Construction of an MRI-based decision tree to differentiate autoimmune and autoinflammatory inner ear disease from chronic otitis media with sensorineural hearing loss.

Autoimmune and autoinflammatory inner ear diseases (AIED/AID) are characterized by the symptom of sensorineural hearing loss (SNHL). To date, standardized diagnostic tools for AIED/AID are lacking, and clinically differentiating AIED/AID from chronic otitis media (COM) with SNHL is challenging. This retrospective study aimed to construct a magnetic resonance imaging (MRI)-based decision tree using classification and regression tree (CART) analysis to distinguish AIED/AID from COM. In total, 67 patients were enrolled between January 2004 and October 2019, comprising AIED/AID (n = 18), COM (n = 24), and control groups (n = 25). All patients underwent 3 T temporal bone MRI, including post-contrast T1-weighted images (postT1WI) and post-contrast FLAIR images (postFLAIR). Two radiologists evaluated the presence of otomastoid effusion and inner ear contrast-enhancement on MRI. A CART decision tree model was constructed using MRI features to differentiate AIED/AID from COM and control groups, and diagnostic performance was analyzed. High-intensity bilateral effusion (61.1%) and inner ear enhancement (postFLAIR, 93.8%; postT1WI, 61.1%) were the most common findings in the AIED/AID group. We constructed two CART decision tree models; the first used effusion amount as the first partitioning node and postT1WI-inner ear enhancement as the second node, whereas the second comprised two partitioning nodes with the degree of postFLAIR-enhancement of the inner ear. The first and second models enabled distinction of AIED/AID from COM with high specificity (100% and 94.3%, respectively). The amount of effusion and the degree of inner ear enhancement on MRI may facilitate the distinction between AIED/AID and COM with SNHL using decision tree models, thereby contributing to early diagnosis and intervention.

Utility and value of pre-operative CT and MRI for cochlear implantation in the elderly.

PURPOSE: To determine the utility and value of pre-operative imaging among the elderly population ≥70 y.o. with bilateral progressive sensorineural hearing loss undergoing cochlear implantation. MATERIALS AND METHODS: A retrospective, cross-sectional review was performed at a tertiary referral center between 2010 and 2018 including patients ≥70 y.o. with bilateral presbycusis who underwent preoperative imaging and cochlear implantation. Primary outcome was whether pre-operative imaging changed the surgeon's surgical plan such as side of implant or abort procedure entirely. Patient characteristics including age, sex, side of implant, imaging modality, whether imaging changed surgical plan, and surgical complications were reviewed. One-way analysis of variance with post-hoc tests using the Bonferroni and Fisher's exact test were used to examine differences between groups. Secondary outcome was cost of preoperative imaging. RESULTS: One hundred thirty-three patients (mean age 79.38 [5.51 SD]) who underwent a total of 142 surgical cases and 147 total scans. There were 92, 27, and 14 patients who underwent CT, MRI, or both, respectfully (n=133). Of the 142 implants that were placed, preoperative imaging did not reveal a contraindication to placing implant on one side over another. Total cost of imaging was $29,694. Estimated cost if 20% of cochlear implant eligible patients ≥70 y.o. underwent imaging is $7,763,490. CONCLUSION: Decreasing unnecessary preoperative imaging can potentially decrease cost in cochlear implantation. In this sample, preoperative imaging did not affect the surgeon's choice of which side to operate on. However, imaging may provide an anatomic roadmap and contribute to either surgical confidence or caution. With the increasing amount of cochlear implant eligible elderly adults, preoperative imaging needs to be more clearly defined in this unique population.

Asymmetric sensorineural hearing loss and vestibular schwannoma: when to image?

PURPOSE OF REVIEW: We review the literature on the use of audiometric protocols in the guidance of when to obtain MRI for detection of vestibular schwannoma. This discussion will focus on the sensitivity, specificity, and cost-effective analysis of audiometric criteria of asymmetric sensorineural hearing loss (ASNHL) when used to decide when MRI scans should be performed. RECENT FINDINGS: The sensitivity for detecting vestibular schwannomas when invoking published audiometric protocols for triggering MRI acquisition in ASNHL ranged from 50 to 100%. Specificity of these protocols ranged from 23 to 83%. Such audiometric protocols are efficient, achieving sensitivity and specificity at these rates while reducing the screening rate to 18 to 35%. The reduced procurement of MRI while using such audiometric protocols is associated with annual cost savings of between 23 and 82%. While no definitive recommendations can be made from this review, some audiometric protocols offer a better balance of sensitivity and specificity than others. SUMMARY: Audiometric protocols for triggering MRI acquisition in ASNHL for evaluation of vestibular schwannoma can be both sensitive and specific. These are competitive measures, and so no protocol is both 100% sensitive and specific. Such protocols become less effective in populations with increased incidence of noise-induced hearing loss. Invocation of such audiometric protocols can considerably reduce the annual cost of MRI evaluation for vestibular schwannomas.

Assessment of the Clinical Benefit of Imaging in Children With Unilateral Sensorineural Hearing Loss: A Systematic Review and Meta-analysis.

IMPORTANCE: Imaging used to determine the cause of unilateral sensorineural hearing loss (USNHL) in children is often justified by the high likelihood of detecting abnormalities, which implies that these abnormalities are associated with hearing loss and that imaging has a positive contribution to patient outcome or well-being by providing information on the prognosis, hereditary factors, or cause of hearing loss. OBJECTIVES: To evaluate the diagnostic yield of computed tomography (CT) and magnetic resonance imaging (MRI) in children with isolated unexplained USNHL and investigate the clinical relevance of these findings. EVIDENCE REVIEW: Cochrane Library, Embase, PubMed, and Web of Science databases were searched for articles published from 1978 to 2017 on studies of children with USNHL who underwent CT and/or MRI of the temporal bone. Two authors (F.G.R. and E.N.B.P.) independently extracted information on population characteristics, imaging modality, and the prevalence of abnormalities and assessed the studies for risk of bias. Eligibility criteria included studies with 20 or more patients with USNHL who had CT and/or MRI scans, a population younger than 18 years, and those published in English. MAIN OUTCOMES AND MEASURES: The pooled prevalence with 95% CI of inner ear abnormalities grouped according to finding and imaging modality. FINDINGS: Of 1562 studies, 18 were included with a total of 1504 participants included in the analysis. Fifteen studies were consecutive case studies and 3 were retrospective cohort studies. The pooled diagnostic yield for pathophysiologic relevant findings in patients with unexplained USNHL was 37% for CT (95% CI, 25%-48%) and 35% for MRI (95% CI, 22%-49%). Cochleovestibular abnormalities were found with a pooled frequency of 19% for CT (95% CI, 14%-25%) and 16% for MRI (95% CI, 7%-25%). Cochlear nerve deficiency and associated cochlear aperture stenosis had a pooled frequency of 16% for MRI (95% CI, 3%-29%) and 44% for CT (95% CI, 36%-53%), respectively. Enlarged vestibular aqueduct (EVA) was detected with a pooled frequency of 7% for CT and 12% for MRI in children with USNHL. CONCLUSIONS AND RELEVANCE: Imaging provided insight into the cause of hearing loss in a pooled frequency of about 35% to 37% in children with isolated unexplained USNHL. However, none of these findings had therapeutic consequences, and imaging provided information on prognosis and hereditary factors only in a small proportion of children, namely those with EVA. Thus, there is currently no convincing evidence supporting a strong recommendation for imaging in children who present with USNHL. The advantages of imaging should be carefully balanced against the drawbacks during shared decision making.

Distortion product oto-acoustic emission: a superior tool for hearing assessment than pure tone audiometry.

OBJECTIVE: Hearing plays a crucial role in the performance of a soldier and is important for communication and speech processing. Service personnel are constantly exposed to high levels of noise and hence predisposed to occupational health disabilities, principally noise-induced hearing loss (NIHL) and tinnitus. NIHL is a significantly common impairment in the military and can affect the warfare performances. Parochial awareness about the effects of uproarious noise which exacerbates their hearing, acceptance rate of use of hearing protection devices and follow-up audiological tests have become the major drawback for prevention of NIHL and are less pondered upon. METHOD: The present study focuses on the effects of noise on the hearing of service crew operating and maintaining military equipment and explores the efficacy of DPOAE's to substitute pure tone audiometry as a quick, easy to operate and implementable test technique for monitoring of hearing status. RESULTS: The results suggest that DPOAE test is important and more coherent than audiometry alone for the early detection of cochlear injury due to noise from military operations and efficacious for detecting NIHL.

Peri-operative electrically evoked auditory brainstem response assessment of facial nerve/cochlea interaction at cochlear implantation.

OBJECTIVES: Dehiscence between the cochlear otic capsule and the facial nerve canal is a rare and relatively newly described pathology. In cochlear implantation (CI), this dehiscence may lead to adverse electric facial nerve stimulation (FNS) already at low levels, rendering its use impossible. Here, we describe an assessment technique to foresee this complication. METHODS: Pre- and postoperative computed tomography (CT) scans and intraoperative electrically evoked auditory brainstem response (e-ABR) measurements were analyzed in two patients with cochlear-facial dehiscence (CFD). RESULTS: Because of the relatively low resolution, the confirmation of CFD with a clinical CT was difficult. The e-ABR displayed a large potential with 6 and 7.5 ms latency, respectively, which did not occur otherwise. DISCUSSION: Potential strategies to resolve and manage FNS are described. CONCLUSION: Prediction of FNS by assessing the distance between the labyrinthine portion of the facial nerve and the cochlea is difficult using conventional CT scans. A large evoked late myogenic potential at low stimulation levels during intraoperative e-ABR measurement may foresee FNS at CI activation.

Assessment of the cochlear nerve to facial nerve size ratio using MR multiplanar reconstruction of the internal auditory canal in patients presenting with acquired long-standing hearing loss.

OBJECTIVE: To test using the facial nerve as a reference for assessment of the cochlear nerve size in patients with acquired long-standing sensorineural hearing loss (SNHL) using MRI multiplanar reconstruction. METHODS: The study was retrospectively performed on 86 patients. Group 1 (study group, n = 53) with bilateral long-standing SNHL. Group 2 (control group, n = 33) without hearing loss. The nerve size was measured by drawing a region of interest around the cross-sectional circumference of the nerve in multiplanar reconstruction images. RESULTS: No significant correlation was noted between the cochlear nerve and facial nerve size, and the patient's age, gender and weight (p > 0.05). In Group 1, the mean ratio of the cochlear to facial nerve size was 0.99 ± 0.30 (range: 0.52-1.86) and 1.12 ± 0.35 (range: 0.34-2.3) for the right and left sides, respectively. In Group 2, it was 1.18 ± 0.23 (range: 0.78-1.71) and 1.25 ± 0.25 (range: 0.85-1.94) for the right and left sides, respectively. The cochlear nerve size was statistically (p = 0.0004) smaller in Group 1 than in Group 2. CONCLUSION: The cochlear nerve size and the cochlear to facial nerve size ratio are significantly smaller in patients with acquired long-standing SNHL. Advances in knowledge: The facial nerve can be used as a reference for assessment of the cochlear nerve in patients with acquired long-standing SNHL.

To image or not to image? A cost-effectiveness analysis of MRI for patients with asymmetric sensorineural hearing loss.

OBJECTIVES/HYPOTHESIS: Our objective was to perform an economic analysis evaluating whether ordering a magnetic resonance imaging (MRI) is a cost-effective practice in the workup of undifferentiated asymmetric sensorineural hearing loss (ASNHL). Use of T1 gadolinium-weighted MR (GdT1W) and T2 weighted MR without contrast (T2MR) was each examined. METHODS: The incremental cost-effectiveness ratio (ICER) of MR imaging among ASNHL patients was evaluated using a decision tree. We calculated what the probability of having a cerebellopontine angle/internal auditory canal lesion would have to be to make MR more cost-effective than observation. The decision pathways included observation, GdT1W, and T2MR. The probability of detecting a lesion and associated costs were employed in constructing our tree. Missing a mass in the observation branch was considered to have an effectiveness of 0. The costs and probabilities were extracted from previously published studies. RESULTS: The ICERs of pursuing GdT1W and T2MR were $27,660 and $15,943, respectively, both below the widely accepted willingness to pay (WTP) thresholds of $30,000 and $50,000. Probabilistic sensitivity analysis with Monte Carlo simulations for GdT1W showed that it is more cost-effective than no imaging, with 54.4% and 83.5% certainty at $30,000 and $50,000 WTP thresholds. Probabilistic sensitivity analysis with Monte Carlo simulations for T2MR showed that it is more cost-effective than no imaging, with 75.2% and 92.6% certainty at $30,000 and $50,000 WTP thresholds. CONCLUSION: This economic evaluation strongly supports pursuing MRI in patients with documented ASNHL as a cost-effective strategy. Both GdT1W and T2MR are more cost-effective than observation. Furthermore, noncontrast T2 imaging may be the more cost-effective modality of these two techniques. LEVEL OF EVIDENCE: 2c. Laryngoscope, 127:939-944, 2017.

Charges associated with imaging techniques in evaluation of pediatric hearing loss.

OBJECTIVE: The best imaging study for evaluation of pediatric hearing loss is debated and it is well known magnetic resonance imaging is more costly than computed tomography. The objective of this study is to evaluate charges of computed tomography temporal bone (CTTB) versus magnetic resonance imaging brain, internal auditory canal/cerebellopontine angle (MRI IAC/CPA), with and without sedation in the pediatric population in order to assess to what extent the charges for the procedure are increased. In addition, differences in need for sedation and duration of sedation will be evaluated. METHODS: All patients, 0-18 years that underwent CTTB or MRI IAC/CPA, between January 2013 through December 2014 within department of otolaryngology. RESULTS: 120 CTTBs (118 non-sedated and 2 sedated) and 51 MRI IAC/CPAs (32 non-sedated and 19 sedated) were performed. Average charge for non-sedated CTTB was $1856. CTTB scan under sedation incurred total additional charges of $2385. Average charges for non-sedated MRI IAC/CPA was $3770. Technical charges for sedated MRI IAC/CPA was $151 lower ($2858) but had additional sedation charges of $2256, a recovery room charge of $250, and additional professional fees of $1496 for total charges of $7621. 37% of MRI IAC/CPAs needed sedation to be completed in comparison to 1.6% of CTTB. CONCLUSION: MRI IAC/CPAs are, on average, twice as costly as CTTBs. Almost 40% of patients need sedation to complete MRI IAC/CPA. These considerations may factor into decision making when choosing imaging modality in evaluation of pediatric hearing loss.

High-frequency sensorineural hearing loss in children.

OBJECTIVES/HYPOTHESIS: Determine the prevalence of high-frequency sensorineural hearing loss (HFSNHL) in our hearing loss population and a diagnostic algorithm for these patients. STUDY DESIGN: Retrospective case series. METHODS: We identified patients diagnosed with sensorineural hearing loss (SNHL) at our pediatric tertiary care institution from 1981 to 2010. Based on audiometric profiles, these patients were subdivided into those with a flat SNHL configuration and those with HFSNHL. Imaging and genetic testing data and data regarding age at diagnosis, laterality, and risk factors were obtained for both groups. Comparisons were then made between the two groups. RESULTS: Of 2,867 patients included in the study, 7.6% had HFSNHL. Age at diagnosis was significantly higher in HFSNHL patients (8.3 years vs. 6.1 years; P < .0001). These patients also had a significantly higher proportion of unilateral versus bilateral loss (49.1% vs. 26.1%; P < .0001); unilateral losses were also less severe. Genetic testing showed no significant difference between groups in the proportion of patients tested or in those who tested positive. Similarly, imaging data revealed no difference in the proportion of patients tested in the two groups; however, overall diagnostic yield was significantly higher in flat SNHL patients (29.5% vs.17.3; P = .02). CONCLUSIONS: The positive predictive value of simple genetic testing is similar to that of imaging studies. However, given cost differences between genetic testing and imaging, it is prudent to perform genetic testing as the initial diagnostic test. Determination of whether high-throughput, multigene diagnostic platforms offer an added benefit in the evaluation of children requires further study. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:1236-1240, 2016.

Evaluating the role of single-photon emission computed tomography in the assessment of neurotologic complaints.

We conducted a retrospective study to reexamine the value of single-photon emission computed tomography (SPECT) in the evaluation of patients with neurotologic complaints, and to assess the intra- and inter-radiologist variability of SPECT readings. Our study population was made up of 63 patients--23 men and 40 women, aged 34 to 91 years (mean: 59)--who had presented to a tertiary care otolaryngology practice and university hospital for evaluation of head trauma, sensorineural hearing loss, tinnitus, and/or vertigo. All patients had undergone brain scanning with SPECT during their evaluation, and almost all had also undergone magnetic resonance imaging (MRI) and standard computed tomography (CT). We compared the findings of all three imaging modalities in terms of their ability to detect neurotologic abnormalities. We found that detection rates were very similar among the three modalities; abnormalities were found in 24% of SPECT scans, 26% of MRIs, and 23% of CTs. Nevertheless, we did find that among 60 patients who underwent all three types of imaging, 13 (22%) exhibited areas of cerebral hypoperfusion on SPECT while their MRIs and CTs were read as either normal or nonspecific. In all, 18 of these 60 patients (30%) exhibited normal or nonspecific findings on all three types of imaging. In addition, when SPECT scans were read by the same radiologist at different times, different results were reported for 17 of the 63 scans (27%). Likewise, when SPECT scans were read by different radiologists, different results were reported for 21 of 63 scans (33%). We conclude that SPECT may be a valuable complementary diagnostic modality for making a comprehensive neurotologic evaluation and that it may detect abnormalities in some patients whose other imaging is read as normal. However, we did not find that SPECT was the most sensitive of the three modalities in neurotologic evaluation, as we had previously found in a preliminary study that the senior author (R.T.S.) published in 1996. In addition, with respect to our radiologists, both their intra- and inter-reader reliability was low, and we recommend additional study on this matter.

Etiologic and diagnostic evaluation: algorithm for severe to profound sensorineural hearing loss in Brazil.

OBJECTIVE: Evaluation of the effectiveness of imaging and genetic testing, and establishment of a cost-effective diagnostic protocol for the etiologic diagnosis of sensorineural hearing loss (SNHL) in Brazil. DESIGN: Prospective cohort study. STUDY SAMPLE: Analysis of 100 unrelated Brazilian patients with severe to profound bilateral SNHL submitted to cochlear implant (CI) between 2002 and 2010 at the University of Campinas hospital. The study was based upon three groups: individuals with congenital, progressive, and sudden SNHL. RESULTS: After the diagnostic investigation, the number of cases with unknown etiology was reduced from 72 to 42 (a 42% reduction); 25% of cases were due to environmental factors, 19% to genetic causes, and 14% to inner-ear abnormalities or other clinical features. The genetic and imaging findings contributed to the diagnosis of SNHL in 19% and 20% of the cases analysed, respectively. Molecular testing mainly contributed to the diagnosis of patients with congenital SNHL, while the contribution of radiologic examination was higher for individuals with progressive or sudden SNHL. A sequential diagnostic protocol was proposed based on these data. CONCLUSIONS: The proposed diagnostic workup algorithm could provide better optimization of etiologic diagnosis, as well as reduced costs, compared to a simultaneous testing approach.

Molecular epidemiology and functional assessment of novel allelic variants of SLC26A4 in non-syndromic hearing loss patients with enlarged vestibular aqueduct in China.

BACKGROUND: Mutations in SLC26A4, which encodes pendrin, are a common cause of deafness. SLC26A4 mutations are responsible for Pendred syndrome and non-syndromic enlarged vestibular aqueduct (EVA). The mutation spectrum of SLC26A4 varies widely among ethnic groups. To investigate the incidence of EVA in Chinese population and to provide appropriate genetic testing and counseling to patients with SLC26A4 variants, we conducted a large-scale molecular epidemiological survey of SLC26A4. METHODS: A total of 2352 unrelated non-syndromic hearing loss patients from 27 different regions of China were included. Hot spot regions of SLC26A4, exons 8, 10 and 19 were sequenced. For patients with one allelic variant in the hot spot regions, the other exons were sequenced one by one until two mutant alleles had been identified. Patients with SLC26A4 variants were then examined by temporal bone computed tomography scan for radiological diagnosis of EVA. Ten SLC26A4 variants were cloned for functional study. Confocal microscopy and radioisotope techniques were used to examine the membrane expression of pendrin and transporter function. RESULTS: Of the 86 types of variants found, 47 have never been reported. The ratio of EVA in the Chinese deaf population was at least 11%, and that in patients of Han ethnicity reached at least 13%. The mutational spectrum and mutation detection rate of SLC26A4 are distinct among both ethnicities and regions of Mainland China. Most of the variants caused retention of pendrin in the intracellular region. All the mutant pendrins showed significantly reduced transport capability. CONCLUSION: An overall description of the molecular epidemiological findings of SLC26A4 in China is provided. The functional assessment procedure can be applied to identification of pathogenicity of variants. These findings are valuable for genetic diagnosis, genetic counseling, prenatal testing and pre-implantation diagnosis in EVA families.

Topics in pediatric head and neck imaging: self-assessment module.

OBJECTIVE: The educational objectives for this self-assessment module are for the participant to exercise, self-assess, and improve his or her understanding of radiology in pediatric congenital sensorineural hearing loss, posterior plagiocephaly, and midline nasofrontal mass. CONCLUSION: The activities in these articles guide the participant to understanding the key concepts of radiology in pediatric congenital sensorineural hearing loss, posterior plagiocephaly, and midline nasofrontal mass.

Assessment of changes in cochlear function with pneumolabyrinth after middle ear trauma.

OBJECTIVE: To describe a case of pneumolabyrinth secondary to tympanic membrane/ossicular trauma and the subsequent recovery of sensorineural hearing loss managed with conservative measures. PATIENTS: A 15-year-old boy presented to an outside hospital with signs and symptoms of acute hearing loss, vertigo, and tinnitus after penetrating injury to his right tympanic membrane. In addition, computed tomography demonstrated air density within the vestibule. INTERVENTIONS: The patient was managed conservatively with bed rest, avoidance of straining, corticosteroids, and antibiotics. MAIN OUTCOME MEASURES: Computed tomography, audiologic testing. RESULTS: Patient recovered near-normal hearing subjectively. There was closure of the air-bone gap (

[Radionuclide tests of cerebral circulation in the assessment of treatment of sensorineural hearing loss]. / Radionuklidnye metody issledovaniia krovoobrashcheniia golovnogo mozga v otsenke rezul'tatov lecheniia neirosensornoi tugoukhosti.

Examination and treatment results are available for 59 patients with acute and chronic neurosensory hypoacusis. Cerebral circulation was investigated at radionuclide carotid arteriography and radionuclide single-photon emission computed tomography. Arterial flow to the brain, microcirculation of the gray matter and venous outflow from the cranial cavity were studied before and after the course of quantum hemotherapy.

Neuro-otological function in X-linked hearing loss: a multipedigree assessment and correlation with other clinical parameters.

Auditory and vestibular investigations were carried out in 19 affected men and 13 obligate female carriers of 7 pedigrees with nonsyndromic hearing loss segregating as an X-linked trait. In addition, high resolution computerised tomographic scanning was carried out in 24 affected males and 12 obligate female carriers. The neuro-otological results confirm that non syndromic X-linked hearing loss is a clinically heterogeneous condition, but radiological assessment of the cochlea revealed two distinct groups: a normal group, and an abnormal group characterised by a bulbous internal auditory meatus, a dilated facial nerve canal and incomplete separation of the basal coil of the cochlea from the internal auditory meatus. Within a given pedigree there was marked consistency of the presence or absence of the CT scan abnormality in the affected males. One third of the obligate female carriers of the radiologically abnormal pedigrees were shown to have a similar abnormal finding, but as two thirds were normal, radiological examination did not predict carrier status. In the affected men, pure tone audiometric data did not correlate with the radiological abnormality, whereas vestibular function was strikingly correlated, being normal in all but one case in pedigrees with normal radiology and absent, or grossly impaired, in the pedigrees with abnormal radiology. Neuro-otological abnormalities were documented in approximately two thirds of the obligate female carriers, but were insufficiently frequent in occurrence or specific in type to be of predictive value.