Safety and effectiveness of the Bonebridge transcutaneous active direct-drive bone-conduction hearing implant at 1-year device use.

The objective of this study is to evaluate the safety and efficacy of a new transcutaneous bone-conduction implant (BCI BB) in patients with conductive and mixed hearing loss or with single-sided deafness (SSD), 1 year after surgical implantation. The study design is multicentric prospective, intra-subject measurements. Each subject is his/her own control. The setting is nine university hospitals: 7 French and 2 Belgian. Sixteen subjects with conductive or mixed hearing loss with bone-conduction hearing thresholds under the upper limit of 45 dB HL for each frequency from 500 to 4000 Hz, and 12 subjects with SSD (contralateral hearing within normal range) were enrolled in the study. All subjects were older than 18 years. The intervention is rehabilitative. The main outcome measure is the evaluation of skin safety, audiological measurements, benefit, and satisfaction questionnaires with a 1-year follow up. Skin safety was rated as good or very good. For the mixed or conductive hearing loss groups, the average functional gain (at 500 Hz, 1, 2, 4 kHz) was 26.1 dB HL (SD 13.7), and mean percentage of speech recognition in quiet at 65 dB was 95 % (vs 74 % unaided). In 5/6 SSD subjects, values of SRT in noise were lower with BB. Questionnaires revealed patient benefit and satisfaction. The transcutaneous BCI is very well tolerated at 1-year follow up, improves audiometric thresholds and intelligibility for speech in quiet and noise, and gives satisfaction to both patients with mixed and conductive hearing loss and patients with SSD.

Quality of life in bimodal hearing users (unilateral cochlear implants and contralateral hearing aids).

The main objective was to evaluate the bimodal self-rated benefits on auditory performance under real conditions and the quality of life in two groups of cochlear-implanted adults, with or without a contralateral hearing aid. The secondary objective was to investigate correlations between the use of a hearing aid and residual hearing on the non-implanted ear. This retrospective study was realized between 2000 and 2010 in two referral centers. A population of 183 postlingually deaf adults, implanted with a cochlear experience superior to 6 months, was selected. The Speech, Spatial, and other Qualities of Hearing Scale were administered to evaluate the auditory performances, and the Nijmegen Cochlear Implant Questionnaire to evaluate the quality of life. The population was divided into two groups: a group with unilateral cochlear implants (Cochlear Implant-alone, n = 54), and a bimodal group with a cochlear implant and a contralateral hearing aid (n = 62). Both groups were similar in terms of auditory deprivation duration, duration of cochlear implant use, and pure-tone average on the implanted ear. There was a significant difference in terms of pure-tone average on low and low-to-mid frequencies on the non-implanted ear. The scores on both questionnaires showed an improvement in the basic sound perception and quality of social activities for the bimodal group. The results suggest that the bimodal stimulation (cochlear implant and contralateral hearing aid) improved auditory perception in quiet and the quality of life domain of social activities.

[Comparison of stapes protheses: A prospective analysis of audiometric results obtained after stapedotomy by implantation of a teflon piston and a Soft-CliP® piston].

A wide variety of pistons are available for stapes surgery in otosclerosis. The objective of this work was to analyze the short-term and medium-term results of Soft-CliP® piston in prima­ry stapes surgery and to compare it with a conventional prosthesis. The study was prospective, monocentric, rando­mi­zed, based on efficiency of Soft-CliP® prosthesis (11 patients) versus Causse® Teflon prosthesis (9 patients) in otosclerosis surgery. Epidemiological data, treatment modalities, pure-tone audiometric results and outcome of patients were studied. Mean follow-up was 10 months. Soft-CliP® placement was statisti­cally longer. The mean postoperative air-bone gap was within 10 dB in 73% and 67% of cases at 2 months and in 82% and 89% of cases at medium-term in Soft-CliP® and Teflon groups respectively. A statistically significant decrease in mean air-bone gap and for all frequencies was observed at 2 months and at medium term, with no difference between the 2 groups. The main result was the demonstration of the reliability and safety of Soft-CliP® prosthesis. With a new design, Soft-CliP® place­ment was easy. The biomechanics of ossicular chain should be better preserved, and the risk of incus erosion and necrosis decreased. A study on a larger population in the long-term would validate these results.

[Middle ear barotraumas due to rhinopharyngeal scar tissue: tubomanometry diagnostic and therapeutic contribution]. / Barotraumatismes de l'oreille moyenne par brides rhinopharyngées: intérêt diagnostique et thérapeutique de la tubomanométrie.

OBJECTIVE: Evaluation of tubomanometry contribution to diagnosis middle ear barotraumas in relation with rhinopharyngeal scar tissue, and contribution to check postoperative effectiveness of scar tissue surgical resection. METHODS: Clinical cases study of two stewardesses who have lost their flight fitness consecutively to barotraumatic otitis during landing, engendered by rhinopharyngeal scar tissue. RESULTS: Pre-operative tubomanometric parameters were abnormal in both cases: decrease of intratympanic pressure in one case, variability of tube opening latency index and lengthening of intratympanic pressure rising time in the other case. Surgical section during endonasal endoscopy results in initially abnormal tubomanometric parameters normalization, allowing resumption of flight fitness without any barotrauma. CONCLUSION: Some abnormal tubomanometric parameters help to establish causality link between middle ear barotrauma and rhinopharyngeal scar tissue for which surgical section is thus indicated. Post-operative tubomanometric parameters normalization prove surgical effectiveness. Then, flight fitness could then be restored.

Transoral surgical treatment of Eagle's syndrome: case report and review of literature.

Eagle's syndrome is a symptomatic abnormal length of the styloid process and/or a calcification of the stylohyoid ligament. Diagnosis is based on common pharyngeal symptoms and is confirmed by radiologic examination, more particularly CT-scan. Medical and surgical treatments are described. Medical treatment consists in repeated local anaesthetics, steroid infiltration or analgesics administration. It is purely symptomatic and has no long-term effectiveness. Surgical treatment remains the treatment of choice and consists of removal of the abnormal process. Both transoral and external approaches have been described. The advantages of the transoral approach is a shorter operative time, the absence of aesthetic prejudice and a lesser risk of neurovascular injury. Few post operative complications have been described (surgical cervical emphysema, cervical swelling, trismus and moderate breathing difficulty) but none of the studies reported neurovascular injury, deep cervical infection nor long-term complication. We present the technique, a case report and a review of the literature of the transoral approach.

Comparison of [¹¹¹In]pentetreotide-SPECT and [¹8F]FDOPA-PET in the localization of extra-adrenal paragangliomas: the case for a patient-tailored use of nuclear imaging modalities.

AIMS AND METHODS: The aim of this prospective study was to compare the diagnostic value of [¹8F]FDOPA-PET and [¹¹¹In]pentetreotide-SPECT somatostatin receptor scintigraphy (SRS) in patients with nonmetastatic extra-adrenal paragangliomas (PGLs). Twenty-five consecutive unrelated patients who were known or suspected of having nonmetastatic extra-adrenal PGLs were prospectively evaluated with SRS and [¹8F]FDOPA-PET. ¹³¹I-MIBG and [¹8F]FDG-PET were added to the work-up in patients with a personal or familial history of PGL, predisposing mutations, abdominal PGLs, metanephrine hypersecretion and abdominal foci on SRS and/or [¹8F]FDOPA-PET. RESULTS: SRS correctly detected 23/45 lesions of which 20 were head or neck lesions (H&N) and 3 were abdominal lesions. [¹8F]FDOPA-PET detected significantly more lesions than SRS (39/45, P < 0·001). Both SRS and ¹8F-DOPA-PET detected significantly more H&N than abdominal lesions (66·7% vs 20%, P = 0·003 and 96·7% vs 67%, P = 0·012, respectively). In two patients with the succinate dehydrogenase D (SDHD) mutation, [¹8F]FDOPA-PET missed five abdominal PGLs which were detected by the combination of SRS, [¹³¹I]MIBG and [¹8F]FDG-PET. A lesion-based analysis using a forward stepwise logistic regression model demonstrates that size ≤ 10 mm (P = 0·002) and abdominal lesions (P = 0·031) were independently associated with "[¹8F]FDOPA-PET diagnosis only". In turn, a previous history of surgery and/or the presence of germline mutation was associated with lower lesion size (P = 0·001). CONCLUSIONS: The sensitivity of SRS for localizing parasympathetic PGLs is lower than originally reported, and [¹8F]FDOPA-PET is better than SRS for localizing small lesions. SRS should be replaced by [¹8F]FDOPA-PET as the first-line imaging procedure in H&N PGL, especially in patients at risk of multifocal disease (predisposing mutations and or previous history of surgery).

The presigmoid retrolabyrinthine approach: Technical note.

BACKGROUND: The retrolabyrinthine approach is classified among the posterior petrosectomies. Its goal is to achieve an enlarged mastoidectomy while sparing the intrapetrous neurotologic structures in order to offer maximal exposure of the posterior cerebellopontine angle compound. METHODS: The stages of the procedure are subsequently the skeletonization of the sigmoid sinus, wide opening of the mastoid antrum and exposure of the semicircular canals. We present herein the technique, indications and limitations of the retrolabyrinthine approach. CONCLUSION: The retrolabyrinthine approach is a demanding technique. Nowadays the retrolabyrinthine approach is routinely combined to additional resections of the petrous bone, so-called "combined petrosectomies", to target the jugular foramen or the petroclival area.

Consensus statement. Corticosteroid therapy in ENT in the context of the COVID-19 pandemic.

This consensus statement about the indications and modalities of corticosteroid treatment in the context of the COVID-19 pandemic was jointly written by experts from the French Association of Otology and Oto-Neurology (AFON) and from the French Society of Otorhinolaryngology, Head and Neck Surgery (SFORL). There is currently not enough data in favour of danger or benefit from corticosteroids in COVID-19, so until this matter is resolved it is advisable to limit their indications to the most serious clinical pictures for which it is well established that this type of treatment has a positive impact on the progression of symptoms. In Grade V and VI Bell's palsy according to the House-Brackmann grading system, a week's course of oral corticosteroids is recommended. Corticosteroid therapy is also recommended in cases of sudden hearing loss of more than 60dB, either in the form of intratympanic injections or a week's course of oral medication. In rhinology, there is no indication for systemic corticosteroid therapy in the current situation. However, patients are advised to continue with their local corticosteroid therapy in the form of a nasal spray or by inhalation. Treatments with corticosteroid nasal sprays can still be prescribed if there is no alternative. Finally, systemic or local corticosteroid therapy is not indicated for bacterial ENT infections.

Improved Assessment of Middle Ear Recurrent Cholesteatomas Using a Fusion of Conventional CT and Non-EPI-DWI MRI.

BACKGROUND AND PURPOSE: Recurrent middle ear cholesteatomas are commonly preoperatively assessed using MR imaging (non-EPI-DWI) and CT. Both modalities are used with the aim of distinguishing scar tissue from cholesteatoma and determining the extent of bone erosions. Inflammation and scar tissue associated with the lesions might hamper a proper delineation of the corresponding extensions on CT images. Using surgical findings as the criterion standard, we assessed the recurrent middle ear cholesteatoma extent using either uncoregistered or fused CT-MR imaging datasets and determined the corresponding accuracy and repeatability. MATERIALS AND METHODS: Twenty consecutive patients with suspected recurrent middle ear cholesteatoma and preoperative CT-MR imaging datasets were prospectively included. A double-blind assessment and coregistration of the recurrent middle ear cholesteatoma extent and manual delineation of 18 presumed recurrent middle ear cholesteatomas were performed by 2 radiologists and compared with the criterion standard. "Reliability score" was defined to qualify radiologists' confidence. For each volume, segmentation repeatability was assessed on the basis of intraclass correlation coefficient and overlap indices. RESULTS: For the whole set of patients, recurrent middle ear cholesteatoma was further supported by surgical results. Two lesions were excluded from the analysis, given that MR imaging did not show a restricted diffusion. Lesions were accurately localized using the fused datasets, whereas significantly fewer lesions (85%) were correctly localized using uncoregistered images. Reliability scores were larger for fused datasets. Segmentation repeatability showed an almost perfect intraclass correlation coefficient regarding volumes, while overlaps were significantly lower in uncoregistered (52%) compared with fused (60%, P < .001) datasets. CONCLUSIONS: The use of coregistered CT-MR images significantly improved the assessment of recurrent middle ear cholesteatoma with a greater accuracy and better reliability and repeatability.

[Paraganglioma of the cerebellopontine angle: report of two cases]. / Paragangliomes de l'angle ponto-cérébelleux: à propos de deux cas.

Paragangliomas of the cerebellopontine angle are exceptional tumours. We report two cases of paragangliomas of the cerebellopontine angle. To our knowledge, including these two cases, only five cases have been reported in the international literature. The emerging field of genetic study of these tumours will prove critical for their diagnosis and prognosis. The presence of paraganglioma in this area is explained by an abnormal embryologic migration of paraganglionic cells into the cerebellopontine angle, associated with tumorogenesis. These tumors can be associated with others cervico-facial tumours and to genomic abnormalities. The preoperative diagnosis of these tumours is very difficult, with clinical signs like hearing loss, vertigo and tinnitus are very ambiguous. Radiologic assessment of the cerebellopontine angle is also vague. The final diagnosis is only made possible peroperatively and by the post-operative histopathologic study. ENT and neurosurgeons should be awarded of the possible existence of paragangliomas of the cerebellopontine angle, and the necessity of a systemic and genetic assessment.

Trigeminal neuralgia (TN): A descriptive literature analysis on the diagnosis and management modalities.

OBJECTIVE: The primary objective of this review is to explore the different aspects of the diagnosis and management of Trigeminal neuralgia (TN). We look at the role of radiological imaging in the work-up of this condition, and based on the findings in the literature, we report data on the medical and surgical management of TN. MATERIALS AND METHODS: A literature review was conducted using PubMed and Cochrane search engines in order to explore the data available on the diagnosis and management of TN. Clinical features and various treatment modalities were analyzed by the authors. The identified studies were evaluated and data was reported on the different aspects of the condition in order to provide an evidence-based update on the topic. DISCUSSION: The diagnosis of TN is based on the patient's clinical history and radiological imaging. The commonest cause of TN is a micro-vascular compression by a looping blood vessel. Radiological evaluation is critical in the work-up of the disorder and in order to eliminate other possible causes. Management of the disorder can be medical or surgical, with micro-vascular decompression having the highest remission rate. CONCLUSION: Patients with TN present paroxysmal pain attacks in the territories innervated by the trigeminal nerve. Diagnostic investigations must allow precise anatomical evaluation of the CPA, and MR imaging is the gold-standard radiological investigation for this purpose. Management of TN can be medical or surgical, with micro-vascular decompression having the highest success rate.

Conservative treatment of vestibular schwannoma: growth and Penn Acoustic Neuroma Quality of Life scale in French language.

The aim of this study was to determine the natural history of growth and quality of life (QoL) outcomes for vestibular schwannoma (VS) managed conservatively, and to validate the disease-specific Penn Acoustic Neuroma Quality-of-Life (PANQOL) scale in French language. We retrospectively studied 26 patients with VS managed conservatively. Patient characteristics and radiological findings were collected. Two scales were used to measure QoL: the Short Form-36 Health Survey (SF-36) and the PANQOL scale translated into French. Internal consistency and scores were compared with previous studies. The mean follow-up was 25 months (range 6-72). We observed tumour growth in 14 patients (53.8%), no growth in 12 patients (46.2%) and no case of tumour shrinkage. The mean tumour growth was 2.22 mm/year. No predictive factor of growth was found. Patients with vertigo or dizziness experienced a poorer QoL according to the SF-36 (Social Functioning and Emotional Role Limitation dimensions) and to the PANQOL scale (Balance and Energy dimensions). Our results were comparable with the literature using the SF-36. With the PANQOL scale, our scores were not statistically different with those from Dutch and North American studies except in the field of hearing (p = 0.019). Quality of life becomes essential in the management of VS. According to these results, we support a non-conservative strategy associated with vestibular rehabilitation for patients with dizziness or vertigo. The PANQOL is a validated specific scale for VS, which can be useful in French.

[Middle ear cholesteatoma]. / Cholestéatome de l'oreille moyenne.

Cholesteatoma is a serious form of chronic otitis media. The aim of this paper is to present the state of the art of disease management, including recent data from the literature and the authors' derived from their Mentors' teaching, Professor Pierre Roulleau (Paris, France) and Professor Robert Charachon (Grenoble, France). The main recent advances concern the use of cartilage grafts to reconstruct the canal wall and/or tympanic membrane (allowing a significant reduction in residual cholesteatoma) and progress in medical imaging allowing more acute preoperative determination of extension of the cholesteatoma (in order to propose an optimally designed surgical technique) and a less invasive postoperative follow-up.

Long-term surgical results in microvascular decompression for hemifacial spasm: efficacy, morbidity and quality of life.

Hemifacial spasm is a condition that may severely reduce patients' quality of life. Microvascular decompression is the neurosurgical treatment of choice. The objective of this work was to describe the efficacy and morbidity of microvascular decompression for hemifacial spasm, evaluate the long-term efficacy on the quality of life and investigate prognostic factors for failure of the procedure. A retrospective study of 446 cases of hemifacial spasm treated by 511 retrosigmoid microvascular decompression over 22 years was conducted. Epidemiological, clinical and imaging findings, treatment modalities and outcomes of patients with pre- and postoperative HSF-8 quality of life questionnaire were studied. Success rate was 82% after first surgery and 91.6% after revision surgery. A low rate of perioperative morbidity was found. Facial palsy was mostly transient (5.5% transient and 0.2% permanent) and cochleovestibular deficit was seen in 4.8% of patients. Revision surgery increased nervous lesions (10.6% to 20.7%). Mean quality of life scores were significantly improved from 18 to 2 over 32, evaluated 7.3 years after surgery. Predictive factors of surgical failure were single conflicts (p = 0.041), atypical vasculo-nervous conflicts involving other vessel than postero-inferior cerebellar artery (p = 0.036), such as vein (p = 0.045), and other compression sites than root exit zone (p = 0.027). Retrosigmoid microvascular decompression is a safe and effective treatment of hemifacial spasm. Revision surgery is not to be excluded in case of failure, but does place patients at risk for more complications. Quality of life is improved in the long-term, indicating objective and subjective satisfaction.

Cerebellopontine angle gangliogliomas: Report of two cases.

BACKGROUND: Gangliogliomas are rare tumors of the central nervous system. We report two unusual cases of gangliogliomas located in the cerebellopontine angle (CPA). POPULATION AND METHODS: The first patient was a 57-year-old woman, who presented with dizziness and harbored a non-enhanced heterogeneous mass located in the cisternal space of the CPA. A partial microsurgical removal was performed, and the pathological examination concluded a grade I ganglioglioma according to the WHO Classification. The postoperative course was uneventful without any adjuvant treatment and the 5-year imaging follow-up indicated a stable remnant tumor. The second patient was a 35-year-old male who presented with acute vertigo and imbalance associated with recent prominent headaches; MR imaging showed a large heterogeneous and post-contrast enhanced tumor mass located in the CPA cistern with a mass effect on the brain. An optimal subtotal surgical resection was performed. The pathologists concluded a WHO grade III ganglioglioma. In spite of adjuvant radiotherapy and chemotherapy, the evolution proved unfavorable and patient died from cancer complications within a 2-year period. In both cases, the precise origin of the tumor could not be clearly identified even if the major component was present in the cisternal space. CONCLUSION: Gangliogliomas growing into the cisternal spaces are exceedingly rare particularly in the CPA. Due to its infiltrating behavior and major difficulties to identify the tumor margins, total resection is not routinely feasible. The histological grading is the most important predictor for oncological prognosis.

[Ossiculoplasty with KURZ titanium prosthesis]. / Ossiculoplastie par prothèse en titane KURZ. A propos de 111 cas.

OBJECTIVES: Report the functional and anatomic results of ossicular reconstruction by titanium prosthesis. MATERIALS AND METHODS: Retrospective chart reviews were performed for 111 patients who had undergone titanium ossicular implants between November 1998 and 2002 (61 PORP, 50 TORP). The anatomical and audiometric data were analyzed on average at 3 and 20 months. RESULTS: At 20 months, the improvement of air-bone-gap mean was 12.7 dB with better results at low frequencies. The global success rate was 66% (PORP 77%, TORP 52%). It decreased significantly in the open techniques. Extrusion rate was low (2/111) and the labyrinthization rate was 3.6%. Twenty patients required a surgical revision (18%). In 9 patients, the prosthesis was too short. At long-term follow-up, the gains were stable in 60 patients, improved in 32 patients and worsened in 19 patients. CONCLUSION: The success rate is higher in the group of the PORP with the closed technique. The stability of the TORP in open technique still remains problematic. In all cases, the risk of extrusion requires a large cartilage graft recovering the plate of the prosthesis. The high rate of luxation (9/111 prosthesis too short) has led us to increase slightly the length of the prosthesis (+1.22 mm mean).

[Implication of mitochondrial apoptosis in neural degeneration of cochlea in a murine model for presbycusis]. / Implication de l'apoptose mitochondriale dans la dégénérescence neurale de la cochlée dans un modèle murin de presbyacousie.

HYPOTHESIS: Pathologies of senescence, in particular those of neurosensory organs represent an important health problem. The improvement of the life expectation entails the fast increase of the frequency of the presbyacusis in the population. The biological and molecular causes of this degenerative pathology of the inner ear are linked to the disappearance of the sensory cells (inner and outer hair cells) and are associated to nervous damages of the spiral ganglion in the cochlea. We were interested in mechanisms causing the cochlear degeneration in a model of mouse CD 1 presenting prematurely auditive losses. MATERIALS AND METHODS: We tried to correlate the evolution of the hearing and the appearance of apoptotic phenomena by marking with specific antibody, activated anti-caspase-3, in the cochlea during time. We studied the role and the involvement of proteins controlling the apoptosis as the P53 protein and from an energy point of view at the level of the mitochondria such as proteins of the Bcl-2 family and the cytochrome c in the various structures of the cochlea. RESULTS: After implantation of electrodes for auditory nerve acoustic thresholds measurements, the audition of mice CD 1 presented a characteristic profile of hearing losses which begins in the high frequencies from the age of 1 month and which quickly evolves towards the low frequencies. The observation (between the 1st and 3rd month of age) of spiral ganglion cells revealed an unchanged number of cellular bodies of type 1 neurons, on the other hand a characteristic morphology of apoptosis of glial cells with the formation of apoptotic body was noted. Indeed, glial cells expressed activated caspase-3. Furthermore, this phenomenon seems to be under the control of the pro-apoptotic protein Bax by its overexpression and a increased release of the cytochrome c. This phenomenon was followed at 3 and 6 months by the disappearance of the outer hair cells by 9 and 48% respectively. CONCLUSION: The apparition of the deafness in the murin model CD 1 allowed us to demonstrate that the degeneration of cochlear structure begins at the level of glial cells of the spiral ganglion from 3 months, followed thereafter by the deterioration of the nervous conduction between the spiral ganglion and the sensory cells. As a consequence, because of the impoverishment in nervous signals, the outer hair cells would begin to disappear during the 6th month. In conclusion, the understanding of the sequence and the cause of these mechanisms responsible for the neural degeneration and the loss of hearing could eventually, allow us to optimize the various treatments of the presbyacusis.

[The outcome of treatments for carcinoma of the external auditory canal]. / Résultats des traitements des tumeurs malignes du conduit auditif externe.

OBJECTIVE: A retrospective analysis of management and survival of patients treated for temporal bone carcinoma. PATIENTS AND METHODS: Thirty patients underwent treatment for carcinoma of the temporal bone. Twenty-five squamous cell carcinomas, 1 melanoma, 2 basocellular carcinomas and 2 adenoid cystic carcinomas were treated. Thirteen patients were treated before for the same disease. RESULTS: Staging revealed 12 T1 and T2, 6 T3 and 12 T4 tumours. The mean follow up was 5 years (2-276 months). The Kaplan Meier survival curves showed survival rates at 2 years of 82%, 67% and 32%, and at 5 years of 82%, 67% and 17%, respectively for the stages T1 or T2, T3 and T4. At the end of follow up at 9 years the survival rates were 66%, 66% and 17% for the stages T1 or T2, T3 and T4 respectively. Overall stages a complete remission was found in 65% and 23%, and deceased was 35% and 77%, respectively for the primary treatment group and the salvage surgery group. CONCLUSION: Long-term prognosis of the carcinoma of the external auditory canal mainly depends on the stage and primary treatment. Surgery (lateral temporal bone or subtotal temporal bone resection, both in combination with a neck dissection and a parotidectomy) and adjuvant radiotherapy is the treatment of choice for part of stage T1 and all T2 and T3 tumours. The improved survival (65%) of patients treated de novo compared with those treated with salvage surgery (23%) suggests that early referral and aggressive primary surgical treatment with postoperative radiotherapy offer the greatest chance of cure.

[The jugulotympanic paragangliomas: 41 cases report]. / Les paragangliomes tympano-jugulaires. A propos de 41 cas.

OBJECTIVE: The jugulotympanic paragangliomas (JTP) represents the most frequent tumour of the middle ear but also of the temporal bone, after the acoustic neurinoma. The management of these vascular tumours remains uncleared. The purpose of this study was to report our experience about JTP in the CHU of Grenoble. MATERIALS AND METHODS: Retrospective study of 41 patients, between 1973 and 1996. Six stages A, 8 stages B and 27 stages C are reported in whom 20 cases (49%) presented an intracranial extension (classification of Fisch). There were 2 familial cases with multiple localisations, in particular carotid. All the patients were divided in 3 groups: surgery or radiation therapy in first intention, surgery followed by radiation therapy. RESULTS: A total tumor removal without recurrence was achieved by surgery in more than 95% of the cases with 6 years follow-up but was associated with significant morbidity (major cranial nerve injury). We noticed one death by laryngospasme (C2Di2 tumour operated by infratemporal A approach). A stabilization of the tumour was obtained with radiotherapy in first intention in 75% of the cases (5 years follow-up) but with a risk of radionecrosis. A revision surgery was necessary in 3 cases. CONCLUSION: The comparaison of our different therapeutic management, surgery (23), radiation therapy (16) or combined (2), encourage us to perform a radical surgery whenever possible. Because of the slow rate of growth, the radiotherapy is indicated for older patients, at risk for surgery or extensive tumors. The objectives of the radiation therapy are to obtain a tumoral stabilization with improvement of the symptoms and low morbidity. The management of this rare pathology must be multidisciplinary. The recent discoveries on genes encoding three succinate dehydrogenase subunits (SDHD, SDHB et SDHC) will allow a genetic detection of asymptomatic case and will define the procedures for their management, coordinated by a national network PGL.NET. A retrospective study could also study the real incidence of familial paragangliomas.

Mini-invasive surgery of infratemporal fossa schwannomas.

BACKGROUND: Infratemporal fossa schwannomas are benign, encapsulated tumours of the trigeminal nerve limited to the infratemporal fossa. Because of the complications and significant morbidity associated with traditional surgical approaches to the infratemporal fossa, which include facial nerve dysfunction, hearing loss, dental malocclusion and cosmetic problems, less invasive alternatives have been sought. METHODS: This paper reports two cases of infratemporal fossa schwannomas treated in 2012 using mini-invasive approaches. The literature regarding different infratemporal fossa approaches was reviewed. RESULTS: The first schwannoma was 30 mm in size and was removed completely by a preauricular subtemporal approach. The second one was 25 mm in size and was removed completely using a purely transnasal endoscopic approach. In both cases, there were no intra-operative or post-operative complications. CONCLUSION: These two approaches allow non-invasive and wide exposure of the infratemporal fossa as compared to classical approaches. Surgical approach should be selected according to the tumour's anatomical location with respect to the maxillary sinus posterior wall. The preauricular subtemporal approach is recommended for tumours localised posterolaterally with respect to the maxillary sinus posterior wall. Medial and anterior tumours near the maxillary sinus posterior wall can be best removed using a transnasal endoscopic approach.