Search

OBJECTIVE The Glasgow Benefit Inventory (GBI) is a health-related quality of life instrument used to detect changes in health status following otolaryngologic interventions. Despite its use in cochlear implant literature, assessment of utility, reliability, and validity of GBI in an adult cochlear implants (CI) patient population has yet to be performed. STUDY DESIGN Retrospective case series. SETTING Academic, tertiary referral center. PATIENTS Postlingually deafened, adult CI patients with at least 1 year of device use. INTERVENTIONS Five hundred fifty-two patients were administered GBI questionnaires at least 1 year following CI activation during follow-up visits. MAIN OUTCOME MEASURES GBI total and subscale scoring were compared to either the Hearing Handicap Inventory for Adults or Hearing Handicap Inventory for the Elderly. Moreover, a factor analysis and Cronbach's alpha were performed to determine GBI validity and internal reliability, respectively. RESULTS The average overall GBI score was 38.6 ± 21.7. This was weakly correlated to the reduction in Hearing Handicap Inventory for Adults/Hearing Handicap Inventory for the Elderly (τb = 0.282, p

Background Homeless individuals frequently experience poor access to healthcare, delayed clinical presentation, and higher disease burden. Providing subspecialty otolaryngology care to this population can be challenging. We previously reported on the prevalence of hearing impairment in Toronto’s homeless community. As a secondary objective of this study, we sought to define otolaryngology specific need for this population. Methods One hundred adult homeless individuals were recruited across ten homeless shelters in Toronto, Canada using a stratified random sampling technique. An audiometric evaluation and head and neck physical examination were performed by an audiologist and otolaryngology resident, respectively. Basic demographic and clinical information was captured through verbal administration of a survey. Descriptive statistics were used to estimate frequency of otolaryngology specific diseases for this population. Results Of the 132 individuals who were initially approached to participant, 100 (76%) agreed. There were 64 males, with median age of 46 years (IQR 37–58 years). The median life duration of homelessness was 24 months (IQR 6–72 months). Participants had a wide range of medical comorbidities, with the most common being current tobacco smoking (67%), depression (36%), alcohol abuse (32%), and other substance abuse (32%). There were 22 patients with otolaryngology needs as demonstrated by one or more abnormal findings on head and neck examination. The most common finding was nasal fracture with significant nasal obstruction (6%). Eleven patients required referral to a staff otolaryngologist based on concerning or suspicious findings, including two head and neck masses, 6 were later seen in follow-up. Conclusion There were substantial otolaryngology needs amongst a homeless population within a universal healthcare system. Future research should focus on further elucidating head and neck related issues in this population and expanding the role of the otolaryngologist in providing care to homeless individuals.

OBJECTIVE Explore patient characteristics associated with tinnitus improvement after cochlear implantation. STUDY DESIGN Retrospective cohort. SETTING Tertiary referral. PATIENTS Adults with bilateral severe-to-profound hearing loss and tinnitus. INTERVENTIONS Unilateral cochlear implantation. RESULTS From 1996 to 2018, 358 patients endorsed pre-implant tinnitus and had ascertainable tinnitus status at 1-year. Clinically significant improvement in Tinnitus Handicap Inventory (THI) (reduction by at least 7-points) was observed in 262 (73.2%) patients, of whom 155 (59.2%) reported complete resolution. Of the 24 characteristics explored, four were identified as independent predictors of improved tinnitus in logistic regression models. In a multivariable model including identified independent predictors, each 10-percentage point increase in baseline hearing in noise test was associated with an 14% reduction in odds of tinnitus resolution or clinically significant improvement (odds ratio [OR] 0.86, 95% confidence limits [CL] 0.77, 0.96) and preoperative use of a hearing aid in the contralateral ear was associated with a 72% reduction (OR 0.28; 95% CL 0.11, 0.73). Each 10-point increase in baseline Hearing Handicap Inventory for Adults (HHI) score was associated with a 28% increase in odds of tinnitus improvement (OR 1.28; 95% CL 1.07, 1.54). Higher baseline burden of tinnitus was associated with higher odds of tinnitus improvement (OR 1.21 per 10-point THI increase, 95% CL 1.04, 1.40). CONCLUSIONS Worse residual hearing and higher baseline hearing and tinnitus handicap are associated with higher odds of tinnitus improvement. Expectations of patients seeking reduced tinnitus burden following cochlear implantation should be managed by counselling regarding odds of tinnitus improvement compared to those with similar residual hearing and tinnitus burden.

BACKGROUND: Given that hearing loss is associated with increased social isolation, reduced earning potential and neurocognitive disease, findings of uncorrected hearing loss in the homeless population have important policy implications. We sought to estimate the prevalence of hearing impairment in an adult homeless population. METHODS: We recruited adult (age ≥ 18 yr) homeless people across 10 homeless shelters in Toronto between April and June 2018 using a 2-stage sampling technique. Participants were interviewed by 1 interviewer using a modified survey that had been used in previous studies looking at other health needs in homeless populations. A comprehensive head and neck examination and audiometric evaluation were performed in each participant by an otolaryngologist and an audiologist. Descriptive statistics were estimated. Audiometric data were standardized directly for age and sex to facilitate direct comparisons with the general Canadian population. RESULTS: Of the 132 people invited, 100 (75.8%) agreed to participate. The median age was 46 (interquartile range [IQR] 37–58) years. The median duration of homelessness was 24 (IQR 6–72) months. Although most participants (78) had some form of extended health care benefits through social assistance, only 22/78 (28%) were aware that hearing tests and hearing aids were covered through these programs. After direct standardization for age and sex, the proportions of participants with a speech-frequency and high-frequency hearing loss were 39.5% (95% confidence interval [CI] 30.4%–49.3%) and 51.9% (95% CI 42.2%–61.4%), respectively. Nineteen participants were hearing aid candidates, only 1 of whom owned functional hearing aids. Rates of speech-frequency hearing loss (39.5%, 95% CI 30.4%–49.3% v. 19.2%, 95% CI 16.9%–21.7%) and high-frequency hearing loss (51.9%, 95% CI 42.2%–61.4% v. 35.5%, 95% CI 33.1%–37.7%) were substantially higher than in the general Canadian population. INTERPRETATION: Our results suggest that homeless adults have a high prevalence of hearing impairment, even when living within a system of universal health insurance; awareness of health care benefits through social assistance programs was poor. Results from this study may prompt initiatives surrounding homeless outreach and health screening.

OBJECTIVES The aim of this study was to prospectively assess pain and associated analgesic consumption after otological surgery comparing two prescription patterns. STUDY DESIGN A prospective nonrandomized consecutive cohort study. METHODS 125 adult patients undergoing ambulatory otologic surgery-cochlear implantation and endaural middle ear surgery, were assigned (according to surgeon's preference) and prospectively studied in two arms: 1) acetaminophen 500 mg + ibuprofen 400 mg; 2) acetaminophen 500 mg + codeine 30 mg. Pain levels, medication dose, disposal patterns of opioids, and suspected side effects were evaluated. RESULTS All patients reported mild to moderate pain. There was a statistically significant reduction of pain from day to day, which was on average 0.26 lower than the day before. Sufficient pain control could be achieved with both drug regimens with no significant difference in pain levels. Only 50% of patients who were prescribed opioids used them. Additionally, the median tablet intake was 3 tablets while 10 to 20 tablets were prescribed. The majority of patients (97%) did not dispose of these drugs safely. CONCLUSION Adequate analgesia was achieved in both arms of this study. Pain control following otologic surgery with a combination of acetaminophen and nonsteroidal anti-inflammatory drugs is recommended unless contraindications or chronic opioid use are present. If opioids such as codeine (30 mg) are prescribed, the amount should be reduced as low as possible, such as five tablets, based on our studied population. LEVEL OF EVIDENCE Level 3-a prospective nonrandomized consecutive cohort study Laryngoscope, 2021.

Background The SVV tests the ability of a person to perceive the gravitational vertical. A tilt in SVV indicates vestibular imbalance in the roll plane, and thus injuries to the utricle or its connecting nerves. A validated bedside method (et, al., 2009, 72(19):1689–1692, Neurol, Zwergal) is the bucket method, in which the subject estimates the true vertical by attempting to properly align a straight line visible on the bottom of a bucket that is rotated at random by the examiner. In our study, the subjects need to align the plumb line on the Visual Vertical iOS app to the vertical direction. Methods Measurements of the SVV were made in 22 healthy subjects (16 females and 6 males). Each subject conducted 10 iterations of bucket test and 10 iterations of iOS app test. The reliability and validity of the iOS app was analyzed by SPSS21. Results Cronbach’s α for the plumb line method was 0.976, and the iOS app was 0.978. Statistical comparison of SVV values measured by the iOS app and the bucket method showed no significant difference in distribution (Mann Whitney U test U = 0.944). Conclusion The Visual Vertical iOS app is an effective and accessible substitute to the plumb line for the measurement of the validated bucket test.

Purpose The effects of image doubling on logarithmic progression chart visual acuity were investigated by simulating diplopia on a computer monitor. Methods Ten participants (6M, 4F) aged 21-28 years (mean 22.4 ± 2.3) were assessed viewing with their left eye while wearing their best correction. Stimuli were eight rows of five Sloan letters, from 0.4 to -0.3 logMAR (6/15 to 6/3) arranged in logarithmic progression format, generated on an HD monitor. Stimuli were generated with different magnitudes of doubling, different directions of doubling, and different intensity ratios between the ghost image and main images. Results When the ghost image had the same intensity as the main image, there was a significant effect of doubling magnitude on visual acuity, with the mean acuity being -0.11 logMAR (6/4.7) for no doubling. Acuity thresholds (logMAR) increased when doubling exceeded 1 min of arc, reaching a level of 0.12 logMAR (6/7.9) for doubling of 16 min of arc. There were no significant effects of orientation on acuity, nor were there significant orientation-doubling magnitude interaction effects of visual acuity. Image doubling magnitude level affected visual acuity differently for different ghost image intensities, with the highest acuity elevation occurring when ghost and main images were equal or nearly equal. For faint ghost image intensities (10% and 20%) image doubling did not significantly affect visual acuity. Conclusions Image doubling will degrade visual acuity if doubling is greater than 1 min of arc, and ghost images are sufficiently intense. However, even with very obvious visually-disturbing image doubling, visual acuity remains only slightly reduced.

OBJECTIVE Assess speech outcomes in unilateral cochlear implant (CI) recipients after addition of a wireless contralateral routing of signals (CROS) microphone. STUDY DESIGN Prospective cohort study. SETTING Ambulatory. PATIENTS Sixteen adult unilateral CI users with nonserviceable hearing on the contralateral side were recruited. Those with AzBio sentence scores of 40 to 80% or Hearing in Noise Test - Quiet (HINT-Q) scores of 60 to 90% with a CI alone were eligible participants. INTERVENTION Speech testing was carried out with the CROS on and off. MAIN OUTCOME MEASURE Speech recognition. RESULTS In the consonant-nucleus-consonant test presented in quiet from the front, word scores were 64.4 (CI) and 63.8% (CI + CROS) (p = 0.72), and phoneme scores were 80.2 (CI) and 80.8% (CI + CROS) (p = 0.65). In AzBio sentence testing in quiet, with the signals projected from the contralateral, front, or ipsilateral to the CI, speech perception with the CI alone was 60.8, 75.9, and 79.1%. With the addition of the CROS microphone, using the same speaker arrangement, speech perception was 69.8 (p

Listening to speech in noise is effortful for individuals with hearing loss, even if they have received a hearing prosthesis such as a hearing aid or cochlear implant (CI). At present, little is known about the neural functions that support listening effort. One form of neural activity that has been suggested to reflect listening effort is the power of 8–12 Hz (alpha) oscillations measured by electroencephalography (EEG). Alpha power in two cortical regions has been associated with effortful listening—left inferior frontal gyrus (IFG), and parietal cortex—but these relationships have not been examined in the same listeners. Further, there are few studies available investigating neural correlates of effort in the individuals with cochlear implants. Here we tested 16 CI users in a novel effort-focused speech-in-noise listening paradigm, and confirm a relationship between alpha power and self-reported effort ratings in parietal regions, but not left IFG. The parietal relationship was not linear but quadratic, with alpha power comparatively lower when effort ratings were at the top and bottom of the effort scale, and higher when effort ratings were in the middle of the scale. Results are discussed in terms of cognitive systems that are engaged in difficult listening situations, and the implication for clinical translation.

ObjectivesHearing loss is the leading human sensory system loss, and one of the leading causes for years lived with disability with significant effects on quality of life, social isolation, and overall health. Coupled with a forecast of increased hearing loss burden worldwide, national and international health organizations have urgently recommended that access to hearing evaluation be expanded to meet demand.MethodsThe objective of this study was to develop ‘AutoAudio’ – a novel deep learning proof-of-concept model that accurately and quickly interprets diagnostic audiograms. Adult audiogram reports representing normal, conductive, mixed and sensorineural morphologies were used to train different neural network architectures. Image augmentation techniques were used to increase the training image set size. Classification accuracy on a separate test set was used to assess model performance.ResultsThe architecture with the highest out-of-training set accuracy was ResNet-101 at 97.5%. Neural network training time varied between 2 to 7 hours depending on the depth of the neural network architecture. Each neural network architecture produced misclassifications that arose from failures of the model to correctly label the audiogram with the appropriate hearing loss type. The most commonly misclassified hearing loss type were mixed losses.ConclusionRe-engineering the process of hearing testing with a machine learning innovation may help enhance access to the growing worldwide population that is expected to require audiologist services. Our results suggest that deep learning may be a transformative technology that enables automatic and accurate audiogram interpretation.

Importance: Medical device performance and safety databases can be analyzed for patterns and novel opportunities for improving patient safety and/or device design. Objective: The objective of this ...

Objectives To predict postoperative cochlear implant performance with heterogeneous text and numerical variables using supervised machine learning techniques. Study design A supervised machine learning approach comprising neural networks and decision tree-based ensemble algorithms were used to predict 1-year postoperative cochlear implant performance based on retrospective data. Setting Tertiary referral center. Patients One thousand six hundred four adults who received one cochlear implant from 1989 to 2019. Two hundred eighty two text and numerical objective demographic, audiometric, and patient-reported outcome survey instrument variables were included. Outcome measures Outcomes for postoperative cochlear implant performance were discrete Hearing in Noise Test (HINT; %) performance and binned HINT performance classification ("High," "Mid," and "Low" performers). Algorithm performance was assessed using hold-out validation datasets and were compared using root mean square error (RMSE) in the units of the target variable and classification accuracy. Results The neural network 1-year HINT prediction RMSE and classification accuracy were 0.57 and 95.4%, respectively, with only numerical variable inputs. Using both text and numerical variables, neural networks predicted postoperative HINT with a RMSE of 25.0%, and classification accuracy of 73.3%. When applied to numerical variables only, the XGBoost algorithm produced a 1-year HINT score prediction performance RMSE of 25.3%. We identified over 20 influential variables including preoperative sentence-test performance, age at surgery, as well as specific tinnitus handicap inventory (THI), Short Form 36 (SF-36), and health utilities index (HUI) question responses as the highest influencers of postoperative HINT. Conclusion Our results suggest that supervised machine learning can predict postoperative cochlear implant performance and identify preoperative factors that significantly influence that performance. These algorithms can help improve the understanding of the diverse factors that impact functional performance from heterogeneous data sources.

Background Hearing loss is one of the leading causes of disability in Canada and worldwide, with more than one million Canadians enduring a hearing-related disability. Meniere’s disease (MD) is a chronic condition of the inner ear, manifesting as a triad of disabling symptoms, including attacks of vertigo, fluctuating sensorineural hearing loss (SNHL), and tinnitus. Impacts on quality of life are severe, particularly with respect to restrictions in social participation and physical activity, fatigue, and reduced capacity to work. Anxiety and other psychological disorders may result from the restrictions imposed on life, the constant uncertainty of vertigo attacks, and fluctuating SNHL, with neuroses and depression affecting 40 to 60% of sufferers of intractable MD. There is a need to establish the benefits of previously studied interventions with greater certainty. The planned systematic review and meta-analyses/network meta-analyses (NMAs) will assess the relative effects of competing pharmacologic and surgical interventions for management of MD in adults. Methods An experienced medical information specialist in consultation with the review team will develop the electronic search strategies. We will search various databases including MEDLINE, Embase, and the Cochrane Library with no date or language restrictions for published literature, and key clinical trial registries for in-progress and completed trials. Screening of the literature will be performed by two reviewers independently using pre-specified eligibility criteria, and quality of the included studies will be assessed using the Cochrane Risk of Bias tool. We will resolve disagreements through consensus or third-party adjudication. When applicable, meta-analyses and NMAs will be pursued to compare interventions in terms of their effects on outcomes, including frequency and severity of vertigo, occurrence and intensity of tinnitus, changes in hearing and speech recognition, quality of life, and harms. Separate analyses exploring the effects of pharmacologic and surgical approaches will be performed. Discussion Our planned systematic review will provide informative evaluations of existing treatments for management of Meniere’s disease. The findings will inform practitioners as to the relative benefits and harms of the existing competing interventions for MD, offer optimal clinical treatment strategies, identify evidence gaps, and determine promising therapies for evaluation in future trials. Systematic review registration PROSPERO CRD42019119129

PURPOSE To develop and validate a smartphone based Virtual Reality Epley Maneuver System (VREMS) for home use. METHODS A smartphone application was designed to produce stereoscopic views of a Virtual Reality (VR) environment, which when viewed after placing a smartphone in a virtual reality headset, allowed the user to be guided step-by-step through the Epley maneuver in a VR environment. Twenty healthy participants were recruited and randomized to undergo either assisted Epleys or self-administered Epleys following reading instructions from an Instructional Handout (IH). All participants were filmed and two expert Otologists reviewed the videos, assigning each participant a score (out of 10) for performance on each step. Participants rated their perceived workload by completing a validated task-load questionnaire (NASA Task Load Index) and averages for both groups were calculated. RESULTS Twenty participants were evaluated with average age 26.4±7.12years old in the VREMS group and 26.1±7.72 in the IH group. The VR assisted group achieved an average score of 7.78±0.99 compared to 6.65±1.72 in the IH group. This result was statistically significant with p=0.0001 and side dominance did not appear to play a factor. Analyzing each step of the Epley maneuver demonstrated that assisted Epleys were done more accurately with statically significant results in steps 2-4. Results of the NASA-TLX scores were variable with no significant findings. CONCLUSION We have developed and demonstrated face validity for VREMS through our randomized controlled trial. The VREMS platform is promising technology, which may improve the accuracy and effectiveness of home Epley treatments. LEVEL OF EVIDENCE N/A.

Objective The use of machine learning technology to automate intellectual processes and boost clinical process efficiency in medicine has exploded in the past 5 years. Machine learning excels in automating pattern recognition and in adapting learned representations to new settings. Moreover, machine learning techniques have the advantage of incorporating complexity and are free from many of the limitations of traditional deterministic approaches. Cochlear implants (CI) are a unique fit for machine learning techniques given the need for optimization of signal processing to fit complex environmental scenarios and individual patients' CI MAPping. However, there are many other opportunities where machine learning may assist in CI beyond signal processing. The objective of this review was to synthesize past applications of machine learning technologies for pediatric and adult CI and describe novel opportunities for research and development. Data sources The PubMed/MEDLINE, EMBASE, Scopus, and ISI Web of Knowledge databases were mined using a directed search strategy to identify the nexus between CI and artificial intelligence/machine learning literature. Study selection Non-English language articles, articles without an available abstract or full-text, and nonrelevant articles were manually appraised and excluded. Included articles were evaluated for specific machine learning methodologies, content, and application success. Data synthesis The database search identified 298 articles. Two hundred fifty-nine articles (86.9%) were excluded based on the available abstract/full-text, language, and relevance. The remaining 39 articles were included in the review analysis. There was a marked increase in year-over-year publications from 2013 to 2018. Applications of machine learning technologies involved speech/signal processing optimization (17; 43.6% of articles), automated evoked potential measurement (6; 15.4%), postoperative performance/efficacy prediction (5; 12.8%), and surgical anatomy location prediction (3; 7.7%), and 2 (5.1%) in each of robotics, electrode placement performance, and biomaterials performance. Conclusion The relationship between CI and artificial intelligence is strengthening with a recent increase in publications reporting successful applications. Considerable effort has been directed toward augmenting signal processing and automating postoperative MAPping using machine learning algorithms. Other promising applications include augmenting CI surgery mechanics and personalized medicine approaches for boosting CI patient performance. Future opportunities include addressing scalability and the research and clinical communities' acceptance of machine learning algorithms as effective techniques.

Determine association of advancements in speech processor technology with improvements in speech recognition outcomes.Retrospective cohort.Tertiary referral center.Adult unilateral cochlear implant (CI) recipients.Increasing novelty of speech processor defined by year of market availability.Consonant-Nucleus-Consonant (CNC) and Hearing in Noise Test (HINT) in quiet.From 1991 to 2016, 1,111 CNC scores and 1,121 HINT scores were collected from 351 patients who had complete data. Mean post-implantation CNC score was 53.8% and increased with more recent era of implantation (p 0.001, analysis of variance [ANOVA]). Median HINT score was 87.0% and did not significantly vary with implantation era (p = 0.06, ANOVA). Multivariable generalized linear models were fitted to estimate the effect of speech processor novelty on CNC and HINT scores, each accounting for clustering of scores within patients and characteristics known to influence speech recognition outcomes. Each 5-year increment in speech processor novelty was independently associated with an increase in CNC score by 2.85% (95% confidence limits [CL] 0.26, 5.44%) and was not associated with change in HINT scores (p = 0.30).Newer speech processors are associated with improved CNC scores independent of the year of device implantation and expanding candidacy criteria. The lack of association with HINT scores can be attributed to a ceiling effect, suggesting that HINT in quiet may not be an informative test of speech recognition in the modern CI recipient. The implications of these findings with respect to appropriate interval of speech processor upgrades are discussed.

In several publicly funded health care systems, including Ontario, Canada, adult tonsillectomies and septoplasties have been suggested to be removed or “delisted” from the government health insurance plan. Thus, the objective of this study was to explore patient perspectives regarding out of pocket (OOP) payment for these procedures. An anonymous survey was administered to patients consented to undergo a tonsillectomy or septoplasty at a community otolaryngology—head and neck surgery (OHNS) practice. The survey asked patients if they would pay the projected cost for their surgery OOP and the maximum amount of time they would wait for their surgery. The survey also contained questions on socioeconomic status and disease severity. Seventy-one patients were included. Overall, 21% of patients were willing to pay OOP for their surgery. Forty-nine percent of patients reported that the maximum amount of time they would be willing to wait for their surgery was 2 to 6 months. There was no significant correlation found between any of the demographic variables or disease severity and willingness to pay OOP for these surgeries. In this study, a small percentage of patients who met the clinical indications for a tonsillectomy or a septoplasty would pay for their surgery in the event that it was not covered by the government health insurance plan. These surgeries are common operations and delisting them could potentially decrease the provision of these services and have a significant impact on Canadian OHNS practices.

Objective To demonstrate the utility of diffusion tensor imaging (DTI) fiber tractography of the facial nerve in patients with cerebellopontine angle (CPA) tumors. Study design Prospective. Setting Tertiary referral center. Patients DTI technique was established in 113 patients without tumors and in 28 patients with CPA tumors. Subsequently, DTI results were compared with intraoperative findings in 21 patients with medium and large-sized tumors, treated surgically via a translabyrinthine approach. Intervention Three Tesla magnetic resonance (MR) was used for DTI tractography. For patients without CPA tumors, the scanning protocol was 32 directions with a 3 × 3 × 3 mm voxel size. For CPA tumor patients, scanning protocol was 32 directions with a 2 × 2 × 2 mm voxel size. DTI data were used to track the facial nerve. Main outcome measures Facial nerve identification rate. Results Facial nerve identification rate in MR-DTI was 97% and 100% in patients without tumors and in patients with tumors of the CPA of the internal auditory canal that were not treated surgically, respectively. MR-DTI identification of the facial nerve was successful in 20 patients who were treated surgically (95%). Good agreement between surgical findings and MR-DTI results was found in 19 patients (90%). Conclusion MR DTI tractography is an effective technique in positively identifying the position of the facial nerve in patients with CPA tumors.

To determine if depriving the use of the first cochlear implant (CI1) impacts adaptation to a sequential implant (CI2).Prospective cohort.Academic center.Sixteen unilateral cochlear implant recipients undergoing contralateral implantation (sequential bilateral) were matched according to age, etiology, duration of deafness, device age, and delay between implants.During a 4-week adaptation period after CI2 activation, patients underwent deprivation of CI1 or were permitted continued use of it.Speech perception scores and subjective quality of life outcomes before CI2 and at 1, 3, 6, and 12-months following activation.Maximal CI2 speech perception scores in quiet were achieved by 1-month postactivation for the "deprivation" group (71.3% for hearing in noise test [HINT], p = 0.767 for change beyond 1-mo) compared with 6-months for the "continued use" group (67.9% for HINT, p = 0.064 for change beyond 6-mo). The "deprivation" group experienced a temporary drop in CI1 scores (67.9% for HINT in quiet at 1-mo versus 78.4% pre-CI2, p = 0.009) recovering to 77.3% by 3-months; unchanged from baseline levels (p = 1.0). A binaural advantage over the better hearing ear was present for HINT sentences with noise (72.4% versus 58.8% for "deprivation", p = 0.001; 71.5% versus 52.7% for "continued use," p = 0.01). Missing data precluded a meaningful analysis of subjective quality of life outcome scales.Bilateral cochlear implantation improves speech perception compared with one implant. A period of deprivation from CI1 shortens time to maximum speech perception by CI2 without long-term consequences on the performance of CI1.

Cochlear implantation has become the standard procedure for restoring substantial hearing in the profoundly deaf. The excellent performance of most of the CI recipients coupled with the rapid evolution of implant technology lead to a distinct expansion in selection criteria for CI. These changes in candidacy primarily include patients with (1) moderate preoperative speech recognition with hearing aids, (2) significant residual hearing, (3) single-sided deafness, and (4) geriatric patients. Many of these patients' conditions were regarded as a clear contraindication to CI only a few years ago. In this article an overview of the current and new aspects of candidacy for cochlear implantation is provided.

Objectives/Hypothesis To evaluate the long-term impact of cochlear implantation on quality of life measured by the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36). Scores were also converted to the SF-6D to derive health utility scores. Study Design Prospective cohort study. Methods Thirty-two patients undergoing cochlear implantation completed the SF-36 preoperatively, 1 year, and 10 years after cochlear implantation. Results SF-36 results showed improvements in seven of the eight attributes when preoperative scores where compared with 1- and 10-year results. Between 1 and 10 years postoperatively, six of eight domains deteriorated in scores. When converted to the SF-6D, the mean preoperative utility scores were 0.592 for standard gamble, 0.636 using the ordinal health state paradigm, and 0.579 using the Bayesian technique. Ten years postoperatively, health utility scores were 0.643 (standard gamble), 0.684 (ordinal health state), and 0.6 (Bayesian). Between preoperatively and 10-year postoperatively, improvements were therefore 0.051, 0.048, and 0.021 for standard gamble, ordinal health state, and Bayesian paradigm, respectively. Conclusions This study establishes the long-term sustained benefits of cochlear implantation on quality of life. Nevertheless, both the SF-36 and SF-6D seem to underestimate the benefit accrued through this intervention. Our data are consistent with others regarding the unsuitability of the SF-36 in benefit assessment, notwithstanding that conversion to the SF-6D is feasible, and the SF-6D seemed to better depict possible benefits from cochlear implantation as compared to the SF-36. Level of Evidence 4. Laryngoscope, 124:278–282, 2014

Objectives/Hypothesis: The purpose of the study was to investigate the potential clinical advantages of incorporating a contralateral routing of signals (CROS) microphone in unilateral cochlear implant (CI) users. Study Design: A prospective study was undertaken on a group of 21 postlingually deafened adults who were all implanted with the same multichannel CI system. Methods: Performance with a unilateral CI was compared with performance using both a unilateral implant and a CROS microphone system worn on the opposite site (CI-CROS). Speech understanding using the AzBio sentence was evaluated in quiet, with speech presented at 0° and 270° azimuth in the horizontal plane. Speech understanding in noise was performed with speech at 0°, and noise at 0°, 90°, and 270°. Results: A significant gain in speech understanding using CI-CROS compared to the unilateral CI alone was found in quiet when speech was presented at 270° (average improvement of 8.8%, P < .01). Participants also demonstrated statistically significant improvement using CI-CROS compared with the unilateral CI alone when noise was presented at 90° and speech at 0° (average improvement of 6.7%, P < .01). Conclusions: Adding a contralateral microphone to a unilateral CI resulted in a significant improvement in speech understanding in different conditions. This method could provide a greater cost/benefit ratio than bilateral CIs and be a potential improvement for those who are not candidates for bilateral CIs. Laryngoscope, 2013

In the present study we sought to evaluate the psychosocial and quality of life (QOL) impacts of cochlear implant (CI) treatment on both CI recipients and their partners in a Canadian population.A qualitative cross-sectional contemporary cohort evaluation was conducted by distributing specifically designed questionnaires to both CI recipients and their partners at their follow-up appointments over a month at Sunnybrook Health Sciences Centre.It was found that both CI recipients and their partners demonstrated substantial benefit and improvement in multiple psychosocial domains.Musical listening was found to show variable improvements between recipients. Further analysis found a simple correlation where the group of recipients who all had a hearing disability for a longer duration had demonstrated a higher proportion of improvement in musical listening. CI recipients endorsed having improved QOL which was in agreement with proxy observation by partners.Overall, CI use has been shown to have significant benefit to psychosocial well-being of CI recipients. This benefit is also conferred to their caregivers/partners; which in our society concerned about caregiver burden is quite important to recognize.

OBJECTIVES To investigate the effects of unilateral multi-channel cochlear implant surgery on health-related quality of life and to determine if there is an age-related impact of cochlear implantation on these effects. DESIGN Prospective study. SETTING Tertiary health-care center. METHODS The Short Form-36 survey (SF-36) was administered to determine the health-related quality of life of 283 age-stratified patients before and after cochlear implant surgery. MAIN OUTCOMES Precochlear to postcochlear implantation changes in health-related quality of life as determined by the SF-36 questionnaire. RESULTS There were significant increases in precochlear and postcochlear implantation scores for 5 of the 8 SF-36 survey domains: vitality, physical role functioning, mental health, emotional role functioning, and social functioning. Significant differences were found between age groups in the domains of social functioning, emotion role functioning, and mental health. CONCLUSION Cochlear implant surgery significantly improves health-related quality of life as categorically stratified by the SF-36 questionnaire. These improvements were most evident in the mental health, emotional and social functioning, and physical functioning at work questions of the survey. Cochlear implant recipients younger than 65 years perceive a greater improvement in their level of energy, mental health, and social function compared with those older than 65 years.

OBJECTIVE: To determine whether there are objective and/or subjective differences in facial function, tongue function and quality of life in patients who have undergone 1) direct facial-to-facial (Primary, 4 patients), 2) direct facial-to-hypoglossal (End-to-End, 7 patients), 3) end-to-side facial-to-hypoglossal nerve anastomoses with sural nerve/greater auricular nerve interposition (End-to-Side with Interposition Graft, 7 patients), and 4) end-to-side facial-to-hypoglossal nerve anastomoses (End-to-Side, 7 patients) after acoustic neuroma excision. SETTING: Tertiary university referral center. STUDY DESIGN: Case series. METHODS: Twenty-five patients with complete facial paralysis after acoustic neuroma surgery who have also undergone 1 of 4 types of facial musculature reinnervation volunteered for the study. The mean duration of follow-up was 11.5 years (range, 1-25 yr). Each patient was globally assessed using the Sunnybrook Facial Grading scale (objective), Facial Disability Index (subjective), Oral-Pharyngeal Disability Index (subjective), Tongue Movement/Muscle symmetry (objective), and short-form SF-36 quality-of-life index (subjective). RESULTS: Patients who underwent direct facial-to-hypoglossal anastomosis had generally poorer outcomes some of which were statistically significant (p < 0.05) in some components of the Oral-Pharyngeal Disability Index. All of these patients had reduced lateral tongue movement as well as ipsilateral tongue atrophy. There was no significant difference in the Sunnybrook Facial Grading scale scores in those patients in whom end-to-side interposition reinnervation surgery was performed compared with those in whom direct end-to-side anastomosis surgery was performed. CONCLUSION: Complete sacrifice of the ipsilateral hypoglossal nerve does present with clinically and statistically significant morbidity in patients. Facial function is statistically similar in patients who underwent an end-to-side anastomosis with or without an interposition graft. These findings suggest thatin the setting of the posttranslabryinthine approach, the technicallyeasier interposition graft procedure is not acompromise.

Objective Microscopic techniques are an essential part of otolaryngologic practice. These procedures demand advanced psychomotor and visuospatial skills, and trainees possess these abilities to varying degrees. No method currently exists to predict who will possess an aptitude for microscopic surgery. Our goal was to determine whether performance can be predicted by background experiences or skills. Study design Retrospective cohort study. Setting Tertiary academic hospital. Subjects Students with no previous surgical experience. Interventions Subjects were surveyed on a wide range characteristics thought to affect surgical aptitude, with a primary focus on video gaming and musical training. Main outcome measure Subjects performed a microsurgical task using a novel simulator and their performance was assessed by blinded investigators. Results Forty-six students were assessed. There was no correlation between video gaming and improved microsurgical performance. Rather, video gamers obtained worse scores, although this difference did not reach significance. The majority of students played a musical instrument. Within this group, musicians who began playing at younger ages obtained higher scores, with the highest scores obtained by musicians who began playing before age 6. However, musicians did not obtain higher scores than non-musicians, regardless of their age of initiation. Conclusions No improvement in microsurgical aptitude was seen in subjects who had a history of video gaming or musical instrument playing.

Introduction: Hearing preservation is invariably a consideration when exploring treatment options for acoustic neuromas. We reviewed the long-term hearing results of patients who were treated using 1) hyperfractionated stereotactic radiotherapy (HSR), 2) hearing preservation tumor excision surgery (HPTES), and 3) expectantly (no treatment). Methods and Materials: Single institution retrospective chart review of 42 patients managed with HSR (1993–2003), 113 patients in whom HPTES was carried out, and 86 patients who were untreated (1974–2003). Hearing levels were graded according to the Gardner-Robertson classification. Results: The percent of patients managed with HSR initially who had serviceable hearing (class 1–2) was 68.8%. This fell to 6.7% in the follow-up interval. Of the group treated with HPTES, 100% had preoperative serviceable hearing. This dropped to 15.9% in the follow-up interval. The percent of patients managed expectantly who initially had serviceable hearing was 77.3%. This dropped to 33.3% during the follow-up interval. Mean follow-up periods were 4.0, 9.5, and 6.8 years in the HSR, HPTES, and expectant groups, respectively. Conclusions: Hearing acuity statistically worsened over the long term (P < .01) in all three groups. There was a significant proportion of patients in whom hearing deteriorated from serviceable to nonserviceable hearing (P < .01) during the follow-up interval. The decline was most significant in the groups treated with HPTES and HSR compared with the group treated expectantly (P < .05). Hearing outcomes, in our experience, continue to be poor, but this is especially so in patients treated with HPTES or HSR.

A 35-year-old man presented to the Department of Otolaryngology with progressive bilateral hearing loss, worse on the right, over the past 12 years. Audiometry determined the hearing loss to be mixed, both conductive and sensorineural. He had no otalgia, vertigo, or otorrhea. There was no history of otologic surgery, noise exposure, or familial hearing loss. He described remote minor head injury at age 4 years that required stitches, and prior fractures, which generally occurred during hockey matches, of his clavicle, fingers, a toe, and the left tibia and fibula. He was not taking any regular medications. Results of a general physical examination were normal. Results of an otologic examination revealed tortuous external auditory canals bilaterally but normal tympanic membranes. High-resolution computed tomography of the temporal bones was performed (Figures 1 and 2).

Cochlear implantation has been shown to result in significant improvements in communication and quality of life, but little is known about the effect of cochlear implantation and changes in a person's employment status and earning potential. The purpose of this study is to measure the extent to which personal income changes in people who receive a cochlear implant.We mailed a survey to a random selection of 150 cochlear implantees who receive health services in a large urban setting. Of the 93 respondents, 65 were eligible for inclusion. Demographics, current income and income prior to implantation were recorded into income categories.With a 6.6-year mean duration from cochlear implantation, it was found that 31% of respondents had increased income enough to move income brackets, with a mean category rise of $10 021. Forty participants reported working pre-implant, while 49 reported working post-implant.Our results suggest preliminary evidence for an association between cochlear implantation and income. Increased accesses to cochlear implantation may provide opportunities for competitive employment and associated economic benefits for the individual, their families, and society.

Objective To determine the safety, efficacy, and outcomes of cochlear implantation in patients with chronic suppurative otitis media (CSOM). Study design Retrospective case review. Setting Tertiary referral center with large cochlear implant program. Patients Nineteen patients with CSOM who underwent cochlear implantation were identified. Case history, timing of surgical procedures, complications, infections, and postimplant audiometric scores (Hearing in Noise Test [HINT], City University of New York Sentences [CUNY], and Central Institute for the Deaf Sentences [CID]) were evaluated. Main outcome measures Rates of postoperative infections and complications as well as postimplant auditory performance. Results Twelve patients underwent a staged procedure involving canal wall down mastoidectomy or radical revision mastoidectomy with middle ear and mastoid obliteration and closure of the external auditory canal followed by cochlear implantation approximately 5 months later. Seven patients were implanted in a single procedure. There were no infections or medical complications after implantation. On average, patients had excellent audiometric scores at 1 year postimplantation (mean sentence test, 79%; SD, 14), and these scores were comparable to our general population (mean sentence test, 71%; SD, 32). Conclusion Cochlear implant patients with CSOM have no increased risk of postoperative infections or complications. These patients have excellent outcomes with audiometric scores comparable to the general cochlear implant population. Cochlear implantation is a safe and effective treatment for patients with profound hearing loss secondary to CSOM.

Objectives/Hypothesis To evaluate the changes in health-related quality of life in unilateral adult cochlear implant patients using the Medical Outcome Study Short-Form Survey-36 (SF-36) and the Health Utility Index Mark 3 (HUI-3). To do so, a health utility index was obtained by converting the SF-36 to the Medical Outcome Study Short-Form Survey-6D (SF-6D) to permit comparison with HUI-3 scores in the context of health preference as measured by quality-adjusted life years. Study Design Prospective cohort study. Methods Eighty-one postlingually deaf adult patients undergoing cochlear implantation completed the SF-36 and HUI-3 preoperatively and at a median of 1.4 years (range, 11 months–5 years) after cochlear implantation. Results The SF-36 improvement was statistically significant in two domains. The SF-36 data were converted to SF-6D. Preoperatively, the mean SF-6D utility score was 0.575 ± 0.056. One year postoperatively this score increased to 0.590 ± 0.064. The improvement of 0.015 ± 0.082 was not statistically significant (P = .1118). Of the HUI-3 attributes, two showed improvement between preoperative and postoperative evaluations. The overall HUI-3 score increased from 0.464 ± 0.207 preoperatively to 0.611 ± 0.190 postoperatively. The gain of 0.146 ± 0.19 was statistically significant (P

Although cochlear implantation has been shown to improve quality of life, the socioeconomic benefit to the individual and society has not been thoroughly investigated. Our objective was to determine the economic impact of profound deafness and subsequent effects of unilateral cochlear implantation.Retrospective analysis of a prospectively collected cochlear implantation database.An academic, tertiary care hospital.A prospectively collected cochlear implantation database of 702 patients was reviewed. Known Canadian economic surrogates were used to estimate the personal economic impact of both deafness and unilateral cochlear implantation.The main outcome measures included employment rates and personal income prior to and following cochlear implantation.A total of 637 patients had sufficient occupational data for inclusion in the study; 36.7% suffered a negative economic impact as a result of their deafness. Cochlear implantation was associated with a significant increase in median yearly income compared to preimplantation ($42 672 vs $30 432; p = .007).Cochlear implantation not only improves quality of life but also translates into significant economic benefits for patients and the Canadian economy. These benefits appear to exceed the overall costs of cochlear implantation.

Objectives The primary purpose of this study was to evaluate a group of postlingually deafened adults, whose aided speech recognition exceeded commonly accepted candidacy criteria for implantation. The study aimed to define performance and qualitative outcomes of cochlear implants in these individuals compared with their optimally fitted hearing aid(s). Study design Retrospective case series. Setting Tertiary referral center. Patients All postlingually deafened subjects (N = 27), who were unsuccessful hearing aid users implanted between 2000 and 2010 with a preimplantation Hearing in Noise Test (HINT) score of 60% or more were included. Intervention We compared patients' preoperative performance (HINT score) with hearing aids to postoperative performance with the cochlear implant after 12 months of device use. In addition, the Hearing Handicap Inventory questionnaire was used to quantify the hearing-related handicap change perceived after the implantation. Results The study group demonstrated significant postoperative improvement on all outcome measures; most notably, the mean HINT score improved from 68.4% (standard deviation, 8.3) to 91.9% (standard deviation, 9.7). Additionally, there was a significant improvement in hearing-related handicap perceived by all patients. Conclusion The envelope of implantation candidacy criteria continues to expand as shown by this study's cohort. Patient satisfaction and speech recognition results are very encouraging in support of treating those who currently perform at a level above the conventional candidacy threshold but struggle with optimally fitted hearing aids.

OBJECTIVE To highlight issues that impact on cochlear implant candidacy and performance in individuals who have severe bilateral sensorineural hearing loss after major blunt head trauma. STUDY DESIGN Retrospective case review. SETTING Tertiary level neurotology center. PATIENTS Twenty-five patients treated at our institution for severe hearing loss after blunt head trauma INTERVENTION Assessment of cochlear implant candidacy criteria and postimplant outcomes in patients presenting with severe hearing loss after major blunt head trauma MAIN OUTCOME MEASURES Cause of injury, pure tone audiology, open set speech perception (preimplant and postimplant), promontory stimulation, and imaging results. RESULTS Of the 25 patients with severe hearing loss, 11 ultimately underwent cochlear implantation. Fall from a height was the most common cause of injury. Five patients experienced bilateral temporal bone fractures. Seventeen patients satisfied candidacy criteria for implantation. For those patients who did undergo cochlear implantation, the mean postimplant open set speech recognition score was 71. Issues related to cochlear implant candidacy and outcomes for implantation will be discussed. CONCLUSION Severe hearing loss after major blunt head trauma presents a number of unique clinical challenges. A significant number of these patients are not suitable for cochlear implantation for a variety of reasons. Clinicians working in the area of cochlear implantation should be aware of the various problems that can impact on effective cochlear implantation in such patients.

OBJECTIVE To assess the prevalence of Connexin 26 (GJB2), Connexin 30 (GJB6), and Pendred (SLC26A4) mutations in a population of adult cochlear implant patients with a history of either early idiopathic or hereditary progressive sensorineural deafness. BACKGROUND Significant efforts have been applied in defining the epidemiology of Connexin 26 (GJB2)-associated hearing impairment in the pediatric population, yet the issue remains ambiguous for adult patients. Causation is important in this population because there are implications to prognosis, risk of associated medical manifestations, and for genetic counseling purposes. PATIENTS Adult patients meeting criteria for cochlear implantation with early-onset hearing loss assessed at an adult cochlear implant center from November 2007 to April 2009. INTERVENTION Genomic DNA samples from whole blood were tested with bidirectional sequence analysis for mutations in the coding region of the GJB2 and SLC26A4 genes and tested for large deletions of the GJB6 gene. RESULTS Fifty-seven patients were analyzed for GJB2 mutations. Eight patients (14%) were found to have GJB2-related hearing impairment; 5 patients were homozygous for the c.35delG mutation (genotype c.35delG/c.35delG), and 3 additional patients were compound heterozygotes with 2 different GJB2 mutations. Of these 8 patients with GJB2-related hearing impairment, 3 had serviceable hearing into their teenage years. One additional patient had 1 GJB2 variant (p.Met195Ile, heterozygous). None had GJB6 mutations. Of the 57 patients, 30 were also analyzed for SLC26A4 mutations. Three of these patients were compound heterozygotes for disease-causing SLC26A4 mutations, confirming SLC26A4-related hearing impairment. Three additional patients were found to have a single variant in SLC26A4. CONCLUSION The prevalence of GJB2- and SLC26A4-related hearing impairment in an adult population with early-onset severe sensorineural hearing loss is significant, suggesting the need for routine assessment for genetic etiologies in this group. We also note 3 individuals with causal connexin 26 mutations with subjective serviceable hearing into adolescence in our cohort.

OBJECTIVE: To describe a new clinical observation of "tactile dysgeusia," a phenomenon associated with otologic and skull base surgery likely caused by injury to the chorda tympani nerve (CTN) or the nervus intermedius (NI) with subsequent aberrant cross-innervation with somatosensory fibers in the surgical field. STUDY DESIGN: Descriptive case series. SETTING: Tertiary university referral center. METHOD: Eight patients described a sensation of touch-evoked dysgeusia after surgery. Seven patients underwent a variety of middle ear procedures including stapedotomies and mastoidectomies. One patient had a large cerebellopontine angle meningioma excised through a translabyrinthine approach. There were no preoperatively sensory symptoms of any kind. RESULTS: In five of seven patients who had an otologic procedure, the CTN nerve was transected, whereas in the remaining two, the nerve was preserved but stretched. In the meningioma patient, the facial nerve was preserved without clear identification of the NI; the facial nerve function was normal postoperatively. All of the otologic patients experienced symptoms of dysgeusia and sensory alteration triggered by touching various parts of the outer ear. One also has a secondary trigger in the V2 dermatome. In the meningioma patient, taste and sensory alteration was induced by stimulating the ipsilateral V2 and V3 dermatome. In all patients, the symptom was located in the lateral aspect of the ipsilateral hemitongue. CONCLUSION: Tactile dysgeusia is an unusual complication after otologic and neurotologic procedures. It is likely related to aberrant re-innervation of the special sensory fibers within the CTN nerve or the NI with somatosensory fibers. Postoperative "tactile dysgeusia" may not be so rare; its true incidence could only be ascertained through a more rigorous postoperative assessment.

Objectives/Hypothesis: Several prominent textbooks have claimed that a plexiform neurofibroma is pathognomonic for neurofibromatosis type I. This is not in agreement with the National Institutes of Health criteria, which require two signs to be present, one of which can be a plexiform neurofibroma. Is a plexiform neurofibroma pathognomonic for neurofibromatosis type I? Study Design: Retrospective chart review. Methods: Fifty-one patients with surgically resected plexiform neurofibromas between the years 1991 and 2001 were identified, and their charts reviewed. The presence and absence of any manifestation of neurofibromatosis type I as detailed in the National Institutes of Health criteria were recorded. The pathology database at the Hospital for Sick Children (HSC) was searched retrospectively. Results: Five of the 51 patients had a single solitary plexiform neurofibroma. They were followed at the neurofibromatosis clinic at the HSC with regular detailed examinations and no other stigmata were found. Conclusion: Although plexiform neurofibromas are highly suggestive of neurofibromatosis type I, they are not pathognomonic as claimed.

Background Many Canadians are affected by sensorineural hearing loss (SNHL) and those with severe or profound hearing loss may have poor hearing function despite optimized hearing aids. Cochlear implants (CI) offer effective hearing rehabilitation for these patients, however, concern continues to exist regarding possible effects of CI on the vestibular system and balance. The objective of this study was to conduct a pilot study assessing the effects of unilateral cochlear implantation (CI) on balance and the vestibular system in post-lingually deafened adults. Methods Twelve patients were included in this pilot study and were assessed pre-operatively and at immediate, 1 week, and 1 month post-operative intervals. Assessments consisted of the dizziness handicap inventory (DHI), subjective visual vertical (SVV), and timed up-and-go testing (TUG). When applicable, testing was repeated with the CI on and off. Results Many patients were found to have deviated SVV at pre-operative and post-operative assessments. However, statistically significant changes were not seen when comparing pre-operative and post-operative SVV or when comparing SVV with the CI on and with the CI off. DHI was found to improve in five patients and worsen in two patients, however, no statistically significant change was found in DHI scores or with TUG testing. Conclusions This current pilot study does not indicate that CI surgery or implant activity influence vestibular or balance function, however, this pilot study is underpowered and greater numbers of patients would need be assessed to confirm these findings.

Background The prognosis for adult patients with Ph(-) B-precursor acute lymphoblastic leukaemia (ALL) who are refractory to treatment or experience relapse (R/R), is poor; over 90 % of these patients die from the disease, typically within a few months. While there are some national guidelines published for the treatment of adult patients with ALL, and local working group recommendations do exist, there is very little detail and no preferred treatment regimens for adult patients with R/R Ph(-) B-precursor ALL. The aim of this study was to describe current real-world clinical practice in Europe for the management and treatment of adult R/R Ph(–) B-precursor ALL. Methods A web-based, double-blind survey was conducted in November/December 2013 in France, Germany, Italy, Spain, and the UK. The survey was developed following consultation with specialist clinicians and a critical review of published literature. Eligible clinicians (15 per country) were board-certified in haemato-oncology or haematology; had at least 4 years of experience in their current role and had treated at least five patients with adult ALL in the 36 months before the survey, including at least one with R/R Ph(−) B-precursor ALL. Results Clinicians across the five countries consulted 16 guidelines and local working group recommendations for the diagnosis and treatment of R/R Ph(–) B-precursor ALL. Thirty three regimens for salvage therapy were reported; the most frequently cited was augmented hyper-CVAD (15 %), with vincristine the most commonly used agent. Salvage therapy regimens involved a range of agents, and most respondents reported using at least one cytotoxic agent; across respondents 10 different cytotoxic agents were cited. All respondents reported that toxicity was common for the regimens they used to treat R/R Ph(–) B-precursor ALL. Conclusions This study provides evidence of current management and treatment patterns of R/R Ph(–) B-precursor ALL in the real-world clinical practice in Europe. The approach to the treatment of R/R Ph(–) B-precursor ALL is heterogeneous, reflecting the lack of any clearly superior chemotherapeutic option, thus it appears that clinicians are trying a wide variety of therapies. These findings show a clear need for effective, tolerable treatments for R/R Ph(–) B-precursor ALL. Electronic supplementary material The online version of this article (doi:10.1186/s12885-015-1745-4) contains supplementary material, which is available to authorized users.