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Biomedical subjects

D Bouccara

Publications and source records attributed to D Bouccara.

At least 37 records · Page 2Linked to original sources

Surgical management of intratemporal lesions.

In order to evaluate the decisional elements in the surgical strategy of deep-seated and/or extensive intratemporal lesions, a retrospective review of cases followed up between 1985 and 1996 in our department was undertaken. Eighty-one adult patients presenting temporal bone lesions located or extending beyond the middle ear limits excluding vestibular schwannomas and surgically treated were included. The population comprised 38 men and 43 women (mean age: 43 years, range: 17-81). Pre-, intra- and postoperative data were collected from medical files. The principal factors influencing the choice of the surgical approach were the location of the lesion and its presumed aggressiveness, the tumour involvement of the internal carotid artery and the labyrinth on preoperative imaging, and the preoperative hearing loss. A coherent algorithm based on these factors can be proposed for the surgical management of intratemporal lesions. High quality preoperative imaging is mandatory for the surgical planning.

Adolescent↗

Hypertrophic neuropathy of the facial nerve.

Hypertrophic neuropathy is a peripheral nerve lesion that is histologically characterized by onion bulb formations around axons. This histologic picture, which is usually seen in generalized hypertrophic neuropathies, can occasionally be observed in single nerves as localized hypertrophic neuropathy. Cranial involvement of such localized hypertrophic neuropathy represents a very rare entity; only a few cases have been reported in the literature. We report the history of a progressive facial paralysis with a tumorous enlargement of the seventh cranial nerve that was clinically suspected of being a schwannoma. Pathological examination permitted the diagnosis of hypertrophic neuropathy.

Adult↗

Extraaxial primitive neuroectodermal tumor mimicking a vestibular schwannoma: diagnostic and therapeutic difficulties. Report of two cases.

Extraaxial cerebellopontine angle (CPA) medulloblastomas and other primitive neuroectodermal tumors (PNETs) are rare tumors. The authors report on two patients with PNETs who presented with progressive audiovestibular symptoms. In each case magnetic resonance (MR) imaging revealed an extraaxial lesion that filled the internal auditory meatus and exhibited the neuroimaging features of a vestibular schwannoma (VS). No high signal intensity was apparent in either the brainstem or adjacent cerebellum on T2-weighted MR images. Surgery with maximum resection (total in one case and subtotal in the other) was performed, followed by craniospinal radiotherapy. One year postoperatively, both patients were free from tumor. A CPA PNET mimicking a VS is a rare entity, the diagnosis of which is important because its treatment differs dramatically from that of VS, including prescribed surgery followed by conventional craniospinal radiotherapy.

Adult↗

Hearing restoration with auditory brainstem implants after radiosurgery for neurofibromatosis type 2.

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.

Adolescent↗

[Small acoustic neuroma: observation or treatment? A review of 207 cases].

The management of small acoustic neuromas (AN) whether localized in an intracanalar position (stage I) or with a small extension of less then 2 cm into the cerebellopontine angle (stage II) remains under debate. Proposed strategies include surgery, stereotactic irradiation and observation. From 1987 to 1997, among 343 AN referred to our department, 207 were small (83 stage I and 124 stage II). Initially, 72 patients were treated conservatively mainly because of their age (over 60-65) and 132 were operated on. Three patients underwent irradiation because of their poor general condition. Significant tumor growth was observed in 15 cases in the conservative treatment group; 14 of these patients underwent secondary surgery and one irradiation. Among the 146 AN operated on (132 initially and 14 secondarily), 142 small AN were operated on via a transpetrosal approach (64% translabyrinthine, 21% middle-fossa and 15% retrosigmoid) and 4 AN, which became large tumors during the observation period, were treated through the translabyrinthine approach. No mortality was observed in our series. Postoperative complications included 11 CSF leakages necessitating reoperation (8%). In 93% of the cases, postoperative facial function at one year was good. Hearing preservation was attempted in 51 selected cases (pure tone average=50 dB, speech discrimination score 100%) with a 51% success rate (53% and 48% through middle-fossa and retrosigmoid approaches respectively). In our opinion, surgery with this risk-benefit ratio is indicated for small AN, except in the elderly for whom conservative management is preferred and in patients in poor clinical condition with a growing AN, for whom irradiation is recommended.

Adult↗

Benefits of cochlear implantation in elderly patients.

Cochlear implantation in elderly patients is a questionable subject. The purpose of this study was to evaluate the procedure and its outcome, the postoperative course, and the audiologic and social benefits of cochlear implantation in this population. Twenty-seven patients older than 60 years were compared with a control group of 15 adult patients. This retrospective study analyzed data concerning the outcome of the procedure, postoperative course, postoperative orthophonic test results, and answers of a questionnaire assessing the changes in communication, perception, and social outcomes. The procedure was uneventful in both groups. Minor complications were not more frequent in elderly patients. Orthophonic test results were comparable in both groups. At 12 months, 83% of the elderly patients had an open-set speech discrimination score above 60%. The benefits of cochlear implantation in terms of the quality of life are not statistically different with younger patients. Cost-utility analysis might support these findings.

Adult↗

[Endolymphatic sac tumor: a rare tumor of internal ear. Report of two cases].

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.

Adult↗

Extensive intratemporal cholesteatoma: surgical strategy.

OBJECTIVE: To evaluate the decisional elements in the surgical strategy for extensive intratemporal cholesteatomas. STUDY DESIGN: A retrospective review of cases followed up between 1985 and 1996. SETTING: Tertiary referral center. PATIENTS: 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. INTERVENTION: 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. MAIN OUTCOME MEASURES: Patients were assessed for postoperative audiologic and facial functions and for recurrence of tumor. RESULTS: 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. CONCLUSION: The surgical strategy, principally based on cholesteatoma location and preoperative auditory function, yielded a high rate of local disease control and facial function preservation.

Adolescent↗

[Clinical application of the multichannel auditory brainstem implant].

OBJECTIVE: To investigate the surgical techniques and speech performance of multichannel auditory brainstem implant (ABI) in patients with bilateral acoustic neuromas (neurofibromatosis type 2). METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Adolescent↗

[Autoimmune deafness].

UNLABELLED: A REAL ENTITY: Deafness resulting from an autoimmune mechanism is suggested by a growing number of clinical and experimental arguments. The audio-vestibular system is known to be involved in a certain number of systemic diseases, particularly Cogan's disease. In other cases, the inner ear alone is involved; deafness may be the first manifestation of a systemic disease or result from a possible immunological mechanism. CLINICAL ASPECTS: Autoimmune deafness is very invalidating. Bilateral perception deafness is observed in 80% of the cases and vestibular involvement is found in 70% DIAGNOSIS: No one simple reliable test is known which can establish the diagnosis of autoimmune deafness. Other causes must be ruled out by appropriate clinical and complementary explorations. For humoral immunity, the western blot method has given promising results suggesting a possible role of the heat shock protein in the underlying immunological mechanism. TREATMENT: Immediate care is needed but no standard treatment has been defined. High-dose corticosteroids can provide symptom relief, particularly in case of abnormal immunological tests. The role of immunosuppressive therapy, sometimes proposed in case of corticosteroid resistance, remains to be defined.

Adrenal Cortex Hormones↗

Surgical treatment of the high jugular bulb in patients with Ménière's disease and pulsatile tinnitus.

The aim of this retrospective study was to evaluate the functional results of surgical lowering of the high jugular bulb in the treatment of patients with Ménière's disease and pulsatile tinnitus. Fifteen patients with disabling Ménière's disease associated with pulsatile tinnitus and a high and medial jugular bulb were included in this study. As treatment a complete mastoidectomy was performed, after which the jugular bulb was freed by an infralabyrinthine and subfacial approach. The bulb was then displaced downwards with surgical wax. Functional results of surgery were assessed by a questionnaire according to the 1995 guidelines of the United States American Academy Committee on Hearing and Equilibrium, audiometric and vestibular tests, and by magnetic resonance and computed tomographic imaging with vascular sequences. Surgical treatment was contraindicated in two cases: one had hypoplasia of the contralateral sigmoid sinus and the other a small petrous hemangioma located around the jugular bulb that was discovered peroperatively. Among the 13 patients treated by definitive surgery, attacks of vertigo were reported as disabling in 12 cases preoperatively (92%) versus 1 (8%) after surgical treatment. No significant change in hearing was observed after surgery. Tinnitus had been reported in all patients preoperatively and decreased in intensity in four (31%) and disappeared in three (23%) after surgery.

Adult↗

[Imaging of the cerebello-pontine angle and skull base].

Lesions of the internal auditory meatus, cerebello-pontine angle and skull base have benefited greatly from the progress made in imaging techniques. There are now three methods of examination at our disposal. MRI scan is the primary method of choice, and includes T1 spin echo sequences without and with the injection of contrast, and the T2-weighted sequence (with spin echo or gradient echo CISS-3DFT); other sequences, such as angio-MRI and FLAIR, are used more rarely. CTscanning is complementary to MRI, but gives a better definition of bony structures. Arteriography is more rarely indicated. Imaging usually allows a diagnosis of the type of lesion encountered. The typical features on MRI and CT scan of the various pathological lesions of the internal auditory meatus, cerebello-pontine angle and skull base are discussed, together with the fact that by far the commonest among them is the vestibular neuroma.

Cerebellopontine Angle↗

Hearing restoration in posterior fossa tumors.

OBJECTIVE: This study aimed to assess the results of hearing restoration with a cochlear or a brainstem implant in posterior fossa tumors. PATIENTS: 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. INTERVENTION: 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. RESULTS: 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. CONCLUSIONS: 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.

Adolescent↗