The impact of multiple deprivation on the management of vestibular schwannomas.

PURPOSE: While some factors have been well-shown to affect the decision-making in treating patients with vestibular schwannomas (VS), little is known on the role of deprivation. Our objective was to assess the effect of socioeconomic background on the management of patients with VS. METHODS: This retrospective cohort study included 460 patients with sporadic VS from West of Scotland. The postcode-based, multifactorial Scottish Index of Multiple Deprivation (SIMD) was used to assess the socioeconomic background of each patient. We performed a multivariate analysis including tumour size, growth and patient age with management modality (observation, stereotactic radiotherapy, microsurgery) being the main outcome measure and outcome (need for additional treatment) an additional measure. RESULTS: We found no significant difference in the demographics, tumour characteristics and primary treatment choice between patients with different SIMD scores. In addition, there was no statistically significant difference in the growth occurrence rates following first-line treatment (p = 0.964) and in the second-line treatment choice (p = 0.460). CONCLUSIONS: Multiple deprivation does not affect decision making in patients with VS in the examined cohort. This is probably linked to the centralisation and uniformity of the service and might not necessarily be applicable to other health services without centralisation.

Long-term growth patterns of vestibular schwannomas after stereotactic radiotherapy: delayed re-growth.

PURPOSE: To determine the long-term outcomes of patients with vestibular schwannomas (VS) after stereotactic radiosurgery (SRS) who experience delayed tumour regrowth. METHODS: We carried out a retrospective case series in tertiary university settings. We included patients with VS with initial response to SRS and delayed regrowth, assessing a database of 735 patients with VS and 159 patients who had SRS as sole treatment. Following SRS, all patients had clinical follow-up and serial magnetic resonance imaging (MRI). We documented the post-SRS clinical assessment, pre- and post-SRS VS size as per MRI in predetermined time periods, response to treatment and rate of (re-) growth and the final outcome in each case. RESULTS: We identified six patients with good initial response but delayed VS regrowth at a faster rate than pre-SRS. The mean growth rate for these VS was 0.347 mm/month (range 0.04-0.78 mm/month) prior to treatment; the mean growth rate at the time of delayed re-growth was 0.48 mm/month (range 0.17-0.75 mm/month); this did not reach the level of statistical significance (p = 0.08). This regrowth occurred at a mean time of 42 months (range 36-66 months) post-SRS and stopped 22 months (mean, range 12-36 months) post regrowth detection in all cases. CONCLUSIONS: Given that delayed post-SRS VS regrowth can occur in approximately 4% of the treated cases, it is important to continue close clinical and radiological follow-up. Despite this abnormal behaviour, VS do stop growing again; still, patients should be made aware of the possibility of this uncommon VS behaviour following SRS.

Duration of symptom control following intratympanic dexamethasone injections in Meniere's disease.

PURPOSE: Intratympanic (IT) injections of corticosteroids have emerged as a non-ablative alternative to gentamicin in the management of refractory Meniere's disease. However, currently, the duration of the symptom control achieved via intratympanic corticosteroids is under reported. METHODS: We retrospectively reviewed the notes of all patients who underwent IT injections of dexamethasone for the treatment of definite Meniere's disease at a single tertiary referral university centre over a 6-year period. We included demographic information, the number of procedures patients required, duration of symptom-control achieved (time interval between repeat IT injections), and the presence of co-morbidities, with a focus on the presence of autoimmune disease. RESULTS: We identified 27 patients who underwent a total of 42 procedures; 23/27 (85.2%) patients demonstrated clinical response with a median period of symptom control of 14.5 months (range 1-64, IQR 10.25). The median longest asymptomatic period per patient was 19 months (range 11-64, IQR: 18). Interestingly, all patients with autoimmune disease (7/27) demonstrated a clinical response; autoimmune disease was found to be a statistically significant predictor of response to treatment (p = 0.002). In patients who received repeated treatment following disease relapse, there was no difference in duration of symptom-control achieved. CONCLUSIONS: IT steroids can provide an effective alternative to gentamicin ablation. Symptom control is achieved for a median of 14.5 months, and treatment can be repeated with no loss of efficacy. Those patients who have an underlying autoimmune co-morbidity are more likely to demonstrate a clinical response to therapy, which may provide insight into the underlying pathophysiology of Meniere's disease.

The significance of vascular loops in the internal auditory meatus: a true incidental imaging finding?

PURPOSE: To determine the clinical significance of vascular loops (VL) in the internal auditory meatus (IAM) and cerebellopontine angle (CPA). METHODS: We carried out a retrospective case series in a tertiary referral centre. Out of 6978 patients undergoing magnetic resonance imaging (MRI) of the IAM for unilateral cochleovestibular symptoms we identified the ones with VLs and reviewed their medical notes. We performed a statistical correlation between the laterality of the VL in the IAM/ CPA as graded according to the Chavda classification (type 1 in the CPA, type 2 extending in the IAM, type 3 extending to the distal IAM end), the laterality of symptoms and the patient's age. RESULTS: A total of 77 VL were identified in 64 patients (0.9%); 39 patients had the VL on the same side of the main symptom, while 25 patients had the VL on the contralateral side. There were 37 Type 1 loops, 29 Type 2 loops and 11 Type 3 loops. The comparison between the grading of the VL and the laterality of symptoms did not reach the level of significance (p = 0.321). There was also no association between the presence of the loop and the patients' age (p = 0.5). All patients were reassured and discharged without any representation in three years follow-up. CONCLUSIONS: We did not identify any significant correlation between the laterality of VLs and the laterality of symptoms, irrespective of the grading of the loop or the patients' age. Such VLs should be considered an incidental rather than causal findings.

Ménière's Disease and Underlying Medical and Mental Conditions: Towards Factors Contributing to the Disease.

PURPOSE OF THE STUDY: To investigate the relation of Ménière's disease (MD) with medical comorbidity or mental conditions. PROCEDURES: Demographic data, medical and mental comorbidities were retrospectively collected and compared from 3 groups of 30 patients each: a group with patients with definite MD, a second one with patients with vestibulopathies other than MD (non-Ménière's vertigo, NMV) and a third one with patients without any vestibular symptoms (control). The level of significance was set at 0.05. RESULTS: The prevalence of mental conditions was 26.7, 23.3 and 6.7% for the MD, the NMV and the control group, respectively. Medical comorbidity was found in 80% of patients in the MD, 63% in the NMV and 20% in the control group. Arthritis was encountered in 8 patients with MD, 3 with NMV and none from the control group. The differences in prevalence of mental disease, comorbidities and arthritis between the MD and the control group were statistically significant (p = 0.02, p < 0.001 and p = 0.008, respectively). CONCLUSION: Psychological conditions play an important role in patients with MD. The significantly increased prevalence of arthritis in the MD group suggests an inflammatory/autoimmune background as a contributing factor to the disease.

Progress of hearing loss in neurofibromatosis type 2: implications for future management.

The objective of this study was to describe changes in hearing over time in patients with neurofibromatosis type 2 (NF2) treated conservatively. A retrospective case review was conducted in a tertiary referral centre. Pure tone audiometry, speech discrimination scores, serviceable hearing (American Academy of Otolaryngology class A or B) and measurement of vestibular schwannoma (VS) size on magnetic resonance imaging were evaluated in 56 patients (89 ears) with NF2 with at least one conservatively managed VS. Over a mean follow-up period of 7 years (range 0.8-21 years) pure tone average thresholds increased gradually with a mean annual rate of 1.3 dB for the right ear (p = 0.0003) and 2 dB for the left ear (p = 0.0009). Speech discrimination scores dropped with an average annual rate of 1.3 and 0.34% in the right and left ear, respectively. Patients maintained serviceable hearing for an average of 7.6 years (range 2.7-19.3 years). The average annual VS growth was 0.4 mm without any correlation with hearing loss. There was a correlation between patients' age and pure tone threshold increase (p < 0.05 for both ears). In this selected population of patients with NF2, hearing threshold increases were very slow. In NF2 patients with indolently behaving tumours, serviceable hearing can be maintained for a significant length of time, making conservative management an attractive option.

The effects of insertion speed on inner ear function during cochlear implantation: a comparison study.

OBJECTIVE: To investigate the insertion speed and its impact on electrode insertion characteristics, hearing preservation and clinical vestibular function in a prospective cohort study with a retrospective control group at a tertiary otology/neurotology centre. INTERVENTIONS: Hearing-preserving cochlear implantation using systemic and topical steroids in conjunction with a round-window approach, a complete cochlear coverage electrode and two different electrode insertion speeds [60 mm/min (n = 18) vs. 15 mm/min (n = 22)] was performed. RESULTS: The insertion speed had a significant impact on various insertion characteristics as well as hearing preservation and vestibular function. In conclusion, a slow electrode insertion speed appears to facilitate full electrode insertion, reduce the occurrence of insertion resistance as well as promote preservation of residual hearing and vestibular function after cochlear implantation.

A systematic review and meta-analysis of stereotactic radiosurgery as a primary treatment in fast-growing vestibular schwannomas.

BACKGROUND: Stereotactic radiosurgery has been shown to be an effective method of managing vestibular schwannomas. The primary aim here is to establish the impact of pre-treatment fast-growing vestibular schwannomas on the efficacy of stereotactic radiosurgery. METHODS: PubMed, Medline and Embase databases were used. The ROBINS-I ('Risk Of Bias In Non-randomised Studies - of Interventions') tool was utilised to assess for risk of bias. Proportionate meta-analysis and sub-analysis for fast-growing tumours were performed to explore the success rate of stereotactic radiosurgery in stabilising or decreasing the tumour burden in vestibular schwannomas. RESULTS: Four moderate risk studies were included in the analysis. Overall, 91 per cent (95 per cent confidence interval = 0.83-0.97, p < 0.01, I2 = 80 per cent) of the tumours demonstrated successful size reduction or stabilisation following stereotactic radiosurgery. Nevertheless, the efficacy of stereotactic radiosurgery in reducing or stabilising fast-growing vestibular schwannomas decreased by 79 per cent (95 per cent confidence interval = 0.64-0.91, p = 0.11, I2 = 62 per cent). CONCLUSION: Stereotactic radiosurgery has a statistically significant success rate in stabilising or decreasing the vestibular schwannoma size. This success rate is diminished in fast-growing vestibular schwannomas.

Investigation of serum calcium and vitamin D levels in superior semicircular canal dehiscence syndrome: A case control study.

Objective: It remains unknown whether calcium metabolism has any effect on the clinical presentation of superior semicircular canal dehiscence (SSCD). Our aim was to analyse the adjusted calcium and vitamin D levels in SSCD patients compared to a control group. Methods: This was a prospective case-control study performed in a tertiary referral center, university teaching hospital in the UK. It included all new patients with SSCD seen in a dedicated skull base clinic over a 5-year period (2015-2019) compared to a gender and age matched control group. The main outcome of the study was adjusted calcium and Vitamin D levels between the two groups. Results: A total of 31 SSCD patients were recruited with a matched number of control patients. The mean Vitamin D level on the SSCD group was 44.8 nmoL/l (SD: 20.8) compared to 47.5 nmoL/l (SD: 27.4) on the control group (p = 0.702). Mean Adjusted calcium level was 2.34 mmoL/l (SD: 0.7) for SSCD compared to 2.41 mmoL/l (SD: 0.11) for controls (p = 0.01), being within normal limits for both the SSCD and the control group. Conclusion: Our study did not identify a link between Vitamin D levels and presence of SSCD. Normal adjusted calcium values were found in both groups. Despite that a statistically significant lower calcium level was found in the SSCD group which could indicate that suboptimal levels of calcium may affect the micro-environment of the otic capsule at the SSC region.

Radiological diagnosis of incomplete partition type I versus type II: significance for cochlear implantation.

OBJECTIVES: To investigate an extended cohort of patients with incomplete partition (IP) and examine the adequacy of the existing classifications based on radiological criteria and on their implications for cochlear implantation. METHODS: Patients with IP admitted to a tertiary referral centre during the period 2000-2010 were retrospectively examined. The subjects were initially classified into IP-I, IP-II and atypical cases. For cochlear implant recipients relevant aspects were analysed. RESULTS: Eighty-three ears (49 patients) with IP were found, 19 with IP-I, 54 with IP-II and 10 atypical. Thirty-three patients received a cochlear implant (11 with IP-I, 19 with IP-II and three atypical) achieving heterogeneous though mostly promising results. Cerebrospinal fluid gushing was the commonest surgical complication, particularly in cases of IP-I. In general, patients with IP-II performed better than those with IP-I. CONCLUSIONS: Cochlear implantation promises adequate hearing rehabilitation for most patients with IP. As we move from IP-I to IP-II better results and lower risk for gushing shall be expected. Based on radiological findings we suggest a modified classification into IP-I, atypical IP-I (with large vestibular aqueduct (LVA) and better partition), IP-II (Mondini deformity) and atypical IP-IIa (without LVA) and b (without LVA but with semicircular canal dysplasia). KEY POINTS: • Radiological (CT and MR) features are of crucial importance for cochlear implantation • Imaging can identify two types of incomplete cochlear partition and atypical cases • Detailed pre-operative radiological assessment can help predict complications and outcome • A more comprehensive radiological classification of these anomalies is proposed.

The vestibulocochlear nerve: aplasia and hypoplasia in combination with inner ear malformations.

OBJECTIVE: To determine features of hypoplasia and aplasia of the vestibulocochlear nerve (VCN) in combination with inner ear malformations (IEMs). METHODS: Index cases were retrospectively selected from all IEMs collected since 1995. CT and MRI data were reviewed by two neuroradiologists. The number and thickness of visible nerves in the cerebellopontine angle cistern and inside the internal auditory canal (IAC) were analysed. RESULTS: MR images for 176 patients with IEMs were analysed. Labyrinthine aplasia and otocyst deformity showed 100% correlation, and IAC malformations exhibited 92% correlation with VCN aplasia. Cochlear aplasia, complete aplasia of the semicircular canals, severe cochlear hypoplasia, common cavity, incomplete partition type 1 and mild cochlear hypoplasia showed decreasing degrees of correlation with hypoplasia of the VCN. The remaining types of IEM did not demonstrate VCN hypoplasia. CONCLUSIONS: Certain forms of IEM show 100% correlation with hypoplasia or aplasia of the VCN, while others correlate less strongly and some do not usually exhibit VCN hypoplasia. MRI should always be carried out for those forms often correlated with VCN hypoplasia. KEY POINTS: Vestibulocochlear nerve deficiency can be strongly suspected in certain inner ear malformations • Bony cochlear aplasia and cochlear nerve aplasia are strongly correlated • In semicircular canal aplasia, hypoplasia of the vestibular nerve can be found • Before cochlear implantation, the type of any IEM should be fully understood.

Controlling the position and the dislocation of the middle ear transducer with high-resolution computed tomography and digital volume tomography: implications for the transducers' design.

A minimal tip dislocation of the middle ear transducer (MET(®), Otologics Ltd) may result in poor hearing performance. Our objective was to examine if a defined MET dislocation can be diagnosed by high-resolution computed tomography (HRCT) or digital volume tomography (DVT). A human cadaver head was sequentially implanted with different MET tips (incus application) including a ceramic tip (T 1c), a titanium tip (T 1t), a new, thinner titanium tip (T 2), and a spherical titanium tip (Ts). HRCT and DVT studies were performed. Afterward, the tips were pulled back 0.5 mm, so that they were not attached to the incus. HRCT and DVT scans were repeated to identify the dislocation. Using the best plain in HRCT images, the dislocation of the transducer could be measured reliably and reproducibly in half of the cases. In particular, the precise positioning and the dislocation could be identified when T 1t and Ts were implanted, with the Ts showing the best visibility. DVT failed in recognizing the dislocation in all cases. The identification of MET tip's dislocation with HRCT depends on the shape, size, and material of the tip. This knowledge is useful for the design of the implants, as determination of the right position of the middle ear transducer may be proven important for the hearing outcome. In some cases, however, surgical exploration may still be required. Although DVT represents a promising imaging method for the otologists, it can barely help when MET dislocation is suspected.

A longitudinal-torsional mode ultrasonic needle for deep penetration into bone.

This work presents a longitudinal-torsional (L-T) composite mode ultrasonic needle device for deep bone penetration. The L-T needle is a geometrically modified version of an L-mode needle whose efficacy as a prototype ultrasonic bone biopsy device has been previously demonstrated by the authors. Finite element analysis (FEA) aided in the design of the L-T needle, with the aim of maximising the achievable torsional displacement while matching the longitudinal displacement achieved by the L-mode needle. Experimental modal analysis (EMA) of the fabricated ultrasonic device was used to identify the modal parameters and validate the FEA model. Harmonic analysis then provided an insight into how the inherent nonlinearities of the high-power transducer are affected by incorporating the geometrical features that degenerate the L mode into an L-T mode. High power characterisation shows that the longitudinal displacement amplitude of the L-T mode needle is larger than that of the L-mode needle. Comparative penetration tests in fresh Wistar rat skull were evaluated by investigating cell death and cell survival. The region of statistically significant cell death was small for both devices, with the combined axial and shear motion of the L-T device causing increased osteocyte necrosis within this region. Nevertheless, the results suggest a promising environment for post-operative healing. It is shown how this technology offers a potential technique for a surgical approach to the petrous apex, an application that requires a deep penetration into bone.

IgG4-Related Sclerosing Disease of the Temporal Bone: A Systematic Review.

OBJECTIVE: IgG4-related disease (IgG4-RD) involving the temporal bone is an uncommon and underrecognized pathology often mistaken for malignancy. This systematic review is the first that aims to thoroughly analyze IgG4-RD of the temporal bone. DATABASES REVIEWED: Ovid MEDLINE, EMBASE, Cochrane Library, and Google Scholar. METHODS: We used the following search keywords: "lgG4-RD," "skull," "skull base," "cranial," "temporal bone," "inner ear." We additionally manually searched the bibliographies of relevant articles. The JBI Critical Appraisal Checklist for Case Reports and Case Series was used to assess the risk of bias; because of the scarcity of the reports, data were available through limited case series and reports; thus, data synthesis was not possible. RESULTS: We identified 17 studies with 22 cases with temporal bone involvement. The most common presenting symptoms were hearing loss, otalgia, and headache. The mastoid and petrous bone were the most affected anatomical areas. Both computed tomography and magnetic resonance imaging were used. Biopsies showed the characteristic lymphoplasmacytic infiltrate in all cases, with histopathology being the diagnostic modality that set the diagnosis. Most patients were treated with corticosteroids ± surgery or a combination of corticosteroids and immunosuppressants with 95.5% symptomatic response and disease control. CONCLUSION: IgG4-RD of the temporal bone radiologically manifests as space-occupying, lytic lesions; clinically, it presents with vague otological symptoms. Diagnosis involves a thorough workup, with histopathology being crucial in setting a definite diagnosis. IgG4-RD tends to respond well to systemic corticosteroids, whereas surgery is mostly required for diagnostic purposes.

Vitamin D Supplementation for Benign Paroxysmal Positional Vertigo: A Systematic Review.

OBJECTIVE: Benign paroxysmal positional vertigo (BPPV) is commonly attributed to displaced otoconia. These have been shown to have biomineralization close to that of bone, and vitamin D deficiency has been associated with BPPV. We aim to systematically review the available literature on vitamin D supplementation and BPPV intensity and recurrence in adults. DATABASES REVIEWED: PubMed, MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials (CENTRAL), Current Controlled Trials, and ClinicalTrials.gov. METHODS: We systematically reviewed the available literature from 1947 to April 2020. The study protocol was registered in the PROSPERO database (trial registration: CRD42020183195). RESULTS: A total of 179 abstracts were identified and screened by two independent reviewers. Based on inclusion and exclusion criteria, six studies were selected and subjected to a quality assessment. In one randomized clinical trial (RCT), vitamin D supplementation was found to reduce annual recurrence rate of vertigo in patient with BPPV and subnormal serum vitamin D levels compared with placebo (odds ratio, 0.69; 95% confidence interval, 0.54-0.90). Non-RCTs demonstrated the possibility of a null effect in the random effects model (odds ratio, 0.08; 95% confidence interval, 0.00-1.56). The RCT considered as low risk of bias. All of the nonrandomized studies were assessed as serious risk of bias. CONCLUSIONS: The intervention studies identified consistently demonstrated a decrease in BPPV recurrence with supplementation of vitamin D in patients with subnormal vitamin D levels. Although there is a paucity of high-quality studies, the present literature does highlight a role for optimization of vitamin D levels in patients with BPPV.

Ear and further anatomic anomalies in children undergoing stapedotomy.

PURPOSE: Aim of this study was to examine any stapes fixation-related clinical, intraoperative and high-resolution computed tomography (HRCT) findings in children undergoing stapedotomy. PROCEDURES: Detailed diagnostic assessment was performed in 12 children (18 ears) who underwent stapedotomy within the period 2005-2008. RESULTS: Ear malformations such as malleus-incus anomalies, malleus-epitympanum fixation and absence of the stapedius tendon, small external auditory canal and auricle malformations were clinically and/or intraoperatively diagnosed. HRCT identified a petrous high jugular bulb and malformed inner auditory canal in 4 ears; however, it failed to recognize ossicular fixation. Systemic anomalies, namely kyphoscoliosis, esophageal atresia and finger malformations were identified in 2 children. Summing up, additional anatomic anomalies were found in 8 patients (66.7%). CONCLUSIONS: Ear malformations may coexist in children with stapes fixation. Ossicular fixation is not easily recognized with HRCT, and therefore, ossicular mobility should always be tested intraoperatively. In a few cases, pediatric stapes fixation can coexist with systemic dysplasias; in such patients, additional referral to geneticists is recommended.