Enlarged Vestibular Aqueduct Research Articles

Vestibular impairment in cochlear implanted children presenting enlarged vestibular aqueduct and enlarged endolymphatic sac

Enlarged vestibular aqueduct (EVA) is a common finding in tomodensitometry. When cranial MRI is performed, enlarged endolymphatic sac (EES) can also be found. Profound hearing loss is a common finding in these patients but a few studies have investigated vestibular function after cochlear implantation (CI) in EVA and EES patients. Our main objective was to find out whether in EVA children candidates to CI, a higher endolymphatic sac (ES) volume was predictive for higher rates of postsurgical vestibular complications. MethodsWe retrospectively included EVA children who benefited from CI, during the last 2 years. Two groups were constituted according to the presence or not of a vestibular impairment (decrease in the VOR gain on the VHIT test on one of the semicircular canals and/or a loss of cVEMPs) 6 months after CI. Endolymphatic volume of both VA and ES was measured for each patient. ResultsFifteen patients were included. The mean endolymph volume was significantly higher in the impaired group (0.40 cm3 ± 0.23, range 0.08–0.70) than in the non-impaired group (0.11 cm3 ± 0.07, range 0.04–0.29; p = 0.029). Four children of the impaired group were followed during one year. At the end of vestibular rehabilitation, all children recovered a lateral canal function and a saccular function. ConclusionIn EVA children, a combined EES appears to increase the risk of severe post CI vestibular impairment. To minimize this risk prior CI surgery, besides tomodensitometry, MRI measurement of the ES volume should be systematically performed.

Enlarged Vestibular Aqueduct: Hearing Progression and Cochlear Implant Candidacy in Pediatric Patients.

Investigate the rate of hearing loss progression and incidence of cochlear implant candidacy in children with enlarged vestibular aqueduct (EVA). EVA is the most common congenital malformation of the inner ear, is responsible for a large percentage of children with hearing loss, and is associated with hearing loss progression. Rates and degree of progression of hearing loss to cochlear implantation candidacy have not been well described. Review of children with EVA who presented to a single academic medical center. Audiometric data were reviewed to determine subjects who met criteria for cochlear implantation (≥75 dB pure-tone average) at presentation. For those not meeting criteria, serial audiometric data were reviewed for progression to candidacy. A total of 257 ears met inclusion criteria. One hundred ninety-two (74.7%) met cochlear implant candidacy criteria by age 12, yet only 117 ears (60.9%) actually received implants before turning 13. One hundred fifty-three ears (59.5%) met implant candidacy criteria at presentation. Nearly 50% of those not initially meeting implantation criteria had a ≥15 dB shift in pure-tone average by age 12, with 37.5% of this subgroup meeting implant candidacy criteria before their teen years at an average age of 7.10 years. The majority of children with EVA reach cochlear implant candidacy before reaching adulthood, yet implantation rates for candidate ears remain at 60% and delay in implantation persist. Parents of children with EVA should be counseled on the likelihood of progression and closely monitored for cochlear implant candidacy.

Computer tomography demonstrations of single-sided deafness

Objective:To investigate the distribution of common inner ear and internal auditory canal malformations in children with single-sided deafness（SSD） ,and to explore the imaging etiology of SSD by comparing the quantitative parameters of key bone structures between deaf and normal ears in children with congenital SSD. Method:Forty children with SSD diagnosed in the Second Hospital of Lanzhou University from September 2016 to March 2019 were collected. All of them underwent HRCT examinations of temporal bone . The area of bone island, the width of vestibular, the width of internal auditory canal, the height of cochlear and the width of cochlear basal axis were measured. Paired t test was used to compare the difference between the hearing abnormality and normal hearing in children with SSD. Result:The rate of inner ear deformity was 62.5% in SSD group,the most common deformity was cochlear nerve canal deformity, 20 cases （50.0%） of cochlear canal stenosis and 3 cases （7.5%） of cochlear canal atresia.The second most common deformity was internal auditory canal deformity, including 5 cases （12.5%） of internal auditory canal stenosis and 1 case （2.5%） of internal auditory canal atresia. Other malformations included 1 case（2.5%） of RO, 2 cases （5.0%） of incomplete partition （IP） type II and 1 case （2.5%） of enlargement of vestibular aqueduct （EVA）. There are no significant difference in the measured results of the key structures of the inner ear between two groups except the width of cochlear nerve canal, internal auditory canal and the area of bone island. Conclusion:The main inner ear deformities in children with SSD are cochlear nerve canal stenosis and inner auditory canal stenosis. HRCT of temporal bone has high diagnostic value for inner ear deformities in children with SSD.

Digenic inheritance of mutations in EPHA2 and SLC26A4 in Pendred syndrome

Enlarged vestibular aqueduct (EVA) is one of the most commonly identified inner ear malformations in hearing loss patients including Pendred syndrome. While biallelic mutations of the SLC26A4 gene, encoding pendrin, causes non-syndromic hearing loss with EVA or Pendred syndrome, a considerable number of patients appear to carry mono-allelic mutation. This suggests faulty pendrin regulatory machinery results in hearing loss. Here we identify EPHA2 as another causative gene of Pendred syndrome with SLC26A4. EphA2 forms a protein complex with pendrin controlling pendrin localization, which is disrupted in some pathogenic forms of pendrin. Moreover, point mutations leading to amino acid substitution in the EPHA2 gene are identified from patients bearing mono-allelic mutation of SLC26A4. Ephrin-B2 binds to EphA2 triggering internalization with pendrin inducing EphA2 autophosphorylation weakly. The identified EphA2 mutants attenuate ephrin-B2- but not ephrin-A1-induced EphA2 internalization with pendrin. Our results uncover an unexpected role of the Eph/ephrin system in epithelial function.

An analysis of surgical management of difficulties during cochlear implant with inner ear anomalies

Objective:The purpose of this study is to review the difficulties that can occur during cochlear implant surgery in patients with inner ear abnormalities and the management. Method:A retrospective analysis was made on 186 cases of cochlear implant with inner ear malformation, the types of inner ear malformations included 6 cases（3.23%） of isolated semicircular dysplasia, 137 cases（73.66%） of isolated large vestibular aqueducts, 26 cases（13.98%） of Mondini malformations, 6 cases（3.23%） of incomplete septal type Ⅲ, 3 cases（1.61%） of internal auditory stenosis, 7 cases（3.76%） of cochlear dysplasia and 1 case（0.54%） of incomplete septal typeⅠ. Two hundred patients with normal inner ear structures were randomly selected as the control group. The data collected included the types of inner ear abnormalities, intraoperative manifestations, clinical management strategies, and postoperative speech rehabilitation, and the literature was reviewed. Result:148 patients（77.49%） with inner ear malformation underwent successful surgery, electrode insertion was incomplete in 6 patients（3.14%）, and cerebrospinal fluid blowout occurred in 29 patients（15.18%）, it was difficult to locate the window because of the abnormal structure of the window in 8 cases（4.19%）. In 191 patients, the facial recess approach was adopted intraoperatively, and 17.8% of the patients had significant structural abnormalities of the facial nerve, significantly more than the group with normal inner ear structure. Only 1 patient showed delayed facial nerve paralysis 1 week after surgery, and recovered well after treatment. 6.81% of the patients adopted the expanded round window approach, which was significantly lower than that of the group with normal inner ear structure（28%）. There was no significant difference between patients with inner ear malformation and patients with extremely severe deafness with normal inner ear structure who received cochlear implant in speech rehabilitation. Conclusion:Cochlear implant is safe, feasible and effective for patients with inner ear malformation. For patients with inner ear malformation, special attention should be paid to the preoperative imaging reading to predict the possible risks during the operation. The safest surgical plan, including the type of electrode and the manner in which the window is opened, must be prepared before the operation, and the operation must be performed or directed by an experienced surgeon who can adjust the optimal surgical plan according to what is seen during the operation.

Absence of Endolymphatic Sac Ion Transport Proteins in Large Vestibular Aqueduct Syndrome—A Human Temporal Bone Study

Epithelial ion transport pathologies of the endolymphatic sac (ES) are associated with large vestibular aqueduct syndrome (LVAS). LVAS is defined by the pathognomonic features of a widened bony vestibular aqueduct (VA) and an enlarged ES. The underlying cause of its associated cochleovestibular symptoms remains elusive. Disturbances in epithelial ion transport in the enlarged ES, affecting inner ear fluid regulation, were proposed as a possible pathophysiology. However, although respective epithelial ion transport pathologies have been demonstrated in the enlarged ES from transgenic LVAS mouse models, these pathologies have not been investigated in human LVAS cases. Histological and immunohistochemical analysis of the enlarged ES epithelium in postmortem temporal bones from two individuals with a clinical diagnosis of LVAS. The enlarged ES epithelium demonstrated an overall atypical epithelial differentiation and a lack of the immunolocalization of signature ion transport proteins. Notably, in both cases, a rudimentary branch of the ES with a typically differentiated ES epithelium was present. The described cellular and molecular pathologies of the enlarged ES in humans provide evidence of epithelial transport pathology as one potential cause of cochleovestibular symptoms in LVAS. The present findings also emphasize the clinical relevance of already established LVAS mouse models.

Hearing Loss in Enlarged Vestibular Aqueduct: A Prognostic Factor Systematic Review of the Literature.

There is a need to highlight individual prognostic factors determining hearing loss in enlarged (wide) vestibular aqueduct, as currently clinicians cannot counsel parents about the expected clinical course, nor provide individualized hearing rehabilitation plans following identification at newborn screening. We apply a novel methodology to specifically outline and assess the accuracy of prognostic factors reporting for hearing loss in enlarged vestibular aqueduct. A preferred reporting items for systematic reviews and meta-analyses compliant systematic review (Prospero ID: CRD42019151199), with searches applied to Medline, EMBASE, and Cochrane. Studies with longitudinal design were included between 1995 and 2019. The CHARMS-PF tool was used to assess robustness of prognostic factor study designs. The QUIPS tool was used to assess for individual study risk of bias. Seventy papers were suitable for data extraction. In the six studies with low risk of bias, the domains of enlarged vestibular aqueduct (EVA) morphology, age, hearing thresholds, sex, head trauma, and genotype provided exploratory prognostic factors for hearing loss associated with enlarged vestibular aqueduct. Overall, study heterogeneity and risk of bias precluded reporting by forest plots and meta-analysis. The majority of exploratory prognostic factor studies for hearing loss associated with enlarged vestibular aqueduct are hampered by risk of bias. However, this systematic review identifies potential independent prognostic factors which should be measured, and adjusted for, in subsequent confirmatory studies utilizing multivariate analysis. This would determine the true independent prognostic effects associated with hearing loss in enlarged vestibular aqueduct, while facilitating prognostic model development and the ability to predict individual hearing loss trajectory.

Air and bone-conducted vestibular evoked myogenic potentials in children with large vestibular aqueduct syndrome

Background There are few studies focused on vestibular symptoms and function of the children with LVAS. Objectives This study aimed to find the characteristics of air and bone-conducted VEMPs among children with LVAS, and to investigate the relationship between VEMPs and vestibular symptoms. Material and methods A total of 44 children with LVAS and 10 healthy children were recruited as the case group and control group. Air and bone-conducted VEMP were performed to the participants. Results For air-conducted measurement, there was elevated amplitude of cVEMP in case group than control group. There was no significant difference at oVEMP parameters between the case group and control group. For bone-conducted measurement, significantly longer P1 latency and shorter P1-N1 latency of cVEMP were observed among the case group; there were a series of changes in oVEMP parameters among the case group. Logistic regression model revealed that air-conducted oVEMP asymmetric ratio was valuable to predict vestibular symptoms’ development among the kids with LVAS. Conclusion Asymmetric ratio of oVEMP could be used as one predictor of developing vestibular symptoms of the children with LVAS. Applying bone-conducted VEMP as one alternative parameter of vestibular syndrome is novel and will certainly remain an area of continued investigation.

A novel genotyping technique for discriminating LVAS-associated high-frequency variants in SLC26A4 gene

An increasing number of biological and epidemiological evidence suggests that c.919-2A > G and c.2168A > G variants of solute carrier family 26, member 4 (SLC26A4) gene play a critical role in the development of large vestibular aqueduct syndrome (LVAS). In this study, we developed a rapid genotyping method for discriminating LVAS-associated high-frequency variants in SLC26A4 gene. The genotyping technique consists of 3′ terminal exonuclease-resistant phosphorothioate-modified allele specific primer extension mediated by exo+ polymerase. In PCR amplification by Pfu polymerase, allelic specific primers perfectly matching wild type allele were extended while no specific products were yielded from primers targeting variant allele. Similarly, allelic specific primers perfectly matching variant allele were extended and no specific products were observed from primers targeting wild type allele. The clinical application of 3′ terminal phosphorothioate-modified allele specific primer extension mediated by Pfu polymerase identified both homozygous for SLC26A4 gene c.919-2A > G variant in two patients clinically diagnosed as LVAS by temporal bone CT scan. The genetic results from this method are consistent with that of DNA sequencing. The data suggest that exo+ polymerase-mediated 3′ terminal phosphorothioate-modified primer extension is reliable in the identification of SLC26A4 gene high-frequency variant prior to high-resolution CT scan. The method is extremely suitable for quickly molecular etiologic screening and early diagnosis and aggressive prevention therapy of LVAS.

Investigation of DFNB4 SLC26A4 mutation in patients with enlarged vestibular aquaduct

ObjectivesMutations of the SLC26A4 gene causing enlarged vestibular aqueduct (EVA) syndrome have not yet been fully elucidated. The study aimed to investigate SLC26A4 mutations in patients with EVA syndrome in the Turkish population. Identifying these mutations may play an essential role in determining the prognosis, follow-up, and management options of these patients. MethodsWhole exome sequencing and/or Sanger sequencing of SLC26A4 in 22 patients with sensorineural hearing loss associated with isolated EVA without inner ear anomalies, and 22 controls were performed. ResultsTwenty-two patients and 22 control subjects were included in the study. The onset of hearing loss was pre-lingual in 15 patients, and post-lingual in 7. The mean (standard deviation) vestibular aqueduct width of the patients was 3.23 mm (1.28). Twenty SLC26A4 variants, 15 of them unique, were identified in 22 patients. Among them, seven variants were heterozygous, and 13 were homozygous. The variants p.E37X (c.109G > T), p.Y27H (c.79T > C), p.C706Y (c.2117G > A) have not been previously reported. ConclusionThe detection of rare and previously unreported mutations in our study showed that studies with a larger number of patients with EVA might reveal more role of the SLC26A4 gene. Besides, to understand the etiopathogenesis of the disease, other related genes also should be investigated.

Enlarged vestibular aqueduct and Mondini Malformation: audiological, clinical, radiologic and genetic features

PurposeWhen referring to enlarged vestibular aqueduct (EVA) we should differentiate between nonsyndromic enlarged vestibular aqueduct (NSEVA) and Pendred Syndrome (PDS), a disease continuum associated with pathogenic sequence variants of Pendrin’s Gene (SLC26A4) in about half of the cases. The study was aimed to analyse the clinical and audiological features of a monocentric cohort of Caucasian patients with NSEVA/PDS, their genetic assessment and morphological inner ear features.MethodsWe retrospectively reviewed the audiologic, genetic and anamnestic data of 66 patients with NSEVA/PDS followed by our audiology service.ResultsSLC26A4 mutations was significantly correlated with the presence of PDS rather than NSEVA (p < 0.019), with the expression of inner ear malformations (p < 0.001) and with different severity of hearing loss (p = 0.001). Furthermore, patients with PDS showed significantly worse pure tone audiometry (PTA) than patients with NSEVA (p = 0.001). Anatomically normal ears presented significantly better PTA than ears associated with Mondini Malformation or isolated EVA (p < 0.001), but no statistically significative differences have been observed in PTA between patients with Mondini Malformation and isolated EVA.ConclusionNSEVA/PDS must be investigated in all the congenital hearing loss, but also in progressive, late onset, stepwise forms. Even mixed or fluctuating hearing loss may constitute a sign of a NSEVA/PDS pathology. Our findings can confirm the important role of SLC26A4 mutations in determining the phenotype of isolated EVA/PDS, both for the type/degree of the malformation, the hearing impairment and the association with thyroid dysfunction.

Biomechanical analysis of the clinical characteristics of enlarged vestibular aqueduct syndrome with Mondini malformation

Backgroundnumerous researches on the pathological mechanism of Enlarged Vestibular Aqueduct (EVA) syndrome mainly focuses on the genetic characteristics of SLC26A4 gene and the function of its encoding protein, Pendrin. One of the limitations with these explanations is that it does not explain why cerebrospinal fluid pressure can affect clinical manifestations.ObjectivesTo establish a new approach to explain the clinical manifestations of EVA syndrome with biomechanical method.Material and methods108 cases of EVA syndrome who received cochlear implantation were analyzed retrospectively. A cochlear model was built to reflect the differences of the structure in EVA syndrome with or without Mondini malformation. The CFD software was used to simulate and display the differences in mechanical pathogenic factors to which the model was subjected.ResultsEVA syndrome patients with Mondini malformation suffer more mechanical damage from the cerebrospinal fluid pressure due to their structural reason and their symptoms appear earlier and progress faster.ConclusionsBiomechanics is an important aspect of pathological mechanism of EVA syndrome, and it provides a new angle for clinical decision-making.

Significance of high signal intensity in the endolymphatic duct on magnetic resonance imaging in ears with otological disorders

BackgroundHigh signal intensity in the endolymphatic duct (ED) is occasionally observed on magnetic resonance imaging (MRI) in ears that have otological disorders.ObjectiveThe signal intensity (SI) in the ED on post-contrast MRI was investigated in subjects with various otological disorders, and the meaning of high SI in the ED was evaluated.Material and methods392 patients with otological disorders and 21 controls without otological symptoms underwent 3 T MRI. The SIs of the ED and the cerebellum were measured, the SI ratio (SIR) was calculated, and ears with SIR ≥4 were identified.ResultsA high SIR was identified in the ED of 3.7% of ears affected by definite Meniere’s disease (dMD), 100% of ears affected by large vestibular aqueduct syndrome (LVAS), and 7.1% of ears with no otological symptoms. On the whole, a significant relationship was found between the existence of vestibular or cochlear EH and the SIR in the ED.ConclusionThe MRI finding of high SI in the ED may indicate the mechanism of inner ear disturbances in ears with otological disorders, especially in those with LVAS, and it may suggest an underlying disorder in some ears in which otological symptoms are not apparent.

Detailed MR imaging assessment of endolymphatic hydrops in patients with SLC26A4 mutations.

ObjectiveMutations in SLC26A4 represent the second most common mutations in deafness patients. The majority of patients with SLC26A4 mutations have a large vestibular aqueduct (LVA). Recently, some reports showed the presence of endolymphatic hydrops (ELH) in patients with LVA on the basis of high-resolution enhanced 3T-MRI. However, detailed evaluation has not been performed. We provide the first report on ELH in LVA patients with biallelic SLC26A4 mutations. In this study, we focused on 1) the findings of ELH in LVA patients with biallelic SLC26A4 mutations, and 2) the findings of the endolymphatic duct (ED) and endolymphatic sac (ES) by using two different gadodimide (Gd) enhancement methods. Subjects and methodsFive patients with SLC26A4 mutations underwent enhanced 3T-MRI using the intratympanic (IT) or intravenous (IV) injection of Gd for the diagnosis ELH. ResultsAll of the patients had ELH in at least one ear. ELH was identified in the vestibule (8/10 ears) as well as in the cochlea (7/10 ears). With regard to the ED and ES, all ears for which MRI was performed with an IT injection of Gd had black areas in the ES or VA or both; however, all of the ears receiving an IV injection had no black areas and were well enhanced. ConclusionsA majority of the patients had severe ELH in the cochleo-vestibular endolymph, with two different patterns observed in the MRI findings of the ED and ES.

Grades of hearing loss affect the presence of acoustically evoked short latency negative responses in children with large vestibular aqueduct syndrome

ObjectivesWe aimed to investigate the relationship between grades of hearing loss and the presence of acoustically evoked short latency negative response (ASNR) in children with large vestibular aqueduct syndrome (LVAS), so as to enhance the reference value of ASNR for the diagnosis of LVAS in children. MethodsTwo hundred sixteen ears from 108 patients (aged 4–90 months) diagnosed with bilateral LVAS, with slight to profound hearing loss, were enrolled in the present study from January 2012 to December 2018. All of the cases were diagnosed with LVAS according to high-resolution computed tomography (HRCT) or magnetic resonance imaging (MRI) scans of the inner ears. The auditory brain stem response (ABR) tests were performed on these subjects with click stimulus (ck-ABR), and the ASNRs were detected based on the method recommended by previous studies. The degree of hearing loss for each ear was classified by the estimated pure-tone average (PTA) thresholds, which were calculated according to the ck-ABR thresholds. ResultsASNRs were present in 40.7% (88/216) ears during ck-ABR tests. Both thresholds of ABR (Z = 2.977, p = 0.003) and estimated PTA (Z = 2.977, p = 0.003) were significantly higher in the ASNR absent group than in the ASNR present group. The frequency of not profound hearing impairment (≤80 dB HL) was much higher in the ASNR present group (44/88; 50%) than in the ASNR absent group (40/128; 31.3%) (χ2 = 7.714, p = 0.005). The results of the logistic regression model, adjusted by cases’ age and gender, showed that compared with those ears with profound hearing impairment (>80 dB HL), the not profound impaired ears were associated with a 2.48-fold increased odds of recording ASNR presence in the ck-ABR test [odds ratio (OR) = 2.48, 95% confidence interval (CI): 1.38–4.46, p = 0.003]. ConclusionsGrades of hearing loss affect the presence of ASNR in children with LVAS, and manifesting as cases with not profound hearing impairment showed increased odds of recording ASNR in the ck-ABR test. Furthermore, more studies should be performed imperatively to determine the diagnosis value of ASNR in children with LVAS.

Progression of hearing loss and cochlear implantation in large vestibular aqueduct syndrome

ObjectivesLarge vestibular aqueduct syndrome (LVAS) is a congenital inner ear malformation that commonly results in progressive sensorineural hearing loss (SNHL) and cochlear implantation (CI). Though LVAS accounts for approximately 15% of pediatric SNHL, little is known regarding the rate and severity of SNHL in these patients. We sought to characterize the timing of SNHL progression to CI in patients with LVAS. MethodsWe performed a retrospective chart review at our institution from 2000 to 2018 using ICD-10 “large vestibular aqueduct syndrome,” and through identifying patients with CI who had LVAS. Demographic, surgical, and audiometric data were collected. Theoretical CI candidacy was approximated using a pure tone average (PTA) HL threshold of 70 dB. ResultsOf 103 patients, 96 had bilateral LVAS, and 7 had unilateral LVAS. Forty-one patients had bilateral implants, 52 had unilateral implants, and 10 were not implanted. The mean age at first implant was 8.62 years old [95%CI = 6.75,10.49], the mean age at second implant was 12.24 years old [95%CI = 8.33,16.15], and the mean time between implants was 4.37 years [95%CI = 3.02,5.73]. LVAS patients reached HL threshold of 70 dB at a mean age of 5.16 years old (SD = 3.04) for the “worse ear” and 9.08 years old (SD = 4.96) for the “better ear.” ConclusionsLVAS patients are a heterogenous population of patients, in which some may undergo progression of HL and some may not. Further, there may be a discrepancy in the timing between patients’ theoretical CI candidacy and when they undergo CI. In order to optimize timing of CI, individual monitoring and close observation of LVAS patients is recommended.

Enlarged vestibular aqueduct syndrome-dehiscence syndromes-honeycomb mastoid : Pathophysiology and evidence for clinical differentiation

Differential diagnosis of dizziness with hearing loss requires standardized procedures for detection and classification of rare congenital and acquired malformations of the petrous part of the temporal bone. The aim of this study was to present the physiology and pathophysiology of endolymphatic and perilymphatic pressure regulation, diagnostic guidelines, and aspects of prognosis and treatment. Relevant publications and guidelines were evaluated and own cases are reported. Enlarged vestibular aqueduct (EVA) is the most frequently observed malformation of the inner ear, which leads to increased internal hydrostatic pressure and cochleovestibular dysfunction. Non-syndromic and syndromic forms, e.g., Pendred syndrome, are known. Other pressure-relevant malformations are semicircular canal dehiscence syndrome (SCDS) and enlargement of the cochlear aqueduct. There are currently no treatment options for EVAS and enlarged cochlear aqueduct. Pendred syndrome generally requires treatment with cochlea implants (CI) in early childhood. Dizziness and autophony in patients with SCDS syndrome can be effectively treated by semicircular canal occlusion and coverage. Complaints in non-syndromic EVA and SCD syndrome are mainly caused by exposure of the inner ear to provoked and spontaneous pressure increases. Deafness and vestibular dysfunction in syndromic EVA (i.e.,Pendred syndrome, branchiootorenal syndrome) are caused by malformation of the cochlea, genetic maldevelopment of the hair cells, and pressure effects.

Pediatric sudden sensorineural hearing loss: Experience in a pediatric ENT emergency care center

ObjectivesSudden sensorineural hearing loss (SSNHL) is relatively rare and its physiopathology remains unclear, particularly in children. Our goal was to evaluate clinical characteristics, etiologies, management, treatment outcomes and prognostic factors in the pediatric population. MethodsWe performed a retrospective chart review of all children registered for SSNHL between August 2004 and September 2017 in a tertiary care pediatric hospital. We analysed data regarding clinical symptoms, audiological characteristics, diagnostic investigations and treatment outcomes. ResultsThirty-five patients were included. Mean age was 12 years (range 4–18 years). Male:female ratio was 15:20. Hearing loss was left-sided for 18 patients, right-sided for 12 patients and bilateral for 5 patients. Degree of hearing loss varied from mild to profound across frequencies in the 40 ears studied. Thirty-four patients had associated otologic symptoms: the most frequent was tinnitus (28 ears), followed by vertigo (23 ears), otalgia (5 ears) and sensation of blocked ear (5 ears). Twenty-nine patients received systemic steroids and 3 intra-tympanic steroids. In the treated group, 69% had improvement on the audiograms (14% total, 55% partial). Vestibular tests were performed in 16 patients and were abnormal in 10 patients. Radiological examination included computed tomography scan (n = 16) and/or magnetic resonance imaging (n = 33). They revealed 2 bilateral enlarged vestibular aqueducts, 1 labyrinthitis, 1 intra-cochlear haemorrhage. ConclusionSSNHL can affect speech and language development in children. There are differences among the pediatric population, including inner ear malformation and immune disease. Specific work up is proposed. Appropriate diagnosis and therapeutic management are discussed.

Long-term cochlear implantation outcomes in patients following head injury.

ObjectiveIn cases of a severe to profound sensorineural hearing loss following head injury, the cochlear implant (CI) is the primary option for auditory rehabilitation. Few studies, however, have investigated long‐term CI outcomes in patients following head trauma, including those without temporal bone fracture (TBF). Herein, the aim of this study is to examine CI outcomes following cases of head injury with and without TBF.MethodsAudiometric outcomes of patients who received a CI due to a head injury resulting in severe to profound hearing loss at two tertiary care hospitals were analyzed. Patients were divided into those who received a CI in a fractured temporal bone (group A, n = 11 patients corresponding to 15 ears) and those who received a CI in a non‐fractured temporal bone (group B, n = 8 patients corresponding to nine ears). Primary outcomes included duration of deafness prior to CI and postoperative consonant‐nucleus‐constant whole word (CNC) scores.ResultsNineteen patients (84% male), corresponding to 24 CIs, were identified. Fifteen CI were performed on ears with TBF (group A), and nine CI were performed on ears without TBF (group B). No patients had an enlarged vestibular aqueduct (EVA). The mean duration of deafness was 5.7 and 11.3 years in group A and group B, respectively. The mean duration of CI follow‐up (CI experience) was 6.5 years in group A and 2.1 years in group B. The overall mean postoperative CNC score for all subjects was 68.6% (±21.2%, n = 19 with CNC testing). There was no difference in CNC score between group A and group B (69.8% and 66% respectively, P = .639).ConclusionThe study is among the largest series examining long‐term outcomes of CI after head injury. CI is an effective method for auditory rehabilitation in patients after head injury.Level of evidenceIV.

Inner ear perilymphaticum gadolinium based MR imaging in large vestibular aqueduct: a pilot study

回顾性分析2013年7月至2018年9月济宁医学院附属医院经临床诊断的13例（21耳）大前庭导水管综合征（LVAS）患者（LVAS组）和23例（23耳）听力正常者（正常组）外淋巴间隙钆成像资料。先行三维稳态构成干扰（3D-CISS）和三维液体衰减反转恢复（3D-FLAIR）序列MRI扫描，经鼓膜向鼓室内注入稀释的钆对比剂，1、2、3、6 d后分别行3D-FLAIR扫描。对两组耳蜗、前庭及半规管外淋巴间隙显示情况进行评分，评分结果比较采用Wilcoxon秩和检验。测量LVAS组内淋巴管（ED）和内淋巴囊（ES）区注射对比剂前后信号强度，计算与同层脑干的信号强度比（SIR），采用单因素方差分析比较注射前后的差异。结果显示注射对比剂前LVAS组和正常组耳蜗、前庭及半规管在3D-FLAIR上呈低信号；注射对比剂后，对比剂分布于内耳耳蜗、前庭、半规管外淋巴间隙，两组间显示情况评分差异无统计学意义（Z=-0.95，P=0.34）。正常组ED和ES均未显示，注射对比剂后亦无信号强度改变；LVAS组ED和ES区在3D-FLAIR上呈完全低信号2耳，呈分界清晰低、高信号19耳。注射对比剂后患侧ED和ES区低信号区信号强度增强，注射对比剂前后SIR值间比较差异有统计学意义（F=111.93，P <0.01）。本研究结果提示：LVAS患耳内耳（耳蜗、前庭和半规管）内淋巴积水不显著；患耳ED和ES可能与前庭外淋巴间隙相通；部分患耳的ED和ES内可能存在间隔。