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Objectives: Intraoperative electrocochleography (ECochG) provides valuable information regarding inner ear function during surgical procedures. Here, we report findings from ECochG collected at three time intervals during the management of a patient with symptoms of inner ear hydrops., Patients: A 38-year-old man with an endolymphatic sac tumor who demonstrated classic Ménière's symptoms., Interventions: Endolymphatic shunt, tumor biopsy, and definitive resection of an endolymphatic sac tumor., Main Outcome Measures: Intraoperative ECochG measurements at three time intervals, using frequency-specific tone bursts and clicks., Results: Across time intervals, the amplitude of ECochG summation potential (SP) responses decreased for tone bursts of all frequencies. On the other hand, the SP response to the click stimulus, and the ratio of the SP to action potential (AP), basically remained unchanged across time points., Conclusions: Results suggest that the absolute amplitude of the SP response to frequency-specific tone bursts may be more sensitive to inner ear hydrops than the traditionally used SP/AP ratio as a response to click stimuli.

Canalolabyrinthine schwannoma is a rare subtype of neuroma. Only 1 case has been described in the literature. We report the clinical case of a 51-year-old Caucasian female with Meniere's disease-like symptoms. Magnetic resonance imaging showed right VIII cranial nerve schwannoma that had different characteristics from the classical described types. The peculiar features of our case of intralabyrithine canalolabyrinthine schwannoma directed the surgeon and radiologist to tailored considerations for follow-up and therapy.

Intralabyrinthine schwannomas (ILSs) are uncommon benign tumors that originate in the Schwann cell sheath of the intralabyrinthine distal branches of the vestibulocochlear nerve. They have no initial involvement in the internal auditory canal, although that might develop later. These lesions can arise inside the cochlea, originate in the vestibule or, in rare cases, develop in the semicircular canals. From these sites, spread might take place via the anatomic connections between the perilymphatic spaces in the scala vestibuli and the anterior vestibule. Thus, ILSs centered in the cochlea might involve the vestibule, and those originating in the vestibular end organs would reach the cochlea. Presenting signs and symptoms include a progressive or sudden sensorineural hearing loss (which occurs in more than 95% of patients), as well as tinnitus and vertigo. Magnetic resonance imaging characteristics include sharp circumscription and hypointensity on thin, heavily T2-weighted 3D images and strong enhancement after gadolinium administration on T1-weighted images. We describe a series of 7 cases of primary ILS that were managed at two of our institutions. We also discuss the need for a comprehensive otoneurologic evaluation that encompasses the functional derangement and the tumor location as delineated by MRI, and we describe the treatment options.

Syringocystadenoma papilliferum is a rare cutaneous adnexal tumor that usually arises in the head and neck region. It may develop de novo or within a nevus sebaceus. Linear syringocystadenoma papilliferum is an uncommon variant of this benign tumor. We report a child with linear retroauricular distribution of syringocystadenoma papilliferum. A background nevus sebaceus was shown histologically. Total excision was curative with no recurrence. An association between the linear variant of syringocystadenoma papilliferum and nevus sebaceus has not been reported previously.

Endolymphatic sac tumors (ELSTs) are rare tumors arising from the epithelium of the endolymphatic sac and duct that can be either sporadic or associated with von Hippel-Lindau (VHL) disease. We report a case of a VHL patient with histologically proven residual ELST who underwent Ga DOTATATE PET/CT showing increased activity (SUVmax, 6.29) by the ELST. The presented case of a VHL-associated ELST with increased Ga DOTATATE uptake indicates cell-surface expression of somatostatin receptors by this tumor, suggesting the potential application of somatostatin receptor imaging using Ga DOTA-conjugated peptides in the workup and management of these patients.

Background: Apocrine hidrocystomas, also known as apocrine cystadenomas, are bening cystic tumours derived from the secretory portion of apocrine sweat glands., Clinical Case: A 78-year old female was referred to our division for assesment an asymptomatic translucent, well-defined cystic lesion located on the upper helix. The histological features were consistent with apocrine hidrocystoma coexisting with gouty tophi., Conclusions: We report the second case of apocrine hydrocystoma located in the pinna, outside of ear canal and the first case of its association with gouty tophi described. It is difficult to know which lesion was first established and if the tophi may lead to ductal obstruction and subsequent cystic retention.

Purpose: The purpose of this research note is to identify and prioritize diseases important for detection in adult hearing health care delivery systems., Method: Through literature review and expert consultation, the authors identified 195 diseases likely to occur in adults complaining of hearing loss. Five neurotologists rated the importance of disease on 3 dimensions related to the necessity of detection prior to adult hearing aid fitting., Results: Ratings of adverse health consequences, diagnostic difficulty, and presence of nonotologic symptoms associated with these diseases resulted in the identification of 104 diseases potentially important for detection prior to adult hearing aid fitting., Conclusions: Current and evolving health care delivery systems, including direct-to-consumer sales, involve inconsistent means of disease detection vigilance prior to device fitting. The first steps in determining the safety of these different delivery methods are to identify and prioritize which diseases present the greatest risk for poor health outcomes and, thus, should be detected in hearing health care delivery systems. Here the authors have developed a novel multidimensional rating system to rank disease importance. The rankings can be used to evaluate the effectiveness of alternative detection methods and to inform public health policy. The authors are currently using this information to validate a consumer questionnaire designed to accurately identify when pre- fitting medical evaluations should be required for hearing aid patients.

Cerebral venous sinus thrombosis (CVST) is a rare complication of surgical treatment of vestibular schwanomma. We present a rare case of extensive venous sinus thrombosis after trans-labyrinthine approach that was refractory to systemic anti-coagulation. Mechanical aspiration thrombectomy was utilized to re-canalize the venous sinuses and resulted in successful resolution of neurological symptoms. Indications of utilizing endovascular approaches are discussed that will enable skull base surgeons to address this uncommon yet potentially fatal complication., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Introduction: Turner syndrome is a chromosomal abnormality where all or a part of one of the X chromosomes is absent or it has other abnormalities. Besides characteristic abnormalities of short stature and infertility, women with Turner syndrome have increased risks for tumors of the central nervous system, especially meningioma and an otologic disease. Meningioma involving the middle ear is extremely rare, and this condition has never been published in association with Turner syndrome., Case Report: We present an otologic manifestation associated with other abnormalities in a patient with Turner syndrome and discuss diagnosis and possible treatment options., Conclusion: Multidisciplinary team approach is essential in these patients in order to evaluate their vulnerability and define therapeutic priorities.

The combined symptoms of vertigo, hearing loss, and facial palsy indicate the presence of lesions in the internal auditory canal (IAC). Differential diagnoses, such as inner/middle ear infections and IAC neoplasms, can make the definitive diagnosis of IAC lymphomas challenging. Lymphomas can infiltrate the central nervous system at various sites; however, IAC involvement in metastatic lymphomas is very rare. Herein we report the case of a patient with IAC lymphoma presenting with aural fullness of the left ear and intractable otalgia followed by symptoms of facial weakness, hearing loss, and vertigo within 48 h. The uncharacteristic clinical manifestations and concurrent middle ear infection meant that the conclusive diagnosis of IAC lymphoma was delayed.

Purpose: Neurofibromatosis type 2 (NF2) is a dominantly inherited genetic condition that clinically manifests through the appearance of multiple meningiomas, ependymomas and bilateral vestibular schwannomas (acoustic neuromas) which lead to progressive hearing loss. Neovascularization is necessary for tumor growth and is driven by tumor-produced angiogenic factors such as vascular endothelial growth factor (VEGF). Bevacizumab is a humanized monoclonal antibody that neutralizes the activity of VEGF. Recent data have shown that VEGF is produced by schwannoma tumor cells. Bevacizumab treatment in patients with NF2 who were considered poor candidates for surgery and radiation therapy was found to result in clinically meaningful hearing improvement and tumor volume reduction in previous studies., Methods: We report the case of a 40-year-old woman with sudden right-sided hearing loss. Magnetic resonance imaging (MRI) revealed multiple meningiomas and neurinomas (C2 and L5 lesions) and a right-sided acoustic neurinoma, confirming the diagnosis of NF2. Bevacizumab was given as infusion every 2 weeks at a dose of 5.0 mg/kg body weight with MRI monitoring every 6 months., Results: After 6 months from the start of therapy the patient reported progressive improvement of hearing response in audiometry, word recognition and face-to-face conversation. MRI evidenced reduction of the volume of the right vestibular schwannoma and the multiple meningiomas as well as attenuation of brain stem compression., Conclusions: At the time of writing the patient is continuing treatment with bevacizumab without adverse events. She has good functional status and quality of life.

Intracochlear schwannomas are rare tumours. Diagnosis is based on high-resolution MRI, which should be used for accurate determination of the location of tumours. We report a case of a cochlear schwannoma that presented with profound hearing loss and intractable paroxysmal positional vertigo, which was diagnosed with gadolinium-enhanced MRI and removed using a transcanal minimally-invasive transotic approach.

Introduction: Dermoids of the Eustachian tube are rare benign developmental tumours that typically occur in female children. General consensus of classification and nomenclature has still not been reached. The treatment of choice consists of a radical surgical excision. Several approaches have been described and few cases are reported in literature. A gross total resection is now safely achievable through an endoscopic mini-invasive approach., Materials and Methods: We have reported a case of dermoid pedicled in the left Eustachian tube and resected with a pure endoscopic transnasal approach. A review of the literature was performed., Case Study: The aim of this paper is to present the first case of pure endoscopic transnasal resection in a 4-day-old infant as emergency treatment of Eustachian tube dermoid presenting an acute respiratory failure at birth., Conclusion: The endoscopic transnasal resection is a safe and feasible technique in selected dermoids of the Eustachian tube, when the middle ear is not involved. This approach could be used also in new-born children, decreasing the morbidity of the classic surgical treatment and avoiding the risk of craniofacial alterations., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objective: To analyze the long term outcomes after surgery in tympanomastoid paragangliomas., Study Design: Retrospective study., Methods: The charts of 145 patients with tympanomastoid paragangliomas managed between 1988 and 2013 were reviewed. The clinical features, audiological data, pre- and postoperative notes were noted. The tumors were staged according to the modified Fish and Mattox classification. The surgical approaches for all patients were formulated according to the surgical algorithm developed at our center., Results: 34 (23.5%), 46 (31.7%), 22 (15.2%), 18 (12.4%) and 25 (17.2%) patients were diagnosed to have TMP class A1, A2, B1, B2 and B3 tumors respectively. Gross tumor resection was achieved in 141 (97.2%) patients. The facial nerve was uncovered in four patients and infiltrated in three. The cochlea was found eroded in seven cases. The mean follow-up was 48.4 months. Recurrence was seen in one patient (0.7%). In the cases where the facial nerve was preserved (n=143), the nerve function was graded as HB grade 1 in 138 patients (97%). Postoperatively, the mean AC showed an improvement in all categories except in class B2 and B3, which corresponds to the classes that include patients who underwent subtotal petrosectomy., Conclusion: We report the long term surgical outcomes in tympanomastoid paragangliomas in the largest series published till date. It is possible to completely eradicate all types of tympanomastoid paragangliomas with minimum sequelae by choosing the correct surgical approach to achieve adequate exposure for individual tumor classes as described in our classification and algorithm., Level of Evidence: IIb., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Inverted papilloma of the middle ear is extremely rare and so far only 25 cases have been published in medical literature. It is a benign, rapidly growing tumour with higher incidence of malignancy and recurrence compared with sinonasal papillomas. The aetiology remains unknown. Typical clinical presentation includes hearing loss, otorrhoea and otalgia. Treatment is surgical radical resection of the lesion and radiotherapy in selected cases. Long-term follow-up, including MRI is recommended, due to the risk of recurrence and malignant degeneration.

Objective: The case of a thirteen-year-old woman showing an atypical teratoid/rhabdoid tumor (AT/RT) primarily occurred in the internal auditory canal was presented., Results: There was a delay in diagnosing AT/RT because of the first histological diagnosis of benign neurofibroma. If we had changed the surgical approach to one which was middle cranial fossa-based or translabyrinthine in the second or third operation, we might have reached an earlier final diagnosis. Although we faced a dilemma about whether to sacrifice facial nerve function for dissection of the tumor, we should have considered the possibility of malignancy at an earlier stage., Conclusion: This is a case report of AT/RT in the internal auditory canal presenting with progressive hearing loss as the initial symptom. Although no previous reports of AT/RT primarily occurring in the internal auditory canal are existent, this rare form of the disease should be considered in future evaluations as a differential diagnosis for internal auditory canal tumor., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Here we report a rare case of Merkel cell carcinoma complicated with nephrosis and malignant lymphoma. A 79-year-old male, who had undergone rectectomy due to colorectal cancer about 10 years previously, was diagnosed as Merkel cell carcinoma of the left ear lobe with lymph node metastases. Tumor resection and lymph node dissection were performed. A year later, follow-up PET-CT revealed a small hot spot at the ileocecum without apparent tumor formation based on examination by colonoscopy. The patient received 56 Gy of radiation. Two months later, he developed new-onset nephrosis followed by renal failure, and was referred to our hospital (Cr 4.26 mg/dL, UA 13.5 mg/dL, Alb 2.1 g/dL). Further examination negated the possibility of vasculitis, collagen disease, or myeloma kidney. Since his renal function continued to decline, causing uremic symptoms, he was hospitalized and underwent hemodialysis soon after referral. Abdominal CT scan revealed an ileocecal mass with multiple abdominal lymphadenopathy, which was later diagnosed as diffuse large B-cell lymphoma (stage IV) by tumor biopsy. Corticosteroid therapy (prednisolone 60 mg/day) was soon initiated with no response. Local skin redness and blister formation at the left shoulder emerged gradually, which strongly suggested a local recurrence of Merkel cell carcinoma. Despite the use of rituximab, the patient's general condition deteriorated without any sign of recovery. Three months after the start of dialysis, we discontinued dialysis therapy due to his poor health status, and eventually he died of cachexia. Autopsy revealed triple cancers: rectal cancer, Merkel cell carcinoma, and malignant lymphoma. In addition to the case report, we will summarize and discuss former similar case reports in the literature.

Objective: Indications for cochlear implantation have extended progressively over the years both from an audiological and clinical point of view, including the introduction of cochlear implants (CI) for hearing rehabilitation following skull base surgeries and neurotological procedures. In the past, cochlear implantation has been used for hearing rehabilitation in radiation-induced sensorineural hearing loss in nasopharyngeal and tonsillar carcinoma with successful outcomes. Here we describe a similar outcome following total deafness after tympanojugular paraganglioma irradiation, which is also the first such report in literature., Design: The irradiated ear of this patient was implanted with a CI through standard posterior tympanotomy., Study Sample: A 26 year old male with multiple paragangliomas with bilateral deafness, secondary to surgery on one ear and radiotherapy for the opposite ear., Results: After a follow-up of 48 months, no local complications have occurred and the hearing results have remained stable with 100% sentence recognition., Conclusions: Bilateral and multiple paragangliomas are rare tumors. Despite the modality of treatment, the hearing is almost always compromised. In these patients, cochlear implantation offers a new perspective for hearing restoration. This report demonstrates that cochlear implantation can be effectively performed after tympanojugular paraganglioma irradiation with long-lasting, satisfactory results, even in the presence of residual tumor.

We describe the case of a 52-year-old man with a history of squamous cell carcinoma (SCC) of the tonsil who presented with right subacute otalgia and otorrhea. Dedicated computed tomography of the temporal bones showed opacification within the mastoid process with destruction of bony mastoid septations consistent with coalescent mastoiditis. Preoperative imaging showed no destruction or expansion of the bony eustachian tube that would indicate that a direct spread had occurred. An urgent cortical mastoidectomy was performed. Intraoperatively, a friable white mass surrounded with purulence and granulation tissue was biopsied and returned as SCC. The discrete metastasis was removed without complication. Postoperatively, the patient was prescribed palliative chemotherapy. This case shows that a metastatic SCC can be masked by an overlying mastoiditis, and thus it should be considered in the differential diagnosis of a patient with a history of oropharyngeal cancer.

Objective: To describe symptoms from intralabyrinthine schwannomas specific from more common vestibular schwannomas of the internal auditory canal or cerebellopontine angle resulting from mass effect from the tumor within the labyrinth., Patients: Eight patients diagnosed as having intralabyrinthine schwannomas from 2000 to 2014 were examined retrospectively from two tertiary neurotologic centers., Interventions: Diagnosis of intralabyrinthine schwannoma was made with gadolinium-enhanced magnetic resonance imaging scans. Heavily T2-weighted sequences were used to verify mass within the fluid-filled labyrinth. Patients then underwent audiometric and vestibular testing when appropriate. Treatment consisted of observation or surgical resection., Main Outcome Measures: Clinical symptoms, magnetic resonance imaging scans, audiometric and videonystagmography data, and operative findings were reviewed., Results: Five of the eight patients had positional vertigo or nystagmus on testing. One patient's only complaint was positional vertigo without auditory symptoms. Three of the patients demonstrated mixed hearing loss. Two patients underwent resection of their tumors, one because of tumor growth and the other because of intractable vertigo., Conclusion: These data show that a high proportion within our series displayed symptoms of positional vertigo and mixed hearing loss, which are symptoms not typical of nonintralabyrinthine schwannomas. One patient's only reported symptom was positional vertigo. These symptoms may arise from the effect of the tumor's mass exerted on the cochlear and vestibular end organs.

Objective: To describe the presentation of intralabyrinthine schwannomas (ILSs)., Study Design and Setting: Retrospective multicenter study involving 12 European skull base surgery tertiary referral centers., Patients: One hundred ten patients with the diagnosis of ILS, either labyrinth confined or extending into the internal auditory meatus for less than 50% of their volume., Main Outcome Measures: Data collected were age, sex, nature and timing of presenting symptoms, hearing (according to the AAO-HNS grading system), results of vestibular tests (caloric tests and cervical vestibular-evoked myogenic potentials [c-VEMPs]), and tumor localization. Presenting symptoms and laboratory test results were studied according to the extension of the lesion into the cochlea (C) and vestibule (V), on one hand, and according to unifocal (L1) or plurifocal (L2) extension into the labyrinth, on the other., Results: Intracochlear type was more common (50%) than vestibular (19.1%) and more diffuse forms (30.9%). The mean delay for diagnosis was long (72.5 mo; SD, 76.6). Mean age was 53.9 years (SD, 13.2). Deafness was the most common symptom (77.8 dB HL [SD, 33.6], with only 24.6% of patients keeping viable hearing. Caloric tests (65.5% of patients) were abnormal in 77.8% of cases. c-VEMPs were abnormal in 65.7% of the 36 cases analyzed. In V forms, hearing was significantly better (class A + B in 21.1% in C and 45.8% in V forms) (p = 0.03), and vestibular function was more altered (C: 57%, V: 100%, p = 0.0009*). L2 forms were diagnosed later (L1: 59.1 mo, L2: 104.5 mo; p = 0.004*) and were associated more frequently with a dead ear (L1: 13.1%, L2: 41.2%, p = 0.002*) than L1 forms., Conclusions: This series, which is the largest in the literature, demonstrates that even very small and localized ILSs heavily compromise labyrinthine functions.

Introduction: Facial nerve weakness is most commonly due to Bell's palsy or cerebrovascular accidents. Rarely, middle ear tumor presents with facial nerve dysfunction., Case Presentation: We report a very unusual case of middle ear osteoma in a 49-year-old Caucasian woman causing progressive facial nerve deficit. A subtle middle ear lesion was observed on otoscopy and computed tomographic images demonstrated an osseous middle ear tumor. Complete surgical excision resulted in the partial recovery of facial nerve function., Conclusions: Facial nerve dysfunction is rarely caused by middle ear tumors. The weakness is typically due to a compressive effect on the middle ear portion of the facial nerve. Early recognition is crucial since removal of these lesions may lead to the recuperation of facial nerve function.

Background: Endolymphatic sac tumors (ELSTs) are rare, slow-growing tumors of the petrous bone. Despite the typical localisation, their radiological diagnosis can be challenging due to the variety of other tumors potentially showing similar features., Case Report: We present a 16-year-old child with progressive hearing loss, vertigo, and tinnitus who had a large petrous bone lesion showing imaging features of both ELSTs and aneurysmal bone cysts (ABCs). The patient underwent preoperative embolization of the tumor-supplying vessels and subsequently a subtotal resection. Histological examination revealed an ELST., Conclusion: Despite the rarity of petrous bone ABCs, they should be considered as a differential diagnostic alternative of ELSTs due to their similar imaging appearance.

Osteomas of the middle ear are rare benign neoplasms. To date, only a few cases have been reported. Osteomas of the middle ear are small, single, usually unilateral, peduncular growths, off-white in color, with a smooth or multilobular surface, asymptomatic or causing functional disorders. The most common symptom is conductive hearing loss because of impingement of the ossicular chain. Some cases are asymptomatic and are diagnosed incidentally. We present two cases of incidentally detected middle ear osteoma. Based on a review of the main articles in the literature and analysis of two cases managed in our department, we describe the clinical spectrum, etiology, and management of middle ear osteomas., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Introduction: Pleomorphic adenoma accounts for 65 percent of all salivary gland tumors. It has been identified in several anatomical regions, but pleomorphic adenoma arising in the ear canal, first described in 1951, is extremely rare., Case Presentation: A 40-year-old Japanese man's left ear canal was obstructed by a pleomorphic adenoma that caused mild conductive hearing loss. The tumor was resected and he remains disease-free two years after surgery., Conclusions: Pleomorphic adenoma usually arises from a major and minor salivary gland, but pleomorphic adenoma of the ear canal is derived from the ceruminous gland. We discuss the present case and 37 other case reports in our effort to clarify the clinical features and the course of pleomorphic adenoma in the ear canal.

We report a case of squamous cell carcinoma of external auditory canal in an Omani man with oculocutaneous albinism. The disease mimicked inflammatory process revealing positive cultures for various microorganisms during the course of his illness. He was eventually biopsied to rule out atypical infective process or presence of malignancy. He was staged as T4N0M0 and treated with radical doses of palliative radiation therapy which was very well tolerated and resulted in a complete resolution of disease clinically and a major soft tissue response on radiological imaging. Another unique finding was the absence of epidermal growth factor receptor (EGFR) protein overexpression in the tumour specimen. More than 90% of mucosal squamous cell carcinoma (SCC) involving the head and neck region overexpress the EGFR protein in normal skin patients. SCC is the predominant cutaneous malignancy in albinos, and the presence of EGFR protein overexpression in cutaneous SCC is believed to be 56-58% in normal skin patients. The scientific literature is scarce on reporting incidence of EGFR overexpression in either cutaneous or mucosal SCC in albinos, and it remains to be defined whether being albino is the cause for its absence., (2014 BMJ Publishing Group Ltd.)

Auricular tumors constitute 6% of all head-neck tumors. Malignant tumors of the auricula are generally squamous or basal cell carcinomas. Myiasis rarely occurs in healthy individuals. In general, it is a parasite that is seen in patients with mental retardation or psychiatric disorders, elderly individuals, those with poor self-care and hygiene, and those with immune system disorders. In humans, it is mainly seen in tropical and subtropical regions; however, in rare instances, it may be seen in other regions of the world. In the literature, there are limited numbers of myiasis cases reported from Turkey. In this study, we aimed to present a myiasis case (Wohlfahrtia magnifica) involving cutaneous and subcutaneous tissues with an underlying head-neck cancer, which, to the best of our knowledge, has not been reported before in the literature.

Objective: To analyze the difference between the endolymphatic sac tumors (ELSTs) in sporadic cases and in von Hippel-Lindau (VHL) disease., Study Design: Retrospective case review in a tertiary referral center., Patients and Methods: Fourteen cases of ELST, occurring since 1998, were reviewed. We analyzed the initial symptoms, characteristics of the tumor, treatment, sequelae, and follow-up for each group., Results: The ELSTs were sporadic in 6 cases and associated with VHL disease in 8 cases. The mean age at the time of the first surgery was 26 years (range, 12-41). All except two of the patients presented with a unilateral tumor. The initial symptoms were hearing loss (n = 9), tinnitus (n = 7), and/or vertigo (n = 5). Hearing loss was more prevalent in the sporadic cases. Preoperative arteriography was performed for 4 patients, with embolization performed for 1 patient. The size of the tumor was significantly larger in the sporadic cases (31.7 mm) than in the cases of VHL disease (19.3 mm). The surgical approach was more extensive in the sporadic cases. The surgeons found 2 types of tumors. Cystic tumors with massive bleeding invading the surrounding structures (the dura mater or jugular bulb) were more common in the sporadic cases. Fibrous tumors that infiltrate the bone and have moderate bleeding were more common in the cases associated with VHL disease.Two patients with small lesions were not operated on but were followed for 6 years without tumor growth. They died of metastasis from gastric and kidney cancer. Four recurrences occurred during the 14 years of follow-up. Four facial palsies and 8 cases of profound deafness were encountered postoperatively., Conclusion: Sporadic tumors are more aggressive than those associated with VHL disease. Complete surgical resection should be the goal of treatment. Preoperative angiography with embolization is recommended. In some cases, embolization may be impossible, and preoperative or postoperative radiotherapy should be discussed.

Background: Tumor of the temporal bone is a rare disease with a very poor prognosis. Surgery and postoperative radiotherapy are usually the recommended treatments for squamous cell carcinoma (SCC) of the external and middle ear, which may cause conductive hearing loss. The purpose of this study was to evaluate the audiologic results and compliance of active middle ear implant (AMEI) and establish the feasibility of the procedure in a patient treated for middle ear cancer., Methods: A 73-year-old patient treated with lateral petrosectomy, neck dissection, reconstruction/obliteration by pedicled pectoralis major myocutaneous flap, and postoperative full dose radiotherapy for external and middle ear SCC was selected for AMEI. Preoperative audiometric and speech audiometry tests were performed on both ears before and after the activation., Main Outcome Measures: Pure tone free field audiometry. Binaural free field speech audiogram., Results: Aided pure tone free field audiometry AMEI results show an increase in air conduction. Speech audiogram showed better discrimination scores in AMEI-aided situations. No complications were observed., Conclusion: AMEI after surgery followed by radiotherapy for middle ear cancer is feasible. Acoustic results in obliterated ear are satisfactory.

The primary goals of surgery of tympanomastoid paragangliomas (TMPs) are tumor eradication and hearing preservation. Though the surgical management of TMPs has been dealt with widely in the literature, the effects of TMPs themselves on preoperative hearing and the audiological outcomes after surgery have not been analyzed in detail. This article comprehensively evaluates the preoperative hearing and the long-term hearing outcomes after surgery of TMPs. This study is based on a study population of 145 patients which is the largest reported in the literature. The surgical approaches for all patients with TMPs were formulated according to an algorithm developed by the authors. Complete tumor removal with excellent hearing results can be achieved by approaching the tumor classes by the right surgical technique. TMPs could possibly induce sensorineural hearing loss in higher frequencies, and future studies could be directed towards this., (© 2014 S. Karger AG, Basel.)

Osteomas of the skull base are rare, benign, slowly progressing growths of dense cortical bone. Osteomas occurring in the internal auditory canal are extremely rare. These lesions have sometimes been linked with dizziness, sensorineural hearing loss, and/or tinnitus. Although there have been documented cases in which surgical excision has improved these symptoms, symptomatic relief is not always achieved with surgical management. Here we present, to the best of our knowledge, only the third reported case of bilateral osteomas of the internal auditory canal. An 82-year-old woman presented with an acute onset of vertigo without a history of trauma or ear infection. She reported two similar episodes occurring a few years earlier, with symptoms persisting for only a few days. Audiometry showed presbycusis. Computed tomography and magnetic resonance imaging identified bilateral internal auditory canal osteomas. The patient was treated conservatively, monitored, and had complete resolution of her symptoms.

Problem: A 58-year-old man presented with transient vertigo and pulsatile tinnitus., Methods: High-resolution computed tomography, magnetic resonance imaging, excision, and subsequent immunohistochemical assays were performed., Results: Imaging showed a soft tissue mass in the epitympanum and mastoid with bone erosion of the tegmen tympani and a dural tail sign, suggesting meningioma. Subsequently, because of signs of clinical progression, a canal-wall-up attico-antromastoidectomy was performed, with near-complete removal of a granulomatous, ossifying, haemorrhagic mass., Conclusions: Radiological imaging was critical in determining the extent of the mass and excluding other pathologies. Due to the atypical clinical and radiological signs, the final diagnosis of capillary haemangioma of the middle ear and temporal bone was made only after surgical resection and histopathological examination with immunohistochemistry, which excluded meningioma. The contiguous occurrence of cutaneous capillary haemangioma of the lateral face and neck was an important clue to the diagnosis.

Malnutrition affects 40-50% of patients with ear, nose and throat (ENT) cancer. The aim of this study was to assess changes induced by a specific nutritional supplement enriched with n-3 polyunsaturated fatty acids, fiber and greater amounts of proteins and electrolytes, as compared with a standard nutritional supplement, on markers of inflammation, oxidative stress and metabolic status of ENT cancer patients undergoing radiotherapy (RT). Fourteen days after starting RT, 26 patients were randomly allocated to one of two groups, 13 supplemented with Prosure, an oncologic formula enriched with n-3 polyunsaturated fatty acids, fiber and greater amounts of proteins and electrolytes (specific supplement), and 13 supplemented with Standard-Isosource (standard supplement). Patients were evaluated before RT, and 14, 28 and 90 days after starting RT. The results showed that there were no significant differences between the groups, but greater changes were observed in the standard supplement group, such as a decline in body mass index (BMI), reductions in hematocrit, erythrocyte, eosinophil and albumin levels, and a rise in creatinine and urea levels. We concluded that metabolic, inflammatory and oxidative stress parameters were altered during RT, and began to normalize at the end of the study. Patients supplemented with Prosure showed an earlier normalization of these parameters, with more favorable changes in oxidative stress markers and a more balanced evolution, although the difference was not significant.

Objectives: Lipomas of the internal auditory canal (IAC) and cerebellopontine angle (CPA) are exceedingly rare lesions. The purpose of this report was to describe our experience with lipomas of the IAC and CPA and perform a review of the literature., Methods: We report 8 cases of lipomas involving the IAC and/or the CPA that were managed at Gruppo Otologico between April 1987 and October 2012., Results: Four cases of entirely intracanalicular lipomas were radiologically misinterpreted as vestibular schwannomas and underwent tumor removal by a translabyrinthine approach. Two of these patients experienced postoperative facial nerve palsy. Lipomas were suspected in 4 patients on the basis of imaging findings and were managed conservatively. Of these 4 cases, 3 did not show any growth after an average period of 28 months, and 1 case demonstrated tumor growth on follow-up imaging., Conclusions: Neuroimaging represents an extremely important tool for this diagnosis. Attempts to achieve complete resection may result in severe neurologic sequelae, especially in large lesions. Observation with repeated imaging in order to detect growth of the lesion is usually recommended. Debulking of the tumor, mainly aimed at brain stem and cranial nerve decompression, should be considered in cases of disabling and uncontrolled neurologic symptoms and signs.

Objective: Endolymphatic sac tumors (ELSTs) are rare in the general population with much higher prevalence in von Hippel-Lindau(VHL) disease. The purpose of this study is to present two cases of endolymphatic sac tumor with VHL disease with analysis of VHL gene and to explore their association with VHL disease using molecular analysis., Methods: Clinical data of these two patients from different VHL families were studied. DNAs extracted from peripheral bloods were amplified by the polymerase chain reaction using oligonucleotide primers corresponding to the VHL gene, then compared the mutations with the Human Gene Mutation Database., Results: In case 1, 6 family members were enrolled in the study. Among them, three had been identified to have a germline missense point mutation at codon 194 of the VHL gene exon 1 (p.S65W). The little sister of the patient (case 1) underwent vitrectomy for retinal hemangioblastoma 5 years ago in another hospital. The mother of the patient (case 1) was further diagnosed to have a cerebellar hemangioblastoma and renal carcinoma in the following physical examination. Case 2 with her parents were also tested. Codon 499 of the VHL gene exon 3 (p.R167W) were detected in case 2 and her mother, but the mother refused further examination., Conclusions: The genetic diagnosis plays an important role in early detection of symptomatic patients and suspected patients. Clinical screening for members of the VHL families, and close follow-up of carriers allow an early detection of tumors and the metastasis, which is the most common cause of death of these patients.

Object: To determine if physiologically based MRI sequences can be used to detect endolymphatic sac tumor (ELST)-associated hydrops, the authors performed contrast-enhanced delayed FLAIR imaging in consecutive ELST patients with clinical findings consistent with hydrops., Methods: Consecutive patients with von Hippel-Lindau (VHL) disease and clinical findings of endolymphatic hydrops and ELSTs underwent contrast-enhanced delayed FLAIR MRI. Clinical, audiological, operative, and imaging findings were analyzed., Results: Three patients (2 male, 1 female) with 4 ELSTs (1 patient had bilateral ELSTs) were identified who had clinical findings consistent with endolymphatic hydrops. Computed tomography and MRI evidence of an ELST was found in all patients. Their mean age at initial evaluation was 39.7 years (range 28-51 years). All patients demonstrated progressive sensorineural hearing loss that was associated with episodic vertigo and tinnitus. Contrast-enhanced delayed FLAIR MRI clearly demonstrated dilation of the membranous labyrinth consistent with hydrops in the affected ears but not the unaffected ears. Two patients underwent resection of the associated ELST that resulted in stabilization of progressive hearing loss, as well as amelioration of tinnitus and vertigo., Conclusions: Contrast-enhanced delayed FLAIR MRI can be used to detect ELST-associated hydrops. Noninvasive MRI detection of hydrops can permit earlier detection of ELSTs in patients with VHL disease and provides direct insight into a mechanism that underlies ELST-associated audiovestibular morbidity.