tumoral extension were not correlated with a higher rate of tumor resection-related complications. No correlation was found between high amount of petrous bone drilling and approach- or resection-related complications., Conclusions: EAPA is a challenging approach that deals with critical neurovascular structures and demands specific skills to be safely performed. Contrary to general belief, its approach-related morbidity seems to be acceptable at dedicated skull base centers. Morbidity can be lowered with careful examination of the preoperative neuroradiological workup, appropriate patient selection, and attention to technical details.

The Anterior transpetrosal approach is a skull base surgical procedure that can provide a wide operative view of the petroclival and the prepontine region. This approach requires one to accurately understand the anatomy of the anterior part of the temporal bone and the histological structure of the dura mater. In addition, it is necessary to learn operative techniques for venous preservation, accurate hemostasis at each step, and prevention of cerebrospinal fluid leakage. In this article, the anatomy and surgical techniques that are required for a beginner to master the anterior transpetrosal approach are explained step by step.

Abstract: Petrous apex cephalocele (PAC) is a rare type of cephalocele. It is usually asymptomatic. It can be unilateral or bilateral. Bilateral cases are less common. They are etiologically and clinically different entities from unilateral ones. A 56-year-old female patient presented to the hospital with the complaint of a severe nonspecific headache. She has been examined in ear nose throat, ophthalmology, and finally neurology clinics. Bilateral PAC was incidentally found in magnetic resonance imaging (MRi) examination. Headache was associated with PAC because no additional pathology could explain the clinical symptomatology. There is a proven relationship between empty sella and PAC. Regarding PAC less than 50 cases are known. Our paper aims to contribute to the literature by exhibiting etiologic and clinic differences between unilateral and bilateral PAC. It highlights the relationship between PAC and broad sella that is a different entity from empty sella and shows this remarkable radiological appearance. The authors presented our case accompanied by clinical and MRi findings., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Many different benign and malignant processes affect the central skull base and petrous apices. Clinical evaluation and tissue sampling are difficult because of its deep location, leaving imaging assessment the primary means for lesion evaluation. Skull base lesions demonstrate a variety of confusing appearances on imaging, creating diagnostic dilemmas. It is important to be familiar with imaging appearances of common mimickers of malignant neoplasm in the skull base. This article familiarizes readers with imaging characteristics of various anatomic variants and benign pathologies that mimic malignant neoplasms, in hopes of increasing confidence of diagnosis, decreasing unnecessary procedures, and allaying patient fear., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: To evaluate the effectiveness of surgical treatment of patients with petrous bone cholesteatoma (PBC) depending on the localization of the pathological process., Material and Methods: 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., Results: 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., Conclusion: 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 authors present a patient and neurosurgical nuances of total resection of recurrent meningioma of posterior surface of petrous bone 65´35´30 mm. The tumor captured two critical zones of posterior cranial fossa with unusual frontal growth and spread from the surface of petrous bone to the fourth ventricle. The neoplasm filled the ventricle without lesion of ependyma. Extensive fibrous meningioma of posterior surface of petrous bone was totally excised 18 years ago. MRI was annually carried out for 5 years. Surgical nuances that ensured total extraction of tumor without cytoreduction were as follows: 1) en-bloc resection of tumor from the fourth ventricle due to smooth surface of tumor and minimum number of adhesions with cerebellum; MR-confirmed CSF strip between the tumor edges and walls of the ventricle; no signs of hydrocephalus in subtotal ventricular tamponade; 2) unusual frontal tumor growth under 45º required appropriate angular traction of tumor with minimal rotation; 3) traction was followed by sequential appearance of 3 segments of tumor: petrous, apertural and ventricular; 4) topography of the area of lateral eversion of the fourth ventricle was established by identifying the narrowing (constriction) of tumor; 5) in extracting the tumor from the fourth ventricle, we performed minimal rotation to avoid damage to ventricular walls and lateral aperture due to difference between the larger and smaller diameters (by 6 mm) of ovoid ventricular segment of tumor; 6) no CSF leakage following appearance of ventricular segment (tumor enlargement) indicated integrity of ependyma of the fourth ventricle. Histological examination confirmed fibrous meningioma. Fast and complete regression of focal symptoms was observed after surgery. A 3-year follow-up after surgery revealed no signs of tumor recurrence.

Intradiploic epidermoid cysts are benign tumors that account for ∼1% of all intracranial space occupying lesions. Because of the indolent growth, symptoms are usually gradual in onset with a prolonged duration. While they can originate from any part of the skull, temporal bone epidermoids are rare. We describe a case of an unusual presentation of a temporal bone epidermoid cyst. A 64-year-old male presented with sudden right-sided hearing loss and worsening right eyelid ptosis was found to have a nonenhancing lobulated mass above the right tegmen tympani with complex signal and possible communication with the apical turn of the cochlea on magnetic resonance imaging (MRI). He underwent subtemporal craniotomy with excision of the cyst lining and matrix without any complications and was discharged home on postoperative day 1. Complete excision was not possible given that the facial nerve and cochlea were improved. He had subjective improvement in his hearing immediately after surgery. Intradiploic epidermoid cysts are benign tumors that can present anywhere in the skull with variable presentation. While complete excision is the goal, neurovascular involvement may make this challenging. The use of computed tomography scan and MRI can prove to be invaluable in determining accurate diagnosis and size for surgical planning.

Objective: Jugular bulb abnormalities (JBA) such as high riding jugular bulb and jugular bulb diverticulum can extend or erode into the middle and inner ear. In this report, we report on a series of 6 patients with jugular bulb anomalies involving the internal auditory canal (IAC)., Methods: A retrospective case series., Results: Six females, ages 6 to 63 presenting with myriad of otologic symptoms including hearing loss, tinnitus, balance disturbance, fullness, and otalgia were discovered to have JB eroding into IAC. Computerized tomography, but not Magnetic Resonance Imaging, was able to identify IAC erosion by a significantly enlarged JB., Conclusion: A significantly enlarged JB eroding into the IAC maybe congenital or acquired. It can present with a variety of common otologic symptoms. Long term follow-up is needed to determine the natural history of JB anomalies involving the IAC and need for intervention.

Objective: To investigate the association between sinus tympani volume and petrous apex pneumatization in this retrospective-archival temporal bone computed tomography study., Methods: We included 46 temporal bones with pneumatized petrous apex from 26 patients and 52 temporal bones without petrous apex pneumatization from 26 other patients. Using OsiriX software for MacOS (version 3.8.1, Pixmeo), we measured the volumes from three-dimensional sinus tympani models and compared the sinus tympani volumes and depths between the temporal bones with and without pneumatized petrous apex., Results: Among 150 patients totally reviewed, 26 (17.3%) had petrous apex pneumatization. The median sinus tympani volume was 16.05 (5.6-59.7) mm 3 in temporal bones with pneumatized petrous apex and 8.7 (1.76-59.7) mm 3 in temporal bones without. The sinus tympani volume was significantly greater in temporal bones with pneumatized petrous apex compared to those without (p < 0.001). Additionally, temporal bones with pneumatized petrous apex had a significantly deeper sinus tympani [median depth = 2.17 (0-3.04) mm] compared to the temporal bones without [median depth = 1.69 (0-3.73) mm] (p = 0.045). We found that petrous apex pneumatization was associated with deeper and larger sinus tympani., Conclusion: Patients with pneumatized petrous apex had a greater sinus tympani volume associated with the increased risk of residual cholesteatoma., Competing Interests: Declaration of Competing Interest The authors declare that there is no conflict of interest., (Copyright © 2020. Published by Elsevier B.V.)

Objective: NF2 patients can develop new meningiomas throughout their lifetime. Little is known about the clinical features of newly developed NF2 meningiomas. In this study, we analyzed newly developed NF2 meningiomas in a large patient population., Patients and Methods: Among 452 NF2 patients, the location patterns of 81 pediatric and 939 adult NF2 meningiomas were compared to find the predominant locations of newly developed meningiomas in adulthood. The clinical features of 39 newly developed meningiomas in 24 NF2 patients were summarized. Clinical risk factors of NF2 meningioma growth rates were analyzed., Results: Pediatric patients had significantly more intracranial meningiomas than adult patients at the skull base (except for the petrosal region) (p < 0.0063). Adult patients had significantly more cranial meningiomas than pediatric patients at the parasagittal, parafalcine (middle & posterior), and frontal/parietal/cerebellar convex surfaces (p < 0.0063). Newly developed NF2 meningiomas in adults tended to occur at different locations than the locations of NF2 meningiomas in pediatric patients. New meningiomas could develop at various ages. Ninety-five NF2 patients were imaged and followed up for at least one year. Twenty-four patients (25.3 %) developed 39 new meningiomas during the follow-up period. They usually had initial meningiomas when new meningiomas occurred. The number of newly developed meningiomas per patient and the petrosal location were significantly associated with both the absolute and relative annual growth rates (p < 0.05)., Conclusions: The number of newly developed NF2 meningiomas seems to be a clinical marker of NF2 disease severity. In adults, new NF2 meningiomas tend to occur in patients with initial meningiomas. The predominant locations of newly developed NF2 meningiomas seem to be the parasagittal, parafalcine (middle/posterior), and frontal/parietal/cerebellar convex surfaces., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Over the last few years, palaeogenomic studies of the petrous bone (the densest part of the temporal bone) have shown that it is a source of DNA in both larger quantities and of better quality than other bones. This dense bone around the otic capsule has therefore been called the choice substrate in palaeogenomics. Because the practice of forensic genetics responds to different imperatives, we implemented a study aimed at (i) understanding how and why the petrous bone is an advantageous substrate in ancient DNA studies and (ii) establishing whether it is advantageous in forensic STR typing. We selected 50 individual skeletal remains and extracted DNA from one tooth and one petrous bone from each. We then amplified 24 STR markers commonly used in forensic identification and compared the quality of that amplification using the RFU intensities of the signal as read on the STR profiles. We also performed histological analyses to compare (i) the microscopic structure of a petrous bone and of a tooth and (ii) the microscopic structure of fresh petrous bone and of an archaeological or forensic sample. We show that the RFU intensities read on STR profiles are systematically higher in experiments using DNA extracted from petrous bones rather than teeth. For this reason, we were more likely to obtain a complete STR profile from petrous bone material, increasing the chance of identification in a forensic setting. Histological analyses revealed peculiar microstructural characteristics (tissue organization), unique to the petrous bone, that might explain the good preservation of DNA in that substrate. Therefore, it appears that despite the necessity of analysing longer fragments in forensic STR typing compared to NGS palaeogenomics, the use of petrous bones in forensic genetics could prove valuable, especially in cases involving infants, toothless individuals or very degraded skeletal remains., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Multiple myeloma is a monoclonal proliferation of plasma cells with common involvement of vertebrae, ribs and skull vault. However, involvement of skull base is relatively uncommon and myeloma manifesting initially as a petrous apex mass is distinctly rare. We report a rare case of non-secretory multiple myeloma in a 52-year-old Egyptian male presenting primarily as a right petrous apex mass with abducens nerve palsy. Additionally, neoplastic cells aberrantly expressed cytokeratin. Although rare, plasma cell myeloma should be considered in the differential diagnosis of petrous apex masses.

A 15-year-old female presented for evaluation of progressive hearing loss over a year. Computed tomographic imaging revealed a 11 x 6 × 6 mm osseous lesion with 'groundglass' appearance within the left posterior petrous bone lateral to vestibular aqueduct suspicious for an endolymphatic sac tumor. Surgical excision revealed fibrous dysplasia on histological analysis. Fibrous dysplasia of the temporal bone is not uncommon, but few cases of extension causing sensorineural hearing loss exist in the literature. We describe the first reported case of fibrous dysplasia mimicking an endolymphatic sac tumor; interestingly, the patient also showed hearing improvement immediately following removal., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Cerebellopontine angle (CPA) meningiomas arise from the petrous face of the temporal bone, which forms the lateral boundary of the CPA. They can be categorized into anterior, middle, and posterior, based on their attachment in relation to the internal acoustic meatus. Each of them presents with their own characteristic clinical syndromes. Because of their close proximity to neurovascular structures, they pose a challenge during surgery. Microsurgery remains the primary treatment modality for large and symptomatic meningiomas. The retrosigmoid approach provides an ideal access for most of the tumors in this location. Radiosurgery is the primary modality of adjuvant therapy for residual, recurrent, and small lesions. Fully fractionated external beam radiotherapy can be used for larger, broader-based residual/recurrent tumors. Management of these complex lesions should include patient preferences and a team approach, including a skull base neurosurgeon, neurotologist, and radiation oncologist., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objectives: Petrous bone cholesteatoma is a rare pathologic entity and may be a difficult surgical challenge because of potential involvement of the facial nerve, carotid artery, dura mater, otic capsule and superior petrosal or lateral sinus. The objective of this article is to present the endoscope-assisted surgery for petrous bone cholesteatoma., Materials and Methods: Eight patients (nine ears) who underwent endoscope-assisted petrous bone surgery for cholesteatoma. Pure tone audiogram, magnetic resonance imaging were performed at preoperatively, and at approximately 12 months postoperatively., Results: Endoscope assisted surgery was performed in 8 patients and 9 ears. Of these patients, 6 were male and 2 were females. Median age was 19,5 (range 7-52) years. Hearing was able to preserved in 8 ears (8/9). Recurrence disease was observed one ear in long term follow up (1/9). In another one patient, cholesteatoma pearl was removed in the office., Conclusion: Endoscope-assisted surgery can allow removal of cholesteatoma of petrous apex with preserving hearing. It also provides to remove the cholesteatoma via transmastoid approach for perilabyrinthine space as "minimally invasive surgery" instead of middle fossa approach that is standard surgical procedure. In apical and peri-labyrinthine cholesteatomas, endoscopes allow to preserve hearing with middle fossa approach instead of trans-otic/ trans-labyrinthine/trans-cochlear approach.

Purpose: Endolymphatic sac tumor (ELST) is a rare, slow-growing, and low-grade malignant tumor arising from the endolymphatic sac in the posterior petrous bone. The purpose of this study is to describe the clinical and radiologic features, and investigate the clinicoradiologic correlation of ELST., Methods: We retrospectively reviewed the clinical, computed tomography (CT), magnetic resonance imaging (MRI), and pathologic findings of 14 patients with 15 ELSTs., Results: Patients comprised of eight women and six men with a mean age of 42.3 years at the time of diagnosis and 35.2 years at the time of initial symptoms. The mean interval between initial symptoms and diagnosis was 84.7 months. The most frequent cochleovestibular symptom was hearing loss in 14 patients (100%); other cochleovestibular symptoms were tinnitus in eight patients (57.1%), vertigo in three patients (21.4%), and aural fullness in three patients (21.4%). Ten patients (71.4%) presented with facial paralyses and five patients (14.3%) presented lower cranial nerve deficits. CT findings revealed spiculated, stippled, or reticular high density within the tumors. The lesions involved mastoid cells, vertical facial nerve canal, semicircular canal, cochlea, tympanum, jugular foramen, internal auditory canal, or petrous apex. On the available MRI, all the eight lesions showed patchy and/or speckled hyperintensity on unenhanced T1WI. Five lesions showed flow voids on T2WI and T1WI. Three lesions had blood fluid levels within cysts., Conclusion: CT and MRI findings of ELSTs are associated with clinical features. Imaging tests should be performed to identify ELSTs early and ensure greater potential for hearing preservation in patients with cochleovestibular symptoms.

We present an unusual case of skull base osteomyelitis in an 88-year-old woman. She presented with gradual onset unilateral headache and diplopia. On examination, there was evidence of a left-sided Horner's and ipsilateral sixth nerve palsy. In addition to persistent raised inflammatory markers, an MRI neck identified signal change in the petrous bone confirming a diagnosis of skull base osteomyelitis. Skull base osteomyelitis should be considered in presentations of subacute raised inflammatory markers in the context of ipsilateral cranial nerve signs., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Laterally located intracranial mature teratomas are rare entities. We report an extremely rare case of an extra-axial mature teratoma of the left petrous temporal bone in an infant who was managed surgically. This is the second reported case of a mature teratoma of the petrous temporal bone in an infant., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Primary intraosseous meningioma is a subset of extradural meningioma that arises in the bone, and only a few cases have been reported to date., Case Presentation: An 80-year-old man presented with decreased hearing on the right side accompanied by a disturbance of balance 10 months prior to admission. Magnetic resonance imaging revealed an 8 × 7 cm osteolytic mass in the right posterior fossa related to the petrous bone, with extension to the cervical region. During surgery, the tumor was found to be located extradurally, with no invasion of the dura. The tumor was removed entirely, apart from a small portion around the jugular foramen to avoid lower cranial nerve injury., Conclusion: The final diagnosis was primary intraosseous osteolytic meningioma with atypical pathology. Here, we report a rare case of an osteolytic skull lesion in the skull base not invading the dura and with extensive bone destruction.

Background: Petrous bone cholesteatoma (PBC) is a rare but local aggressive lesion which can lead to severe complications. Surgery is the mainstay for its treatment. Objectives: To analyse the clinical characteristics and surgical outcomes in a series of patients with PBC, paying special attention to cochlea preservation and use of endoscope. Materials and methods: Retrospective study of 51 patients with PBC who underwent surgery at our centre. Results: Hearing loss (72.5%) and facial paralysis (58.8%) were the two most common symptoms. According to Sanna's classification, supralabyrinthine subtype (51.0%) was the most common subtype, followed by the massive subtype (33.3%). In most patients, PBC was radically removed using subtotal petrosectomy (80.4%). Endoscope was used for assistance in six cases. Various managements of facial nerve were used in different cases. Hearing rehabilitation was not emphasized (44 postoperative dead ear); however, cochlea was preserved as far as possible (45.3%). Recurrence was identified in five patients by MRI with diffusion weighted imaging (DWI) sequence. No recurrence was detected in patients underwent surgery with endoscope assistance. Conclusions and significance: radical excision and functional reconstruction constitute the framework of PBC surgery. Cochlea preservation is critical for possible cochlear implantation in the future. Use of endoscope has the potential to enhance surgical precision and reduce recurrence.

Objective: To describe the clinical course and treatment outcomes for patients with petrous apex epidermoid with skull base erosion., Study Design: Retrospective case series., Setting: Tertiary-care academic center., Patients: Patients surgically managed for petrous apex epidermoids at a single-tertiary care institution from 2001 to 2017., Interventions: Surgical management of primary petrous apex epidermoids., Main Outcome Measures: The presenting symptoms, imaging, pre- and postoperative clinical course, and complications were reviewed., Results: Seven patients were identified. The most common presenting symptoms included: sudden sensorineural hearing loss (n = 3), headaches (n = 1), vertigo (n = 2), and facial paralysis/paresis (n = 2). An epidermoid was discovered in two patients as an incidental finding after a head trauma and one patient after admission for altered mental status and meningitis. Two patients presented with complete facial paralysis (House-Brackmann 6/6). Preoperative normal hearing (pure-tone average < 25 db) was identified in three patients and serviceable hearing (pure-tone average 25-60 dB) in one patient. Three patients with nonserviceable hearing underwent a transcochlear or transotic approach. The remaining four patients underwent an attempted hearing preservation approach. Postoperatively, one patient developed delayed facial paralysis, improving within 3 months. Hearing was preserved in three patients. Two patients developed recurrence of disease within 4 and 7 years respectively., Conclusions: Surgical management of aggressive petrous apex epidermoid is effective to preserve facial function and prevent recurrence. In our series different surgical approaches were used with variable outcomes.

Objective: This study gives details of a rare case of petrous apicitis that presented as Gradenigo's syndrome and was managed surgically., Method: This study presents a case report and review of the literature., Results: A four-year-old female was admitted for failure to thrive following recent sinusitis. Physical examination was positive for right sided facial pain, photophobia and right abducens nerve palsy. Subsequent magnetic resonance imaging revealed a 1.3 × 1.7 × 1.4 cm abscess encompassing the right Meckel's cave. A computed tomography scan showed petrous apicitis and otomastoiditis, confirming Gradenigo's syndrome. The patient was taken to the operating theatre for right intact canal wall mastoidectomy with myringotomy and tube placement. She was discharged on six weeks of ceftriaxone administered by a peripherally inserted central catheter line. At a two-week post-operative visit, she showed notable improvement in neuropathic symptoms., Conclusion: This study presents a rare case of petrous apicitis managed surgically without the need for a craniotomy or transcochlear procedure.

Background: The endoscopic endonasal approach (EEA) was recently added to the neurosurgical armamentarium as an alternative approach to the petrous apex (PA) region. However, the maximal extension, anatomical landmarks, and indications of this procedure remain to be established., Objective: To investigate the limitations and suggest a classification of PA lesions for endoscopic petrosectomy., Methods: Five anatomical specimens were dissected with EEA to the PA. Anatomical landmarks for the surgical steps and maximal limits were noted. Pre- and postprocedural computed tomographic scan and image-guidance were used. Relevant surgical cases were reviewed and presented., Results: We defined 3 types of petrosectomy: medial, inferior, and inferomedial. Medial petrosectomy was limited within the paraclival internal carotid artery (ICA) anteriorly, lacerum ICA inferiorly, abducens nerve superiorly, and petrous ICA laterally. Among those, abducens nerve and petrous ICA are surgical limits. Full skeletonization of the paraclival ICA and removal of the lingual process are essential for better access to the medial aspect of PA. Inferior petrosectomy was defined by the lacerum foramen synchondrosis anteriorly, jugular foramen inferiorly, internal acoustic canal posteriorly, and PA superolaterally. Those are surgical limits except for the foramen lacerum synchondrosis. The connective tissue at the pterygosphenoidal fissure was a key landmark for the sublacerum approach. Clinical cases in 3 types of PA lesions were presented., Conclusion: The EEA provides access to the medial and inferior aspects of the PA. Several technical maneuvers, including paraclival and lacerum ICA skeletonization, sublacerum approach, and lingual process removal, are key to maximize PA drilling., (Copyright © 2018 by the Congress of Neurological Surgeons.)

Background: Outcomes of petroclival meningiomas (PCM) (morbidity, permanent cranial nerves deficit, tumor removal and recurrence) are inconsistent in the literature, making it a challenge to predict surgical morbidity., Methods: A multicenter study of patients with PCMs larger than 2.5cm between 1984 and 2017 was conducted. The authors retrospectively reviewed the patients' medical records, imaging studies and pathology reports to analyze presentation, surgical approach, neurological outcomes, complications, recurrence rates and predictive factors., Results: There were 154 patients. The follow-up was 76.8 months on average (range 8-380 months). Gross total resection (GTR) was achieved in 40 (26.0%) patients, subtotal resection (STR) in 101 (65.6%), and partial resection in 13 (8.3%). Six (2.6%) perioperative deaths occurred. The 5-year, 10-year and 15-year progression-free survival (PFS) of GTR and STR with radiation therapy (RT) was similar (100%, 90% and 75%). PFS of STR without adjuvant radiation was associated with progression in 71%, 51% and 31%, respectively. Anterior petrosectomy and combined petrosectomy were associated with higher postoperative CN V and CN VI deficits compared to the retrosigmoid approach. The latter had a significantly higher risk of CN VII, CN VIII and LCN deficit. Temporal lobe dysfunction (seizure and aphasia) were significantly associated with the anterior petrosectomy approach., Conclusions: Our study shows that optimal subtotal resection of PCMs associated with postoperative RT or stereotactic radiosurgery results in long-term tumor control to equivalent radical surgery. Case selection and appropriate intraoperative judgement are required to reduce the morbidity., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Objective: The endoscopic endonasal transpterygoid route has been widely evaluated in cadavers, and it is currently used during surgery for specific diseases involving the lateral skull base. Identification of the petrous segment of the internal carotid artery (ICA) is a key step during this approach, and the vidian nerve (VN) has been described as a principal landmark for safe endonasal localization of the petrous ICA at the level of the foramen lacerum. However, the relationship of the VN to the ICA at this level is complex as well as variable and has not been described in the pertinent literature. Accordingly, the authors undertook this purely anatomical study to detail and quantify the peri-lacerum anatomy as seen via an endoscopic endonasal transpterygoid pathway., Methods: Eight human anatomical specimens (16 sides) were dissected endonasally under direct endoscopic visualization. Anatomical landmarks of the VN and the posterior end of the vidian canal (VC) during the endoscopic endonasal transpterygoid approach were described, quantitative anatomical data were compiled, and a schematic classification of the most relevant structures encountered was proposed., Results: The endoscopic endonasal transpterygoid approach was used to describe the different anatomical structures surrounding the anterior genu of the petrous ICA. Five key anatomical structures were identified and described: the VN, the eustachian tube, the foramen lacerum, the petroclival fissure, and the pharyngobasilar fascia. These structures were specifically quantified and summarized in a schematic acronym-VELPPHA-to describe the area. The VELPPHA area is a dense fibrocartilaginous space around the inferior compartment of the foramen lacerum that can be reached by following the VC posteriorly; this area represents the posterior limits of the transpterygoid approach and, of utmost importance, no neurovascular structures were observed through the VELPPHA area in this study, indicating that it should be a safe zone for surgery in the posterior end of the endoscopic endonasal transpterygoid approach., Conclusions: The VELPPHA area represents the posterior limits of the endoscopic endonasal transpterygoid approach. Early identification of this area can enhance the safety of the endoscopic endonasal transpterygoid approach expanded to the lateral aspect of the skull base, especially when treating patients with poorly pneumatized sphenoid sinuses.

Object: This study proposes a variation of the transorbital endoscopic approach (TOEA) that uses the lateral orbit as the primary surgical corridor, in a minimally invasive fashion, for the posterior fossa (PF) access. The versatility of this technique was quantitatively analyzed in comparison with the anterior transpetrosal approach (ATPA), which is commonly used for managing lesions in the PF., Methods: Anatomical dissections were carried out in 5 latex-injected human cadaveric heads (10 sides). During dissection, the PF was first accessed by TOEAs through the anterior petrosectomy, both with and without lateral orbital rim osteotomies (herein referred as the lateral transorbital approach [LTOA] and the lateral orbital wall approach [LOWA], respectively). ATPAs were performed following the orbital approaches. The stereotactic measurements of the area of exposure, surgical freedom, and angles of attack to 5 anatomical targets were obtained for statistical comparison by the neuronavigator., Results: The LTOA provided the smallest area of exposure (1.51 ± 0.5 cm2, p = 0.07), while areas of exposure were similar between LOWA and ATPA (1.99 ± 0.7 cm2 and 2.01 ± 1.0 cm2, respectively; p = 0.99). ATPA had the largest surgical freedom, whereas that of LTOA was the most restricted. Similarly, for all targets, the vertical and horizontal angles of attack achieved with ATPA were significantly broader than those achieved with LTOA. However, in LOWA, the removal of the lateral orbital rim allowed a broader range of movement in the horizontal plane, thus granting a similar horizontal angle for 3 of the 5 targets in comparison with ATPA., Conclusions: The TOEAs using the lateral orbital corridor for PF access are feasible techniques that may provide a comparable surgical exposure to the ATPA. Furthermore, the removal of the orbital rim showed an additional benefit in an enhancement of the surgical maneuverability in the PF.

Objective: The cervical carotid segment is used routinely as donor site for high-flow bypass procedures. The horizontal petrous segment would offer a shorter graft distance, complete graft protection intracranially, and avoid the need for surgical neck exposure. In a morphometric cadaveric study, we aimed to investigate variations of the petrous carotid anatomy, especially the incidence of bony dehiscence of the roof of the horizontal petrous carotid segment canal, which may facilitate exposure of the vessel and thereby potentially lower the morbidity of high-flow bypass procedures., Methods: A subtemporal approach was used to expose the horizontal petrous internal carotid artery (ICA) on each side of 4 alcohol-embedded, silicone-injected human cadaver heads to perform a morphometric analysis of the vessel segment and surrounding bony anatomy., Results: The following measurements were obtained of the horizontal petrous ICA (millimeters): long axis mean 9.6 (standard deviation [SD] 4.4, MIN 4.2, MAX 19.5), diameter mean 4.9 (SD 0.6, MIN 4, MAX 5.7), thickness of canal roof mean 2.1 (SD 1.7, MIN 0, MAX 5), and distance from temporal squama mean 22.5 (SD 6, MIN 17, MAX 35). Dehiscence of the bony roof of the horizontal petrous carotid canal was found in 25% of specimen investigated., Conclusions: A dehiscent bony roof of the horizontal petrous carotid canal potentially facilitates exposure of the vessel for high-flow bypass procedures and was observed in 25% of specimens. This feature could be identified on preoperative high-resolution imaging and thus aid in patient selection., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: The current study investigated the incidence, morphology and morphometry of the ossified ligaments expanding between petrous bone and posterior clinoid processes and in between the anterior, middle and posterior clinoid processes. Side symmetry, gender dimorphism and age influence were also studied., Materials and Methods: A total of 123 adult Greek dry skulls were observed., Results: A caroticoclinoid bar (CCB) was found in 60.2%. Partial CCBs appeared more commonly (36.6%) than complete (23.6%). The caroticoclinoid foramen (CCF) was symmetrical on both sides and genders. An anterior interclinoid, a posterior petroclinoid and a partial posterior interclinoid bar appeared in 19.5%, 6.5% and 2.4%, respectively. Osseous spurs posterolateral to the posterior clinoid process were present in 5.7%., Conclusion: The study highlights important morphometric details about osseous bars of the sella region and the related CCF in Greek skulls. Notable differences in the incidence of these bars in Greek individuals compared with findings from other populations highlight the growing awareness of ethnic differences in skull base landmarks. Variations and surgically oriented measurements provided by this study may benefit clinicians involved in the treatment of the middle cranial fossa pathology, enriching understanding of the complicated regional anatomy. Preoperative sellar area mapping is essential, by using computed tomography images, since modification of the surgical approach may be required in cases of severe ossification., (Copyright © 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Introduction: Endolymphatic sac tumours are benign, slowly growing tumours that invade the temporal bone, and present clinically in the form of unilateral hearing loss. They can be sporadic or occur in the context of Von Hippel-Lindau disease (VHL)., Case Summary: The authors report a case of endolymphatic sac tumour arising in the utricle presenting histological and immunohistochemical features corresponding to endolymphatic sac tumour in a patient without VHL., Discussion: Endolymphatic sac tumours invade the posterior part of the petrous temporal bone. According to two studies concerning patients with Von Hippel-Lindau disease, endolymphatic sac tumours arise from the endolymphatic duct. This case of intralabyrinthine sporadic endolymphatic sac tumour supports this hypothesis for sporadic forms, indicating the need for labyrinthectomy associated with tumour resection to avoid recurrence., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)