Customized Cochlear Implant Positioning in a Patient With a Low- Grade Glioma: Towards the Best MRI Artifact Management.

OBJECTIVE: To report the personalized decision-making pro- cess adopted for a cochlear implant (CI) candidate requiring magnetic resonance imaging (MRI) brain surveillance. STUDY DESIGN: Clinical capsule report. SETTING: Tertiary academic referral center. PATIENT: A 23-year-old man affected by posttraumatic bilat- eral profound hearing loss, already in radiological follow-up for a suspected small left cuneal low-grade glioma. INTERVENTIONS: A multidisciplinary approach involving preoperative MRI simulations and 3D printed (3DP) models aiming to adapt the CI position to facilitate MRI brain lesion visibility. MAIN OUTCOME MEASURES: MRI visibility and surgical approach. RESULTS: Preoperative MRI scans with the placement of an Ultra 3D CI were performed simulating different implant location to assess the brain lesion visibility in MRI. CI was positioned 9 cm away from the external auditory canal with an angle of 90 degrees. To assess the technical feasibility of the surgical procedure, a patient-specific 3DP head model was produced preoperatively. The postoperative course was uneventful, the patient showed a significant benefit from CI, and the brain lesion was highly visible at the MRI follow-up. CONCLUSIONS: The employment of strategies aimed at improving the MRI quality in CI recipients still represents a topic requiring attention. Thanks to multidisciplinary team collaboration, in our case, the CI position was successfully determined to allow unhindered MRI visibility of a specific intracranial structure.

The role of cochlear implant positioning on MR imaging quality: a preclinical in vivo study with a novel implant magnet system.

PURPOSES: To investigate the effects for Ultra 3D cochlear implant (CI) positioning on MR imaging quality, looking at a comprehensive description of intracranial structures in cases of unilateral and bilateral CI placement. METHODS: Four CI angular positions (90°, 120°, 135° and 160°) at 9 cm distance from the outer-ear canal were explored. The 1.5 T MRI assessment included our institutional protocol for the investigation of brain pathologies without gadolinium application. Three investigators (two experienced neuroradiologists and one experienced otoneurosurgeon) independently evaluated the MR findings. A 4-point scale was adopted to describe 14 intracranial structures and to determine which CI positioning allowed the best image quality score and how bilateral CI placement modified MRI scan visibility. RESULTS: A high positive correlation was found between the three blinded observers. Structures situated contralateral from the CI showed high-quality values in all four placements. Structures situated ipsilaterally provided results suitable for diagnostic purposes for at least one position. At 90°, artifacts mainly involved brain structures located cranially and anteriorly (e.g., temporal lobe); on the contrary, at 160°, artifacts mostly influenced the posterior fossa structures (e.g., occipital lobe). For the bilateral CI condition, MR imaging examination revealed additional artifacts involving all structures located close to either CI, where there was a signal void/distortion area. CONCLUSIONS: Suitable unilateral CI positioning can allow the visualization of intracranial structures with sufficient visibility for diagnostic purposes. Bilateral CI positioning significantly deteriorates the anatomical visibility. CI positioning might play a crucial role for patients who need post-operative MRI surveillance.

Retrosigmoidal placement of an active transcutaneous bone conduction implant: surgical and audiological perspectives in a multicentre study.

INTRODUCTION: The retrosigmoidal (RS) placement of the Bonebridge system (BB) has been advocated for cases of unfavourable anatomical or clinical conditions which contraindicate transmastoid-presigmoidal positioning. However, these disadvantageous conditions, combined with the considerable dimensions of the implant, may represent a challenge, especially for surgeons with no skull base experience. Moreover, the literature reports only limited experience concerning RS implantation of the BB system. METHODS: A multicentre, retrospective study was conducted to analyse the surgical and functional outcomes of a wide population of patients undergoing RS placement of the BB system by means of a surgical technique specifically developed to overcome the intraoperative issues related to this surgery. Twenty patients with conductive or mixed hearing loss and single sided deafness were submitted to RS implantation of the BB system. RESULTS: Audiological assessment concerning the measurement of the functional and effective gain by pure-tone audiometry (28 dB HL and -12.25 dB HL, respectively) and speech audiometry (24.7 dB HL and -21 dB HL, respectively) was conducted. A high overall subjective improvement of quality of life was recorded with the Glasgow Benefit Inventory questionnaire. No major complications, such as device extrusions or other conditions requiring revision surgery, were reported during the follow-up period (median: 42 months). CONCLUSIONS: In our study, which has one of the largest cohort of patients reported in the literature, RS placement of the BB system was safe and effective. Our functional results showed comparable hearing outcomes with presigmoidal placement. The effective gain, rarely investigated in this field, may be the object of further research to improve our understanding of bone conduction mechanisms exploited by bone conduction hearing implants.

MRI-induced artifact by a cochlear implant with a novel magnet system: an experimental cadaver study.

PURPOSES: To primarily evaluate MRI-induced effects for Ultra 3D cochlear implantation in human cadavers in terms of artifact generation and MR image quality. METHODS: Three human cadaveric heads were submitted to imaging after unilateral and bilateral cochlear implantation. The 1.5 T MR examination protocol was chosen in accordance with our institutional protocol for the assessment of brain pathology. The maximal signal void size was measured according to each sequence and plane. Two experienced neuro-radiologists and one experienced otoneurosurgeon independently evaluated the MR image quality findings. A 4-point scale was used to describe the diagnostic usefulness of 14 brain structures. RESULTS: Shape and size of the artifacts were found to be highly related to MRI sequences and acquisition planes. MRI sequences and processing algorithms affected the ability to assess anatomical visibility. Image quality appeared either high or assessable for diagnostic purposes in 9 out of 14 of the ipsilateral structures, in at least one plane. Anatomical structures contralateral to the cochlear implant were highly visible in all conditions. Artifact intrusion clearly improved after application of metal artifact-reduction techniques. In the case of bilateral cochlear implant, a mutual interaction between the two implant magnets produced an additional artifact. CONCLUSIONS: We performed the first cadaver study aimed at systematically evaluating the MRI-induced artifacts produced by a cochlear implant with a novel four bar magnet system. Specific brain structures can be assessable for diagnostic purposes under 1.5 T MRI, with the cochlear implant magnet in place.

Upright BPPV Protocol: Feasibility of a New Diagnostic Paradigm for Lateral Semicircular Canal Benign Paroxysmal Positional Vertigo Compared to Standard Diagnostic Maneuvers.

Background: The diagnosis of benign paroxysmal positional vertigo (BPPV) involving the lateral semicircular canal (LSC) is traditionally entrusted to the supine head roll test, also known as supine head yaw test (SHYT), which usually allows identification of the pathologic side and BPPV form (geotropic vs. apogeotropic). Nevertheless, SHYT may not always allow easy detection of the affected canal, resulting in similar responses on both sides and intense autonomic symptoms in patients with recent onset of vertigo. The newly introduced upright head roll test (UHRT) represents a diagnostic maneuver for LSC-BPPV, supplementing the already-known head pitch test (HPT) in the sitting position. The combination of these two tests should enable clinicians to determine the precise location of debris within LSC, avoiding disturbing symptoms related to supine positionings. Therefore, we proposed the upright BPPV protocol (UBP), a test battery exclusively performed in the upright position, including the evaluation of pseudo-spontaneous nystagmus (PSN), HPT and UHRT. The purpose of this multicenter study is to determine the feasibility of UBP in the diagnosis of LSC-BPPV. Methods: We retrospectively reviewed the clinical data of 134 consecutive patients diagnosed with LSC-BPPV. All of them received both UBP and the complete diagnostic protocol (CDP), including the evaluation of PSN and data resulting from HPT, UHRT, seated-supine positioning test (SSPT), and SHYT. Results: A correct diagnosis for LSC-BPPV was achieved in 95.5% of cases using exclusively the UBP, with a highly significant concordance with the CDP (p < 0.000, Cohen's kappa = 0.94), regardless of the time elapsed from symptom onset to diagnosis. The concordance between UBP and CDP was not impaired even when cases in which HPT and/or UHRT provided incomplete results were included (p < 0.000). Correct diagnosis using the supine diagnostic protocol (SDP, including SSPT + SHYT) or the sole SHYT was achieved in 85.1% of cases, with similar statistical concordance (p < 0.000) and weaker strength of relationship (Cohen's kappa = 0.80). Conclusion: UBP allows correct diagnosis in LSC-BPPV from the sitting position in most cases, sparing the patient supine positionings and related symptoms. UBP could also allow clinicians to proceed directly with repositioning maneuvers from the upright position.

Isolated Eustachian Tube Osteoma: Common Lesion in Uncommon Site.

Isolated Eustachian tube osteomata are a very rare entity indeed, with the last case being reported in 1979. We describe a case of the otoscopic finding of a blue-yellowish eardrum and successive identification by computed tomography scan of an osteoma localized in the Eustachian tube. Clinical presentation and diagnostic and therapeutic phases in comparison with the literature will be discussed.

Feasibility of Using the Video-Head Impulse Test to Detect the Involved Canal in Benign Paroxysmal Positional Vertigo Presenting With Positional Downbeat Nystagmus.

Positional downbeat nystagmus (pDBN) represents a relatively frequent finding. Its possible peripheral origin has been widely ascertained. Nevertheless, distinguishing features of peripheral positional nystagmus, including latency, paroxysm and torsional components, may be missing, resulting in challenging differential diagnosis with central pDBN. Moreover, in case of benign paroxysmal positional vertigo (BPPV), detection of the affected canal may be challenging as involvement of the non-ampullary arm of posterior semicircular canal (PSC) results in the same oculomotor responses generated by contralateral anterior canal (ASC)-canalolithiasis. Recent acquisitions suggest that patients with persistent pDBN due to vertical canal-BPPV may exhibit impaired vestibulo-ocular reflex (VOR) for the involved canal on video-head impulse test (vHIT). Since canal hypofunction normalizes following proper canalith repositioning procedures (CRP), an incomplete canalith jam acting as a "low-pass filter" for the affected ampullary receptor has been hypothesized. This study aims to determine the sensitivity of vHIT in detecting canal involvement in patients presenting with pDBN due to vertical canal-BPPV. We retrospectively reviewed the clinical records of 59 consecutive subjects presenting with peripheral pDBN. All patients were tested with video-Frenzel examination and vHIT at presentation and after resolution of symptoms or transformation in typical BPPV-variant. BPPV involving non-ampullary tract of PSC was diagnosed in 78%, ASC-BPPV in 11.9% whereas in 6 cases the involved canal remained unidentified. Presenting VOR-gain values for the affected canal were greatly impaired in cases with persistent pDBN compared to subjects with paroxysmal/transitory nystagmus (p < 0.001). Each patient received CRP for BPPV involving the hypoactive canal or, in case of normal VOR-gain, the assumed affected canal. Each subject exhibiting VOR-gain reduction for the involved canal developed normalization of vHIT data after proper repositioning (p < 0.001), proving a close relationship with otoliths altering high-frequency cupular responses. According to our results, overall vHIT sensitivity in detecting the affected SC was 72.9%, increasing up to 88.6% when considering only cases with persistent pDBN where an incomplete canal plug is more likely to occur. vHIT should be routinely used in patients with pDBN as it may enable to localize otoconia within the labyrinth, providing further insights to the pathophysiology of peripheral pDBN.

Gradenigo's syndrome with abscess of the petrous apex in pediatric patients: what is the best treatment?

BACKGROUND: Gradenigo's syndrome is defined by the classic clinical triad of ear discharge, trigeminal pain, and abducens nerve palsy. It has become a very rare nosological entity after the introduction of antibiotics, so that has been defined as the "forgotten syndrome." However, the underlying pathological process (apical petrositis) still represents a life-threatening condition that shall be immediately recognized in order to address the patient to the proper therapy. The therapy itself may be an argument of discussion: on a historical background ruled by surgery, reports of successful conservative antibiotic treatment have risen in recent years. METHODS AND RESULTS: We reported a case of Gradenigo's syndrome in a child with an abscess of the left petrous apex and initial involvement of the carotid artery. After multidisciplinary evaluation, we decided to encourage conservative treatment, until complete regression was observed. DISCUSSION: The available literature of the last 10 years was reviewed, with particular attention to the presence of an apical abscess and the therapeutic approach. The principles of management with regard to conservative therapy versus surgical indications are therefore examined and discussed.

Anatomic variations of the round window niche: radiological study and related endoscopic anatomy.

PURPOSE: In the last decades, literature has shown an increasing interest in round windows (RW) anatomy due to its pivotal role in deafness surgery. The high variability of this anatomical region, with particular regard to the round windows niche (RWN), has been studied by several authors through different methods of investigation. The aim of the present research was to radiologically examine the morphological variability of the RWN and to link the imaging findings to the endoscopic view. METHODS: High-resolution CT scans of 300 temporal bones without neuro-otological pathologies were retrospectively reviewed by 2 neuroradiologist and 1 ENT surgeon who independently evaluated the RWN morphological variations. To link the radiological to the endoscopic data, 45 cadaveric human temporal bones were submitted to a radiological evaluation and to an otoendoscopy conducted through a posterior tympanotomy approach. RESULTS: Three variants of the RWN were detected on coronal CT scan reconstructions: 155 "cylindrical-type", 97 "j-type" and 48 "truncated cone-type". For each radiological type the endoscopic findings showed a specific endoscopic position of the RW chamber, which results in different degrees of RW membrane visibility when analysed through a posterior tympanotomy approach. CONCLUSIONS: To the best of our knowledge, this is the first description of the above-mentioned RWN radiological variations supported by endoscopic data. This study suggests an additional anatomical evaluation that could be useful to predict the RW membrane visibility through a posterior tympanotomy approach. Further studies are required to support the clinical implications of our observations.

"Emergency" Cochlear Implantation in Labyrinthitis Ossificans Secondary to Polyarteritis Nodosa: How to Face a Rare Entity.

Polyarteritis nodosa (PAN) is a systemic vasculitis affecting the small- and medium-sized arteries that may present with hearing impairment. In rare cases, PAN may be associated with progressive labyrinthitis ossificans (LO), an otologic emergency requiring early cochlear implantation (CI) to restore hearing before the complete, irreversible cochlear ossification. We report the first case in the literature of a patient affected by PAN with bilateral sudden sensorineural hearing loss and rapid LO who underwent "emergency" bilateral simultaneous CI. This case report emphasizes the importance of an early audiological evaluation in patients with PAN when LO is suspected. Multidisciplinary approach is mandatory when facing organ-specific manifestations in patients with PAN. Detailed discussion is provided with particular regard to clinical and radiological presentation as well as CI outcomes in such a rare and challenging case.

Validation of the Italian Version of the Dizziness Handicap Inventory, the Situational Vertigo Questionnaire, and the Activity-Specific Balance Confidence Scale for Peripheral and Central Vestibular Symptoms.

Neurophysiological measurements of the vestibular function for diagnosis and follow-up evaluations provide an objective assessment, which, unfortunately, does not necessarily correlate with the patients' self-feeling. The literature provides many questionnaires to assess the outcome of rehabilitation programs for disequilibrium, but only for the Dizziness Handicap Inventory (DHI) is an Italian translation available, validated on a small group of patients suffering from a peripheral acute vertigo. We translated and validated the reliability and validity of the DHI, the Situational Vertigo Questionnaire (SVQ), and the Activities-Specific Balance Confidence Scale (ABC) in 316 Italian patients complaining of dizziness due either to a peripheral or to a central vestibular deficit, or in whom vestibular signs were undetectable by means of instrumental testing or clinical evaluation. Cronbach's coefficient alpha, the homogeneity index, and test-retest reproducibility, confirmed reliability of the Italian version of the three questionnaires. Validity was confirmed by correlation test between questionnaire scores. Correlations with clinical variables suggested that they can be used as a complementary tool for the assessment of vestibular symptoms. In conclusion, the Italian versions of DHI, SVQ, and ABC are reliable and valid questionnaires for assessing the impact of dizziness on the quality of life of Italian patients with peripheral or central vestibular deficit.

New insights into vestibular-saccade interaction based on covert corrective saccades in patients with unilateral vestibular deficits.

In response to passive high-acceleration head impulses, patients with low vestibulo-ocular reflex (VOR) gains often produce covert (executed while the head is still moving) corrective saccades in the direction of deficient slow phases. Here we examined 23 patients using passive, and 9 also active, head impulses with acute (< 10 days from onset) unilateral vestibular neuritis and low VOR gains. We found that when corrective saccades are larger than 10°, the slow-phase component of the VOR is inhibited, even though inhibition increases further the time to reacquire the fixation target. We also found that 1) saccades are faster and more accurate if the residual VOR gain is higher, 2) saccades also compensate for the head displacement that occurs during the saccade, and 3) the amplitude-peak velocity relationship of the larger corrective saccades deviates from that of head-fixed saccades of the same size. We propose a mathematical model to account for these findings hypothesizing that covert saccades are driven by a desired gaze position signal based on a prediction of head displacement using vestibular and extravestibular signals, covert saccades are controlled by a gaze feedback loop, and the VOR command is modulated according to predicted saccade amplitude. A central and novel feature of the model is that the brain develops two separate estimates of head rotation, one for generating saccades while the head is moving and the other for generating slow phases. Furthermore, while the model was developed for gaze-stabilizing behavior during passively induced head impulses, it also simulates both active gaze-stabilizing and active gaze-shifting eye movements.NEW & NOTEWORTHY During active or passive head impulses while fixating stationary targets, low vestibulo-ocular gain subjects produce corrective saccades when the head is still moving. The mechanisms driving these covert saccades are poorly understood. We propose a mathematical model showing that the brain develops two separate estimates of head rotation: a lower level one, presumably in the vestibular nuclei, used to generate the slow-phase component of the response, and a higher level one, within a gaze feedback loop, used to drive corrective saccades.

Translabyrinthine vestibular neurectomy and simultaneous cochlear implant for Ménière's disease.

BACKGROUND: Surgical management of Ménière's disease (MD) is recommended in case of medical and intratympanic treatment failures. Translabyrinthine vestibular nerve section has been considered the gold standard for denervation procedures in order to control vertigo attacks, although at the cost of sacrificing residual hearing. To the best of our knowledge, no work has been published with regard to a group of patients submitted to translabyrinthine vestibular neurectomy and simultaneous cochlear implant for MD. The aim of the present study was to assess the effectiveness of translabyrinthine vestibular nerve section and simultaneous cochlear implant in a prospective study. METHODS: All adult patients (over 18 years of age) with a diagnosis of intractable unilateral definite MD and useless residual hearing function were enrolled after medical and intratympanic treatment failures. Pre- and postoperative otoneurological evaluation concerned: frequency of vertigo attacks, head impulse test and caloric testing, pure tone average and speech perception audiometry in quiet conditions, tinnitus handicap inventory test, functional level scale and rate of vertigo control, dizziness handicap inventory test, and MD patient-oriented severity index. At least 6 months of follow-up were needed to be enrolled in the study. RESULTS: Four patients were included in the study. Translabyrinthine vestibular nerve section and simultaneous cochlear implant seemed to considerably improve the disabling effects of MD, achieving a good control of vestibular symptoms (mean pre/postoperative vertigo attacks per month: 16.5/0), resolving hearing loss (mean pre/postoperative pure tone average in the affected ear: 86.2/32.5 dB), improving the tinnitus (mean pre/postoperative tinnitus handicap inventory test: 77.2/6), and finally increasing the overall quality-of-life parameters. CONCLUSIONS: In our preliminary report, translabyrinthine vestibular nerve section and simultaneous cochlear implant showed encouraging results in order to definitively control both vestibular and cochlear symptoms during the same therapeutic procedure.

Intratympanic gentamicin treatment 'as needed' for Meniere's disease. Long-term analysis using the Kaplan-Meier method.

In recent years, several titration or on-demand protocols using low-dose repeated intratympanic (IT) gentamicin injections have been adopted for the vertigo control in unilateral medical refractory Menière's disease (MD). Because of the frequent recurrence and the need to treat the patients several times, it is difficult to strictly follow the 1995 AAO-HNS criteria to classify the results. The Kaplan-Meier analysis provides an effective and simpler method to address these concerns. We report the results of a long-term study (7 years) on a large population of MD patients (174) treated with on-request low-dose delayed IT gentamicin injections analysed using the Kaplan-Meier survival method. Effective vertigo control was obtained with a single injection in 40.2% of the patients (excellent responders) and with repeated injections (2-9) in 43.7% of the patients (moderate responders). Only six patients (3.5%) needed to be submitted to vestibular neurectomy because of the persistence of vertigo attacks (non-responders). A subgroup of 22 patients (12.6%) reporting a late recurrence of vertigo attacks after an initial vertigo-free interval lasting more than 2 years (short-term responders) were successfully treated with a further cycle of injections. In no cases, we observed significant signs of cochlear or vestibular toxicity. Kaplan-Meier survival analysis provided an excellent method for reporting treatment success or failure in patients followed for variable length of time with our kind of protocol.

Reading while moving: the functional assessment of VOR.

The head impulse test (HIT) is nowadays recognized as the gold standard for clinical testing of the angular vestibulo-ocular reflex (VOR). By imposing unpredictable, abrupt head rotations in canal pairs' planes it aims at unveiling the dysfunction of the semicircular canal towards which the head is rotated based on Ewald's II law. Functional testing of the VOR aims at assessing the ability of the reflex to stabilize gaze in space and thus allow clear vision during head movements. The HIT device (HITD) approach exploits impulsive head rotations spawning a range of angular accelerations while requiring subjects to identify optotypes briefly displayed on a screen. Here we also recorded eye movements, so that the evaluation of the individual subject is based both on the VOR gain and on the percentage of correct answers with respect to a population of controls. Here we used the HITD to study 14 patients suffering from vestibular neuritis and 7 of those were re-tested after three months. We found that the HITD was able to unveil the ipsilesional deficit and the contralesional impairment, together with the improvement in the follow-up test.

Intratympanic gentamicin in monolateral Meniere's disease: our experience.

The aim of this study was to verify the efficacy of a modified Odkvist titration protocol of intratympanic gentamicin application in the control of vertigo attacks and the effects on the auditory and vestibular function in a group of 71 patients affected by monolateral MD resistant to medical therapy. All the patients underwent an intratympanic administration of a 1-ml solution containing 26.6 mg of gentamicin sulfate. The treatment protocol provided one to three injections for a total amount of gentamicin varying from 26.6 to 80 mg. Five days after the first gentamicin administration, cochlear and vestibular function tests were performed. The worsening of the PTA greater than 15 dB, the appearance of clinical signs of vestibulotoxicity such as imbalance or persistent spontaneous nystagmus beating away from the injected ear or of a "curative vertigo" were the criteria taken into consideration to stop the treatment. In the absence of any sign, a second and third injection were performed. The presence of an unchanged frequency of the attacks at least 3 months after the previous cycle was the parameter considered to perform a second or third cycle. Seventeen (24%) patients were submitted to a second cycle of therapy and two (3%) to a third cycle. After a mean follow-up period of 20.3 months (range: 3 to 48) all 71 patients experienced good control of the vertigo attacks: grade A in 46 cases and grade B in 25 cases according to the AAO-HNS CoHE criteria. The pure tone average (PTA) hearing threshold (500-3,000 Hz) worsened in 19 patients, improved in 5 and was unchanged in 47. On the basis of the experience acquired during the treatment, we progressively decreased the number of injections from 3/cycle to a 1-2/cycle of therapy. Moreover, in the later phase of the study re-injections were administered 1 or 2 weeks after the previous application and avoided in the presence of signs of depression of the vestibular and/or cochlear function. A residual caloric excitability was found in 30% of the cases. Vertigo control doesn't seem to be linked to the achievement of vestibular inexcitability. The marker of successful gentamicin treatment at short-term is the appearance of signs of curative vertigo and/or vestibular imbalance, and at long-term the disappearance of vertigo attacks.