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We report the MRI findings of six unusual lesions of the internal auditory canal: three haemangiomas, one lipoma, one metastasis and one traumatic neuroma. We compare the findings to those of 20 intracanalicular schwannomas. We noted the site and size of the tumour, its signal intensity, borders and the homogeneity of enhancement were studied on T1-weighted images before and after intravenous contrast medium and T2-weighted images. Most schwannomas were homogeneous lesions, isointense on T1- and T2-weighted images, and strongly enhancing. Spontaneous high signal on T1-weighted images, heterogeneous contrast enhancement and extranodular enhancement were helpful for recognising lesions other then schwannomas; site, size and signal on T2-weighted images were not. All the haemangiomas had a specific pattern of contrast enhancement, with an anterior core intensely enhancing portion and a posterior portion which enhanced moderately or not at all.

To present three pediatric cases of auditory brainstem implantation (ABI) and review literature data concerning this topic.The first two children had a neurofibromatosis type II with bilateral sensorineural deafness; in both cases, the implant was inserted during the surgical removal of a vestibular schwannoma; the third patient had profound deafness due to bilateral cochlear nerve insufficiency associated with inner ear malformation.Two postoperative complications were observed: patient 1 had a persistent fever which required the replacement of the fat graft used to seal the translabyrinthine approach; patient 3 had a CSF leakage requiring additional surgery and lumbar external drainage. In our three patients, the numbers of active electrodes were 6/22 (Cochlear ABI 24M ABI), 11/12 (Medel Opus II ABI) and 11/12 (implant Medel), respectively. Due to additional major surgical procedures and to disappointing functional results of the ABI, patient 1 stopped wearing her implant 18 months after implantation. Nine months after surgery, patient 2 achieved open-set speech recognition and was very satisfied with the implant. Six months after implantation, patient 3 (cochlear nerve deficiency), who was 3.5 years-old at the time, clearly reacted to some environmental sounds but was not yet able to achieve speech recognition.ABI has now entered the list of treatments that can be proposed in pediatric profound sensorineural deafness. Its major risks of complications are CSF leakage and non-auditory side effects. Its outcomes are worse and less predictable than cochlear implants. Thus, its indications must remain restricted to cases meeting the following conditions: absence of alternative option to restore hearing, patients and parents high level of motivation and realistic expectations.

Neurofibromatosis 2 (NF2) is a rare autosomal dominant disease whose hallmark is the development of bilateral vestibular schwannomas.Other features of NF2 include schwannomas, meningiomas, ependymomas, localized along the central nervous system, schwannomas of the peripheral nerves, cutaneous and ophthalmological manifestations. NF2 can be diagnosed in patients without bilateral vestibular schwannoma with sets of diagnostic criteria. Disease phenotype is variable among patients. Main negative prognostic factors are a young age at onset of symptoms and a high number of tumors at diagnosis. NF2 tumor suppressor gene encodes Merlin/Schwannomin, and is also involved in most sporadic schwannomas and meningiomas. Its functions remains largely unknown.Treatment and follow of NF2 patients up require oto-neurosurgical teams experienced in NF2. Yearly and life time surveillance is mandatory. A clinical screening protocol is suggested. Classically, only symptomatic lesions are to be treated. Some authors advocate an early proactive strategy against vestibular schwannoma in order to preserve hearing. When a treatment is advisable, surgery remains the treatment of choice for tumors. Auditory brainstem implant must be taken into account in hearing rehabilitation.

To expose the results obtained at short and middle terms and the tolerance of over pressure treatment with Meniett in Menière disease.A pressure treatment using Meniett device was initiated for 53 patients suffering of unilateral or bilateral active Menière's disease, despite medical treatment and physiotherapy.Retrospective analysis of the data demonstrate i) no complications due to the tympanostomy tube, ii) with a 4 months follow up after the treatment, a total control or reduction of vertigo in respectively 65% and 24.5% of the initially treated patients.The overpressure treatment with Meniett has been well tolerated. It can be repeated in case of recurrence of the symptoms. It is an interesting alternative for the treatment of Menière disease.

To evaluate results of cochlear implantation in patients with far-advanced otosclerosis.Sixteen patients with far-advanced otosclerosis had undergone unilateral (n = 13) or bilateral (n = 3) cochlear implantation. Surgical difficulties, incidence of complications and postoperative benefit were analyzed.A full electrode insertion was achieved in all patients without surgical difficulties. All patients demonstrated excellent benefit of cochlear implantation. Binaural implantation still improves speech performances, compared to unilateral implantation. In case of residual cochlear function of one nonoperated side, a stapes surgery, performed during the same surgical time as cochlear implantation, can improve speech scores and restore bilateral hearing. Facial nerve stimulation occurred only in 1 patient.Cochlear implantation is the method of choice for rehabilitation of patients with otosclerosis, presenting profound or total hearing loss. Patients obtain excellent benefit with a low rate of complications.

Skull base osteomyelitis is an uncommon but severe condition generally secondary to necrotizing otitis externa. The aim of this study was to determine the patients demographics, clinical and radiological findings, and outcomes of this condition.We prospectively follow 5 patients with a diagnosis of skull base osteomyelitis between 2004 and 2005. Clinical, biologic and imaging data were collected, and also follow-up during treatment.The five patients were men and the average age at presentation was 75 years (63-89). All except one had a previous diabetes mellitus, with a more or less recent clinical story of otitis externa. All of them suffered of headaches and cranial nerve(s) deficits, particularly facial nerve. Diagnosis was made on MRI with gadolinium injection and fat saturation, and Pseudomonas aeruginosa identified as pathogen in all cases. One patient deceased of cranial nerves palsy complication. In 4 cases we found previously a too short or non adapted treatment of necrotizing otitis externa.In the setting of headache, cranial nerves deficit and abnormal skull base imaging on MRI with Fat sat, osteomyelitis should be consider as the likely diagnosis. This complication should be avoided by optimal management of necrotizing otitis externa.

To investigate the surgical techniques and speech performance of multichannel auditory brainstem implant (ABI) in patients with bilateral acoustic neuromas (neurofibromatosis type 2).The nucleus 21 channel auditory brainstem implant was implanted into the lateral recess of the fourth ventricle through the translabyrinthine approach in 7 patients after removal of the tumor. The accurate placement of electrode array was ensured by the electromyogram monitoring of the 7th and 9th nerves and the electrically evoked auditory brainstem responses (EABR). Initial switch-on occurred six weeks postoperatively. Speech evaluation was performed every 3 months for the first year and annually thereafter.During the surgery, the lateral recess could be found and the typical EABR could be recorded in 6 cases. They later reported a significant benefit from the device. Two of the cases have achieved functional open-set speech understanding. In contrast, one patient with no EABR because of difficulty of the anatomic location during the surgery had no sensations postoperatively.The multichannel ABI could effectively restore auditory sensations in patients deafened by bilateral acoustic neuromas. The accurate location of the cochlear nucleus complex during surgery was the key factor for the success of the operation.

Otogenic pneumocephalus is uncommon. Typical causes include trauma, tumor, infection, and nasosinusal or mastoid surgery but spontaneous otogenic pneumocephalus is very exceptional. We report a case of spontaneous otogenic pneumocephalus located in the left temporal lobe revealed by sudden onset aphasia. The temporal pneumatocele was cured through an epidural subtemporal approach with needle puncture of the aeroma and duroplasty. Two years after surgery, no recurrence was observed and the patient remained symptom free.

The principal objectives of acoustic neuroma surgery in the order of priority are absence of mortality and neurological morbidity, total removal of the lesion, preservation of facial function, and preservation of hearing. The aim of this work was to evaluate the selection criteria and the therapeutic strategy for hearing preservation in acoustic neuroma management. This retrospective study included 436 consecutive patients referred to our department for an acoustic neuroma. The population comprised 365 patients (84%) treated surgically, 66 patients (15%) managed conservatively, and 5 irradiated patients (1%). The mean age was 54 years (range: 13-87). The mean follow-up period was 24 months (range: 1-120). The surgical approaches were translabyrinthine in 294 (81%), retrosigmoid in 37 (10%), and through the middle fossa in 34 cases (9%). Approaches preserving the labyrinth were employed in patients aged less than 60 years with lesions measuringor=2 cm and a serviceable hearing (pure tone average50 dB and speech discrimination scoreor=50%). A serviceable hearing was preserved in 53% of the cases with a serviceable hearing in 44% of the cases. Postoperative normal or subnormal facial function was obtained in 72 to 98% of cases depending on tumor size. Two cases (0.5%) of recurrence were reported. In conclusion, our surgical strategy based on age, tumor size and pre-operative hearing function permitted hearing preservation in 50% of selected cases, a high rate of facial function preservation and a low risk of recurrence.

Middle ear adenoma is an uncommon tumor. We report two cases and review the relevant literature concerning this rare entity.Two patients with a middle ear adenoma were operated on in our department. Pre- and postoperative data concerning clinical examination, audiometry, and radiology were obtained. Information concerning intraoperative observations and the pathological examination were also gathered.The diagnosis of middle ear adenomas is based on clinical, radiological, and pathological confrontation. The microscopic examination should be combined with histochemical and immuno-histochemical methods in order to evidence the glandular and neuroendocrin components of these lesions.Today, classified as a benign epithelial tumor, the middle ear adenoma is associated with an excellent prognosis provided total excision.

We report the case of a patient presenting a sudden bilateral hearing loss. Four years before, a bladder carcinoma was resected and a chemotherapy was started. Gadolinium-enhanced magnetic resonance images revealed enhancement of both acoustic nerves. Cerebrospinal fluid analysis showed malignant cells consistent with the initial bladder cancer. Meningeal metastases from bladder carcinoma are extremely rare. Systemic chemotherapy and its low meningeal diffusion may enhance the incidence of this complication. Bilateral hearing loss is a rare initial manifestation of meningeal carcinomatosis.

To analyze outcome after otosclerosis surgery with stamedeotomy with blood clot sealing.Otosclerosis surgery was performed in 150 adult patients between 1997 and 1999 by five surgical teams (70% of the procedures were performed by senior surgeons) and followed for 18 months. Stapedotomy was carried out under general anesthesia with an intrameatal approach in 96% of the cases. Stapedotomy (n=120, 80%) was performed with a drill in 141 cases and by laser in 9 (6%). Ninety percent of the Teflon prostheses had a 0.4 mm diameter and a 4.5 mm length. The footplate opening was sealed with blood clots. Venous interposition (n=30, 20%) was performed in the event of partial or total stapedectomy which occurred in spite of an initial stapedotomy attempt.The preoperative air-bone gap (ABG) was 32 +/- 10.3 dB. The gain in air conduction was 25 +/- 11.7 dB with 75% of the patients having more than 15 dB gain. The ABG was 10 +/- 5.4 dB with 73% of the patients having less than 5 dB gain. The interaural difference was 0.5 +/- 14.1 dB and the bone conduction (BC) variation was 1 +/- 7.5 dB. Functional failures were related to significant intralabyrinthine bleeding and revision procedure. The following factors had not effect on outcome: i) stapedotomy versus partial or total stapedectomy, footplate opening sealed by clots or vein, ii) diameter of the stapedotomy and/or the prosthesis, iii) surgical procedure performed by a junior surgeon.Sealing the stapedotomy opening with blood clots appears to provide reliable and reproducible functional outcome that remains stable over time. In this study, changing from partial to total stapedectomy with vein interposition did not modify the functional outcome.

The auditory brainstem implant (ABI) is designed to restore useful auditory sensations in patients with neurofibromatosis Type 2 (NF2). The implantation is usually performed at the time of tumor removal in patients who do not undergo radiation treatment. The authors evaluated the performance of ABIs in three patients with NF2 in whom vestibular schwannoma continued to grow after radiation treatment. These three patients with NF2 received a 21-channel ABI; a translabyrinthine approach was used for both the tumor removal and the ABI placement. The interval between radiosurgery and the tumor removal plus device implantation ranged from 2 to 11 years. In all cases, the tumor was growing and the patients presented with total deafness. The mean number of active electrodes in these three patients was equivalent to the average results reported in other patients who received ABIs. The patients in this study used the ABI regularly for everyday life and obtained useful levels of environmental sound recognition. It is concluded that hearing function can be rehabilitated using ABIs in patients with NF2, even if radiosurgery fails to control the tumor growth.

Three patients with neurofibromatosis type 2 who had undergone previous cochlear nerve tumour removal were implanted with the Nucleus 21-channel auditory brainstem implant (ABI). The time intervals between tumour removal and implantation were 4, 5, and 7 years, respectively. Total bilateral deafness was confirmed before implantation. One patient was also blind after acute intracranial hypertension. The translabyrinthine approach was used in all cases. The choice of side for implantation depended on pre-operative magnetic resonance imaging study, the facial nerve function, the presence of recurrent and/or other lesions, and the patient's preference. Although the scarring of prior surgical procedure largely changed the anatomical structures in the cerebello-pontine angle, various landmarks could be found to locate the foramen of Luschka, where the ABI electrode was inserted into the lateral recess of the fourth ventricle. During surgery, the electrically evoked auditory brainstem responses were recorded to confirm that the ABI stimulation activated the auditory system; the electromyogram of the 7th and 9th nerves was helpful in finding the landmarks and minimising the triggering of the cranial nerves with ABI stimulation. The number of active electrodes was 21, 7, and 4 in the three patients. All obtained meaningful pitch scaling and useful auditory sensations. One patient, with 21 activated electrodes, has achieved functional open-set speech understanding. The second patient, with 7 activated electrodes, has benefited from environmental sound awareness and improved lipreading. The last, blind, patient, with 4 electrodes activated, achieved only perception of environmental sounds.

To evaluate the decisional elements in the surgical strategy for extensive intratemporal cholesteatomas.A retrospective review of cases followed up between 1985 and 1996.Tertiary referral center.Nineteen patients with temporal bone cholesteatoma extending beyond the middle ear limits and surgically treated were included. Preoperative imaging distinguished apical (8), infralabyrinthine (3), supralabyrinthine (3), retrolabyrinthine (1), and translabyrinthine (4) cholesteatomas.Apical and supralabyrinthine lesions were treated through a middle fossa approach. Infralabyrinthine and translabyrinthine locations were exposed through a subtotal petrosectomy or a transotic route, depending on the preoperative audiovestibular status and labyrinthine destruction on computed tomography. The retrolabyrinthine lesion was approached through a retrolabyrinthine route.Patients were assessed for postoperative audiologic and facial functions and for recurrence of tumor.The facial nerve was neither rerouted nor interrupted during surgery. Among the 12 patients with preoperative facial palsy (FP), 5 cases of improvement (42%), 6 cases of stable function (50%), and 1 case of mild deterioration (8%) were observed postoperatively. In patients without preoperative FP, facial function remained unchanged postoperatively. The labyrinth could be preserved in three patients (16%), with postoperative stable hearing function in two (11%), and a 40-dB mean auditory deterioration in one (5%). Complete macroscopic resection was obtained in all patients. Two cases (11%) of postoperative recurrence were observed.The surgical strategy, principally based on cholesteatoma location and preoperative auditory function, yielded a high rate of local disease control and facial function preservation.

Papillary tumors of the temporal bone are rare and aggressive neoplasms. Recently described, these tumors had initially a presumed middle-ear origin. Only recently, convincing anatomic, morphological and immunohistochemical arguments exist for an endolymphatic sac origin (inner-ear origin). We report two cases of endolymphatic sac tumor. These tumors can be encountered sporadically or in Von Hippel-Lindau disease. They classically grow very slowly, resulting in late clinical manifestations with expansive mass invading temporal bone and extending in posterior fossa. Radiologically, these endolymphatic sac tumors can mimic metastatic carcinoma, paraganglioma, or cerebellar haemangioblastoma specially in von Hippel-Lindau disease. Histology shows a papillary epithelial tumor with hypervascular stroma, without atypia. The treatment for these tumors is surgical and curative when early diagnosed. In apparently sporadic cases, genetic analysis for Von Hippel-Lindau disease should be considered.

Between 1991 and 1996, 35 patients had an intracanalicular tumor: 26 patients were operated via middle cranial fossa approach (MFA: 23 cases) or translabyrinthine approach (TL: 3 cases), 9 cases were supervised by audiovestibular testing and MRI. For the operated patients through MFA the functional hearing (pure tone loss50 dB and 100% vocal discrimination) was preserved in 47.8% of cases, facial function was grade I and II in 83% of cases and grade III in 17% of cases. One recurrence was noted and reoperated by TL. For the TL operated patients, the postoperative facial function was normal (grade I) in all cases. Among the 9 supervised cases for reasons of age (more than 60 years), or the tumoral size (than 5 mm): the tumor in 3 cases did not increase in size, 5 cases increased by 1 mm/year and 1 case increased 1 cm/year (and must be operated). These results show the importance of the middle cranial fossa approach for excision of intracanalicular neuromas and supervising patients with the same tumor according to age, and tumoral size.

In this report of a retrospective study of 144 cases of idiopathic sudden hearing loss, two important prognostic indicators appear: the elapsed time since the onset of symptoms and the degree of hearing loss. According to these indicators, the authors define three group of patients with a recovery rate of 65%: patients with a threshold hearing level of less than 40 dB, treated within two months of the onset of their hearing loss, patients with a threshold hearing level between 40 and 70 dB treated within one month and patients with a threshold hearing level of more than 70 dB treated within 7 days. Patients with a threshold hearing level of more than 70 dB, treated between 7 and 30 days, have only a partial recovery in 40%. The evaluation of efficacy of various treatment demonstrates a slight improvement of the hearing loss using steroids associated to vasodilatators, even if no significant difference is found between the different therapies. The normovolemic hemodilution and the treatment included mannitol seem to be ineffectual.

Intralabyrinthine schwannomas are rare tumors of vestibule, cochlea, semicircular canals, or some combination of these three. In the past, they have been found at autopsy or as incidental finding at surgery. Since the advent of magnetic resonance imaging (MRI) with intravenous gadolinium contrast, the preoperative diagnosis is possible. We report two cases of intralabyrinthine schwannoma: one case of intralabyrinthine schwannoma extended into the internal auditory canal (IAC), a second case of tumor restricted to the vestibule. No characteristic clinical presentation of this tumor is reported in the literature. In patients with vestibular weakness (vertigo and no response of caloric testing), MRI with gadolinium contrast can make the diagnosis of intravestibular tumor. In patients with the more common IAC acoustic neuromas, MRI can demonstrate extension of tumor into the labyrinth; diagnosing this extension preoperatively is important to plan surgical treatment.

This study aimed to assess the results of hearing restoration with a cochlear or a brainstem implant in posterior fossa tumors.Six patients were selected. Two patients with an acoustic neuroma in the only-hearing ear (cases 1 and 2), one patient with a posterior fossa meningioma (case 3), one patient with bilateral facial neuroma (case 4), and two patients with bilateral acoustic neuroma (cases 5 and 6) participated.In cases 1 and 2, the patients had a cochlear implant inserted on the only-hearing ear opposite the acoustic neuroma. In case 3, the patient presented with total deafness on the left side and a 10-mm meningioma on the right side. A cochlear implantation was performed after removal of the meningioma on the right side. In case 4, the patient was operated on on both sides with bilateral postoperative deafness. A cochlear implantation was performed on the better hearing ear. In cases 5 and 6, patients underwent an auditory brainstem implantation after the exeresis of the second tumor.Promontory test results were positive for patients 1, 2, 3, and 4. After implantation, patients 1, 2, 3, and 4 scored 98%, 13%, 70%, and 30%, respectively, in open-set sentence recognition tests, whereas patients 5 and 6 scored 0% and 20%, respectively.In case of nonfunctional cochlear nerve, in acoustic neuroma, either bilateral and in the only-hearing ear, promontory test should be performed. If positive results, a cochlear implantation should be performed, because successful results could be expected. Overall results of cochlear implantation on speech discrimination are better than those obtained with a brainstem implant.

The results of facial nerve surgery in the temporal bone have been evaluated in a review of 34 cases (19 decompressions and 15 reparations). Decompressions of immediate and complete facial paralysis by fracture of the temporal bone that have achieved a good motor recovery resulted from early surgery before the 21st day (50% of grades I and II, 50% of grades III). After this delay, facial recovery did not reach grades I and II any longer. In case of nervous interruption of traumatic origin or after tumoral ablation, direct restoration of continuity by an intermediary grafting fixed with fibrin glue enabled the recovery of a facial function of grade III in 87.5% of the cases. These results point out the importance of an early exploration of traumatic facial paralysis before the end of the third week, and the interest of using a cable graft fixed with fibrin glue to repair directly the facial nerve.

Between 1987 and february 1994, 162 consecutive patients with acoustic neuroma were operated on by an otoneurosurgery team, using transpetrous approaches (89% translabyrinthine, 8% middle fossa and 3% retrosigmoid). The relationship between the clinical, audiometric and vestibulographic characteristics and the post-operative facial nerve function were evaluated. In acoustic neuromas with cerebello-pontine component inferior to 3 cm without central neurologic signs (ic: central controlateral auditory and/or ipsilateral vestibular pathway alteration), good post-operative facial nerve function was achieved in 80% of cases. In acoustic neuromas superior to 3 cm with alteration of the central vestibular and auditory pathways, a good result was obtained in only 30% of cases which correlated negatively with preoperative facial dysfunction. These results underline the value of preoperative facial and audiovestibular examinations in predicting the postoperative facial nerve function following surgery for acoustic neuroma.

We performed a retrospective review of the audiologic, radiologic and clinical data on 239 patients operated on for acoustic neuroma from 1987 to 1995 at Beaujon Hospital. In 16 patients or 6.6% of the total group, we identified as having experienced sudden hearing loss by history. The 16 tumors involved were 1 stade I; 8 stade II, 5 stade III and 2 stade IV. In 14 cases, the tumor was removed by translabyrinthine approach, 1 case by masto-retrosigmoid approach and 1 case by middle fossa approach. The hearing was preserved in the two latter cases. The purpose of this study is to illustrate and reaffirm the importance of a thorough neurotologic evaluation and to discuss the diagnostic methods in this select patient population.

Twenty five cases of malignant tumors of the petrous bone are presented. Prognosis depends on the histology and on the local invasion which dictate the possibility of curative surgery. Squamous cell carcinoma of the middle ear and other invasive tumors have a bad prognosis, and in spite of the progress of modern image technology, the peripetrous extension of the tumors are often underestimated. Actuarial two years survival for squamous cell carcinoma was 38%. Total excision needs at least a subtotal petrous resection with, in some cases meningal and internal carotid resection. In all cases, surgery was followed by radiation. Low grade malignant tumors had a better prognosis and required a more limited resection. In these cases, when necessary, facial nerve can be repaired by a cable graft at the time of initial surgery.

The position of the jugular bulb is extremely variable. A high jugular fossa with a diverticulum of the jugular bulb can alter the inner ear function with sensorineural hearing loss, vertigo and tinnitus. Nine cases of jugular bulb diverticulum with vertigo mimicking Menière's disease were operated on and followed up from 3 months to 4 years. Eight patients were treated surgically. The jugular bulb was approached through a mastoïdectomy and the diverticulum was compressed downwards using bone wax. The vertigo disappeared after surgery in all cases. These observations suggest that an abnormally of the jugular bulb should be considered as a possible symptom of Ménière's disease and that vertigo can be cured by downward compression of the diverticulum.

The position of the jugular bulb is extremely variable. A high jugular fossa with a diverticulum of the jugular bulb can alter the inner ear function with neurosensory hearing loss, vertigo and tinnitus. Six cases of jugular bulb diverticulum with vertigo mimicking Meniere's disease were operated on and followed up from 6 months to 4 years. The jugular bulb was approached through a mastoidectomy and the diverticulum was decompressed downward using bone wax. The vertigo disappeared after surgery in all the cases. These observations suggest that an abnormality of the jugular bulb should be considered as a possible origin of Meniere's disease and that vertigo can be cured by downward decompression of the diverticulum.

When confronted with complete chemotherapy-induced histological/clinical regression of a tumor, should the initial therapeutical strategy be modified? Such situations occur more and more frequently. Additional treatment must be carried out in all instances. Indeed, a correlation between complete clinical and histological regression was demonstrated in 66% of cases by histological examination of surgical specimens. It is doubtless recommended to avoid mutilating surgery and to continue chemotherapy for another year. However, whenever partial surgery had been programmed initially, the same indications and excision limits should be maintained. Combination chemical and surgical treatments yield better local results. Nonetheless, this is not always the case and the patient's tolerance to chemotherapy and background should influence the final decision. Radiotherapy then constitutes a choice alternative.

The Chinese flap has already been recommended in the context of circular pharyngolaryngectomy. The authors studied its vascularization from an anatomy-focusing project carried out on 40 dissection cases. They broaden the applications of this material to posterior reconstructive pharyngectomy, for which they specify the methods and routes of access. Other applications are proposed: this flap may represent an alternative for reconstitution of the oral cavity, or even as a second-intent surgical procedure where other technics have failed.