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Objectives Delayed post-gadolinium magnetic resonance imaging (MRI) detects changes of endolymphatic hydrops (EH) within the inner ear in Meniere’s disease (MD). A systematic review with meta-analysis was conducted to summarise the diagnostic performance of MRI descriptors across the range of MD clinical classifications. Materials and methods Case-controlled studies documenting the diagnostic performance of MRI descriptors in distinguishing MD ears from asymptomatic ears or ears with other audio-vestibular conditions were identified (MEDLINE, EMBASE, Web of Science, Scopus databases: updated 17/2/2022). Methodological quality was evaluated with Quality Assessment of Diagnostic Accuracy Studies version 2. Results were pooled using a bivariate random-effects model for evaluation of sensitivity, specificity and diagnostic odds ratio (DOR). Meta-regression evaluated sources of heterogeneity, and subgroup analysis for individual clinical classifications was performed. Results The meta-analysis included 66 unique studies and 3073 ears with MD (mean age 40.2–67.2 years), evaluating 11 MRI descriptors. The combination of increased perilymphatic enhancement (PLE) and EH (3 studies, 122 MD ears) achieved the highest sensitivity (87% (95% CI: 79.92%)) whilst maintaining high specificity (91% (95% CI: 85.95%)). The diagnostic performance of “high grade cochlear EH” and “any EH” descriptors did not significantly differ between monosymptomatic cochlear MD and the latest reference standard for definite MD (p = 0.3; p = 0.09). Potential sources of bias were case-controlled design, unblinded observers and variable reference standard, whilst differing MRI techniques introduced heterogeneity. Conclusions The combination of increased PLE and EH optimised sensitivity and specificity for MD, whilst some MRI descriptors also performed well in diagnosing monosymptomatic cochlear MD. Key Points • A meta-analysis of delayed post-gadolinium magnetic resonance imaging (MRI) for the diagnosis of Meniere’s disease is reported for the first time and comprised 66 studies (3073 ears). • Increased enhancement of the perilymphatic space of the inner ear is shown to be a key MRI feature for the diagnosis of Meniere’s disease. • MRI diagnosis of Meniere’s disease can be usefully applied across a range of clinical classifications including patients with cochlear symptoms alone.

Supplemental Digital Content is available in the text., Objectives: The primary objective of this study was to establish the reproducibility of cochlear microphonic (CM) recordings obtained from a cochlear implant (CI) electrode contact during and immediately after insertion. This was achieved by evaluating the insertion angle and calculating the position of the apical electrode contact during insertion, using postoperative cone beam computed tomography (CBCT). The secondary objective was to create individualized patient maps of electrode contacts located within acoustically sensitive regions by correlating the CM amplitude to the electrode position determined using CBCT. Methods: CMs were recorded from a CI electrode contact during and immediately after insertion in 12 patients (n = 14 ears). Intraoperative recordings were made for a 0.5 kHz tone burst stimulus and were recorded from the apical electrode contact. Postinsertion recordings were made from the odd-numbered electrode contacts (1–15) along the array, using a range of stimulus frequencies (from 0.125 to 2 kHz). The time point at which each electrode contact passed through the round window was noted throughout the insertion, and the CM amplitude at this point was correlated to postoperative CBCT. This correlation was then used to estimate the CM amplitude at particular points within the cochlea, which was in turn compared with the amplitudes recorded from each electrode postoperatively to assess the reproducibility of the recordings. Results: Significant correlation was shown between intraoperative insertion and postinsertion angles at two amplitude events (maximum amplitude: 29° mean absolute error, r = 0.77, p = 0.006; 10% of maximum amplitude: 52° mean absolute error, r = 0.85, p = 0.002). Conclusion: We have developed a novel method to demonstrate the reproducibility of the CM responses recorded from a CI electrode during insertion. By correlating the CM amplitude with the postoperative CBCT, we have also been able to create individualized maps of CM responses, categorizing the cochlea into acoustically responsive and unresponsive regions. If the electrode contacts within the acoustically sensitive regions are shown to be associated with improved loudness discrimination, it could have implications for optimal electrode mapping and placement.

Purpose The association of internal auditory canal (IAC) fundal diverticula with otospongiosis (OS) and their clinical significance remain unclear. We explored whether isolated IAC diverticula were morphologically different from those with additional CT features of OS, and whether IAC diverticula morphology influenced patterns of hearing loss. Methods Consecutive temporal bone CT studies with (n = 978) and without (n = 306) features of OS were retrospectively assessed. Two independent observers evaluated the presence of IAC diverticula morphological features (depth, neck:depth ratio, definition of contour and angulation of shape), and these were correlated with the presence of fenestral and pericochlear OS. Audiometric profiles were analysed for the isolated IAC diverticula and those with fenestral OS alone. Continuous data was compared using Wilcoxon rank sum tests and categorical data with chi-squared and Fisher’s exact tests. Results Ninety-five isolated IAC diverticula were demonstrated in 54/978 patients (5.5%) without CT evidence of OS (31M, 23F, mean age 46), and 119 IAC diverticula were demonstrated in 71/306 patients (23%) with CT evidence of OS (23M, 48F, mean age 55). Reduced neck:depth ratio, ill definition and angulation were all significantly associated with the presence of pericochlear OS (p p Conclusion IAC diverticula associated with pericochlear OS demonstrate different morphological features from isolated IAC diverticula. There are no clear audiometric implications of these morphological features.

Percutaneous bone-anchored hearing devices (pBAHDs) are the most commonly used bone conduction implants (BCI). Concerns surround the long-term complications, notably skin-related, in patients with percutaneous abutments. The active transcutaneous BCI Bonebridge system can help avoid some of these pitfalls but is often considered a second-line option due to various factors including perceived increased overall costs.Longitudinal economic analysis of Bonebridge BCI 601 versus pBAHD over a 5-year follow-up period.A specialist hearing implant centre.Adult patients (≥16 years) with conductive hearing loss, mixed hearing loss or single-sided deafness, who received a Bonebridge or pBAHD implant between 1/7/2013 and 1/12/2018 with a minimum 12-month follow-up.We compared the mean costs per implanted patient for both implants at 1, 3 and 5 years postoperative time points. Clinical effectiveness was evaluated using objective and patient-reported outcome measures.The mean total cost per patient of Bonebridge was significantly higher than pBAHD at 1-year post-implantation (£8512 standard deviation [SD] £715 vs £5590 SD £1394, P .001); however, by 5-years post-implantation this difference was no longer statistically significant (£12 453 SD £2159 vs £12 575 SD £3854, P .05). The overall cost convergence was mainly accounted for by the increased long-term complications, revision surgery rates and higher cost of the pBAHD external processor compared to Bonebridge.Long-term costs of Bonebridge to healthcare providers are comparable to pBAHDs, whilst offering lower complication rates, comparable audiological benefit and patient satisfaction. Bonebridge should be considered as a first-line BCI option in appropriate cases.

Purpose: ‘Black Bone’ magnetic resonance imaging (BB MRI) is a novel sequence developed as an alternative to computed tomography (CT) for osseous imaging. We explored its potential utilisation in t...

Objective: To determine the impact of Human Papilloma Virus (HPV) oropharyngeal cancer (OPC) status on the prediction of head and neck squamous cell cancer (HNSCC) chemoradiotherapy (CRT) outcomes with pre-treatment quantitative diffusion-weighted magnetic resonance imaging (DW-MRI). Methods: Following ethical approval, 65 participants (53 male, age 59.9 ± 7.86) underwent pre-treatment DW-MRI in this prospective cohort observational study. There were 46 HPV OPC and 19 other HNSCC cases with Stage III/IV HNSCC. Regions of interest (ROIs) (volume, largest area, core) at the primary tumour (n = 57) and largest pathological node (n = 59) were placed to analyse ADCmean and ADCmin. Unpaired t-test or Mann–Whitney test evaluated the impact of HPV OPC status and clinical parameters on their prediction of post-CRT 2 year locoregional and disease-free survival (LRFS and DFS). Multivariate logistic regression compared significant variables with 2 year outcomes. Results: On univariate analysis of all participants, the primary tumour area ADCmean was predictive of 2 year LRFS (p = 0.04). However, only the HPV OPC diagnosis (LFRS p = 0.03; DFS p = 0.02) predicted outcomes on multivariate analysis. None of the pre-treatment ADC values were predictive of 2 year DFS in the HPV OPC subgroup (p = 0.21–0.68). Amongst participants without 2 year disease-free survival, HPV-OPC was found to have much lower primary tumour ADCmean values than other HNSCC. Conclusion: Knowledge of HPV OPC status is required in order to determine the impact of the pre-treatment ADC values on post-CRT outcomes in HNSCC. Advances in knowledge: Pre-treatment ADCmean and ADCmin values acquired using different ROI methods are not predictive of 2 year survival outcomes in HPV OPC.

Background Epithelioid haemangiomas are rare, benign, red-brown lesions of uncertain pathogenesis, mostly arising in the head and neck. Case reports in the literature demonstrate considerable variability in their clinical appearance, and rarely describe any associated lymphadenopathy. Case report We report a case of a 26-year-old female who presented with a mass in the right external auditory meatus (EAM), causing progressive occlusion and conductive hearing loss. Imaging demonstrated an enhancing EAM mass with ipsilateral lymphadenopathy. The lesion was surgically excised, restoring the patient's hearing, and the final diagnosis was made on histopathology. Conclusion This case portrays the variable clinical presentation and heterogenous macroscopic appearances of epithelioid haemangiomas, which clinicians should consider when diagnosing EAM lesions, along with radiological and histopathological features. Epithelioid haemangiomas often recur, warranting regular post-operative follow-up.

Objective: CHARGE syndrome presents with a collection of congenital anomalies affecting multiple organs. Ear and temporal bone anomalies, including hearing loss are highly prevalent.We present an a...

OBJECTIVE To evaluate the growth rate and late detection of residual cholesteatoma on long-term follow-up with diffusion weighted magnetic resonance imaging (DWI MRI) in clinically stable ears following definitive surgery, in order to define surveillance imaging protocols. STUDY DESIGN Retrospective case review. SETTING Tertiary referral center. PATIENTS Patients who underwent DWI MRI at our institution between February 2007 and May 2013 for postoperative cholesteatoma follow-up. INTERVENTION Non-echo planar imaging (non-EPI) Half-Fourier Acquisition Single-shot Turbo spin Echo (HASTE) DWI MRI. MAIN OUTCOME MEASURES Intervals between the definitive surgery and the first and subsequent DWI MRI, the maximum coronal dimension of the lesion on DWI and length of follow-up. RESULTS The study evaluated 152 postoperative DWI studies performed for 88 patients. In 12 cases, DWI was initially negative but became positive on repeat imaging after a mean interval of 3.8 years from the initial cholesteatoma surgery (median 3.7 years, range 1.6-7.9). Of these, 3/12 had more than one negative/indeterminate DWI before disease was eventually detected on imaging; in this subgroup, the mean interval between surgery and positive DWI was 3.2 years (median 2.6 years, range 2.3-4.2). 39 DWI positive foci with serial imaging demonstrated a mean growth rate of 4 mm/year (median 2 mm, range 0-18). CONCLUSIONS After negative initial DWI, it is proposed that interval imaging should be considered for a minimum of 5 years in stable ears following definitive cholesteatoma surgery. In view of the marked variability in growth rate, an additional interval scan between 2 and 3 years postoperatively is indicated.

Introduction Dizziness is a common disorder, particularly among the elderly population. Aim of this paper is to revise the current concepts surrounding the relationship between dizziness, psychological disorders and cognitive decline. Evidence acquisition PRISMA-compliant systematic review, including observational studies in people with dizziness. Database inception, Medline/Cochrane/Embase/Web of Science/Scopus/NHS evidence, last search 30th October 2019. Evidence synthesis Overall 22 studies, and 65730 participants were included. 11 studies were cross-sectional, 7 cross-sectional controlled, 2 prospective case-control, 1 retrospective case series, and 1 cohort study. The persistence of vestibular impairment (for 6 months or more) was correlated to the presence of psychological disorders affecting patient's quality of life and causing social anxiety, particularly in some conditions such as Meniere's Disease. Interestingly, vestibular loss has been also correlated to cognitive impairment, with certain vestibular dysfunctions reported to be more prevalent in cognitive impaired individuals. Conclusions The current literature suggests that there is an association between vestibular function, psychological disorders and cognitive functions. The findings from this review could be useful in informing on the need for a multidimensional diagnostic and rehabilitative programs for patients with dizziness. More studies could explore the role of counselling or behavioral therapy with an aim to reduce the perceived dizziness-related disability.

OBJECTIVE The number of cochlear implant (CI) users is ever increasing worldwide, as is the utilization of magnetic resonance imaging (MRI) as a key diagnostic modality for pathology of the brain and surrounding structures. Despite advances in MRI compatibility with CI, metal artefact remains a significant issue that needs to be addressed. We test our hypothesis that the slice encoding for metal artefact correction and view angle tilting (SEMAC-VAT) metal artefact reduction technique improves demonstration of posterior fossa structures on MRI in CI recipients. STUDY DESIGN A retrospective case review. SETTING A tertiary referral hearing implant and skull base center. INTERVENTIONS Dedicated MRI of the posterior fossa using T1 spin echo post-gadolinium sequences with and without the application of SEMAC-VAT in CI recipients. MAIN OUTCOME MEASURES Extent and severity of the artefact and visualization of surrounding anatomic structures with and without the application of SEMAC-VAT, allowing for direct comparison. RESULTS Eight CI recipients with nine CI devices were analyzed. We noted a significant reduction in signal void and improved visibility of the ipsilateral hemisphere in every case. Penumbra size increased although there was improved visibility through the penumbra. There was improved visualization of key intracranial structures, such as the ipsilateral internal auditory canal, cerebellopontine angle, cerebellar hemisphere, and brainstem. CONCLUSIONS Application of SEMAC-VAT produces a significant reduction in signal void and improved visualization of key structures within the temporal bone and posterior cranial fossa in patients with CIs without the need for removal of the internal magnet.

Objectives:Delayed post-gadolinium 3D fluid-attenuated inversion recovery (FLAIR) MRI is used to support a diagnosis of Ménière’s disease (MD) with the ratio of the endolymphatic space (ES) to the sum of the endolymphatic and perilymphatic spaces (SEPS) on a cross-section through the vestibule being a key diagnostic criterion. It was hypothesised that the exact definition of the vestibular cross-section would influence the ES: SEPS ratio, its ability to diagnose MD, and its reproducibility.Methods:Following institutional approval, 22 patients (five male, 17 female; mean age 52.1) with unilateral MD and delayed post-gadolinium 3D FLAIR MRI were retrospectively analysed. Two observers measured the ES and SEPS on predefined axial (superior and inferior) and sagittal vestibular cross-sections. Receiver operating characteristic (ROC) curves, Bland-Altman plots and intraclass correlation (ICC) were analysed for the ES:SEPS ratios.Results:The area under the curve (AUC) was decreased for the ES:SEPS ratios on the superior axial section through the vestibule (AUC 0.737) compared to the inferior axial (AUC 0.874) and sagittal sections (AUC 0.878). The resulting optimal thresholds (sensitivities/specificities) were 0.21 (0.66/0.75), 0.16 (0.77/0.9) and 0.285 (0.75/0.96). The reproducibility was excellent for all measures with ICCs of 0.97, 0.98 and 0.99.Conclusion:Inferior axial or sagittal vestibular cross-sections are more accurate for the diagnosis of MD ears and have excellent reproducibility.Advances in knowledge:The choice of vestibular cross-section influences both the ability to distinguish MD from asymptomatic contralateral ears, and the optimum threshold ES:SEPS value.

Objectives The primary objective was to determine whether the narrowest dimensions of the labyrinthine facial nerve (LFN) canal on the symptomatic side in patients with unilateral recurrent Bell's palsy (BP) differ from those on the contralateral side or in asymptomatic, age‐ and gender‐matched controls on computed tomography (CT). The secondary objectives were to assess the extent of bony covering at the geniculate ganglion and to record inter‐observer reliability of the CT measurements. Methods The dimensions of the LFN canal at its narrowest point perpendicular to the long axis and the extent of bony covering at the geniculate ganglion were assessed by two radiologists. Statistical analysis was performed using the Wilcoxon signed‐rank and Mann‐Whitney U tests (LFN canal dimensions) and the Chi‐squared test (bony covering at the geniculate ganglion). Inter‐observer reliability was evaluated using Intra‐Class Correlation (ICC) and Cohen's kappa. Results The study included 21 patients with unilateral recurrent BP and 21 asymptomatic controls. There was no significant difference in the narrowest dimensions of the ipsilateral LFN canal when compared to the contralateral side or controls (P = .43‐.94). Similarly, there was no significant difference in the extent of bony covering at the geniculate ganglion when compared to either group (P = .19‐.8). Good inter‐observer reliability was observed for LFN measurements (ICC = 0.75‐0.88) but not for the bony covering at the geniculate ganglion (Cohen's kappa = 0.53). Conclusion The narrowest dimensions of the LFN canal and the extent of bony covering at the geniculate ganglion do not differ in unilateral recurrent BP, casting doubt over their etiological significance. Level of Evidence Level IV., Two observers evaluated the narrowest dimensions of the labyrinthine facial nerve canal and the extent of bony covering at the geniculate ganglion on computed tomography (CT), in 21 patients with unilateral recurrent Bell's Palsy and 21 matched asymptomatic controls. There was no significant difference in the dimensions of the ipsilateral (symptomatic) labyrinthine facial nerve canal or the geniculate ganglion bony covering in patients with unilateral recurrent Bell's Palsy, either when comparing to the contralateral side or to asymptomatic controls. This casts doubt over their aetiological significance of these CT findings in cases of recurrent BP.

This review is designed to help radiologists interested in developing a magnetic resonance imaging service for patients with symptoms of Meniere's disease. Examples are selected from our experience with delayed post-gadolinium three-dimensional (3D) fluid attenuated inversion recovery (FLAIR) inner ear imaging of endolymphatic hydrops. The imaging features of the normal and hydropic endolymphatic structures, semiquantitative grading systems, normal variations, and differential diagnoses will be illustrated, whilst appropriate clinical referrals, approaches to reporting and diagnostic pitfalls will be discussed.

Background and purpose We explored whether there was a difference between measurements obtained with CT and MRI for the diagnosis of large vestibular aqueduct syndrome or large endolymphatic sac anomaly, and whether this influenced diagnosis on the basis of previously published threshold values (Valvassori and Cincinnati). We also investigated whether isolated dilated extra-osseous endolymphatic sac occurred on MRI. Secondary objectives were to compare inter-observer reproducibility for the measurements, and to investigate any mismatch between the diagnoses using the different criteria. Materials/methods Subjects diagnosed with large vestibular aqueduct syndrome or large endolymphatic sac anomalies were retrospectively analysed. For subjects with both CT and MRI available (n = 58), two independent observers measured the midpoint and operculum widths. For subjects with MRI (± CT) available (n = 84), extra-osseous sac widths were also measured. Results There was no significant difference between the width measurements obtained with CT versus MRI. CT alone diagnosed large vestibular aqueduct syndrome or large endolymphatic sac anomalies in 2/58 (Valvassori) and 4/58 (Cincinnati), whilst MRI alone diagnosed them in 2/58 (Valvassori). There was 93% CT/MRI diagnostic agreement using both criteria. Only 1/84 demonstrated isolated extra-osseous endolymphatic sac dilatation. The MRI-based LVAS/LESA diagnosis was less dependent on which criteria were used. Midpoint measurements are more reproducible between observers and between CT/MR imaging modalities. Conclusion Supplementing MRI with CT results in additional diagnoses using either criterion, however, there is no net increased diagnostic sensitivity for CT versus MRI when applying the Valvassori criteria. Isolated enlargement of the extra-osseous endolymphatic sac is rare.

BackgroundRecent developments in magnetic resonance imaging have enabled demonstration of endolymphatic hydrops, and the clinical application of these imaging studies in Ménière's disease is being explored.ObjectiveTo evaluate our centre's experience to date of hydrops magnetic resonance imaging in patients with episodic vertigo.MethodsMagnetic resonance imaging was performed using a high-resolution three-dimensional fluid-attenuated inversion recovery sequence on a 3 Tesla scanner at 4 hours following double-dose gadolinium administration.ResultsThe study included 31 patients, 28 of whom had a clinical diagnosis of Ménière's disease. In unilateral Ménière's disease, magnetic resonance imaging was able to lateralise endolymphatic hydrops to the clinically symptomatic ear in all cases. Mild hydrops was often seen in clinically asymptomatic ears.ConclusionThere is a good correlation between the clinical symptoms and lateralisation of hydropic changes on magnetic resonance imaging. Further refinements of imaging techniques and grading system will likely improve the diagnostic accuracy and clinical utilisation of hydrops magnetic resonance imaging.

Objectives: The average life expectancy in the United Kingdom is currently nearly 80 years for a newborn baby with nearly 15% of the population, by 2040, being >75 years old. Hearing impairment is a common disability in the elderly individual, and there have been significant drives to support this population to lead longer and healthier working lives. We aimed to assess the long-term audiological and health-related quality-of-life benefits of cochlear implants (CI) in elderly individuals. Methods: A retrospective and cross-sectional study of patients who received a CI at ≥70 years. Data extracted included speech perception scores, adverse events, telephone use, and patient-reported outcome measures using the Glasgow Benefit Inventory questionnaire with a minimum of 12 months follow-up. Results: Sixty-four patients aged ≥70 years received a unilateral CI. A significant improvement in speech perception scores in all conditions was noted with no significant difference between differing age groups. Glasgow Benefit Inventory scores showed a significant positive impact on patients’ lives and their health status, with no significant difference between the differing age groups. Conclusions: Cochlear implantation is a safe and well-tolerated procedure in the elderly patients with significant improvements observed in audiological performance, health status, and social interactions.

ObjectiveThis case report illustrates an unusual case of a dural arteriovenous fistula and an associated encephalocele presenting as otitis media with effusion.Case reportA 53-year-old man presented with right-sided hearing loss and aural fullness of 2 years’ duration. Examination revealed ipsilateral post-auricular pulsatile tenderness. Computed tomography showed transcalvarial channels suggestive of dural arteriovenous fistula. Further magnetic resonance imaging demonstrated the presence of a temporal encephalocele herniating through the tegmen tympani defect, as well as the abnormal vascularity. Angiography confirmed a Cognard type I dural arteriovenous fistula, which is being managed conservatively. Surgical repair of the encephalocele was recommended but declined by the patient.ConclusionDural arteriovenous fistula is an uncommon intracranial vascular malformation rarely seen by otolaryngologists, with pulsatile tinnitus being the usual presentation. To our knowledge, this is the first reported case of dural arteriovenous fistula presenting with conductive hearing loss and otalgia.

We prospectively evaluated the surgical, audiologic, and quality-of-life outcomes in 5 patients—2 men and 3 women, aged 22 to 64 years (mean: 41.8)—who were implanted with the Sophono Alpha 2 MPO Processor. The indications for implantation of this bone-conduction device included recurrent ear canal infections with hearing aids (n = 3), single-sided deafness (n = 1), and patient preference in view of difficulty using a conventional hearing aid (n = 1). In addition to the patient with single-sided deafness, 3 patients had a bilateral mixed hearing loss and 1 had a bilateral conductive hearing loss. Outcomes measures included surgical complications, functional gain (FG), speech discrimination in quiet and noise, and patient satisfaction as determined by the Glasgow Benefit Inventory (GBI) and the Entific Medical Systems bone-anchored hearing aid questionnaire (BAQ). The only postsurgical complication noted was a minor skin reaction and pain in 1 patient that resolved with conservative management. In the 3 patients with the mixed hearing loss, the mean FG was 13.3, 20.0, 11.7, and 11.7 dB at 0.5, 1, 2, and 4 kHz, respectively; in the patient with the bilateral conductive hearing loss, the FG was 10, 25, 10, and 15 dB at the same frequencies. Speech discrimination scores with the Sophono device were comparable to those seen with conventional hearing aids. After implantation, all 5 patients experienced a positive quality-of-life outcome according to the GBI, although 1 of them had only a marginal improvement. On follow-up, all patients reported that they remained satisfied with their implant and that they used their device all day long. We conclude that the Sophono bone-conduction system is a safe and effective option that should be considered for patients with a mixed or conductive hearing loss who are unable to use a conventional hearing aid, as well as for those with single-sided deafness.

Congenital Cytomegalovirus (cCMV) is a well-defined cause for neonatal mortality and morbidity, particularly sensorineural hearing loss and other neurodevelopmental disruption. We present a retrospective study which provides an overview of the assessment and preoperative work-up for patients diagnosed with cCMV and their cochlear implant (CI) outcomes.This was a retrospective case series study of all children with a confirmed diagnosis of cCMV who underwent cochlear implantation at St Thomas' Hospital from 2003 to 2015. Data were collected on the preoperative audiology, imaging findings, and neurological assessment. CI outcomes were measured using the Speech Intelligibility Rating (SIR), Category of Auditory Performance (CAP), and Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS).Eleven patients underwent cochlear implantation, 45% had severe-to-profound hearing loss, and 55% had bilateral profound hearing loss. The mean age at initial assessment was 2.1 years (median 1.7, range 0.6-7.5) and the mean age of implantation was 4.0 years (median 2.5, range 0.9-11.8). The mean length of follow-up was 4.8 years (median 2.3, range 1.5-14). Six patients had bilateral simultaneous implantation (55%), four bilateral sequential (36%), and one unilateral (9%). Nine patients had white matter changes on magnetic resonance imaging, largely in the periventricular and cortical regions. Of the 11 patients, 4 (36%) had associated neurological comorbidities and 3 (27%) had additional neurocognitive developmental delay of varying severity. The majority of patients showed improvement in auditory outcomes. No statistically significant correlation was found between age of implantation, neurocognitive, and neurological comorbidities or length of follow-up and hearing outcomes.While the overall outcomes were mixed, most children in our cohort were found to benefit from cochlear implantation. Our data also highlight the significant neurodevelopmental comorbidities associated with cCMV and their negative impact on CI outcomes. With the recent advances in medical treatment, this underlines the importance of multidisciplinary management of these patients.

Active middle ear implants augment sound waves and directly stimulate the middle ear structures. The most frequently utilised active middle ear implant is the Vibrant Soundbridge TM (VSB). CT plays a vital role in appropriate patient selection and surgical planning of active middle ear implant surgery. The VSB TM offers a number of options for implant placement. The ideal location is influenced by the patient’s middle ear and mastoid anatomy as well as the type and severity of the hearing loss. CT provides important information on the surgical access to the middle ear and helps determine the most appropriate implant site by assessing the adjacent middle ear anatomy and the continuity of the ossicular chain. Post-operative active middle ear implant imaging may be indicated in the setting of poor auditory outcomes and when revision surgery is being considered so as to assess for suboptimal implant placement or migration. This pictorial review will describe the VSB TM middle ear device and explain the role of imaging in both the pre-operative and post-operative settings.

Objective: We aimed to compare a newer readout-segmented echoplanar imaging (RS-EPI) technique with the established single shot turbo spin echo (SS-TSE) non-EPI diffusion-weighted imaging (DWI) in detecting surgically validated cholesteatoma. Methods: We retrospectively reviewed 358 consecutive MRI studies in 285 patients in which both RS-EPI and non-EPI DWI sequences were performed. Each diffusion sequence was reviewed independently and scored negative, indeterminate or positive for cholesteatoma in isolation and after reviewing the T 1W sequence. Average artefacts scores were evaluated and the lesion size measured as a distortion indicator. The imaging scores were correlated with surgical validation, clinical and imaging follow-up. Results: There were 239 middle ear and central mastoid tract and 34 peripheral mastoid lesions. 102 tympanomastoid operations were performed. The positive predictive value ( PPV), post-operative PPV, primary PPV, negative predictive value were 93%, 95%, 87.5%, 70% for RS-EPI and 92.5%, 93.6%, 90%, 79% for non-EPI DWI. There was good agreement between the two techniques (k = 0.75). Non-EPI DWI is less susceptible to skull base artefacts although the mean cholesteatoma measurement difference was only 0.53 mm. Conclusion: RS-EPI has comparable PPV with non-EPI DWI in both primary and post-operative cholesteatoma but slightly lower negative predictive value. When there is a mismatch, non-EPI DWI better predicts the presence of cholesteatoma. There is good agreement between the sequences for cholesteatoma diagnosis. The T 1W sequence is very important in downgrading indeterminate DWI signal lesions to a negative score. Advances in knowledge: This is, to our knowledge, the first study to compare a multishot EPI DWI technique with the established non- EPI DWI in cholesteatoma diagnosis.

Objective:To report a novel management strategy for mixed hearing loss in advanced otosclerosis.Methods:A 50-year-old male was referred to St Thomas’ Hearing Implant Centre with otosclerosis; he was no longer able to wear conventional hearing aids because of recurrent otitis externa. The patient underwent short process incus vibroplasty (using the Med-El Vibrant Soundbridge device), followed at a suitable interval (six weeks) by stapes surgery. The main outcome measures were: pure tone audiometry, functional gain and monosyllabic word recognition scores.Results:Post-operative pure tone audiometry showed a reduction of the mean air–bone gap from 55 dB HL to 20 dB HL. The residual mixed hearing loss was rehabilitated with the Vibrant Soundbridge, with an average device gain of 32 dB. The monosyllabic word recognition scores in quiet at 65 dB improved from 37 to 100 per cent when using the Vibrant Soundbridge at six months after switch-on of the device.Conclusion:Stapedotomy in conjunction with incus short process vibroplasty (i.e. inner-ear vibroplasty) is a safe and promising procedure for managing advanced otosclerosis with mixed hearing loss in selected patients.

Perioperative Minnesota Multiphasic Personality Inventory (MMPI) scores may be beneficial for predicting prognosis of cochlear implantation (CI). A positive attitude for social interaction in particular correlates with a better speech outcome. Proper perioperative psychological management may, therefore, assist in the auditory rehabilitation of CI patients.To determine the perioperative psychological state of CI patients and its relationship with patient prognosis after CI.This study prospectively enrolled 29 patients who underwent CI from 2005-2013. The MMPI was administered to assess psychosocial and emotional issues surrounding CI and the Korean version of the Central Institute of Deafness (K-CID) score was used to measure speech perception.CI resulted in a significant improvement on the MMPI Paranoia scale (p = 0.02). Patients with abnormal pre-operative and post-operative MMPI scores also had an earlier onset of deafness, longer duration of deafness, and lower K-CID scores than patients with normal MMPI scores (all p 0.05). The post-CI K-CID score had a significant negative correlation with the pre-operative MMPI Schizophrenia score (p 0.01) and significant negative correlations with the post-operative MMPI Paranoia (p = 0.02), Psychasthenia (p = 0.02), Schizophrenia (p = 0.04), Hypomania (p = 0.02) and Social Introversion (p = 0.03) scores.

The retrotympanic anatomy is complex and variable but has received little attention in the radiological literature. With advances in CT technology and the application of cone beam CT to temporal bone imaging, there is now a detailed depiction of the retrotympanic bony structures. With the increasing use of endoscopes in middle ear surgery, it is important for the radiologist to appreciate the nomenclature of the retrotympanic compartments in order to aid communication with the surgeon. For instance, in the context of cholesteatoma, clear imaging descriptions of retrotympanic variability and pathological involvement are valuable in pre-operative planning. The endoscopic anatomy has recently been described and the variants classified. The retrotympanum is divided into medial and lateral compartments with multiple described potential sinuses separated by bony crests. This pictorial review will describe the complex anatomy and variants of the retrotympanum. We will describe optimum reformatting techniques to demonstrate the structures of the retrotympanum and illustrate the associated anatomical landmarks and variants with CT. The implications of anatomical variants with regards to otologic surgery will be discussed.

Objective: We aimed to compare a newer readout-segmented echoplanar imaging (RS-EPI) technique with the established single shot turbo spin echo (SS-TSE) non-EPI diffusion-weighted imaging (DWI) in detecting surgically validated cholesteatoma. Methods: We retrospectively reviewed 358 consecutive MRI studies in 285 patients in which both RS-EPI and non-EPI DWI sequences were performed. Each diffusion sequence was reviewed independently and scored negative, indeterminate or positive for cholesteatoma in isolation and after reviewing the T1W sequence. Average artefacts scores were evaluated and the lesion size measured as a distortion indicator. The imaging scores were correlated with surgical validation, clinical and imaging follow-up. Results: There were 239 middle ear and central mastoid tract and 34 peripheral mastoid lesions. 102 tympanomastoid operations were performed. The positive predictive value ( PPV), post-operative PPV, primary PPV, negative predictive value were 93%, 95%, 87.5%, 70% for RS-EPI and 92.5%, 93.6%, 90%, 79% for non-EPI DWI. There was good agreement between the two techniques (k = 0.75). Non-EPI DWI is less susceptible to skull base artefacts although the mean cholesteatoma measurement difference was only 0.53 mm. Conclusion: RS-EPI has comparable PPV with non-EPI DWI in both primary and post-operative cholesteatoma but slightly lower negative predictive value. When there is a mismatch, non-EPI DWI better predicts the presence of cholesteatoma. There is good agreement between the sequences for cholesteatoma diagnosis. The T1W sequence is very important in downgrading indeterminate DWI signal lesions to a negative score. Advances in knowledge: This is, to our knowledge, the first study to compare a multishot EPI DWI technique with the established non- EPI DWI in cholesteatoma diagnosis.

Objectives/Hypothesis To evaluate the outcome of cochlear implantation (CI) in patients with vestibular schwannoma (VS). Study Design A retrospective case series from a tertiary auditory implant center. Methods A retrospective case note review was carried out to evaluate patients with bilateral profound hearing loss and VS who underwent unilateral CI within the Auditory Implant Centre at St. Thomas' Hospital, London, between 2000 and 2012. This included both bilateral VS with neurofibromatosis type 2 (NF2) and unilateral sporadic VS. Outcome measures included speech perception with Bamford-Kowal-Bench and City University of New York sentences, sound-field thresholds with warble tones, and the subjective benefits reported by patients. Results The study included five patients with NF2 and bilateral VS and two patients with sporadic unilateral VS. The standard preoperative audiologic assessment for CI often could not be carried out in NF2 patients. Preoperative testing was more complete in the two patients with sporadic VS. The audiologic outcome was variable. Open-set speech perception was achieved in three out of five NF2 patients, and another reported significant improvement in environmental sound perception and ease of communication. The outcome was overall better in patients with sporadic VS, both of whom were able to use the telephone in their implanted ear. Conclusions Good speech perception can be achieved in some cases, and CI should be considered as an option for auditory rehabilitation in patients with VS. Level of Evidence 4. Laryngoscope, 123:2019–2023, 2013

Most studies on development of the human auditory system and hearing loss have to date focused on the sensory apparatus, the cochlea. With advancements in magnetic resonance imaging (MRI), there has recently been increasing interest in the subject of cochlear nerve deficiency (CND) and dysfunction. Cochlear nerve deficiency (CND) amongst individuals with congenital sensorineural hearing loss (SNHL) is not as rare as previously thought, with prevalence as high as 18–21 % reported amongst cochlear implant recipients. Cochlear nerve (CN) morphogenesis is a complex process involving cell populations from two disparate progenitors of the otic placode and neural crest cells. In the first trimester, the basic foundation of the auditory pathway is laid down, with the vestibulocochlear ganglion cells delaminating from the otocyst and establishing peripheral and central connections with the developing cochlea and brainstem, respectively. The second trimester is a period of proliferation, growth and myelination. As the number of axons is pruned back closer to the adult level, myelination begins in the intra-cochlear portion of CN and extends proximally. In the third trimester, further maturation of the neuronal connections in conjunction with paralleled development of the cochlea and brainstem leads to emergence of foetal responses to auditory stimuli. Based on the currently available knowledge of the embryological development of CN, various phenotypes of CND are discussed. It is hoped that better understanding of CN ontogenesis will not only lead to further refinement of auditory implant candidacy but also open doors to potential regeneration therapies such as stem cell therapy in the future.

The bone-anchored hearing aid (BAHA) system can offer significant benefits to patients with single-sided deafness (SSD), primarily by lifting the head shadow effect.To evaluate the efficacy of BAHA for SSD by comparing pre- and postoperative speech, spatial and qualities of hearing scale (SSQ) scores.This was a prospective study conducted within a tertiary auditory implant department. The inclusion criteria were unilateral profound hearing loss with normal or mild high frequency hearing loss in the hearing ear (pure tone average better than or equal to 25 dBHL measured at 0.5, 1, 2 and 3 kHz) and subjective benefits reported by patients following a home trial with a BAHA Softband. Patients who met the above criteria and opted for surgery were asked to complete the SSQ questionnaire. The postoperative SSQ response was collected after at least 6 months of consistent BAHA usage.This study included 25 adult patients (mean age at implantation 57.5 years). There was a statistically significant improvement in the average SSQ score in all three sections of the questionnaire with the use of the BAHA. Our patients experienced most marked benefits in speech hearing in challenging listening situations. All patients remain consistent users and there has been no explantation to date.

Sickle cell disease (SCD) is associated with sensorineural hearing loss (SNHL). Although the hearing loss is usually mild, some develop severe-to-profound hearing loss, in whom cochlear implants (CI) may be an option. We present the cases of two children with SCD who developed bilateral severe-to-profound SNHL and underwent cochlear implantation. One patient became profoundly deaf after an acute episode of dizziness. Imaging indicated bilateral cochlear ossification, making subsequent cochlear implant surgery challenging. The second patient developed bilateral severe-to-profound SNHL following acute vaso-occlusive crises. She went on to have uncomplicated cochlear implant surgery. These cases illustrate the variable manner in which children with SCD may develop SNHL, and the difficulties associated with managing such cases. We recommend that children with SCD should undergo regular audiological assessment. Furthermore, clinicians should be aware of the risk of cochlear fibrosis and ossification and ensure prompt assessment following an acute vaso-occlusive crisis or unexplained vestibulocochlear event.

The study was conducted to analyze outcomes following surgical management of large and giant vestibular schwannomas and management options for residual disease. This retrospective case note study includes patients who had undergone microsurgical resection of sporadic, large, or giant vestibular schwannomas from 1986 to 2008. Tumors are classified as large if the largest extracanalicular diameter was 3.5 cm or greater and giant if 4.5 cm or greater. The study included 45 patients (33 large, 12 giant tumors), mean tumor size 4.1 cm. Total excision was achieved in 14 cases (31.1%), near-total in 26 (57.8%), and subtotal in 5 (11.1%). Facial nerve outcome was House-Brackmann Grade I/II in 25 cases (55.6%), III/IV in 16 (35.6%), and V/VI in 4 (8.9%). No recurrence has been detected in those undergoing a complete resection. No residual tumor growth been observed in 15 of 26 who underwent near-total resection (57.7%). Of 11 patients, 10 received further treatment as their residual tumors showed growth. In the subtotal excision group, one patient died, three have demonstrated no growth, and one residual tumor has grown slightly but not required intervention. Optimal management for patients with large or giant vestibular schwannomas has yet to be determined. Management decisions must balance long term function with tumor control.

Carpal tunnel decompression is a procedure commonly performed by many orthopaedic surgeons. Although there is no general consensus on the best technique in terms of acceptability to patients and operative outcome, some surgeons have a specific personal preference, in particular with regard to the use of a tourniquet and the timing of its release. A prospective randomised controlled trial was performed to determine whether or not deflation of the tourniquet and achieving haemostasis prior to wound closure influenced the outcome of carpal tunnel decompression under local anaesthesia. After division of the retinaculum, 26 wrists were randomised to tourniquet released prior to closure and haemostasis with bipolar diathermy and 24 after closure and dressing application. Immediately after surgery, all patients were asked to indicate the maximum level of pain at the operative and tourniquet sites experienced intra-operatively using a visual analogue score. Patients were assessed for wound healing and symptom relief at 2 and 6 weeks post-operatively. Results were analysed using the Wilcoxon 2-sample test. The use of local anaesthesia and tourniquet was well tolerated in both groups. There was no statistically significant difference between the two groups in pain at the operative or tourniquet site or in wound healing and symptom relief at 2 and 6 weeks post-operatively. Open carpal tunnel decompression under local anaesthesia with tourniquet control is a safe, effective and acceptable technique. The timing of tourniquet release does not affect patient satisfaction, wound healing or symptom relief.

Computed tomography (CT) of the neck, chest, abdomen and pelvis is the most appropriate initial investigation following a fine needle aspiration cytology (FNAC) diagnosis of metastatic adenocarcinoma in cervical lymph nodes with unknown primary. PET-CT should be considered as the next step if the initial CT fails to identify the primary site, but its true value is yet to be determined.To review investigation strategies for metastatic adenocarcinoma of unknown primary presenting as cervical lymphadenopathy, and to develop a management algorithm.This was a retrospective case note study from two regional head and neck cancer centres in the UK. Adult patients with FNAC diagnosis of metastatic adenocarcinoma in cervical lymph nodes between 1998 and 2008, with a minimum 5-year follow-up, were included. Patients with a clinically obvious primary tumour or a previous history of adenocarcinoma were excluded.This study examined 41 cases. CT of the neck, chest, abdomen and pelvis was the most useful initial investigation. It identified the primary tumour site in 16/28 cases (57%), detected the primary tumour and led to revision of the FNAC diagnosis in 1 case (2.4%), and was necessary for the final diagnosis of true unknown primary in 12 cases (29.3%). Targeted imaging was not helpful.

Aim To investigate the aetiology and clinical consequences of incudo-stapedial (IS) discontinuity when it is demonstrated on computed tomography (CT) within a fully aerated middle ear and mastoid. Methods and materials Patients with CT evidence of IS discontinuity within a fully aerated middle ear and mastoid were prospectively identified. Clinical history, otoscopic findings, audiometry, and CT data were evaluated. Predefined criteria were used to determine the likely aetiology of IS discontinuity, whether it was diagnosed prior to the CT study, and the clinical consequences in terms of degree of conductive hearing loss and requirement for surgical correction. The range of CT appearances was evaluated. Results The IS discontinuity in 34/36 ears was felt to be due to incus erosion secondary to chronic otitis, on the basis of clinical history and otoscopic findings. The IS discontinuity was rarely evident prior to CT with long-process deficiency being identified in only 5/36 cases. The mean air bone gap was only 22.5 dB. The ossicular defect was surgically addressed in only four cases. The incus deficiency was confined to the lower-third on CT in 19/36 cases. Conclusion When IS discontinuity is demonstrated within a fully aerated middle ear and mastoid, the most likely aetiology is of acquired incus erosion due to chronic otitis media. The IS discontinuity on CT is usually not evident otoscopically. It usually results in only mild conductive hearing loss and the ossicular discontinuity was rarely surgically addressed in the present series.

Objective To determine whether hydrogen peroxide (H 2 O 2 ) mouthwash influences the outcome of secondary post-tonsillectomy hemorrhage in children. Study design Ten-year retrospective study of all children with secondary post-tonsillectomy hemorrhage. Setting Tertiary otolaryngology center. Results Of the 156 patients, 59 received H 2 O 2 and 97 did not. All patients received broad-spectrum intravenous antibiotics. The average rehospitalization duration due to hemorrhage was 1.7 days (H 2 O 2 group) and 1.6 days (control group). In the H 2 O 2 group, 8.5% required surgery, compared with 10.3% in the control group. Further hemorrhage episodes requiring readmission occurred in 3.4% of the H 2 O 2 group and 3.1% of controls. There was no difference between the 2 groups in rehospitalization duration ( P = 0.49), rate of surgical intervention ( P = 0.85), and rate of readmission with further hemorrhage ( P = 0.92). Conclusion Hydrogen peroxide mouthwash does not improve the outcome of secondary post-tonsillectomy hemorrhage in pediatric patients. Significance This study does not support the common practice of treating post-tonsillectomy hemorrhage with H 2 O 2 .

The commonest subglottic mass in infants is a congenital haemangioma, which is usually managed conservatively without a histological diagnosis. Ectopic cervical thymus is rare and usually presents as a cervical mass, with only one case of subglottic ectopic thymus reported to date. Due to its rarity, the diagnosis in most cases relies on surgical excision and histological examination. However, histological diagnosis may not always be easily reached, as is demonstrated in this case report. In this article, an infant with congenital stridor secondary to a subglottic mass is described and the clinical and diagnostic difficulties in its management are discussed.