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.

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.

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.

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.

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.

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.

Effects of microvascular decompression surgery on posture control: A case report in an elderly patient.

AIMS OF THE STUDY: We report the case of an 84-year-old woman with neurovascular conflict of the left cochleovestibular nerve. Prior to surgery, the patient complained of positional vertigo and dizziness for the past two years, and marked instability. Hearing loss and tinnitus were reported on the left side. The aim of this study was to investigate the effects of microvascular decompression of the cochleovestibular nerve on posture control. MATERIALS AND METHODS: Evaluation of cochleovestibular function and posture control was performed before and after surgery. Postural performance was analyzed in static and dynamic conditions, with and without vision, and with optokinetic stimulation. Perception of the static visual vertical (SVV) was recorded in darkness. RESULTS: Positional vertigo and tinnitus disappeared immediately after the decompression. The SVV remained unchanged one week after the surgery. Speech intelligibility of the left ear was improved (30 dB), and the vestibular deficit on this side was also significantly reduced (54% versus 18%). However, the more spectacular result was the effect on postural control. This elderly patient improved her postural balance in both static and dynamic conditions, and became able to maintain her equilibrium in the more challenging dynamic conditions, with and without vision, as early as one week after the surgery, a postural performance that she had been unable to do preoperatively. CONCLUSION: This work is the first to show that the postural deficits resulting from neurovascular conflict of the cochleovestibular nerve are strongly improved after microvascular decompression.

Effects of haptic supplementation on postural stability in unilateral vestibular loss patients.

The effects of haptic supplementation on postural stability during upright standing with vision were investigated in 11 unilateral vestibular loss (UVL) patients and compared to 12 age-marched controls. All the participants were instructed to grip a fixed or mobile stick. In the mobile conditions, the lower extremity of the stick was in contact with a slippery or a rough surface. Postural stability was assessed by the root mean square (RMS) and the velocity of the center of foot pressure (CoP) displacements in both antero-posterior (AP) and medio-lateral (ML) directions. Results showed that RMS was larger in UVL patients than in aged-matched participants in AP direction. However, in the mobile stick conditions, only the rough surface improved postural stability. In addition, RMS was more sensitive to haptic supplementation than CoP velocity. The present study confirms the importance of haptic information in the multi-sensory (re) weighting process underlying postural control in UVL patients.

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.

[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.

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.

Vestibular compensation and vestibular rehabilitation. Current concepts and new trends.

The aim of this review is to present the current knowledge of the mechanisms underlying the vestibular compensation and demonstrating how the vestibular rehabilitation is conducted to help the recovery of balance function. Vestibular rehabilitation is based on improving the natural phenomenon called vestibular compensation that occurs after acute vestibular disturbance or chronic and gradual misbalance. Central compensation implies three main mechanisms namely adaptation, substitution and habituation. The compensation, aided by the rehabilitation aimed to compensate and/or to correct the underused or misused of the visual, proprioceptive and vestibular inputs involved in the postural control. As the strategy of equilibration is not corrected, the patient is incompletely cured and remains with inappropriate balance control with its significance on the risk of fall and impact on quality of life. The vestibular rehabilitation helps to correct inappropriate strategy of equilibrium or to accelerate a good but slow compensation phenomenon. Nowadays, new tools are more and more employed for the diagnosis of vestibular deficit (that may include various sources of impairment), the assessment of postural deficit, the control of the appropriate strategy as well to facilitate the efficiency of the rehabilitation especially in elderly people.

Late pneumolabyrinth after undiagnosed post-traumatic perilymphatic fistula. Case report illustrating the importance of systematic emergency management.

INTRODUCTION: Temporal bone fracture is a common complication of high-energy cranial trauma. Labyrinth involvement is rare, but there is a risk of perilymphatic rupture that is often underestimated on initial clinical examination due to the predominance of neurological and/or somatic symptoms. CASE REPORT: A patient presented with overlooked perilymphatic fistula, decompensated by hyperpressure effort due to poorly adapted management. DISCUSSION: Following a review of the literature on post-traumatic pneumolabyrinth, overall management (from diagnosis to treatment, via prevention advice) was analyzed. A constructive critique of the patient's treatment was thus made. CONCLUSION: We argue for a systematic management protocol in cranial trauma with temporal bone fracture, comprising ENT examination, millimetric-scale cross-sectional imaging of the fracture site, and standardized counseling to prevent late complications.

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.

[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.

Titanium ossicular chain replacement prostheses: prognostic factors and preliminary functional results.

OBJECTIVE: To assess the efficiency of ossiculoplasty procedures with the Kurz titanium ossicular prosthesis and evaluate prognostic factors for the functional results. METHODS: Retrospective chart reviews were performed for ossiculoplasty involving Kurz titanium prostheses between 2006 and 2009 in the ENT Head and Neck Department of the hôpital Nord, Marseille, France. RESULTS: The population studied was 70 patients, with 37 procedures using the partial (PORP) and 33 the total ossicular replacement prosthesis (TORP). Mean follow-up was 9 months. Pre- and postoperative audiological parameters on four frequency averages (0.5, 1, 2, and 3kHz) were compared according to AAO-HNS guidelines. A postoperative air-bone gap (ABG)≤20dB was obtained in 71.43% of the patients (86.49% for PORP, and 54.55% for TORP). The mean change in ABG was 12.45dB in cholesteatomatous otitis versus 13.41dB in non-cholesteatomatous otitis. CONCLUSION: Increasing the length of the ossicular prosthesis, especially TORP, may improve postoperative functional results.

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).

[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.