One Year Assessment of the Hearing Preservation Potential of the EVO Electrode Array.

BACKGROUND: A prospective longitudinal multicentre study was conducted to assess the one-year postsurgical hearing preservation profile of the EVOTM electrode array. METHODS: Fifteen adults presenting indications of electro-acoustic stimulation (pure-tone audiometry (PTA) thresholds ≤70 dB below 750 Hz) were implanted with the EVO™ electrode array. Hearing thresholds were collected at five time-points from CI activation to twelve months (12M) after activation. Hearing thresholds and hearing preservation profiles (HEARRING group classification) were assessed. RESULTS: All subjects had measurable hearing thresholds at follow-up. No case of complete loss of hearing or minimal hearing preservation was reported at any time point. At activation (Nact = 15), five participants had complete hearing preservation, and ten participants had partial hearing preservation. At the 12M time point (N12m = 6), three participants had complete hearing preservation, and three participants had partial hearing preservation. Mean hearing loss at activation was 11 dB for full range PTA and 25 dB for PTAs low-frequency (125-500 Hz). CONCLUSIONS: This study provides the first longitudinal follow-up on associated hearing profiles to the EVO™ electrode array, which are comparable to the literature. However, other studies on larger populations should be performed.

Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis.

Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors. Here, we assessed the contribution to presbycusis of ultrarare pathogenic variants, considered indicative of Mendelian forms. We focused on severe presbycusis without environmental or comorbidity risk factors and studied multiplex family age-related hearing loss (mARHL) and simplex/sporadic age-related hearing loss (sARHL) cases and controls with normal hearing by whole-exome sequencing. Ultrarare variants (allele frequency [AF] < 0.0001) of 35 genes responsible for autosomal dominant early-onset forms of deafness, predicted to be pathogenic, were detected in 25.7% of mARHL and 22.7% of sARHL cases vs. 7.5% of controls (P = 0.001); half were previously unknown (AF < 0.000002). MYO6, MYO7A, PTPRQ, and TECTA variants were present in 8.9% of ARHL cases but less than 1% of controls. Evidence for a causal role of variants in presbycusis was provided by pathogenicity prediction programs, documented haploinsufficiency, three-dimensional structure/function analyses, cell biology experiments, and reported early effects. We also established Tmc1N321I/+ mice, carrying the TMC1:p.(Asn327Ile) variant detected in an mARHL case, as a mouse model for a monogenic form of presbycusis. Deafness gene variants can thus result in a continuum of auditory phenotypes. Our findings demonstrate that the genetics of presbycusis is shaped by not only well-studied polygenic risk factors of small effect size revealed by common variants but also, ultrarare variants likely resulting in monogenic forms, thereby paving the way for treatment with emerging inner ear gene therapy.

The Oticon Medical Neuro Zti cochlear implant and the Neuro 2 sound processor: multicentric evaluation of outcomes in adults and children.

Objective: This study evaluated the outcomes of the Oticon Medical Neuro Zti cochlear implant and the Neuro 2 sound processor.Design: Neuro One users were upgraded to Neuro 2. Monosyllabic word identification was evaluated in adults with Neuro One after ≥5 months, with Neuro 2 at upgrade, and with Neuro 2 after 3 months. Self-reported listening ability, satisfaction, and usability were measured in adults and children.Study sample: Participants were 44 adults and 26 children.Results: Speech identification scores in quiet and noise were 58% and 45% with Neuro One and 67% and 55% with Neuro 2 after 3 months, respectively. Hearing impairment duration and number of active electrodes significantly predicted speech identification in noise with Neuro 2. Significantly higher questionnaire ratings were obtained for Neuro 2 than Neuro One regarding listening ability in complex listening situations, comfort and music, as well as nine aspects of satisfaction and usability.Conclusion: This study demonstrates the clinical superiority of the Neuro 2 sound processor over Neuro One in terms of speech identification in quiet and in noise and reported patient benefit and satisfaction. Given the study design, sources of improvement may include factors unrelated to the sound processor itself.

Transtympanic pseudoaneurysm of the internal carotid artery complicating a myringotomy in a four-year old child: Case report and literature review.

We report the first case of a transtympanic iatrogenic internal carotid artery (ICA) pseudoaneurysm diagnosed in a 4-year-old child following a myringotomy. An endovascular treatment with a covered-stent was decided; spontaneous thrombosis was found during the therapeutic arteriography, and the procedure was aborted. Otoscopy and computed tomography (CT) scan monitoring showed a prolonged thrombosis and the disappearance of the pseudoaneurysm 18months after the diagnostic arteriography. Based on literature review, endovascular techniques seem to be preferred to the surgical approach for treatment of intrapetrous ICA pseudoaneurysm, however clinical and CT scan monitoring may also be a valid option.

Long-term results of middle fossa plugging of superior semicircular canal dehiscences: clinically and instrumentally demonstrated efficiency in a retrospective series of 16 ears.

The objective of this study is to report the surgical outcome after middle fossa approach (MFA) plugging in patients suffering from a superior semi-circular canal dehiscence (SCD) syndrome. This is a retrospective case review. Tertiary referral center. Sixteen ears in 13 patients with a SCD syndrome suffering from severe and disabling vestibular symptoms with a bony dehiscence on CT scan >3 mm and decreased threshold of cervical vestibular evoked potentials (cVEMPs). We assessed preoperatively: clinical symptoms, hearing, cVEMPs threshold, size of dehiscence and videonystagmography (VNG) with caloric and 100 Hz vibratory tests. Postoperatively, we noted occurrences of neurosurgical complication, evolution of audiological and vestibular symptoms, and evaluation of cVEMP data. Tullio's phenomenon was observed in 13 cases (81.3 %) and subjectively reported hearing loss in seven (43.7 %). All patients were so disabled that they had to stop working. No neurosurgical complications were observed in the postoperative course. In three cases (16.6 %), an ipsilateral and transitory immediate postoperative vestibular deficit associated with a sensorineural hearing loss (SNHL) was noted, which totally resolved with steroids and bed rest. All patients were relieved of audiological and vestibular symptoms and could return to normal activity with a mean follow-up of 31.1 months (range 3-95). No patient had residual SNHL. cVEMPs were performed in 14 ears postoperatively and were normalized in 12 (85.7 %). Two of the three patients operated on both sides kept some degree of unsteadiness and oscillopsia. MFA plugging of the superior semi-circular canal is an efficient and non-hearing deteriorating procedure.

Prognostic Factors of Balance Quality After Transpetrosal Vestibular Schwannoma Microsurgery: An Instrumentally and DHI-based Prospective Cohort Study of 48 Patients.

OBJECTIVES: To evaluate short- and mid-term level of imbalance after vestibular schwannoma (VS) microsurgery by the transpetrosal approach, to search for factors predictive of vestibular compensation, and to determine which patient categories need a postoperative vestibular rehabilitation program. STUDY DESIGN: Prospective cohort study at a tertiary referral center. METHODS: Between 2010 and 2011, patients aged 18 to 75 operated on for VS by transpetrosal approaches were included. VS was characterized by its size (Koos classification) and the presence or not of a cystic component. Hearing was classified according to the Gardner Robertson grading. The preoperative workup included an audiogram, computerized video nystagmography (VNG) with caloric testing, gaze study, rotatory tests, click-evoked cervical vestibular evoked myogenic potential measurements (cVEMPs), and subjective visual vertical test. Patients were asked to complete a Dizziness Handicap Inventory (DHI). Postoperatively, patients were reevaluated on D7 (clinical status), D90 (VNG and DHI), and D180 (DHI). Timing and duration of vestibular rehabilitation were also recorded. RESULTS: Forty-eight patients were included. Preoperatively, 77% experienced mild instability problems with a mean DHI score of 14.1 (range 4-32). Postoperatively, 71% reported stable or even improved perceived stability. Mean DHI scores were 28.1 on D90 and 19.8 on D180. Serviceable hearing, cystic transformation, normal cVEMPs, diplopia, and vestibular syndrome on D7 were found to be predictive of worse equilibrium outcome than when absent. A preoperative caloric deficit greater than 75% seemed to be a good prognostic factor. Vestibular rehabilitation was conducted in 56% of patients. Starting it early (<1 mo) seemed to be beneficial for final equilibrium outcome. CONCLUSION: VS microsurgery provides good stability results. Some preoperative parameters may be predictive of worse or improved balance recovery, as is clinical status on D7.

Effects of body tilt on multifrequency admittance tympanometry.

INTRODUCTION: Hydrops and abnormalities of inner fluid pressure are involved in some otologic diseases such as Ménière's disease (MD). However, demonstrating abnormal perilymphatic or endolymphatic pressure is challenging. Multifrequency tympanometry studies in MD patients demonstrated an increase of the width of conductance tympanograms (outside an attack) compared with controls. To confirm that the increase in conductance width is caused by hyperpressure and not hypopressure in these patients tested outside an attack, we assessed the effect of changes in inner ear fluid pressure caused by body tilt on the results of multifrequency admittancemetry tympanograms. MATERIALS AND METHODS: A multifrequency tympanometry including conductance (G) tympanogram at 2 kHz and resonance frequency measurements were performed in 20 volunteers (40 ears) free of otologic or neurologic disease. The measures were collected in three different positions: vertical, supine, and Trendelenburg positions. RESULTS: Changes in inner ear fluid pressure caused by body tilt induced an increase in the width of G tympanograms. In the vertical position, the mean value was 141.7 ± 56.5 daPa; in the supine position, it increased to 158 ± 58.3 daPa; and increased even more in the Trendelenburg position (20 degrees), with a mean of 184 ± 69.6 daPa (p < 0.01). Resonance frequency also increased in the Trendelenburg position.We conclude that the increased width of G tympanograms in MD patients outside an attack may be caused by an increase in inner ear fluid pressure.

Stitchless fibrin glue-aided facial nerve grafting after cerebellopontine angle schwannoma removal: technique and results in 15 cases.

AIM OF THE STUDY: To evaluate the results of facial nerve (FN) grafting using great auricular cable graft and fibrin glue without suturing to palliate FN disruption after removal of large cerebellopontine angle (CPA) vestibular schwannoma (VS) or facial nerve schwannoma (FNS). To assess whether tumor size and origin influenced the results. STUDY DESIGN AND SETTING: Retrospective review of all patients having undergone removal of FNS/VS and needing intraoperative FN repair between 2001 and 2011. INTERVENTION: FN was rehabilitated using great auricular nerve cable graft and fibrin glue (Tisseal) without stitching suture. MAIN OUTCOME MEASURES: All data recorded were reviewed to access age, sex, tumor type, and tumor size according to the Koos classification and presenting symptoms. FN function was evaluated preoperatively and at 18 months using the House-Brackmann (HB) grading system. RESULTS: Among the 595 patients operated for CPA schwannomas in this period, 15 patients (2.5%) underwent FN repair, including 7 cases of FNS and 8 cases of VS. Tumor removal was total in all cases. FN recovery was HB3 in 13 cases (86.7%) and HB4 in 2. The mean time to the first clinical signs of facial reinnervation was 10 months (6-12 mo). No significant relation was found between postoperative facial function and tumor size or type, even if all cases of preoperative FP were noted in FNS. CONCLUSION: Immediate FN reconstruction with fibrin glue-aided greater auricular nerve graft can effectively restore FN function with excellent outcomes. The results seem better than those observed by other authors using sutured grafts or delayed hypoglossal-facial nerve anastomosis.

Clinical presentation of intralabyrinthine schwannomas: a multicenter study of 110 cases.

OBJECTIVE: To describe the presentation of intralabyrinthine schwannomas (ILSs). STUDY DESIGN AND SETTING: Retrospective multicenter study involving 12 European skull base surgery tertiary referral centers. PATIENTS: One hundred ten patients with the diagnosis of ILS, either labyrinth confined or extending into the internal auditory meatus for less than 50% of their volume. MAIN OUTCOME MEASURES: Data collected were age, sex, nature and timing of presenting symptoms, hearing (according to the AAO-HNS grading system), results of vestibular tests (caloric tests and cervical vestibular-evoked myogenic potentials [c-VEMPs]), and tumor localization. Presenting symptoms and laboratory test results were studied according to the extension of the lesion into the cochlea (C) and vestibule (V), on one hand, and according to unifocal (L1) or plurifocal (L2) extension into the labyrinth, on the other. RESULTS: Intracochlear type was more common (50%) than vestibular (19.1%) and more diffuse forms (30.9%). The mean delay for diagnosis was long (72.5 mo; SD, 76.6). Mean age was 53.9 years (SD, 13.2). Deafness was the most common symptom (77.8 dB HL [SD, 33.6], with only 24.6% of patients keeping viable hearing. Caloric tests (65.5% of patients) were abnormal in 77.8% of cases. c-VEMPs were abnormal in 65.7% of the 36 cases analyzed. In V forms, hearing was significantly better (class A + B in 21.1% in C and 45.8% in V forms) (p = 0.03), and vestibular function was more altered (C: 57%, V: 100%, p = 0.0009*). L2 forms were diagnosed later (L1: 59.1 mo, L2: 104.5 mo; p = 0.004*) and were associated more frequently with a dead ear (L1: 13.1%, L2: 41.2%, p = 0.002*) than L1 forms. CONCLUSIONS: This series, which is the largest in the literature, demonstrates that even very small and localized ILSs heavily compromise labyrinthine functions.

Meningeal carcinomatosis of the internal auditory meatus: clinical and imagery-aided differentiation.

OBJECTIVES: To describe difficulties in diagnosing meningeal carcinomatosis of the internal auditory meatus (IAM-MC). STUDY DESIGN: Retrospective case review. SETTING: Tertiary skull base surgery referral center. PATIENTS: A series of six cases of patients diagnosed with IAM-CM over 14 years. RESULTS: The primary cancer was known before diagnosis in three cases (adenocarcinoma of lung, breast, and melanoma). In two others, it was discovered at workup (lung, ethmoid adenocarcinoma). In the last case, no primary cancer was identified. Vestibular symptoms were the first complaint in five of the six cases. Referral symptom was facial paralysis in five cases and rapid-onset total deafness associated with severe unsteadiness in one. Rapid hearing decrease and symptoms of bilateralization were observed in five cases. Diagnosis was ascertained by discovery of malignant cells in the CSF in three cases and at tumor biopsy in one case. In the two other cases, the association of brain metastases and increased dura mater infiltration was convincing. Treatment consisted in radiation therapy to the whole brain in five cases and intrathecal chemotherapy in one case. Evolution was rapidly lethal in five cases. The last died free of disease 4 years after treatment. CONCLUSION: IAM-MC is difficult to demonstrate. Progressive facial paralysis associated with aggressive and rapidly bilateral cochlear and vestibular symptoms are highly evocative in the event of cancer in the patient's history. If there is no history of cancer, lumbar puncture is to be repeated until malignant cells are discovered in the CSF before beginning radiotherapy.

Tolerance and osteointegration of TricOs(TM)/MBCP(®) in association with fibrin sealant in mastoid obliteration after canal wall-down technique for cholesteatoma.

CONCLUSION: The TricOs™/MBCP(®) and fibrin sealant composite was a convenient, effective, and well-tolerated material for mastoid cavity filling and immediate reconstruction of the external auditory meatus after cholesteatoma surgery with canal wall down (CWD). OBJECTIVE: To assess the tolerance and osteointegration of a bone graft substitute, TricOs™/MBCP(®), in association with fibrin sealant for filling the mastoid cavity after cholesteatoma surgery using the CWD technique. METHODS: In this prospective observational study 57 patients with cholesteatoma suitable for CWD were recruited from April 2006 to April 2008 and followed up for 1 year. The mastoid cavity was filled with TricOs™/MBCP(®) followed by immediate reconstruction of the external auditory meatus covered with fascia temporalis and/or cartilage. The main outcome was skin tolerance assessed by a novel weighted score emphasizing long-term results. The typical weighted reference score was 1.67; skin tolerance was considered acceptable if 75% of patients had a score ≤ 1.67. Secondary outcomes were otorrhea and/or otalgia, hearing, and osteointegration assessed through computed tomography scanning at 12 months. RESULTS: Forty-one patients had a complete follow-up; 34 (82.3%) patients achieved the main end point with scores ≤ 1.67. Otorrhea decreased postoperatively. No otalgia interfering with daily tasks was reported. Ossicular reconstruction was carried out in 29 patients. Absence of cochlear toxicity was confirmed by unimpaired bone conduction. Preoperative and postoperative speech audiometry results were similar. No serious adverse events were observed. Osteointegration was satisfactory with hyperdensity or intermediate density in 95% of patients at 12 months.

Germline mutations in FH confer predisposition to malignant pheochromocytomas and paragangliomas.

Malignant pheochromocytoma (PCC) and paraganglioma (PGL) are mostly caused by germline mutations of SDHB, encoding a subunit of succinate dehydrogenase. Using whole-exome sequencing, we recently identified a mutation in the FH gene encoding fumarate hydratase, in a PCC with an 'SDH-like' molecular phenotype. Here, we investigated the role of FH in PCC/PGL predisposition, by screening for germline FH mutations in a large international cohort of patients. We screened 598 patients with PCC/PGL without mutations in known PCC/PGL susceptibility genes. We searched for FH germline mutations and large deletions, by direct sequencing and multiplex ligation-dependent probe amplification methods. Global alterations in DNA methylation and protein succination were assessed by immunohistochemical staining for 5-hydroxymethylcytosine (5-hmC) and S-(2-succinyl) cysteine (2SC), respectively. We identified five pathogenic germline FH mutations (four missense and one splice mutation) in five patients. Somatic inactivation of the second allele, resulting in a loss of fumarate hydratase activity, was demonstrated in tumors with FH mutations. Low tumor levels of 5-hmC, resembling those in SDHB-deficient tumors, and positive 2SC staining were detected in tumors with FH mutations. Clinically, metastatic phenotype (P = 0.007) and multiple tumors (P = 0.02) were significantly more frequent in patients with FH mutations than those without such mutations. This study reveals a new role for FH in susceptibility to malignant and/or multiple PCC/PGL. Remarkably, FH-deficient PCC/PGLs display the same pattern of epigenetic deregulation as SDHB-mutated malignant PCC/PGL. Therefore, we propose that mutation screening for FH should be included in PCC/PGL genetic testing, at least for tumors with malignant behavior.

Inflammatory myofibroblastic tumour of the skull base.

Inflammatory myofibroblastic tumors (IMTs) are rare benign clinical and pathological entities. IMTs have been described in the lungs, abdomen, retroperitoneum, and extremities but rarely in the head and neck region. A 38-year-old man presented with headache, right exophthalmia, and right 6th nerve palsy. A CT scan revealed enlargement of the right cavernous sinus and osteolytic lesions of the right sphenoid and clivus. MR imaging showed a large tumor of the skull base which was invading the sella turcica, right cavernous sinus, and sphenoidal sinus. A biopsy was performed and revealed an IMT. Corticosteroids were given for 3 months but were inefficient. In the framework of our pluridisciplinary consultation, fractionated conformal radiotherapy (FRT) was indicated at a low dose; 20 Gy in 10 fractions of 2 Gy over 12 days were delivered. Clinical response was complete 3 months after FRT. Radiological response was subtotal 6 months after FRT. Two years later, the patient is well.

Lipochoristomas of the cerebellopontine angle and internal acoustic meatus: a seven-case review.

BACKGROUND: Intracranial lipomas are extremely rare tumors, with certain clinical and radiological characteristics. METHODS: We considered the diagnostic evaluation and treatment options of seven patients presenting with lipoma in the cerebellopontine angle or internal acoustic meatus in our department. RESULTS: Mean age was 51, with four out of seven cases being women. Balance disorders (vertigo, dizziness) were the predominant symptoms (in six out of seven patients), followed by hearing loss. The diagnosis of intracranial lipomas was based on the results of imaging studies, especially on magnetic resonance imaging. The management of lipomas of the CPA and of the IAM should initially be conservative, including close follow-up of the patient and evaluation of a potential change in tumor size. Surgical management of CPA lipomas should be reserved for patients with intractable clinical symptoms due to tumor overgrowth. CONCLUSIONS: Diagnosis of CPA and IAM lipomas is considered to be a clinical and imaging challenge. As the complete resection of such lipomas is a risky choice and taking into account the benign course of such tumors, the only absolute surgical indication should be uncontrolled tumor growth.

Anatomo-radiological study of the superior semicircular canal dehiscence of 37 cadaver temporal bones.

PURPOSE: To assess the presence of dehiscence of the superior semicircular canal (SSCC) on computed tomography (CT) scanning and to study the microscopic anatomo-radiological correlation. MATERIALS AND METHODS: Thirty-seven temporal bones preserved in formalin, regardless of the clinical history of cadavers, were studied. A microscopic anatomical study was conducted with an operative microscope (20×). The settings of the CT permitted to obtain 0.6 mm slices contiguous reconstruction in Pöschl plane and in Stenvers plane. Three-dimensional (3D) reconstructions were performed if a radiological dehiscence was observed. The apex thickness was measured in Pöschl plane. The radiological positive criterion of SSCC dehiscence was an absence of bone coverage of more than 1 mm long in Pöschl and Stenvers planes. RESULTS: We observed three dehiscences of the 37 temporal bones on CT in Pöschl and Stenvers planes. However, no dehiscence was found microscopically. The 3D reconstruction was also positive in these three cases. Reconstructions in the Pöschl plane offered good results up to a bone thickness of 0.6 mm. When it was lower than 0.6 mm, the interpretation of the images appeared to be subjective. CONCLUSION: This study emphasizes the limitations of CT imaging, with a risk of false positives to take into account when interpreting the images. The 3D reconstructions also give too many false positives to be used alone and make an accurate diagnosis. The diagnosis of SSCC dehiscence will therefore remain clinical. Complementary and instrumental radiological examinations should be performed only to confirm this clinical suspicion.

Endolymphatic sac tumors: experience of three cases.

OBJECTIVES: To describe the clinical features, radiological findings, treatment and outcomes of three cases of endolymphatic sac tumors (ELST). METHODS: Retrospective analysis of three cases of ELST. RESULTS: The first patient had a large ELST invading the labyrinth after a long history of vertigo. He was recurrence-free 1 year after retrolabyrinthine surgical removal. In the second case, an acute peripheral facial nerve paralysis associated with ipsilateral sensorineural hearing loss led to the diagnosis. A translabyrinthine approach was used to remove the tumor, which recurred three times over 10 years. The third patient was a young woman suffering from von Hippel-Lindau (VHL) disease and referred for a sudden sensorineural hearing loss due to an intralabyrinthine hemorrhage secondary to a 2 mm-large endolymphatic sac-confined ELST. Her hearing was totally lost after the deafness recurred 1 month after this first episode. MRI demonstrated a small bilateral ELST. The patient refused surgery on the deaf side. CONCLUSION: ELST are difficult to diagnose due to the wide variety of their presentations. Patients with ELST should be screened for VHL disease. Dural invasion and tumor hypervascularization increase the risk of local recurrences after surgery. Early surgical resection may lead to complete tumor removal and inner ear preservation.

Unique case of malignant transformation of a vestibular schwannoma after fractionated radiotherapy.

OBJECTIVE: Malignant transformation of vestibular schwannoma is considered a rare clinical entity. Radiotherapy, as a treatment option for vestibular schwannoma, is regarded as a potential risk factor for secondary malignancy. Recently, radiotherapy with dose fractionation has been proposed, intended to diminish the risk of radiation-induced neuropathy. CASE PRESENTATION: The aim of the present study is to report the first case, to the best of our knowledge, of malignant transformation of a residual vestibular schwannoma 19 years after fractionated radiotherapy, describing its characteristics with regard to those previously reported in the literature. CONCLUSIONS: The main purpose of the present work is to state that the knowledge of the iatrogenic potential pitfalls of any technique of radiotherapy in clinical oncology is becoming a necessity. Finally, our report demonstrates that the irradiated patients must be monitored for life because a secondary malignancy may appear after a very long delay.

Spontaneous osteodural defects of the temporal bone: diagnosis and management of 12 cases.

OBJECTIVES: The objectives of the study were to describe the clinical presentation, diagnostic process, surgical treatment, and outcome of patients with spontaneous dural defect of temporal bone and to explore the possible contribution of idiopathic intracranial hypertension. METHODS: Medical records of consecutive patients with spontaneous defects of the temporal bone were reviewed. Clinical presentation, diagnostic process, exploration of benign intracranial hypertension, surgical management, and outcome of patients are presented. RESULTS: Six of the 12 patients presenting with spontaneous cerebrospinal fluid otorrhea were women. Ages ranged from 38 to 76 years. Seven patients presented with meningitis. The location and the extent of the occurring defect were detected by computed tomography in all cases. Radiologic signs of empty sella syndrome, indicator of benign intracranial hypertension, were revealed in 3 cases using magnetic resonance imaging. Six tegmen defects were repaired using a middle fossa approach without recurrence. Four patients received the combined approach. All patients had complete resolution of the cerebrospinal fluid leak, although 2 cases developed adverse effects attributable to surgical procedure. CONCLUSION: The diagnosis of spontaneous cerebrospinal fluid otorrhea requires clinical suspicion in the setting of meningitis and persistent serous otitis media. High-resolution computed tomography can confirm the diagnosis. The authors' findings advocate the multilayered closure technique through a middle fossa approach.

Corticosteroids in the treatment of vestibular neuritis: a systematic review and meta-analysis.

OBJECTIVE: To systematically review and meta-analyze the results of all randomized controlled trials comparing corticosteroids with placebo for the treatment of patients with vestibular neuritis. DATA SOURCES: An electronic search was performed in MEDLINE, EMBASE, Cochrane Library, and CENTRAL databases, and then extensive hand-searching was performed for the identification of relevant studies. No time and language limitations were applied. STUDY SELECTION: Prospective randomized controlled trials comparing corticosteroids with placebo for the treatment of patients with vestibular neuritis. DATA EXTRACTION: Odds ratios (ORs), weighted mean differences (WMD), 95% confidence intervals (CIs), and tests for heterogeneity were reported. DATA SYNTHESIS: Four studies were eventually identified and systematically reviewed. Meta-analysis was feasible for 3 studies. Regarding the recovery of clinical symptoms, the proportion of patients with clinical recovery at 1 month after the initiation of therapy did not differ significantly between the corticosteroids and placebo groups (OR, 1.45; 95% CI, 0.26-8.01; p = 0.67). The proportion of patients with caloric complete recovery was significantly different between the corticosteroids and placebo groups both at 1 (OR, 12.64; 95% CI, 2.6-61.52; p = 0.002; heterogeneity, p = 0.53; fixed effects model) and 12 months (OR, 3.35; 95% CI, 1.45-7.76; p = 0.005; heterogeneity, p = 0.03; random effects model) after the initiation of therapy. The caloric extent of canal paresis at 12 months after the initiation of therapy seemed to differ significantly between patients who received corticosteroids and those who received placebo (WMD, -12.15; 95% CI, -19.85 to -4.46; p < 0.05; heterogeneity, p < 0.05; random effects model). CONCLUSION: The present systematic review and meta-analysis, based on the currently available evidence, suggests that corticosteroids improve only the caloric extent and recovery of canal paresis of patients with vestibular neuritis. At present, clinical recovery does not seem be better in patients receiving corticosteroids.

Gradenigo syndrome without acute otitis media.

Acute otitis media complications are less and less common, thanks to the more widespread availability and use of adequate antibiotics. Nevertheless, when they occur, they may be inconspicuous or silent but with dangerous evolution. Described here is the case of a 5-year-old child suffering from incomplete Gradenigo syndrome without acute otitis media, which was managed medically with favorable outcome.