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Tumours of the middle ear are rare. Among them glomus tympanicum is the most frequent, followed by adenoma of the middle ear (AME). This insidious pathology entity displays unspecific clinical and audiological features. Advances in histology and immunohistochemistry have greatly contributed to an accurate diagnosis. We report our experience with two patients, a middle-aged man and a woman, diagnosed in our hospital. Both patients underwent surgical treatment and have shown no signs of recurrence after a 27- and 28-month follow-up. In both cases an accurate diagnosis was confirmed by histological and immunohistochemical analysis.

We report a case of endolymphatic sac tumor (ELST). A 48-year-old female had recurrent and slowly progressive hearing loss, accompanied with dizziness like Meniere's disease. A tumor was located around the semicircular canals, and was detected on CT and MRI. The patient underwent total removal of the tumor using a transmastoid approach. Histopathological examinations agreed with features of an ELST. The tumor was highly suspected to have originated from the rugose portion of the endolymphatic sac or the endolymphatic duct, based on surgical and imaging studies. Structure of the membranous labyrinth was preserved regardless of the existence of the tumor around semicircular canals with bone destruction. ELSTs seem to have an osteolytic or osteophilic nature, by examining patterns of tumor infiltration.

Objective: To determine whether the appearance of the inner ear on T2-weighted follow-up magnetic resonance imaging correlates with hearing status after hearing-preservation surgery for vestibular schwannoma., Study Design: Retrospective chart review., Setting: Tertiary referral medical center., Patients: The study includes patients undergoing hearing-preservation surgery for vestibular schwannoma from 1998 to 2003., Intervention: Diagnostic evaluation with magnetic resonance imaging and audiometric evaluation., Main Outcome Measures: Hearing results as reported in charts was correlated with appearance of membranous labyrinth on T2-weighted magnetic resonance images obtained at least 1 year after surgery., Results: Twenty-nine patients were identified, 16 of whom satisfied the inclusion criteria. All 16 of the patients underwent middle fossa removal of vestibular schwannoma. Serviceable hearing according to American Academy of Otolaryngology-Head and Neck Surgery criteria was preserved in eight patients (50%). Of the eight patients without serviceable hearing, six had the cochlear nerve sacrificed at the time of surgery. All patients with serviceable hearing had normal appearing cochleovestibular signal on T2-weighted images, whereas six of eight patients (75%) with no hearing or poor hearing had abnormal low signal in the inner ear, suggesting inner ear ossification. The positive predictive value of a normal labyrinth for preserved hearing was 90%, whereas the negative predictive value of an abnormal labyrinth for no hearing was 100%. All but one patient who had the cochlear nerve sacrificed showed abnormal morphology of the labyrinth on T2-weighted magnetic resonance imaging., Conclusion: We describe the T2-weighted magnetic resonance findings after hearing-preservation surgery for acoustic tumor removal. Loss of inner ear signal on T2-weighted images correlates with loss of hearing postoperatively, whereas preserved inner ear signal correlates with hearing preservation after middle fossa surgery for vestibular schwannoma removal.

Perineural invasion of cranial nerves, including the facial nerve by squamous cell carcinoma of the skin, is a poor prognostic factor for recurrent disease and disease-specific mortality. We discuss 2 patients who presented to the Otolaryngology/Head and Neck Surgery service with complete facial nerve paralysis and a history of skin cancer. Based on our experience with these patients, we recommend that patients who present with facial nerve paralysis and a history of cutaneous malignancy undergo aggressive management including early intervention and complete exploration of the facial nerve from the geniculate ganglion out to the periphery. Such rigorous care achieves the best possible oncologic outcomes for a proven aggressive disease.

Auditory brainstem implants (ABIs) are currently indicated for patients older than 12 years with neurofibromatosis type 2 (NF2) who had bilateral schwannoma removed. Over the last 10 years, we have extended the indications for ABIs to nontumor children and adult patients with cochlear or cochlear nerve injuries or malfunctions who would not benefit from a cochlear implant. We have provided ABIs for patients with cochlear nerve aplasia and other injuries, and patients in whom any benefit was, or would be, severely compromised as in extensive cochlear ossification. In the present chapter we report our recent findings in adult ABI patients and compare the psychophysical and speech perception outcomes in tumor with those in nontumor patients. We demonstrate that the ABI can stimulate the central auditory system in a way that gives the ability of open set speech understanding, and can thus be indicated in nontumor adult patients who are not candidates for a cochlear implant. From April 1997 to January 2006, a total of 80 patients, 62 adults and 18 children, were fitted with ABIs in the University of Verona ENT Department; age ranged from 14 months to 70 years. Twenty-six patients had NF2 with bilateral vestibular schwannoma removal, and 54 had nontumor diseases of the cochlear nerve or cochlea. The retrosigmoid approach was used in all patients. All patients had a functioning implantation, and no complications were observed during the operation, activation as well as long-term use of the ABI. All patients, except 1 (NF2), reported auditory sensations with activation of various numbers of electrodes (from 5 to 21). Different electrodes elicited different pitch sensations. At 1 year after implantations nontumor adults scored from 12 to 100% in open set speech perception tests (average 59%), and tumor (NF2) patients scored from 5 to 30% (average of 11%). The differences between these results are statistically significantly (p < 0.01). To investigate the cause of the differences in performance between tumor and nontumor ABI recipients, a series of psychophysical tests were done consecutively in 39 adult patients with implants (25 nontumor and 14 tumor patients) from May 1999 to April 2004 and with a follow-up of at least 1 year. The outcome of this study shows that: (1) The ABIs allow most tumor and nontumor patients to experience improved communication as well as awareness of environmental sounds. (2) Nontumor patients had better hearing outcomes than tumor patients when the variation in the auditory benefit with the ABI in relation to the patient's underlying pathological conditions were taken into consideration. (3) A significant number of nontumor patients are able understand speech at a level comparable to that of the most successful cochlear implant users including conversational telephone use. (4) The ABI represents the tool for hearing rehabilitation in patients with profound hearing loss who cannot be fitted with cochlear implants.

Background: Facial nerve schwannoma of the internal auditory canal is a very rare tumour., Aim: While Bell's palsy is the commonest cause of a facial paresis, more serious causes should be excluded if recovery is delayed., Method: A case report of a young man who presented with a long standing facial palsy., Conclusion: Any facial palsy that does not show evidence of recovery within six weeks should be investigated radiologically to exclude a tumour.

Middle-ear ceruminous adenomas are rare benign neoplasms arising from the epithelium of the middle ear. Progressive hearing loss, ear fullness and tinnitus are common symptoms of this tumour; facial nerve paresis and vestibular disturbances occur very infrequently. We present two cases of middle-ear ceruminous adenomas, one showed rapid unilateral hearing loss with aural fullness, followed by purulent aural discharge and vertigo. In the second case, the disease affected an already deaf ear and the only symptom of the disease was increasing vertigo. The clinical features, intraoperative findings, and histological and radiological findings are presented. The cases are compared to those described in the literature.

A 42-year-old woman complained of tinnitus, hearing loss and headache. Many years ago she had been treated for a vestibular schwannoma and cutaneous neurofibroma. MRI revealed bilateral vestibular schwannomas and multiple meningiomas due to neurofibromatosis type 2.

Objective: Present four new cases of carcinoid tumor of the middle ear, two of which developed late recurrences and regional metastases. Review the literature to identify the clinical features, rate of recurrence, and incidence of metastasis of carcinoid tumor of the middle ear., Study Design: Retrospective chart review., Setting: Tertiary referral hospital., Patients: Eligibility criteria consist of a diagnosis of carcinoid tumor of middle ear., Intervention: Surgical excision of primary and metastatic disease., Main Outcome Measure: Clinical characteristics, rate of recurrence, and incidence of metastasis of carcinoid tumor of the middle ear., Results: Forty-six patients with carcinoid tumor of the middle ear are included in this report, 42 patients were identified from a review of the literature, and 4 new patients are presented. The most common presenting symptom was hearing loss. Surgical excision was the treatment with radical mastoidectomy being the most common procedure. Ten (22%) patients developed locally recurrent disease, and four (9%) developed regional metastases., Conclusions: Carcinoid tumor of the middle ear is an infrequent cause of a middle ear mass, with only 46 cases published. Despite previous assertions of benignancy, the findings of this study suggest that carcinoid tumor of the middle ear is indeed a potential low-grade malignancy with documented metastatic potential. Almost all middle ear adenomatous tumors ("adenoma" and "carcinoid") show evidence of neuroendocrine differentiation, and so at least some middle ear carcinoids ("adenomas") appear to represent well-differentiated neuroendocrine carcinomas. Presentation and symptoms are consistent with a middle ear mass and rarely include carcinoid syndrome. Surgical treatment is recommended and tailored to the extent of disease. Patients with carcinoid tumor of the middle ear require indefinite follow-up for possible recurrence or metastasis.

Objectives/hypothesis: Described is the first reported case of an angiofibrolipoma of the ear canal in a patient who presented with right-sided conductive hearing loss and a medial canal stenosis., Study Design: Case report., Results/conclusion: This variant of lipoma contains mature adipocytes, blood vessels, and dense collagenous tissue. The physical examination can be misleading, and the diagnosis requires histopathological examination. The patient was treated with complete surgical excision, tympanoplasty, canalplasty, and skin grafting to the external auditory canal. His pure-tone average improved from 37 to 11 dB, and his air-bone gap was closed completely. The lipoma has not recurred in the 6-month period following surgery.

Unlabelled: The etiology of sudden deafness can remain undetermined despite extensive investigation. This study addresses the value of magnetic resonance imaging in the analysis of sudden deafness patients., Study Design: Transversal cohort., Material and Method: In a prospective study, 49 patients attended at otolaryngology emergency room of Federal University of Sao Paulo--Escola Paulista de Medicina, from April 2001 to May 2003, were submitted to magnetic resonance imaging., Results: Magnetic Resonance abnormalities were seen in 23 (46.9%) patients and revealed two tumors suggestive of meningioma, three vestibular schwannomas, thirteen microangiopathic changes of the brain and five (21.7%) pathological conditions of the labyrinth., Conclusion: Sudden deafness should be approached as a symptom common to different diseases. The presence of cerebellopontine angle tumors in 10.2% of our cases, among other treatable causes, justifies the recommendation of gadolinium-enhanced magnetic resonance use, not only to study the auditory peripheral pathway, but to study the whole auditory pathway including the brain.

Patients with acoustic neuroma in their only hearing ear are not frequently seen in clinical practice. Managing this group of patients is a challenge to both the patient and surgeon. In this study we report on five cases of acoustic neuroma in an only hearing ear. Our decision for conservative management of those patients with regular follow-up using auditory brain stem response and magnetic resonance imaging is discussed. Other management options currently available are reviewed as well.

We report the 25-year follow-up on the first reported case of odontoma in the middle ear. Diagnosis of odontoma had been made on the basis of radiography films that showed a middle ear mass with multiple toothlike areas of radiopacity. No clinical intervention was recommended. At 25-year follow-up, audiometry showed progressive mixed hearing loss on the affected side and mild ipsilateral sensorineural hearing loss. Computed tomography better characterized the discrete mass, which was shaped similar to a dental crown. To avoid jeopardizing cochlear and facial nerve function, no surgical intervention was pursued, and we recommended use of a BiCROS (bilateral contralateral routing of signal) hearing aid.

Objective: Endolymphatic sac tumors are aggressive papillary tumors of the temporal bone frequently associated with von Hippel-Lindau disease. The goal of this study was to use a newly devised classification system as a means to analyze differences between endolymphatic sac tumor extension in von Hippel-Lindau disease and non-von Hippel-Lindau disease patients., Methods: Previously reported cases of endolymphatic sac tumor and two new cases were retrospectively reviewed and assigned to a new classification system consisting of four grades based on tumor extent and location., Results: Mean age of 103 patients without von Hippel-Lindau disease was 52.5 years, whereas in 46 patients with VHL the mean age was 31.3 years. Patients with von Hippel-Lindau disease were more likely to be female (female-to male ratio of 2:1 for von Hippel-Lindau disease patients versus 1:1 for non-von Hippel-Lindau disease patients). Symptoms consisted of hearing loss (100% [mean duration, 10 yr] for VHL patients versus 97% [mean duration, 7.8 yr] for non-von Hippel-Lindau disease patients), facial weakness (38% versus 49%), and tinnitus or vertigo (41% versus 60%). Bilateral tumors were common in von Hippel-Lindau disease patients (28% versus 1%). Tumors in von Hippel-Lindau disease patients were significantly more likely to be lower grade than tumors in non-von Hippel-Lindau disease patients (Grade I, 40% versus 25%; Grade II, 50% versus 58%; Grade III, 8% versus 14%; and Grade IV, 2% versus 4%; p < 0.05). Before 1988, there were relatively fewer Grade I (15% versus 33%) and relatively more Grade II (69% versus 47%) endolymphatic sac tumors in non-von Hippel-Lindau disease patients than after 1988., Conclusions: Increased usefulness of intracranial imaging since 1988 has led to the diagnosis of sporadic endolymphatic sac tumors with lower grades. Surveillance imaging in von Hippel-Lindau disease may account for the greater proportion of endolymphatic sac tumors diagnosed with lower grades. Endolymphatic sac tumors associated with a diagnosis of von Hippel-Lindau disease appear to affect a younger population of patients than non-von Hippel-Lindau disease cases and occur in women twice as often as in men when associated with von Hippel-Lindau disease. In addition, tumors are more frequently bilateral and less advanced in the von Hippel-Lindau disease patient as opposed to the non-von Hippel-Lindau disease patient.

A 62-year-old female was admitted to our clinic in October 1999 with a 6-year history of hearing loss, tinnitus and otorrhea in the right ear. Physical examination showed a polypoid mass arising from the right middle ear and extending into the external auditory canal. Serohemorrhagic otorrhea was also observed. Serious hemorrhage occurred while taking a biopsy. The histopathologic diagnosis was a glomangioma. MRI demonstrated a hypervascular mass (10 x 15 mm(2)) completely filling the right middle ear. Preoperative embolization of the ascending pharyngeal artery was carried out. The tumor was completely resected via a transmastoid approach. No complications were observed postoperatively. At the most recent follow-up examination, 72 months after the operation, no sign of disease was present. The modified radical mastoidectomy approach used in this case proved to be a safe and efficacious method for removing the glomangioma.

Schwannoma of the eighth cranial nerve (acoustic neuroma) is, by far, the most common cerebellopontine angle lesion. There is, however, a wide variety of differential diagnostic possibilities that must be considered when viewing images of patients with sensorineural hearing loss (SNHL), vertigo, and dizziness. This communication is intended to provide the reader with an approach to this problem. Detailed anatomy of the region is also included in this communication.

We examined the clinical presentation in patients with a histologically proven ingrowth of the cochlear nerve by acoustic neuroma to see whether this differs from what is known from large acoustic neuroma series. In total, 85 acoustic neuromas had an en bloc dissection to study histologically the relation between the cochlear nerve and the acoustic neuroma. In 21 of these 85 specimens, there was histologic proof of invasion of the cochlear nerve by the tumor. For 13 of these 21 tumors, sufficient clinical data could be retrieved to describe the clinical presentation in these patients. We collected clinical data such as age, sex, presenting symptoms, duration of symptoms, tone audiograms, tumor size measurements and volumetric calculations, and latency interval data I-V of brain stem evoked response audiometry and calculated whether there was any correlation among those data. We also compared these clinical data with the data from some large acoustic neuroma series. No clear difference could be shown between the clinical presentation of acoustic neuroma patients with cochlear nerve ingrowth and the clinical presentations in large acoustic neuroma series. This outcome favors the theory that the hearing impairment in acoustic neuroma patients is mainly the result of compression on the vessels of the cochlea and/or on the cochlear nerve.

Objective: Meningiomas arising primarily within the internal auditory canal (IAC) are notably rare. By far the most common tumors that are encountered in this region are neuromas. We report a series of eight patients with meningiomas of the IAC, analyzing the clinical presentations, surgical management strategies, and clinical outcomes., Methods: The charts of the patients, including histories and audiograms, imaging studies, surgical records, discharge letters, histological records, and follow-up records, were reviewed., Results: One thousand eight hundred meningiomas were operated on between 1978 and 2002 at the Neurosurgical Department of Nordstadt Hospital. Among them, there were 421 cerebellopontine angle meningiomas; 7 of these (1.7% of cerebellopontine angle meningiomas) were limited to the IAC. One additional patient underwent surgery at the Neurosurgical Department of the International Neuroscience Institute, where a total of 21 cerebellopontine angle meningiomas were treated surgically from 2001 to 2003. As a comparison, the incidence of intrameatal vestibular schwannomas during the same period, 1978 to 2002, was 168 of 2400 (7%). There were five women and three men, and the mean age was 49.3 years (range, 27-59 yr). Most patients had signs and symptoms of vestibulocochlear nerve disturbance at presentation. One patient had sought treatment previously for total hearing loss before surgery. No patient had a facial paresis at presentation. The neuroradiological workup revealed a homogeneously contrast-enhancing tumor on magnetic resonance imaging in all patients with hypointense or isointense signal intensity on T1- and T2-weighted images. Some intrameatal meningiomas showed broad attachment, and some showed a dural tail at the porus. In all patients, the tumor was removed through the lateral suboccipital retrosigmoid approach with drilling of the posterior wall of the IAC. Total removal was achieved in all cases. Severe infiltration of the facial and vestibulocochlear nerve was encountered in two patients. There was no operative mortality. Hearing was preserved in five of seven patients; one patient was deaf before surgery. Postoperative facial weakness was encountered temporarily in one patient., Conclusion: Although intrameatal meningiomas are quite rare, they must be considered in the differential diagnosis of intrameatal mass lesions. The clinical symptoms are very similar to those of vestibular schwannomas. A radiological differentiation from vestibular schwannomas is not always possible. Surgical removal of intrameatal meningiomas should aim at wide excision, including involved dura and bone, to prevent recurrences. The variation in the anatomy of the faciocochlear nerve bundle in relation to the tumor has to be kept in mind, and preservation of these structures should be the goal in every case.

Background: Implantable electrical devices are becoming increasingly common in the patient population presenting for Mohs micrographic surgery. In addition to understanding the potential intraoperative complications with implantable cardioverter-defibrillators and pacemakers, the Mohs surgeon needs to be aware of the relatively new treatment of movement disorders using implanted deep brain stimulators., Objective: We present only the second reported case of Mohs surgery in a patient with a deep brain stimulator. In an attempt to help minimize adverse events during a procedure, we review the more commonly encountered electrical devices as well as the newer deep brain stimulators. We provide guidelines for the avoidance of electromagnetic interference during an electrosurgical procedure., Methods: This 76-year-old patient with Parkinson's disease and an implanted deep brain stimulator underwent Mohs surgery for excision of a squamous cell carcinoma on the ear. In an attempt to minimize electromagnetic interference with his implanted device, hemostasis was obtained with the aid of a battery-operated heat-generating handheld electrocautery device., Results: The patient tolerated the procedure well without complications or reports of discomfort., Conclusion: Patients with implanted electrical devices are subject to electromagnetic interference during an electrosurgical procedure. Care must be taken in this expanding patient population during a Mohs surgical procedure.

Non-surgical stereotactic gamma ionizing irradiation was developed in order to produce a lethal effect on unilateral acoustic neurinomas (vestibular schvannomas). We present a study of postural vestibular dependent performances in 218 patients before and after curative irradiation. Subjects were asked to stand at ease on a static dynamometric foot-plate, gazing at a fixed point in front of them (EO condition) or to stand eyes closed (EC condition). Statokinesigrams were registered during two consecutive sessions of 51.2 sec, under EO and EC conditions. These sessions were first managed the days before (d-1), and after (d+1) irradiation; others were performed later (d+1 to 5 Years; n=37). Center of pressure (CofP) mediolateral (X) and sagittal (Y) positions were quantified every 100 msec. Mean Xm session value (with SD) was taken as a personal parameter for left or right body inclination. Area S for 90% of the XY successive placements of the CofP observed during a session was taken as an index for 2D postural way. Before irradiation the overall average of the 218 personal Xm mean values demonstrated a statistically significant body inclination toward the affected side, however under EC condition only. The day after irradiation, a significant reduction of the overall mean value of body inclination was observed. Paired Xm statistics of confirmed this trend toward usual symmetry. The day after stereo-irradiation, averaging areas S of ellipses indicated a shift of instability toward normality. For the two parameters, the pseudo-Romberg ratios (performance EC/performance EO) indicated that a special visual contribution to balance control is present in patients with a neurinoma tumor (here I and II grades). The relative importance of this visual support declined shortly after ionizing treatment. Because the radiation is neither noxious nor excitatory we think such a rapid recovery is due to some recovery of vestibular nerve afferent conduction, and a rapid neural reprogramming of the balance control. These attractive results call for deeper investigations of both vestibular and auditory functions.

Rationale: Patients with cystic vestibular schwannomas (VS) are a radiologically well-defined subgroup of patients who classically have poor outcome after microsurgical resection. Since Pendl's report of a high rate of failures, they are also considered as poor candidates for radiosurgery., Material and Method: Among the 1000 consecutive patients who underwent Gamma Knife surgery in Marseilles, France between July 1992 and January 2002, we have collected and studied 54 patients with cystic VS at the time of treatment., Results: The median follow-up of this group was 26 Months (mean: 33, range: 6-90). Failure (6.4%) led to microsurgical removal in 2 patients and a radiosurgery in 1 patient with a delay of 2 Years for 2 of them and 3 Years for the third. No facial palsy has been reported. Two patients developed transient hypesthesia. Among the 32 patients with functional hearing at the time of treatment, 53% preserved their hearing function at 3 Years., Conclusions: We found an increased risk of failure in this group compared to patients with no cyst at time of radiosurgery (93.6% instead of 98%). But this is also a group were we observe most dramatic shrinkage. Prudent radiosurgical treatment of cystic vestibular schwannomas remains mandatory: strict follow-up is specially important.

Objective: The majority of the patients still lose hearing function in spite of the technical advances in microsurgery. We wanted to evaluate preservation of hearing function potential after Gamma Knife Surgery., Material and Method: In Marseille, we performed 1000 Gamma-Knife procedures for vestibular schwannomas between July 1992 and January 2002. This population included 175 patients undergoing first intention surgery for a unilateral schwannoma with functional preoperative hearing (Gardner and Robertson 1 or 2) who were studied with a follow-up longer than 3 Years. Univariate and multivariate analysis was performed., Results: Numerous parameters significantly influenced the probability of functional hearing preservation at 3 years. The overall rate of preservation was 60%. The main parameters of predictability were limited hearing loss (Gardner and Robertson stage 1 versus 2) before radiosurgery, presence of tinnitus as the initial symptom, young age and the small tumor size. Preservation of functional hearing at 3 years was 77.8% when the patient was initially in stage I, 80% when the patient's first symptom was tinnitus, and 95% when the patient had both. In these patients, the probability of functional preservation at 5 years was 84%., Conclusion: We report a large population of patients treated by radiosurgery with initial functional hearing. These results demonstrate that a large percentage of selected patients can preserve functional hearing after Gamma Knife Surgery, their chances of functional preservation being greater than after microsurgery or simple surveillance.

Rationale: To evaluate the functional results of Gamma Knife surgery of vestibular schwannomas relying on a large and prospective series of consecutive cases., Material and Method: The first 1000 patients with cerebello-pontine angle schwannomas were consecutively treated by Gamma Knife in Marseille Timone University Hospital between July 1992 and March 2001. Patients without NF2 and or clinico-radiological arguments in favor of a facial origin accounted for a population of 927 patients (414 males, for 513 females) including 843 treated in first intention. In this series the Koos classification was: stage I 77 patients, stage II 520 patients, stage III 287 patients and stage IV 42 patients. The average Volume was 12.7mm3. Haring was usefull (Gardner and Robertson) before radiosurgery in 47% of the patients (subnormal in 20.3%)., Results: Tumor control at last follow-up was 97%. Globally, a clinical trigeminal injury was observed in 0.6% of the patients and a facial palsy in 1.3%. There was clearly a decrease of the incidence of neuropathies with time; no facial palsy being reported among the last 258 patients. The rate of functional hearing preservation (Gardner) for patients initially in class I was 77.8% (47.6% for class II) at 3 Years. This rate of functional preservation reached 95% among patients with tinnitus as a first symptom., Conclusion: Today, strong evidence surports the superiority of Gamma Knife surgery in term of functional perservation and equal efficacy compared with microsurgical removal. Consequently, radiosurgery must be preferred as a first intention choice for young patients with few symptoms presenting with a small to middle size vestibular schwannomas (Koos I-III).

The coexistence of paraganglioma with cholesteatoma is a very rare clinical disorder. The clinical presentations are nonspecific. There may be radiological characteristics of either cholesteatoma or paraganglioma in the middle ear area, but the diagnosis of the coexistence of the 2 is usually made only postoperatively. Here is such a case that is made more interesting not only because it initially presented with conductive hearing loss but also because the clinical picture mimicked chronic otitis media. The patient underwent postauricular tympanomastoidectomy with extended facial recess approach to remove the tumor. No evidence of recurrence and complications were noted.

Objective: To describe vertigo and hearing disturbance as a first sign of glioblastoma., Study Design: Case report., Setting: Ear, Nose, and Throat Department of the University of Regensberg, Germany. Primary Care Center., Patients: A patient with a left temporal glioblastoma., Results: A 67-year-old man presented with a 2-month history of vertigo and hearing disturbance. Radiological imaging revealed a left temporal tumor with dural inflation and erosion of the petrous bone and superior semicircular duct. The surgery involved total resection of the tumor and resurfacing of the gap in the superior canal. The histopathological examination revealed World Health Trade Organization IV glioblastoma. Postoperatively, the debilitating symptoms were relieved and the patient received radiation therapy. Tumor progression indicated a recraniotomy and a mastoidectomy. The tumor was only partially resected, and required chemotherapy. It subsequently developed otoliquorrhea and required a remastoidectomy. Histopathology of a pathological fracture of the X thoracic vertebra revealed a metastasis of the known glioblastoma. The patient died from respiratory distress syndrome., Conclusion: To the best of our knowledge, we are presenting the first case with transdural infiltration of bony structures by a glioblastoma at the moment of diagnosis. The transdural spread could be via the sinus petrosus and along the nervous petrosus major in the petrosal bone. Superior canal dehiscence syndrome should be considered in the differential diagnosis of vertigo and hearing disturbance. Two different processes for the etiology of the superior canal dehiscence syndrome are discussed previously in the literature; however, we present a new entity with a tumor-cause dehiscence of the bone overlying the superior canal.

A case is presented where a left jugulotympanic paraganglioma (JTP) extended to the nasopharynx and the patient presented with recurrent epistaxis. Although initial biopsy of an aural polyp had been suggestive of the diagnosis several years previously, the diagnosis was not confirmed until the patient presented with recurrent epistaxis and severe anaemia. To the best of our knowledge, this is the first case reported of such a presentation of JTP.

The patient presenting with otalgia poses a diagnostic challenge for which orderly and diligent evaluation and management is needed. The etiology of otalgia can be either primary or referred, and a detailed history and physical examination with directed studies as indicated can elucidate the cause of the pain.

The entity which has come to be known as an endolymphatic sac tumor (ELST) has, in the past, been known as adenocarcinoma of endolymphatic sac origin, aggressive papillary tumor of the temporal bone and Heffner's tumor. ELSTs arise in the vicinity of the inner ear and may extend to involve both the posterior fossa as well as the middle ear and the external ear canal, which may complicate the differential diagnosis ELSTs are typically seen in adults, with only rare descriptions in pediatric patients. They may be sporadic tumors or they may arise as part of the symptom complex of von Hippel-Lindau disease. Clinical signs at presentation range from a mass in the external ear canal to sensorineural deafness to cranial nerve palsies. Imaging studies reveal a destructive lesion of the petrous bone which is heterogeneous on MR scanning. Light microscopy reveals two chief patterns: a follicular pattern, reminiscent of thyroid parenchyma; and a papillary/solid pattern. Both patterns are often admixed in the same tumor, and the individual tumor cells are cytologically bland. Immunohistochemically, ELSTs are typically keratin-, vimentin- and epithelial membrane antigen-positive; they are often S-100 protein-positive and neuron-specific enolase-positive as well. ELSTs are difficult to extirpate surgically (owing to their locally aggressive nature); nevertheless, surgical excision remains the mainstay of current therapy. These are slow-growing (albeit locally aggressive) tumors which have only rarely been reported to metastasize; as such, they remain principally a problem of local control.

The authors report on a patient with a rare schwannoma that arose from the cochlear division of the vestibulocochlear nerve. Distinctively, the lesion appeared to arise from the cochlea itself and was monitored with clinical and neuroimaging studies for 12 years before it was diagnosed and treated. The atypical occurrence of schwannomas of the vestibulocochlear nerve originating in the inner ear structures underscores the high level of clinical suspicion required for the diagnosis of these lesions in patients presenting with persistent auditory and vestibular symptoms.

Objective: To describe inflammatory polyps of the middle ear in 5 dogs., Study Design: Case series., Animals: Five dogs with ear disease., Methods: Medical records (1995-2001) were reviewed to identify dogs with inflammatory polyps of the middle ear. Signalment, clinical signs, ancillary diagnostic procedures, treatment, postoperative complications, and outcome were recorded. Owners and referring veterinarians were contacted to document outcome., Results: Dogs with inflammatory polyps of the middle ear were male and aged 4 to 13 years. Two dogs had bilateral polyps, whereas 3 had unilateral polyps. The most common clinical presentation was otitis externa and media, with radiographic evidence of otitis media. Polyps were treated by ventral bulla osteotomy (VBO) in 1 dog and total ear canal ablation with lateral bulla osteotomy (TECA-LBO) in 4 dogs. Polyps consisted of a fibrovascular stroma infiltrated with neutrophils, macrophages, lymphocytes, and plasma cells. The overlying epithelium was frequently ulcerated. Immediate postoperative complications included a seroma after VBO (1 dog) and transient unilateral facial nerve paralysis after bilateral TECA-LBO (1 dog). No recurrence occurred within 9 to 69 months., Conclusions: Unilateral or bilateral, inflammatory polyps can occur in the middle ear of dogs in association with otitis externa and media. No recurrence occurred after surgical removal of the polyps., Clinical Relevance: Inflammatory polyps of the middle ear in dogs can be a cause of otitis externa/media. Surgical removal of aural polyps has a good prognosis., (Copyright 2003 by The American College of Veterinary Surgeons)

The case of a five year old boy who presented with a lower motor neurone facial nerve palsy secondary to primary non-Hodgkin's lymphoma (NHL) of the middle ear is discussed. Any child who presents with a facial nerve palsy and conductive hearing loss requires thorough evaluation to exclude the possibility of temporal bone malignancy.

We report a case of mucoepidermoid carcinoma (MEC) originating from the eustachian tube and middle ear. A 31-year-old male who presented with otorrhoea and methicillin-resistant Staphylococcus aureus (MRSA) in the right ear was admitted to hospital due to cerebral infarction and deep vein thrombosis. After recovery, biopsies from a granulomatous mass found in the middle ear during operation for chronic otitis media revealed intermediate-grade MEC and a nasopharyngeal mass identified after surgery also revealed the same result. He received combined radiation therapy and chemotherapy and no residual or recurrent tumour was detected after two years of follow-up.

Background and Purpose: Intracranial lipomas are uncommon congenital malformations that most often are asymptomatic. A rare subset of intracranial lipomas arises within the vestibule of the inner ear, which can cause sensorineural hearing loss. We present the CT and MR imaging characteristics of lipomas within the vestibule of the inner ear and propose a pathogenetic mechanism for this malformation., Methods: We retrospectively reviewed five cases of vestibular lipoma. T1-weighted axial conventional spin-echo and T2-weighted axial fast spin-echo sequences were performed in all five cases. Four patients underwent T1-weighted fat-saturated imaging, and one underwent CT scanning with Hounsfield units measured., Results: Four female patients ranging in age from 10 to 40 years and one 26-year-old male patient had sensorineural hearing loss. Two patients had progressive high-frequency loss; one, sudden-onset high-frequency loss; and two, congenital high-frequency loss. All had lesions within the vestibule that displayed hyperintensity on T1-weighted images, isointensity to slight hypointensity on T2-weighted fast spin-echo images, hypointensity with fat saturation, and fat attenuation on CT scans. Three of the cases were associated with lipoma of the cerebellopontine angle. In none of these cases were the cerebellopontine angle and labyrinthine lipomas contiguous., Conclusion: Intravestibular lipoma is considered when a focal hyperintense lesion is identified within the vestibule of the inner ear on T1-weighted MR images. Lesion hypointensity on fat-saturated T1-weighted MR images verifies its lipomatous nature and separates it from intralabyrinthine hemorrhage or highly proteinaceous fluid.

Objective: To determine the incidence of bilateral endolymphatic sac tumors in von Hippel-Lindau syndrome and to describe the technique and results of hearing preservation surgery for small endolymphatic sac tumors in a series of patients with von Hippel-Lindau syndrome., Study Design: Analysis of the literature to determine the incidence of bilateral endolymphatic sac tumors and a retrospective case review of hearing preservation surgery for removal of small endolymphatic sac tumors in four patients with von Hippel-Lindau syndrome., Setting: Tertiary care academic medical centers., Patients: Four patients with von Hippel-Lindau syndrome (three with bilateral endolymphatic sac tumors) and progressive sensorineural hearing loss in which preoperative imaging studies revealed in situ or small endolymphatic sac tumors without ipsilateral labyrinthine destruction., Intervention: All four patients had complete surgical excisions of the endolymphatic sac tumor via one of three surgical approaches with the goal of hearing preservation. One patient had bilateral surgery., Main Outcome Measures: Audiometric and radiographic., Results: Nearly one-third (30.2%) of patients with von Hippel-Lindau syndrome and endolymphatic sac tumors have bilateral disease. All four patients (five ears) maintained serviceable hearing postoperatively after surgical excision of the endolymphatic sac tumor via a variety of approach options., Conclusion: The discovery of a small or in situ endolymphatic sac tumor affords the patient the option of surgical removal with hearing preservation. This is critical in the patient with von Hippel-Lindau syndrome who is at risk for bilateral disease and complete bilateral anacusis if tumor growth progresses.

A glomus tympanicum tumor that is associated with a visible retrotympanic mass is the most frequent cause of pulsatile tinnitus. The preoperative diagnostic approach to this lesion includes a meticulous physical examination as well as high-resolution CT, magnetic resonance angiography, and digital angiography, which can also be used for preoperative embolization. We report the use of color transcranial Doppler sonography in the evaluation of glomus tympanicum tumor in a 67-year-old woman with a 3-year history of left tinnitus. An otoscopic examination revealed a reddish pulsatile mass behind an intact tympanic membrane. No lesions were visualized on gray-scale sonography. Contrast-enhanced color transcranial Doppler sonography showed a vascular ovoid mass that measured 2 x 1 x 1 cm; spectral analysis of the lesion revealed arterial flow with a low resistance index. Color transcranial Doppler sonography helped define the dimensions and vascular characteristics of the lesion., (Copyright 2002 Wiley Periodicals, Inc.)

Patients with vestibular schwannomas (VSs) most commonly present with sensorineural hearing loss, which is often insidious or gradual. Up to 26% of patients may present with sudden hearing loss, however, which poses an important surgical challenge. Sudden hearing loss has been attributed to spasm or occlusion of the labyrinthine artery resulting from tumor compression, and it is usually treated with corticosteroids. Hearing preservation surgery is not usually attempted in patients who have poor or nonserviceable hearing preoperatively. The authors describe a 68-year-old man with complete deafness of the left ear since childhood, who developed sudden, profound sensorineural hearing loss in the right ear. Magnetic resonance imaging revealed a small right-sided intracanalicular tumor. Treatment with high-dose corticosteroids produced only minimal improvement in hearing. Subsequent emergency decompression and resection of a VS resulted in rapid improvement and restoration of hearing, with facial nerve preservation. Although most neurotologic lesions in patients with hearing in only one ear are managed nonsurgically, resection of small tumors in the setting of sudden hearing loss should be considered in selected cases. This finding indicates that a therapeutic window may exist during which sudden hearing loss caused by intracanalicular tumors is reversible.

Merkel cell carcinoma (MCC) is a rare malignant tumour that develops in sun-exposed areas in immunocompromised patients (chronic lymphocytic leukaemia, transplant recipients) older than 50 years. We report MCC in a young black woman with human immunodeficiency virus (HIV) infection. A 2-cm binodular violaceous lesion developed on her left ear lobe. Extensive work-up, including computed tomographic scans of the neck, chest, abdomen and pelvis, octreotide scan and sentinel node biopsy, did not demonstrate any metastasis. A wide excision was performed and the patient remained free of disease after 9 months. This case is the fourth observation of MCC in an HIV-infected patient.

Trauma and tumor are the causes of acquired ear defects that surgeons are frequently called on to treat. Trauma may result in hematoma or laceration of the ear. In addition, both trauma and tumor excision may result in skin or cartilage loss. Prevention of recurrence depends on complete excision of the tumor m both lateral margins and depth. Tumors of the ear (as well as the nose) are often incompletely excised because of the difficulty in reconstruction. The absence of subcutaneous tissue in the ear allows fixation and perichondrial involvement to occur early. The tumor affinity for perichondrium usually prohibits penetration into the cartilage itself.