Unilateral Multifocal Inner Ear and Internal Auditory Canal or Cerebellopontine Angle Cochleovestibular Schwannomas-Genetic Analysis and Management by Surgical Resection and Cochlear Implantation.

OBJECTIVE: To describe the genetic characteristics and the management of two very rare cases of unilateral multifocal inner ear and internal auditory canal or cerebellopontine angle cochleovestibular schwannomas not being associated to full neurofibromatosis type 2-related schwannomatosis. PATIENTS: In a 29-year-old man and a 55-year-old woman with single-sided deafness multifocal unilateral cochleovestibular schwannomas were surgically resected, and hearing was rehabilitated with a cochlear implant (CI). Unaffected tissue was analyzed using next generation sequencing of the NF2 gene. Tumor tissue was analyzed using a 340-parallel sequencing gene panel. MAIN OUTCOME MEASURES: Mutations in the NF2 gene, word recognition score for monosyllables at 65 dB SPL (WRS 65 ) with CI. RESULTS: No disease-causing mutation was detected in the examined sequences in blood leucokytes. All tumor samples revealed, among others, somatic pathogenic NF2 mutations. While the anatomically separate tumors in case 1 were likely molecular identical, the tumors in case 2 showed different genetic patterns. WRS 65 was 55% at 6 years of follow-up and 60% at 4.5 years of follow-up, respectively. CONCLUSIONS: The occurrence of multifocal unilateral cochleovestibular schwannomas without pathogenic variants in NF2 in non-affected blood leucocytes can be associated with mosaic NF2 -related schwannomatosis (case 1), or with likely sporadic mutations (case 2) and may be overlooked due to their extreme rarity. Although challenging, successful hearing rehabilitation could be achieved through surgical resection of the tumors and cochlear implantation.

[To the origins of surgery for acoustic neuromas]. / K istokam khirurgii nevrinom slukhovogo nerva.

Acoustic neuroma is one of the most common tumors of the posterior cranial fossa. Its removal is always a challenge for the neurosurgeon and the patient. The history of surgery for acoustic neuromas is inextricably linked with the history of neurosurgery in general. The modern surgical community must know history and be able to use it. Only then will the development of surgery lead to the preservation of the quality of life of patients. In the history of surgery for acoustic neuromas, the stages of its development are clearly visible from the description of the clinical picture through the study of the anatomy of the cerebellopontine angle to modern microsurgical removal.

Arachnoid Cyst of the Cerebellopontine Angle: A Systematic Literature Review.

OBJECTIVE: The role of surgical management of arachnoid cyst (AC) of the cerebellopontine angle (CPA) is uncertain. This topic has remained controversial with varying contradictory recommendations in the literature, which is limited to mostly case reports. We aimed to provide a comprehensive summary and analysis of symptoms, operative techniques, outcomes, and recurrence of all available surgical cases of AC of the CPA to date. METHODS: A systematic literature search was performed in May 2022 querying several scientific databases. Inclusion criteria specified all studies and case reports of patients with AC located at the CPA for which any relevant surgical procedures were performed. RESULTS: A total of 55 patients from the literature and 5 treated at our institution were included. Mean patient age was 29 years (range, 0.08-79 years), with nearly twice (1.7×) as many female as male patients (37 female, 22 male). Headaches (35%), hearing loss (30%), vertigo (22%), and ataxia (22%) were the most common presentations. Following surgery, 95% experienced symptom improvement, with complete resolution in 64%. Of patients with hearing loss, 44% reported a return to normal. The rate of mortality was 1.69%, and 10% of tumors recurred (mean follow-up 2.3 years [range, 0-15 years]. CONCLUSIONS: Symptomatic AC of the CPA is rare. It exhibits a proclivity for females and commonly manifests with headache, hearing loss, vertigo, and ataxia. While careful selection for surgical candidacy is needed and intervention should be reserved for patients with severe symptoms, surgical decompression is an effective tool for symptom alleviation and recovery.

Introducing endoscopic assistance on routinary basis for vestibular schwannomas resection: A single centre acceptance analysis.

OBJECTIVE: The use of endoscopic assistance in retrosigmoid approach for tumors of the cerebellopontine angle brought undoubted technological advantages in skull base surgery. Nonetheless, the use of the endoscope is not as widespread as it could be. The aim of the study is to analyze the impressions of neurosurgeons and otologists with different experience in vestibular schwannoma surgery, experiencing the introduction of the endoscope in surgical daily practice. METHODS: All patients undergoing vestibular schwannoma surgery were recruited in the period from January 2019 to December 2020. The endoscope-assistance and a minimum follow-up of 12 months were considered inclusion criteria. An eight items questionnaire was administered to the surgeons who used endoscope-assistance during surgery. RESULTS: A total number of 20 patients were recruited. Five surgeons experienced the use of 0° and 45° optics in the "pre-resection" and "intra-meatal" phases of the procedures. The survey gave positive feedbacks on the introduction of the endoscope in vestibular schwannoma resection. The main drawback was the difficulty to manage the use of angled optics. CONCLUSIONS: Despite the known limitations of the study, the idea of investigating surgeons' impressions on the use of the endoscope could be another motif to explain why this instrument and its diffusion is limited despite its advantages in vestibular schwannoma surgery.

Fully neuroendoscopic resection of cerebellopontine angle tumors through a retrosigmoid approach: a retrospective single-center study.

The objective of this study is to preliminarily investigate the surgical safety, efficacy, techniques, and clinical value of fully neuroendoscopic surgery for the resection of cerebellopontine angle (CPA) tumors via a retrosigmoid approach. The clinical data of 47 cerebellopontine angle area (CPA) tumors that were treated by full neuroendoscopic surgery from June 2014 to June 2023 were retrospectively analyzed. The efficacy and advantages of the surgical techniques were evaluated based on indicators such as duration of the surgery, neuroendoscopic techniques, intraoperative integrity of nerves and blood vessels, extent of tumor resection, outcomes or postoperative symptoms, and incidence of complications. The 47 cases of cerebellopontine angle tumors include 34 cases of epidermoid cysts, 7 cases of vestibular schwannomas, and 6 cases of meningiomas. All patients underwent fully neuroendoscopic surgery. Twenty tumors were removed using the one-surgeon two-hands technique, and 27 tumors were removed using the two-surgeons four-hands technique. The anatomical integrity of the affected cranial nerves was preserved in all 47 cases. None of the patients suffered a postoperative hemorrhage, cerebrospinal fluid leak, and aseptic or septic meningitis, or died. The rate of total tumor resection was 72.3% (34/47), and the symptom improvement rate was 89.4% (42/47). All patients were followed up for 2 to 12 months, and none died nor showed any signs of tumor recurrence. By analyzing 47 fully neuroendoscopic resections of CPA tumors using the posterior sigmoid sinus approach in our center, we believe that such method allows complete, safe, and effective resection of CPA tumors and is thereby worthy of clinical promotion.

Fully endoscopic microvascular decompression for trigeminal neuralgia: technical note and early outcomes.

Microscopic microvascular decompression (MVD) has been considered a curative and reliable method for treating classical trigeminal neuralgia (TN) for decades. Endoscopy can provide bright illumination and a panoramic view, which enhances the visualization of the posterior fossa. In view of the above advantages of endoscopy, it gradually became an option for MVD for treating TN. This study was performed to evaluate the advantages of fully endoscopic MVD for treating TN and is presented with a description of our operative technique. From January 2020 to January 2022, 95 classical TN patients underwent fully endoscopic MVD performed by the same surgeon and assistant in our department. The assistant held the endoscope, and the surgeon operated. Brain stem auditory evoked potentials (BEMPs) were routinely monitored. For every patient, the neurovascular conflict was identified, and complete decompression was achieved. The Barrow Neurological Institute (BNI) pain intensity score was used to evaluate the degree of facial pain. The intraoperative findings, postoperative outcomes, and complications were analyzed. Immediately after the operation, 93 patients (97.9%) achieved complete pain relief (BNI score of I). Two patients (2.1%) still had some pain, but it could be adequately controlled with medicine (BNI score of III). During the 12-36 months of follow-up, recurrence was found in 3 patients (3.2%), including one patient (1.1%) with a BNI score of II and 2 patients (2.1%) with a BNI score of III. Complications were found in 5 patients (5.3%), including facial numbness in 3 patients (3.2%), vertigo in one patient (1.1%), and headache in one patient (1.1%). There were no cases of mortality, stroke, hearing impairment, facial paralysis, or other complications. Fully endoscopic MVD is a safe and effective method for treating TN. It provides bright illumination and a panoramic view for surgeons to better observe neurovascular conflicts in deep areas of the cerebellopontine angle (CPA).

Simultaneous cochlear implantation during resection of a cerebellopontine angle meningioma: case report.

Cerebellopontine angle (CPA) meningiomas commonly involve the internal auditory canal (IAC). We report a case of a 68-year-old lady with idiopathic profound bilateral deafness with a meningioma which was discovered on workup for cochlear implantation. We performed simultaneous excision of her CPA and IAC meningioma with insertion of a cochlear implant (CI). She regained functional hearing with marked improvement in quality of life. Intraoperative electrophysiological testing can be used to confirm preservation of the cochlear nerve enabling simultaneous implantation which is preferable for clinical and logistical reasons. This creates an option for hearing rehabilitation at the time of IAC/CPA tumour surgery in appropriate patients.

The cerebellopontine angle cistern volumetric differences in trigeminal neuralgia patients with and without vertebrobasilar compression: a case-matched study.

Previous studies have indicated that the small cerebellopontine angle (CPA) cistern plays a role in the pathogenesis of trigeminal neuralgia (TN), but they are likely not involved in TN associated with vertebrobasilar artery (VBA) compression because of its rarity. Forty-four patients with VBA-associated TN and 44 age-, sex-, and hypertension-matched TN patients without VBA compression (non-VBA-associated) were included. All patients underwent high-resolution MRI. The CPA cistern volumes were measured bilaterally. The presence of vertebrobasilar dolichoectasia (VBD) and laterality of the vertebrobasilar junction (VBJ) were observed. The CPA cistern volume on the affected side was smaller than the unaffected side (714.4 ± 372.8 vs 890.2 ± 462.2 mm3, p < 0.001) in non-VBA-associated TN patients, while VBA-associated TN patients show a larger CPA cistern on the affected side than the unffected side (1107.0 ± 500.5 vs 845.3 ± 314.8 mm3, p < 0.001). The prevalence of VBD was higher in patients with VBA-associated TN than in matched non-VBA-associated TN patients (90.9% vs 4.5%, p < 0.001). A positive correlation between the laterality of VBJ and the affected side was found in the VBA-associated TN group (p < 0.0001). Large CPA cistern may be a neuroradiological feature of VBA-associated TN, and most of the VBA-associated TN is accompanied by VBD. The presence of VBD and the lateral shift of VBJ may expand the CPA cistern by squeezing the surrounding tissue on the affected side and also increase the chance of VBA compression on the trigeminal nerve, resulting in the genesis of VBA-associated TN.

Bruns Nystagmus in Cerebellopontine Angle Hemangioblastoma.

Bruns nystagmus is a form of jerk nystagmus that has a localizing value in cerebellopontine angle (CPA) tumors. Hemangioblastomas involving the CPA is rare. A case of a 57-year-old male presented with Bruns nystagmus which led to the discovery of a large CPA hemangioblastoma is described. The nystagmus was compatible with the laterality of the tumor. High quality video of Bruns nystagmus was recorded.

Microsurgical anatomy and pathoanatomy of the outer arachnoid membranes in the cerebellopontine angle: cadaveric and intraoperative observations.

PURPOSE: The cerebellopontine angle (CPA) is a frequent region of skull base pathologies and therefore a target for neurosurgical operations. The outer arachnoid is the key structure to approach the here located lesions. The goal of our study was to describe the microsurgical anatomy of the outer arachnoid of the CPA and its pathoanatomy in case of space-occupying lesions. METHODS: Our examinations were performed on 35 fresh human cadaveric specimens. Macroscopic dissections and microsurgical and endoscopic examinations were performed. Retrospective analysis of the video documentations of 35 CPA operations was performed to describe the pathoanatomical behavior of the outer arachnoid. RESULTS: The outer arachnoid cover is loosely attached to the inner surface of the dura of the CPA. At the petrosal surface of the cerebellum the pia mater is strongly adhered to the outer arachnoid. At the level of the dural penetration of the cranial nerves, the outer arachnoid forms sheath-like structures around the nerves. In the midline, the outer arachnoid became detached from the pial surface and forms the base of the posterior fossa cisterns. In pathological cases, the outer arachnoid became displaced. The way of displacement depends on the origin of the lesion. The most characteristic patterns of changes of the outer arachnoid were described in case of meningiomas, vestibular schwannomas, and epidermoid cysts of the CPA. CONCLUSION: The knowledge of the anatomy of the outer arachnoid of the cerebellopontine region is essential to safely perform microsurgical approaches as well as of dissections during resection of pathological lesions.

Diluted intracisternal papaverine for microvascular protection of cranial nerves during vestibular schwannoma and cerebello-pontine angle surgery. Commentary and review of the literature.

Vasospasm after resection of skull base tumors is a rare complication that often produces relevant ischemic sequelae. This review of the literature reports a number of published experiences that can help determine the potential causes of vasospasm after cerebello-pontine angle (CPA) tumor and -in particular-vestibular schwannoma (VS) resection, the ways to prevent it, and the methods to obtain the correct diagnosis. The cause appears to be multifactorial and the surgical approach may contribute to the pathogenesis of vasospasm. Neurosurgeons must pay attention to detect possible vasospasm at an early stage of cerebello-pontine. Cerebral blood flow measurement and transcranial Doppler are useful monitoring tools. Intra-operative prevention of vasospasm during CPA tumor resection with papaverine hydrochloride (PPV) seems to play a relevant role. In particular, PPV is a direct-acting vasodilator used to manage vasospasm during various neurosurgical operations. There is large uncertainty about intracisternal PPV dose-related efficacy and side effects. Dilution of PPV in saline prior to application is recommended to avoid complications. In our experience, in line with the literature, we use a pure PPV without excipients 60 mg/2 ml diluted in 20 cc of 0,9% saline solution (0,3%) to prevent Hearing Loss during Posterior Fossa Microvascular Decompression for Typical Trigeminal Neuralgia and other cranial nerves potentially involved during VS and other CPA tumor resection. The aim of this commentary is to analyze and discuss the role of diluted intracisternal PPV for microvascular protection of cranial nerves during CPA tumor surgery.

Radical resection of trigeminal schwannoma at the cerebellopontine angle with support of the digital robotic exoscope Synaptive Modus V system: A case report and literature review.

RATIONALE AND PATIENT CONCERNS: We report on a 67-year-old woman who experienced drowsy mental status, facial numbness, and hearing loss on the right side; the symptom gradually worsened over the last 4 years. Brain magnetic resonance imaging revealed a 4.8 × 1.8 × 2.6 cm lesion located in the right cerebellopontine angle. INTERVENTIONS: She underwent surgery with the retrosigmoid suboccipital approach and support from the digital robotic exoscope Synaptive Modus V system. To the best of our knowledge, this is the first reported case that used the robotic exoscope system in Vietnam, and also in Asia. DIAGNOSIS: We performed radical resection of the tumor, the surgery position and the pathology result concluded the diagnosis was trigeminal schwannoma. OUTCOMES: After 30 months of follow-up, she fully recovered and the magnetic resonance imaging showed radical resection of the tumor. LESSONS: The aim of this study is to share our experience with the robotic exoscope system, which can improve optical field and image resolution, hence creating an opportunity for surgery that otherwise is impossible. The application of this robotic exoscope system is a breakthrough in neurosurgery in developing countries, such as Vietnam.

Solitary Sclerotic Fibroma of Right Cerebellopontine Angle.

Sclerotic fibroma (storiform collagenoma) is a fibrotic tumor that occurs mainly in patients with Cowden syndrome, but it can also occur in isolation, as detailed in previous reports. Here we present a case of a solitary sclerotic fibroma in cerebellopontine angle. Brain magnetic resonance imaging revealed a lesion showing hypointense signal on both T1 and T2. The lesion was not enhanced after administering gadolinium. The tumor was removed integrally by surgery.

Microsurgical transcranial approaches to the posterior surface of petrosal portion of the temporal bone: quantitative analysis of surgical volumes and exposed areas.

Different microsurgical transcranial approaches (MTAs) have been described to expose the posterior surface of the petrous bone (PPB). A quantitative, anatomical comparison of the most used MTAs, for specific areas of the PPB, is not available. Anatomical dissections were performed on five formalin-fixed, latex-injected cadaver heads (10 sides). Six MTAs were analyzed: Kawase approach (KWA), retrosigmoid approach (RSA), retrosigmoid approach with suprameatal extension (RSAS), retrolabyrinthine approach (RLA), translabyrinthine approach (TLA), and transcochlear approach (TCA). Surgical volumes and exposed areas of each approach were quantified with a dedicated neuronavigation system (ApproachViewer, part of GTx-Eyes II, University Health Network, Toronto, Canada) and adjuvant software (ITK-SNAP and Autodesk Meshmixer 3.5). Areas and volumes were compared using linear mixed models. TCA provided the best exposure of Trautmann's triangle and the retromeatal, suprameatal, meatal, and premeatal regions. RSAs provided the best exposure of the inframeatal region, with RSAS gaining significant exposure of the suprameatal region. KWA had the highest surgical volume, and RLA the lowest. Transpetrosal approaches offer the widest exposure of PPB proportionally to their invasiveness. Retrosigmoid approaches, which get to the studied region through a postero-lateral path, are paramount for the exposure of the inframeatal and suprameatal region and, given the adequate exposure of the remaining PPB, represent an effective approach for the cerebellopontine angle (CPA). These anatomical findings must be considered with approach-related morbidity and the pathological features in order to choose the most appropriate approach in clinical practice.

Fascicular-Sparing Technique for Resection of Cerebellopontine Angle Facial Nerve Schwannoma.

Surgery of cerebellopontine angle (CPA) facial nerve schwannoma (FNS) in patients with good facial nerve function is a challenge.1-10Video 1 highlights the fascicular-sparing technique for resection of a CPA FNS. A 41-year-old male patient symptomatic with persistent headaches and tinnitus underwent a retrosigmoid approach for a right cystic CPA tumor, presumed vestibular schwannoma. Intraoperatively, the facial nerve was identified as fine multiple strands splayed around the perimetry of the tumor, which elicited a motor response at a low threshold stimulation. This finding led to the intraoperative diagnosis of FNS according to the reported criteria.5 Neuromonitoring-assisted fascicular-sparing resection technique was performed. It involved the gradual separation of the uninvolved nerve fibers using a fine-stimulating dissector at a threshold of 0.2 mA. Entry into the tumor was at a stimulation silent cyst. The tumor was debulked with preservation of the endoneurium and pulse irrigation hemostasis. A near-total resection was performed. The patient was discharged on the second postoperative day with a House-Brackman III facial nerve deficit. The deficit remained stable during the following annual follow-up visits. Resection of CPA FNS is indicated at the earliest sign of deficit. However, it might be encountered as masquerading at the surgery of an acoustic tumor. The fascicular-sparing technique is critical in avoiding injuries to the endoneurium during the resection and with the ability to preserve function. The sparing of endoneurium avoids collagenization, fibrosis, and ischemia of the nerve, which are known to be the pathologic substrate of worse functional outcomes.

Analysis of Imaging Results for Semisitting Compared with Supine Positioning in the Retrosigmoid Approach for Resection of Cerebellopontine Angle Vestibular Schwannomas.

OBJECTIVE: To compare the completeness of resection of vestibular schwannomas using three-dimensional segmented volumetric analysis of pre- and postoperative magnetic resonance imaging (MRI) of patients undergoing supine and semisitting positioning for the retrosigmoid approach. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary medical center. PATIENTS: Patients with vestibular schwannomas undergoing surgical resection via the retrosigmoid approach. INTERVENTIONS: Tumor resection via the retrosigmoid approach with different patient positioning: standard supine versus semisitting. MAIN OUTCOME MEASURES: Preoperative versus postoperative three-dimensional segmented volumetric MRI analysis of vestibular schwannomas. RESULTS: A total of 43 patients (15 supine and 28 semisitting) underwent retrosigmoid craniotomy for resection of vestibular schwannomas. For the conventional supine and semisitting positioning, mean preoperative tumor volumes were 12.65 and 8.73 cm 3 ( p = 0.15), respectively. Postoperative mean tumor volumes for the supine and semisitting positions were 2.09 and 0.48 cm 3 ( p = 0.13), respectively. There were 11 cases of postoperative sigmoid sinus thrombosis, 3 in the conventional supine group and 8 in the semisitting groups, and there were 6 cases of postoperative cerebrospinal fluid leaks, all in the semisitting group. The mean House-Brackmann scores for the supine and semisitting groups were 2.9 and 2.3, respectively. There was no statistically significant difference between groups in the rates of these or any other postoperative complications. CONCLUSIONS: The semisitting position for the suboccipital retrosigmoid approach for vestibular schwannoma resection does not compromise the ability to adequately resect the tumor as seen by volumetric MRI results. Further studies are needed to establish the safety of this position compared with the traditional supine approach.

The Influence of Trigeminocardiac Reflex on Postoperative Cardiac Adverse Events in Patients Undergoing Cerebellopontine Angle Tumor Resections: A Case-Control Study.

OBJECTIVE: To assess the potential impact of the trigeminocardiac reflex (TCR) on postoperative adverse cardiac events and to identify predictors of the TCR in cerebellopontine angle surgery. METHODS: Patients undergoing elective cerebellopontine angle surgery from October 1, 2015, to September 30, 2020, were recruited consecutively for this retrospective case-control study. The TCR was evaluated by reviewing electronic anesthesia records and defined as a drop in heart rate was >20%. Controls were identified from the same retrospective cohort and matched by age, sex, and similar (±5 days) surgery date in the ratio of 1:2. RESULTS: Of 2446 patients, 68 (2.78%) experienced TCR episodes. A total of 97 TCR episodes occurred among the 68 patients. In 2 TCR episodes, severe cardiac complications developed after surgery-myocardial injury in one case and cardiac arrest in the other case. The prevalence of adverse cardiovascular events was higher in the TCR group (60.3% vs. 36.0%, P = 0.001) than in the control group. The independent risk factor for the TCR in the multivariate condition logistic regression was tumor compression of the brainstem (odds ratio = 2.36, 95% confidence interval 1.40-3.95; P = 0.001). CONCLUSIONS: Intraoperative TCR episodes seemed to be associated with postoperative adverse cardiac events in patients undergoing cerebellopontine angle surgery. Moreover, tumor compression of the brainstem might be a risk factor for TCR episodes.

Pre- and intra-operative prognostic factors of facial nerve function in cerebellopontine angle surgery.

PURPOSE: The study assesses whether pre- and intraoperative factors linked to electromyography and direct electrical stimulation (DES) of facial nerve can predict facial nerve function in the short- (12 days) and long-term (1 year) after cerebellopontine angle (CPA) tumor resection. METHODS: 157 patients who underwent surgical resection of CPA tumors with facial nerve monitoring. Pre-operative factors (age, tumor size, pure tone average), surgical time and intra-operative parameters regarding facial function, minimum stimulation threshold (MST), compound muscle action potential (CMAP) and the difference between proximal and distal CMAP (DPDC) were evaluated. RESULTS: A correlation between tumor size, MST, CMAP and facial function in both short and long term was found. A higher grade of immediate facial paralysis corresponded to a higher risk of poor outcome after one year. A postoperative House-Brackmann (HB) score of V or VI was correlated with poor outcome in 88.8% and 93.8% of cases. A risk of HB 3 or more, in the long term, was correlated with a tumor size of 20.2 mm. Using an MST of 0.1 mA, for long-term predictions, sensitivity and specificity were 0.62 (95% CI 0.46-0.75) and 0.73 (95% CI 0.61-0.82), respectively. With a CMAP cut-off < 200 µV, for long-term prediction, sensitivity was 0.73 (95% CI 0.53-0.87) and specificity 0.73 (95% CI 0.55-0.85). CONCLUSION: The assessment based on the cut-offs described increases the ability to predict facial function. Improving predictive accuracy enables surgeons to address patients' expectations and to establish an intervention timeline for planning facial reanimation.

How I do it: cerebellopontine angle epidermoid cyst removal with endoscopic assistance.

BACKGROUND: Epidermoid cyst in the cerebellopontine angle (CPA) can involve numerous critical structures. Endoscopic assistance following microscopic removal via a retrosigmoid approach can expand the surgical corridor, allowing for more complete resection. METHOD: We describe in a stepwise fashion the surgical steps for the microscopic removal of an epidermoid cyst of the CPA with endoscopic assistance. CONCLUSION: Endoscopically assisted microscopic removal for CPA epidermoid cysts provides wide access to the CPA and its adjacent structures and shows to be an effective option in selected cases.

Cerebellopontine angle epidermoid with ipsilateral external ear atresia: an embryological association or a coincidence?

The cerebellopontine angle (CPA) is the most common site for intracranial epidermoids. They may have varied presentations viz facial pain, hemifacial spasms, headache and uncommonly features of raised intracranial pressure. Their association with external ear atresia has not been reported in literature to date. We present a very rare case of CPA epidermoid with ipsilateral external ear atresia.