A Multicenter Validity Study of Four Smartphone Hearing Test Apps in Optimized and Home Environments.

OBJECTIVE: Pure tone audiometry (PTA) is the gold standard for hearing assessment. However, it requires access to specialized equipment. Smartphone audiometry applications (apps) have been developed to perform automated threshold audiometry and could allow patients to perform self-administered screening or monitoring. This study aimed to assess the validity and feasibility of patients using apps to self-assess hearing thresholds at home, with comparison to PTA. METHODS: A multi-center, prospective randomized study was conducted amongst patients undergoing PTA in clinics. Participants were randomly allocated to one of four publicly-available apps designed to measure pure tone thresholds. Participants used an app once in optimal sound-treated conditions and a further three times at home. Ear-specific frequency-specific thresholds and pure tone average were compared using Pearson correlation coefficient. The percentage of app hearing tests with results within ±10 dB of PTA was calculated. Patient acceptability was assessed via an online survey. RESULTS: One hundred thirty-nine participants submitted data. The results of two at-home automated smartphone apps correlated strongly/very strongly with PTA average and their frequency-specific median was within ±10 dB accuracy. Smartphone audiometry performed in sound-treated and home conditions were very strongly correlated. The apps were rated as easy/very easy to use by 90% of participants and 90% would be happy/very happy to use an app to monitor their hearing. CONCLUSION: Judicious use of self-performed smartphone audiometry was both valid and feasible for two of four apps. It could provide frequency-specific threshold estimates at home, potentially allowing assessments of patients remotely or monitoring of fluctuating hearing loss. LEVEL OF EVIDENCE: 2 Laryngoscope, 134:2864-2870, 2024.

Technical considerations and long-term results of endovascular venous stenting to control venous hypertension from meningiomas invading intracranial venous sinuses.

OBJECTIVE: Meningiomas invading the intracranial venous sinuses may cause intracranial venous hypertension, papilledema, and visual compromise. Sinus resection and graft reconstructions, however, add significant complexity to tumor surgery, with the potential for increased morbidity. In this study, the authors explored whether venous sinus stenting might provide an alternative means of controlling venous hypertension that would be sustainable over the long term. METHODS: The authors performed a retrospective review of all 16 patients with intracranial meningiomas who underwent stenting at their institution for venous sinus compromise. At presentation, all had headache and 9 had papilledema. Thirteen patients had 1 meningioma and 3 had 2 or more. Three patients had had previous tumor resection and radiotherapy. One patient had been treated with a lumboperitoneal shunt and radiotherapy. The median length of clinical follow-up was 8 years (range 4 months-18 years). RESULTS: Venous sinus narrowing was often not confined to the site of meningioma, and bilateral transverse sinus narrowing, reminiscent of that seen in idiopathic intracranial hypertension, was present in 7 patients with sagittal sinus meningiomas. Eleven patients had stents placed solely across sinus narrowing caused by meningioma. Five patients had additional stents placed at other sites of venous narrowing at the same time: in one of these patients, a stent was placed across a defect in the sagittal sinus caused by previous surgery, and in the 4 other patients, stents were placed across nontumor narrowings of the transverse sinuses. In 1 patient, the jugular vein was also stented. Nine patients developed symptomatic in-stent restenosis at the meningioma site. Eight had further stenting procedures with variable success in restoring the in-stent lumen. The remaining patient, with a late partial relapse, is being reinvestigated. Papilledema resolved in all patients after stenting. Six patients experienced prolonged and very substantial relief of all symptoms. Five patients had persistent headache despite restoration of the sinus lumen. Five had persistent symptoms associated with resistant in-stent stenosis. There were no significant complications from any of the diagnostic or therapeutic procedures. CONCLUSIONS: In patients who are symptomatic with meningiomas obstructing the venous sinuses, successful stenting of the affected segment can give a good outcome, especially in terms of relieving papilledema. However, further procedures are often necessary to maintain stent patency, other areas of venous compromise frequently coexist, and some patients remain symptomatic despite apparently successful treatment of the index lesion. Long-term surveillance is a requirement.

Changes in audiovestibular handicap following treatment of vestibular schwannomas.

OBJECTIVE: This study aimed to assess degree of audiovestibular handicap in patients with vestibular schwannoma. METHODS: Audiovestibular handicap was assessed using the Hearing Handicap Inventory, Tinnitus Handicap Inventory and Dizziness Handicap Inventory. Patients completed questionnaires at presentation and at least one year following treatment with microsurgery, stereotactic radiosurgery or observation. Changes in audiovestibular handicap and factors affecting audiovestibular handicap were assessed. RESULTS: All handicap scores increased at follow up, but not significantly. The Tinnitus Handicap Inventory and Dizziness Handicap Inventory scores predicted tinnitus and dizziness respectively. The Hearing Handicap Inventory was not predictive of hearing loss. Age predicted Tinnitus Handicap Inventory score and microsurgery was associated with a deterioration in Dizziness Handicap Inventory score. CONCLUSION: Audiovestibular handicap is common in patients with vestibular schwannoma, with 75 per cent having some degree of handicap in at least one inventory. The overall burden of handicap was, however, low. The increased audiovestibular handicap over time was not statistically significant, irrespective of treatment modality.

Incidence and presentation of vestibular schwannoma: a 3-year cohort registry study.

BACKGROUND: Vestibular schwannoma (VS) is the most common benign tumour arising in the lateral skull base. Reported incidence rates of VS vary across geographical locations and over time. There is scarce updated evidence over the past decade on the epidemiology and mode of presentation of VS. OBJECTIVE: To describe the epidemiology and mode of presentation of VS in the East of England between 2013 and 2016. METHODS: A retrospective epidemiological analysis of data from a national VS registry and electronic patient records was conducted, including all newly diagnosed adult patients in a UK tertiary referral centre, between April 1st, 2013, and March 31st, 2016. RESULTS: There were 391 new cases identified resulting in an overall mean incidence of 2.2 VS cases per 100,000 person-year. The incidence rate for all patients in the <40 age group ranged between 0.3 and 0.7 per 100,000 person-year, increasing to a range of 5.7 to 6.1 per 100,000 person-year in the 60-69 age group. The top three combinations of symptoms on presentation per patient were hearing loss and tinnitus (97, 24.8%), hearing loss alone (79, 20.2%) and hearing loss, tinnitus, and balance symptoms (61, 15.6%). The median duration of symptoms was 12 months, with a wide range from 1.4 to 300 months. Age was negatively correlated with tumour size (r = -0.14 [-0.24 to -0.04], p=0.01) and positively correlated with symptom duration (r = 0.16 [0.03-0.29], p=0.02). CONCLUSIONS: The incidence of vestibular schwannoma has increased compared to previous studies in the UK and is similar to incidence rates reported in other countries during the past decade. It peaks in the seventh decade of life, mainly because of an increase in the diagnosis of small tumours with a long duration of audio-vestibular symptoms in older patients, compared to earlier studies.

Meningitis Risk in Patients with Inner Ear Malformations after Cochlear Implants: A Systematic Review and Meta-Analysis.

OBJECTIVE: The objective of this study is to determine the rate of postoperative meningitis after cochlear implantation in those with inner ear malformations (IEMs) via meta-analysis. DATA SOURCES: Medline, EMBASE, and the Cochrane Library. METHODS: This study was reported following the preferred reporting items for systematic reviews and meta-analyses (PRISMA) checklist. Proportion meta-analysis was conducted through an inverse variance random-effect model based on arcsin transformation and presented as forest plots. Quality assessment of the included studies was performed through the National Institutes of Health Quality Assessment Tool. RESULTS: Overall, 38 of 2966 studies met the inclusion criteria and were included in the analysis. There were 10 cases of meningitis after cochlear implantation in 1300 malformed ears. The overall rate of meningitis after cochlear implantation in IEMs was 0.12% (95% confidence interval, 0.006-0.380%; I2 = 0%). Cases occurred in incomplete partition (n = 5), Mondini deformity (n = 2), common cavity (n = 2), and enlarged internal auditory canal (n = 1). Six of 10 cases of postoperative meningitis occurred with an intraoperative cerebrospinal fluid leak. CONCLUSION: In those with IEMs, the risk of meningitis after cochlear implantation is very low.

Life changing response to successive surgical interventions on cranial venous outflow: A case report on chronic fatigue syndrome.

Recognition of similarities between chronic fatigue syndrome and idiopathic intracranial hypertension (IIH) has raised suggestions that they might be connected, with chronic fatigue syndrome representing a mild version of IIH, sharing many of its symptoms, but without the signature features of elevated intracranial pressure that characterize the complete syndrome. A further development of this idea factors in the effects of a cerebrospinal fluid leak, a known complication of IIH, to explain cases where symptoms seem out of proportion to the apparent physiological disturbance. Cranial venous outflow obstruction has been proposed as the pathological substrate. We describe a patient with multiple symptoms, including headache and disabling fatigue, in which this model guided investigation and treatment. Specifically, CT and catheter venography identified focal narrowings of both jugular and the left brachiocephalic veins. Treatment of brachiocephalic obstruction was not feasible. However, in separate surgical procedures, relief of jugular venous obstruction produced incremental and significant clinical improvements which have proven durable over the length of follow-up. We suggest that investigating chronic fatigue syndrome under this model might not only bring benefit to individual patients but also will provide new insights into IIH and its relationship with spontaneous intracranial hypotension.

Radiation treatment of benign tumors in NF2-related-schwannomatosis: A national study of 266 irradiated patients showing a significant increase in malignancy/malignant progression.

Background: Radiation treatment of benign tumors in tumor predisposition syndromes is controversial, but short-term studies from treatment centers suggest safety despite apparent radiation-associated malignancy being reported. We determined whether radiation treatment in NF2-related schwannomatosis patients is associated with increased rates of subsequent malignancy (M)/malignant progression (MP). Methods: All UK patients with NF2 were eligible if they had a clinical/molecular diagnosis. Cases were NF2 patients treated with radiation for benign tumors. Controls were matched for treatment location with surgical/medical treatments based on age and year of treatment. Prospective data collection began in 1990 with addition of retrospective cases in 1969. Kaplan-Meier analysis was performed for malignancy incidence and survival. Outcomes were central nervous system (CNS) M/MP (2cm annualized diameter growth) and survival from index tumor treatment. Results: In total, 1345 NF2 patients, 266 (133-Male) underwent radiation treatments between 1969 and 2021 with median first radiotherapy age of 32.9 (IQR = 22.4-46.0). Nine subsequent CNS malignancies/MPs were identified in cases with only 4 in 1079 untreated (P < .001). Lifetime and 20-year CNS M/MP was ~6% in all irradiated patients-(4.9% for vestibular schwannomas [VS] radiotherapy) versus <1% in the non-irradiated population (P < .001/.01). Controls were well matched for age at NF2 diagnosis and treatment (Males = 133%-50%) and had no M/MP in the CNS post-index tumor treatment (P = .0016). Thirty-year survival from index tumor treatment was 45.62% (95% CI = 34.0-56.5) for cases and 66.4% (57.3-74.0) for controls (P = .02), but was nonsignificantly worse for VS radiotherapy. Conclusion: NF2 patients should not be offered radiotherapy as first-line treatment of benign tumors and should be given a frank discussion of the potential 5% excess absolute risk of M/MP.

Frequency of electrode migration after cochlear implantation in the early postoperative period. What are associated risk factors?

OBJECTIVES: To estimate the prevalence of, and risk factors associated with electrode migration (EM) in cochlear implant (CI) recipients. DESIGN: Historical cohort study of all CIs performed between 1 January 2018 and 1 August 2021 in a single tertiary adult and paediatric CI centre in the UK. MAIN OUTCOME MEASURES: The primary aim is to determine the prevalence of electrode migration, comparing intraoperative surgeon report and examination of a routine plain radiograph performed 2 weeks after surgery. EM is defined as the detection of movement of two or more electrodes out of the cochlea from the time of surgery. Multivariate analysis was performed to investigate preoperative and intraoperative risk factors that might predispose to migration. RESULTS: Four hundred and sixty-five patients, having 516 distinct surgeries, with 628 implants were analysed. EM occurred following 11.5% of implant operations. Pre-existing cochlear abnormality was an independent associated risk factor for EM (OR: 3.40 ⟨1.20-9.62⟩ p = .021). Demographics, surgical technique, usage of a precurved electrode, CSF leak, surgeon seniority and intraoperative telemetry did not influence risk of migration. There were 5 implants (0.8%) which migrated later than 2 weeks, with a median date of imaging diagnosis (x-ray or CT scan) of 263 days ⟨IQR:198⟩, for which head injury was a common precipitating factor. There were differences in the risk of migration between different lateral wall electrodes. CONCLUSION: EM in the early postoperative period is a common occurrence and is more likely in implant recipients with obstructed or malformed cochleae.

The Risk of Meningitis After Cochlear Implantation: A Systematic Review and Meta-Analysis.

OBJECTIVE: This study aims to estimate the rate of postoperative meningitis (both immediate and long-term) in patients following cochlear implants (CIs). It aims to do so through a systematic review and meta-analysis of published studies tracking complications after CIs. DATA SOURCES: MEDLINE, Embase, and Cochrane Library. REVIEW METHODS: This review was performed in line with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Studies tracking complications following CIs in patients were included. Exclusion criteria included non-English language studies and case series reporting <10 patients. Bias risk was evaluated using the Newcastle-Ottawa Scale. Meta-analysis was performed through DerSimonian and Laird random-effects models. RESULTS: A total of 116/1931 studies met the inclusion criteria and were included in the meta-analysis. Overall, there were 112 cases of meningitis in 58,940 patients after CIs. Meta-analysis estimated an overall rate of postoperative meningitis of 0.07% (95% confidence interval [CIs], 0.03%-0.1%; I2 = 55%). Subgroup meta-analysis showed this rate had 95% CIs crossing 0% in implanted patients who received the pneumococcal vaccine, antibiotic prophylaxis, those with postoperative acute otitis media (AOM), and those implanted less than 5 years. CONCLUSION: Meningitis is a rare complication following CIs. Our estimated rates of meningitis after CIs appear lower than prior estimates based on epidemiological studies in the early 2000s. However, the rate still appears higher than the baseline rate in the general population. The risk was very low in implanted patients who received the pneumococcal vaccine, antibiotic prophylaxis, received unilateral or bilateral implantations, developed AOM, those implanted with a round window or cochleostomy techniques, and those under 5 years.

Facial Nerve Function Outcome and Risk Factors in Resection of Large Cystic Vestibular Schwannomas.

Objective Cystic vestibular schwannomas (VS) in contrast to solid VS tend to have accelerated growth, larger volume, rapid/atypical presentation, lobulated/adherent surface, and unpredictable course of the cranial nerves. Cystic VS are surgically challenging, with worse clinical outcomes and higher rate of subtotal resection (STR). Methods We retrospectively analyzed postoperative outcomes of 125 patients with cystic VS, operated between years 2005 and 2019 in our center. We confronted the extent of the resection and House-Brackmann (HB) grade of facial palsy with the results of comparable cohort of patients with solid VS operated in our center and literature review by Thakur et al. 1 Results Translabyrinthine approach was preferred for resection of large, cystic VS (97.6%). Gross-total resection (GTR) was achieved in 78 patients (62.4%), near-total resection (NTR) with remnant (<4 × 4 × 2 mm) in 43 patients (34.4%), and STR in 4 patients (3.2%). NTR/STR were significantly associated with higher age, tumor volume >5 cm 3 , retrosigmoid approach, high-riding jugular bulb, tumor adherence to the brain stem, and facial nerve ( p = 0.016; 0.003; 0.005; 0.025; 0.001; and <0.00001, respectively). One year after the surgery, 76% of patients had HB grades 1 to 2, 16% had HB grades 3 to 4, and 8% had HB grades 5 to 6 palsy. Worse outcome (HB grades 3 to 6) was associated with preoperative facial palsy, tumor volume >25 cm 3 , and cyst over the brain stem ( p = 0.045; 0.014; and 0.05, respectively). Comparable solid VS operated in our center had significantly higher HB grades 1 to 2 rate than our cystic VS (94% versus 76%; p = 0.03). Comparing our results with literature review, our HB grades 1 to 2 rate was significantly higher (76% versus 39%; p = 0.0001). Tumor control rate 5 years after surgery was 95.8%. Conclusion Our study confirmed that microsurgery of cystic VS has worse outcomes of facial nerve preservation and extent of resection compared with solid VS. Greater attention should be paid to the above-mentioned risk factors.

Detecting and managing partial shorts in Cochlear implants: A validation of scalp surface potential testing.

OBJECTIVE: To investigate the value of scalp surface potentials to identify and manage partial short circuits to ground in cochlear implant electrodes. DESIGN: A retrospective review of patients with suspected partial short circuits. MAIN OUTCOME MEASURE: Electrical output of individual electrodes was measured using scalp surface potentials for patients reporting a change in hearing function. Electrical output was compared to functional performance and impedance measurements to determine if devices with suspected partial short circuits were experiencing a decrease in performance as a result of reduced electrical output. Electrical output was checked in an artificial cochlea for two implants following explant surgery to confirm scalp surface potential results. RESULTS: All patients with suspected partial short circuits (n = 49) had reduced electrical output, a drop in impedances to approximately ½ of previously stable measurements or to below 2 kΩ, an atypical electrical field measurement (EFI) and a decline in hearing function. Only devices with an atypical EFI showed reduced electrical output. Results of scalp based surface potentials could be replicated in an artificial cochlea following explantation of the device. All explant reports received to date (n = 42) have confirmed partial short circuits, with an additional four devices failing integrity tests. CONCLUSION: Surface potential measurements can detect partial shorts and had 100% correlation with atypical EFI measurements, which are characteristic of a partial short to ground in this device. Surface potentials can help determine the degree to which the electrode array is affected, particularly when behavioural testing is limited or not possible.

A 2 and 5-Year Longitudinal Analysis of 671 Consecutive Patients Diagnosed with Unilateral Vestibular Schwannoma.

OBJECTIVE: To analyse the 2 and 5-year outcomes of a cohort of patients with newly diagnosed vestibular schwannoma patients. STUDY DESIGN: Longitudinal cohort study. SETTING: Tertiary skull base referral unit. PATIENTS: Six hundred and seventy-one consecutive patients referred to the Cambridge Skull Base Unit between 2005 and 2010. MAIN OUTCOME MEASURES: 1) Treatment outcomes, 2) hearing preservation, 3) facial nerve function, and 4) gross tumor control. RESULTS: At initial diagnosis, 440/671(65.6%) of patients entered a watch, wait and rescan (WWR) pathway, 208/671 (31%) underwent primary microsurgical excision and 23/671 (3.4%) primary radiotherapy. Of patients who entered WWR, 144/440 (32.7%) exhibited tumor growth necessitating secondary intervention at a mean of 22.7 months; 67.4% had radiotherapy and 32.6% surgery. Radiotherapy was successful at achieving tumor control in 95% of cases. Primary surgery via the translabyrinthine route was performed in the majority of cases allowing for a radiologically clear surgical resection in 93% of cases. CONCLUSION: The Cambridge philosophy of treating the newly diagnosed vestibular schwannoma aims at preserving functional status where possible. In patients with small tumors it is recommended that they are placed on to a WWR pathway, of which 67.3% will not exhibit any growth by 5 years. If small tumors grow more than 2 mm by serial scanning, radiotherapy offers a high rate of tumor control. In patients with larger tumors more than 20 mm we would advocate microsurgical excision by the translabyrinthine route which offers excellent functional outcomes and gross tumor control.

Prevalence of occult nodal metastases in squamous cell carcinoma of the temporal bone: a systematic review and meta-analysis.

PURPOSE: Primary: To determine the rate of occult cervical metastases in primary temporal bone squamous cell carcinomas (TBSSC). Secondary: to perform a subgroup meta-analysis of the risk of occult metastases based on the clinical stage of the tumour and its risk based on corresponding levels of the neck. METHODS: A systematic review and meta-analysis of papers searched through Medline, Cochrane, Embase, Scopus and Web of Science up to November 2021 to determine the pooled rate of occult lymph node/parotid metastases. Quality assessment of the included studies was assessed through the Newcastle-Ottawa scale. RESULTS: Overall, 13 out of 3301 screened studies met the inclusion criteria, for a total of 1120 patients of which 550 had TBSCC. Out of the 267 patients who underwent a neck dissection, 33 had positive lymph nodes giving a pooled rate of occult metastases of 14% (95% CI 10-19%). Occult metastases rate varied according to Modified Pittsburg staging system, being 0% (0-16%) among 12 pT1, 7% (2-20%) among 43 pT2 cases, 21% (11-38%) among 45 pT3, and 18% (11-27%) among 102 pT4 cases. Data available showed that most of the positive nodes were in Level II. CONCLUSION: The rate of occult cervical metastases in TBSCC increases with pathological T category with majority of nodal disease found in level II of the neck.

Cochlear Implantation in Neurofibromatosis Type 2: Experience From the UK Neurofibromatosis Type 2 Service.

OBJECTIVE: To review the outcomes of cochlear implants (CI) in patients with neurofibromatosis type 2 (NF2) in a large cohort, and identify factors associated with poor hearing benefit. STUDY DESIGN: Fifteen-year retrospective national observational case series. SETTING: United Kingdom regional NF2 multidisciplinary teams. PATIENTS: Consecutive patients with NF2 receiving a CI. INTERVENTIONS: CI for hearing rehabilitation. MAIN OUTCOME MEASURES: 1) Audiometric performance at 9 to 12 months after implantation using City University of New York (CUNY) sentence recognition score, and Bamford- Kowal-Bench (BKB) word recognition score in quiet (BKBq), and in noise (BKBn). 2) CI use at most recent review. RESULTS: Sixty four consecutive patients, median age 43 years, were included. Nine to 12 months mean audiometric scores were: CUNY 60.9%, BKBq 45.8%, BKBn 41.6%. There was no difference in audiometric outcomes between VS treatment modalities. At most recent review (median 3.6 years from implantation), 84.9% with device in situ/available data were full or part-time users. Between 9 and 12 months and most recent review there was an interval reduction in mean audiometric scores: CUNY -12.9%, BKBq -3.3%, BKBn -4.9%. Larger tumor size and shorter duration of profound hearing loss were the only variables associated with poorer audiometric scores. Tumor growth at the time of surgery was the only variable associated with CI non-use. Individual patient response was highly variable. CONCLUSIONS: CI can provide significant and sustained auditory benefits to patients with NF2 independent of tumor treatment modality, with the majority of those implanted becoming at least part-time users. Larger datasets are required to reliably assess the role of independent variables.

Evidence-based surveillance protocol for vestibular schwannomas: a long-term analysis of tumor growth using conditional probability.

OBJECTIVE: The growth characteristics of vestibular schwannomas (VSs) under surveillance can be studied using a Bayesian method of growth risk stratification by time after surveillance onset, allowing dynamic evaluations of growth risks. There is no consensus on the optimum surveillance strategy in terms of frequency and duration, particularly for long-term growth risks. In this study, the long-term conditional probability of new VS growth was reported for patients after 5 years of demonstrated nongrowth. This allowed modeling of long-term VS growth risks, the creation of an evidence-based surveillance protocol, and the proposal of a cost-benefit analysis decision aid. METHODS: The authors performed an international multicenter retrospective analysis of prospectively collected databases from five tertiary care referral skull base units. Patients diagnosed with sporadic unilateral VS between 1990 and 2010 who had a minimum of 10 years of surveillance MRI showing VS nongrowth in the first 5 years of follow-up were included in the analysis. Conditional probabilities of growth were calculated according to Bayes' theorem, and nonlinear regression analyses allowed modeling of growth. A cost-benefit analysis was also performed. RESULTS: A total of 354 patients were included in the study. Across the surveillance period from 6 to 10 years postdiagnosis, a total of 12 tumors were seen to grow (3.4%). There was no significant difference in long-term growth risk for intracanalicular versus extracanalicular VSs (p = 0.41). At 6 years, the residual conditional probability of growth from this point onward was seen to be 2.28% (95% CI 0.70%-5.44%); at 7 years, 1.35% (95% CI 0.25%-4.10%); at 8 years, 0.80% (95% CI 0.07%-3.25%); at 9 years, 0.47% (95% CI 0.01%-2.71%); and at 10 years, 0.28% (95% CI 0.00%-2.37%). Modeling determined that the remaining lifetime risk of growth would be less than 1% at 7 years 7 months, less than 0.5% at 8 years 11 months, and less than 0.25% at 10 years 4 months. CONCLUSIONS: This multicenter study evaluates the conditional probability of VS growth in patients with long-term VS surveillance (6-10 years). On the basis of these growth risks, the authors posited a surveillance protocol with imaging at 6 months (t = 0.5), annually for 3 years (t = 1.5, 2.5, 3.5), twice at 2-year intervals (t = 5.5, 7.5), and a final scan after 3 years (t = 10.5). This can be used to better inform patients of their risk of growth at particular points along their surveillance timeline, balancing the risk of missing late growth with the costs of repeated imaging. A cost-benefit analysis decision aid was also proposed to allow units to make their own decisions regarding the cessation of surveillance.

Cochlear Implantation in Children with Autism Spectrum Disorder: A Systematic Review and Pooled Analysis.

OBJECTIVE: To determine outcomes following cochlear implantation (CI) in children with autism spectrum disorder (ASD). DATABASES REVIEWED: MEDLINE, Embase, Web of science, Cochrane Library, and Clinicaltrial.gov. METHODS: The review was performed according to the PRISMA statement. Primary outcomes measures were changes in speech perception and speech production scores. Secondary outcome measures included communication mode, device use, parental recommendation of implant, postoperative hyperacusis, and quality of life measures. Pooled analysis of outcomes was performed if possible. RESULTS: Twenty-four studies reported on 159 children with ASD. There were improvements in speech perception in 78% of cases and in speech expression in 63% of cases, though the extent of this improvement was variable. Seventy-four percent of children with ASD and CI are nonoral communicators. Intermittent/nonuse rate was 31%. Hearing outcomes are worse compared to children with other disabilities. The vast majority of parents would recommend CI based on their experiences. CONCLUSION: Outcome in children with ASD and CI are highly variable and significantly poorer compared to non-ASD children. Despite this, most parents report positive experiences and the evidence supports the use of CI in children with ASD.

Cochlear Implantation in Elderly Patients: Survival Duration, Hearing Outcomes, Complication Rates, and Cost Utility.

INTRODUCTION: The prevalence of hearing loss and its consequences is increasing as the elderly population grows. As the guidelines for cochlear implantation (CI) expand, the number of elderly CI recipients is also increasing. We report complication rates, survival duration, and audiological outcomes for CI recipients aged 80 years and over and discuss the cost utility of CI in this age group. METHODS: A retrospective cohort study was undertaken of all CI recipients (126 cases), aged 80 years and over at the time of their surgery, implanted at our institution (Cambridge University Hospitals) during a period from January 1, 2001, to March 31, 2019. Data on survival at 1, 3, and 5 years post-implantation, post-operative complications and functional hearing outcomes including audiometric and speech discrimination outcomes (Bamford-Kowal-Bench sentence test) have been reported. RESULTS: The mean age at implantation was 84 years. The mean audiometric score improved from 108 dB HL to 28 dB HL post-implantation. The mean Bamford-Kowal-Bench score improved from 14% to 66% and 73% at 2 and 12 months post-implantation, respectively. The complication rate was 15.3%. The survival probability at 1 year post-implantation was 0.95 for females and 0.93 for males, at 3 years was 0.89 for females and 0.81 for males, and at 5 years was 0.74 for females and 0.54 for males. CONCLUSION: CI is safe and well-tolerated in this age group and elderly patients gain similar audiometric and functional benefit as found for younger age groups.

Assessment of the cochlear implant pathway for newborn hearing screening referrals.

OBJECTIVE: To evaluate the cochlear implant (CI) assessment pathway for children referred through the newborn hearing screening programme (NHSP) and identify potential reasons for delay in CI surgery at a large UK centre. METHODS: Retrospective analysis of electronic notes of children referred through NHSP from 2016 to 2019. Key points in the assessment pathway from referral to switch-on were assessed. RESULTS: Of the 46 referrals, 44 went on to have CI surgery. Median age at CI surgery was 15 months (interquartile range (IQR) 13 to 17 months). Only 5% of referrals were implanted before the age of 12 months. Delayed referral and the presence of otitis media with effusion (OME) were associated with delayed implantation. The median period of monitoring for OME was 48 days (IQR 20 to 93 days), with a natural resolution rate of only 11%. The median time from surgery to switch-on was 5 weeks (IQR 4.1 to 5.4 weeks). CONCLUSION: Achieving implantation prior to the age of 12 months for NHSP referrals can be challenging for a variety of reasons. The delays in the system and the steps that our implant programme has taken to address these will be highlighted.

Cochlear Implantation in NF2 Patients Without Intracochlear Schwannoma Removal.

OBJECTIVE: To determine if cochlear implantation without removal of inner ear schwannomas (IES) is an effective treatment option for Neurofibromatosis 2 (NF2) patients. To determine how the presence of an intracochlear schwannoma might impact cochlear implant (CI) outcomes and programming parameters. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary center for cochlear and auditory brainstem implantation. PATIENTS: Of 10 NF2 patients with IES, 8 are reported with no previous tumor removal on the implanted ear. INTERVENTIONS: Cochlear implant without tumor removal. MAIN OUTCOME MEASURES: Performance outcomes with CI at least 1-year post intervention. Programming parameters, including impedances, for patients with IES. RESULTS: All patients had full insertion of the electrode arrays with round window approaches. Performance outcomes ranged from 0 to 100% for Bamford-Kowal-Bench sentences. Impedance measurements for active electrodes for patients with IES were comparable to those measured in patients without vestibular schwannoma (VS). Only patients who had radiation treatment before receiving their implant had elevated threshold requirements for CI programming compared with CI recipients without VS. CONCLUSION: Cochlear implantation without tumor removal is an effective option for treating NF2 patients with IES. The presence of an intracochlear tumor did not have an impact on CI performance or programming requirements compared with patients without VS; however, previous treatment with radiation may be related to elevated current requirements in the CI settings.

Determinants of Vestibular Schwannoma Growth.

OBJECTIVE: Management of vestibular schwannomas (VS) involves surgery, radiotherapy, or surveillance, based on patient and tumor factors. We recently described conditional probability as a more accurate method for stratifying VS growth risk. Building on this, we now describe determinants of VS growth, allowing clinicians to move toward a more personalized approach to growth-risk profiling. METHODS: Retrospective analysis of a prospectively collected database in a tertiary referral skull base unit between 2005 and 2014. Inclusion of patients with unilateral VS managed on surveillance protocol for a minimum of 5 years. Analysis of patient age, sex, tumor location, tumor size, and symptomology using conditional probability. RESULTS: A total of 340 patients met inclusion criteria. The conditional probability of growth of extracanalicular VS was significantly higher versus intracanalicular (IC) VS (30% versus 13%, p < 0.001) as was small-sized VS versus IC VS (28 versus 13%, p = 0.002), but only in the first year after diagnosis. Sex, age, and presenting symptoms did not significantly affect VS growth. CONCLUSION: In our series, extracanalicular VS were more likely to grow than IC VS and small-sized VS more likely to grow than IC VS, but only in the first year after diagnosis. Conversely, sex, age, and presenting symptoms did not affect the conditional probability of VS growth.