Obstructive ureteric calculus with superimposed infections causing reversible posterior leukoencephalopathy syndrome: A case report.

RATIONALE: Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinicoradiological phenomenon first observed 2 decades ago. Reversibility is the hallmark of this rare clinical phenomenon once the triggering pathology is aptly and adequately treated. Tinnitus preceding bilateral hearing loss as a symptomatology of RPLS has not been reported in the literature. Furthermore, chronic obstructive ureteric calculus with superimposed infections as a cause of RPLS has not been reported in the literature. PATIENT CONCERNS: A 57-year-old female was admitted at our facility because of 2 days history of hearing loss in both ears. She experienced tinnitus in both ears 2 weeks prior to the hearing loss. She is a known hypertensive. She has also undergone multiple surgical treatments for urinary calculi. DIAGNOSIS: Computed tomography (CT) scan of the urinary system revealed a calculus at the right ureter. Magnetic resonance imaging (MRI) showed abnormal signals at both temporo-parieto-occipital (TPO) cortices, the subcortical area, as well as the left hippocampus which was consistent with the diagnosis of RPLS. INTERVENTIONS: While on antibiotics for treatment of infections, the patient went into hypertensive encephalopathy and pneumonia was also established necessitating intensive care. OUTCOMES: We observed a resolution of the patient's temperature and hypertension when the right ureteric stone finally descended into the bladder. Also, we observed disappearance of the abnormal signals at both TPO cortices, the subcortical area, as well as the left hippocampus. Two years follow-up revealed no recurrence of her symptomatology. LESIONS: Patients who present with hypertensive encephalopathy maybe more prone to developing RPLS. Renal insufficiency alone or hypertension alone may not be single predisposing entities to RPLS but rather multiple predisposing factors.

Scalar Translocation Comparison Between Lateral Wall and Perimodiolar Cochlear Implant Arrays - A Meta-Analysis.

OBJECTIVES/HYPOTHESIS: Two types of electrode arrays for cochlear implants (CIs) are distinguished: lateral wall and perimodiolar. Scalar translocation of the array can lead to intracochlear trauma by penetrating from the scala tympani into the scala vestibuli or scala media, potentially negatively affecting hearing performance of CI users. This systematic review compares the lateral wall and perimodiolar arrays with respect to scalar translocation. STUDY DESIGN: Systematic review. METHODS: PubMed, Embase, and Cochrane databases were reviewed for studies published within the last 11 years. No other limitations were set. All studies with original data that evaluated the occurrence of scalar translocation or tip fold-over (TF) with postoperative computed tomography (CT) following primary cochlear implantation in bilateral sensorineuronal hearing loss patients were considered to be eligible. Data were extracted independently by two reviewers. RESULTS: We included 33 studies, of which none were randomized controlled trials. Meta-analysis of five cohort studies comparing scalar translocation between lateral wall and perimodiolar arrays showed that lateral wall arrays have significantly lower translocation rates (7% vs. 43%; pooled odds ratio = 0.12). Translocation was negatively associated with speech perception scores (weighted mean 41% vs. 55%). Tip fold-over of the array was more frequent with perimodiolar arrays (X2  = 6.8, P < .01). CONCLUSIONS: Scalar translocation and tip fold-overs occurred more frequently with perimodiolar arrays than with lateral wall arrays. In addition, translocation of the array negatively affects hearing with the cochlear implant. Therefore, if one aims to minimize clinically relevant intracochlear trauma, lateral wall arrays would be the preferred option for cochlear implantation. Laryngoscope, 131:1358-1368, 2021.

Evaluation of the outcome of CT and MR imaging in pediatric patients with bilateral sensorineural hearing loss.

OBJECTIVE: To evaluate the clinically relevant abnormalities as visualized on CT and MR imaging in children with symmetric and asymmetric bilateral sensorineural hearing loss (SNHL), in relation to age and the severity of hearing loss. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral otology and audiology center. PATIENTS AND DIAGNOSTIC INTERVENTIONS: From January 2006 until January 2016, a total of 207 children diagnosed with symmetric and asymmetric bilateral SNHL were included. They underwent CT and/or MR imaging for the evaluation of the etiology of their hearing loss. MAIN OUTCOME MEASURES: Radiologic abnormalities associated with SNHL. RESULTS: 302 scans were performed in 207 children (median age of 0.8 years old) with bilateral SNHL. The most frequently identified cause of bilateral SNHL was a malformation of the labyrinth. The combined diagnostic yield of CT and MR imaging was 32%. The diagnostic yield of MR (34%) was considerably higher than that of CT (20%). We found a higher rate of abnormalities in children with profound hearing loss (41%) compared to milder hearing loss (8-29%), and in asymmetric SNHL (52%) compared to symmetric SNHL (30%). CONCLUSION: Imaging is essential in the etiologic evaluation of children with bilateral SNHL. The highest diagnostic yield is found in children with bilateral asymmetric SNHL or profound SNHL. Based on our findings, MR is the primary imaging modality of choice in the etiological evaluation of children with bilateral SNHL because of its high diagnostic yield.

Case Report: Neurobrucellosis with Plastered Spinal Arachnoiditis: A Magnetic Resonance Imaging-Based Report.

Diffuse spinal arachnoiditis in neurobrucellosis is a rare manifestation. We report a boy aged 17, presenting with hearing impairment and recurrent vomiting for 18 months, weight loss for 12 months, dysphagia, dysarthria, hypophonia for 6 months, and gait unsteadiness for 5 months. He had bilateral 5th (motor) to 12th cranial nerve palsy, wasting and weakness of limbs, fasciculations, absent tendon reflexes, and positive Babinski's sign. Cerebrospinal fluid (CSF) showed raised protein and pleocytosis. Magnetic resonance imaging (MRI) showed extensive enhancing exudates in cisterns and post-contrast enhancement of bilateral 5th, 6th, 7th, and 8th nerves. Spine showed clumping with contrast enhancement of the cauda equina roots and encasement of the cord with exudates. Serum and CSF were positive for anti-Brucella antibodies. He showed significant improvement with antibiotics. At 4 months follow-up, MRI demonstrated near complete resolution of cranial and spinal arachnoiditis. It is important to recognize such rare atypical presentations of neurobrucellosis.

Duplicated internal auditory canal with inner ear malformation: Case report and literature review.

Internal auditory canal anomalies are rare. Narrow internal auditory canal is believed to occur as a result of aplasia or hypoplasia of the vestibulocochlear nerve. Narrow duplication of the internal auditory canal is considered to be very rare. Narrow duplication of the internal auditory canal with inner ear malformation has been reported in only 3 cases. We present 2 cases of narrow duplication of the internal auditory canal with inner ear malformation. The first case had inner ear malformation on only one side and the second case had inner ear malformation on both sides. The embryogenesis may be different between internal auditory canal and inner ear.

Malformation of the eighth cranial nerve in children. / Malformaciones del octavo par en niños.

INTRODUCTION AND OBJECTIVES: Prevalence of congenital sensorineural hearing loss (SNHL) is approximately 1.5-6 in every 1,000 newborns. Dysfunction of the auditory nerve (auditory neuropathy) may be involved in up to 1%-10% of cases; hearing losses because of vestibulocochlear nerve (VCN) aplasia are less frequent. The objectives of this study were to describe clinical manifestations, hearing thresholds and aetiology of children with SNHL and VCN aplasia. METHODOLOGY: We present 34 children (mean age 20 months) with auditory nerve malformation and profound HL taken from a sample of 385 children implanted in a 10-year period. We studied demographic characteristics, hearing, genetics, risk factors and associated malformations (Casselman's and Sennaroglu's classifications). Data were processed using a bivariate descriptive statistical analysis (P<.05). RESULTS: Of all the cases, 58.8% were bilateral (IIa/IIa and I/I were the most common). Of the unilateral cases, IIb was the most frequent. Auditory screening showed a sensitivity of 77.4%. A relationship among bilateral cases and systemic pathology was observed. We found a statistically significant difference when comparing hearing loss impairment and patients with different types of aplasia as defined by Casselman's classification. Computed tomography (CT) scan yielded a sensitivity of 46.3% and a specificity of 85.7%. However, magnetic resonance imaging (MRI) was the most sensitive imaging test. CONCLUSIONS: Ten percent of the children in a cochlear implant study had aplasia or hypoplasia of the auditory nerve. The degree of auditory loss was directly related to the different types of aplasia (Casselman's classification) Although CT scan and MRI are complementary, the MRI is the test of choice for detecting auditory nerve malformation.

A novel CHST3 allele associated with spondyloepiphyseal dysplasia and hearing loss in Pakistani kindred.

Skeletal dysplasias (SDs) are highly heterogeneous disorders composed of 40 clinical sub-types that are part of 456 well-delineated syndromes in humans. Here, we enrolled consanguineous kindred from a remote area of Sindh province of Pakistan, with 14 affected individuals suffering with short stature, kyphoscoliosis, joint dislocations, clubfoot, heart valve anomalies and progressive bilateral mixed hearing loss. To identify pathogenic variants in this family, whole exome sequencing (WES) was performed in one affected and one normal individual, which revealed a novel transversion mutation (c.802G>T; p.Glu268*) in CHST3 associated with the phenotype. CHST3 encodes a chondroitin 6-O-sulfotransferase-1 (C6ST-1) enzyme that is essential for the sulfation of proteoglycans found in cartilages. Previously, mutations in CHST3 have largely been reported in sporadic cases of SD, primarily with severe spinal abnormalities, joint dislocations, joint contractures, and clubfoot. Clinical and radiological examination of the affected individuals in this family provides new insights into phenotypic spectrum of CHST3 alleles and disease progression with age.

Improvement of sudden bilateral hearing loss after vertebral artery stenting.

Bilateral deafness is a rare but possible symptom of vertebrobasilar ischemia. We report a case of sudden bilateral sensorineural hearing loss caused by bilateral vertebral artery (VA) occlusion which dramatically improved after stenting. A 54-year-old man was admitted with sudden onset of bilateral deafness, vertigo, and drowsy mental status. Brain diffusion-weighted MRI showed acute infarction involving both the posterior inferior cerebellar artery and left posterior cerebral artery territory. Cerebral angiography showed bilateral distal VA occlusion, and emergency intracranial stenting was performed in the left VA. After reperfusion therapy his symptoms gradually improved, including hearing impairment. Endovascular stenting may be helpful in a patient with sudden deafness caused by bilateral VA occlusion.

Pontine Tegmental Cap Dysplasia: MR Evaluation of Vestibulocochlear Neuropathy.

Pontine tegmental cap dysplasia (PTCD) is recently recognized as a rare congenital brain stem malformation with typical neuroimaging hallmarks of ventral pontine hypoplasia and vaulted pontine tegmentum projecting into the fourth ventricle. PTCD patients also demonstrate variable cranial neuropathy with predilection for involvement of the vestibulocochlear and facial nerves. We present a case of PTCD diagnosed on MRI in the neonatal period. During early infancy, the patient displayed features of multiple cranial neuropathies and bilateral hearing loss. At the age of 2, the patient underwent further MRI assessment with dedicated high resolution T2 SPACE sequence to delineate the cranial nerve deficiencies.

Oval window atresia: a novel surgical approach and pathognomonic radiological finding.

OBJECTIVES: The facial nerve usually occupies the oval window area in patients with oval window atresia. During exploration, if the facial nerve is discovered to lie in the oval window area, this is usually regarded as a contraindication for further surgical intervention. The aim of the present paper is to demonstrate the preoperative pathognomonic radiological sign and describe a new surgical approach for this difficult situation. METHODS: 3 patients and 4 ears were operated due to conductive hearing loss by the same surgeon in a tertiary referral center. Their clinical presentation, radiological findings, surgical findings and final outcomes were evaluated and correlated. RESULTS: Surgical findings were identical in all 4 ears: facial nerve was running over the oval window and tympanic portion was completely dehiscent. Incus long arm was medially displaced due to abnormal development of the stapes suprastructure. In each ear a successful vestibulotomy and teflon piston placement was achieved. Preoperative mean air-bone gap of 47.5dB was improved to 21.5dB. There were no complications. CONCLUSION: Oval window atresia is a rare middle ear anomaly usually regarded as a contraindication for surgical intervention. In this study we present a novel surgical approach with succesful results. However the best approach is to inform the family by showing the nerve on tomography, showing the operation video, informing the family about the sensorineural hearing loss and letting the family choose the treatment option.

Cochlear nerve deficiency and associated clinical features in patients with bilateral and unilateral hearing loss.

OBJECTIVE: To clarify the prevalence and clinical characteristics of cochlear nerve deficiency (CND) in patients with congenital bilateral and unilateral hearing loss. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: One hundred fourteen children with bilateral and 56 children with congenital unilateral sensoneural hearing loss. MAIN OUTCOME MEASURES: Review of medical records, audiologic tests, and imaging studies. Imaging studies were evaluated for the presence or absence of abnormalities in the bony cochlear nerve canal (BCNC), internal auditory canal (IAC), and inner ear. RESULTS: The prevalence of CND, whether unilateral or bilateral, was much higher in the unilateral than in the bilateral hearing loss group: 50% (28/56) versus 5.3% (6/114). Among the 6 children with bilateral hearing loss and CND, 2 had bilateral BCNC stenosis alone, 2 had bilateral BCNC stenosis and unilateral IAC stenosis, 1 had unilateral BCNC stenosis alone, and 1 had unilateral IAC stenosis alone. All 28 children with unilateral hearing loss and CND had BCNC stenosis, whereas 9 (32.1%) also had concurrent IAC stenosis. Three of the 6 children with CND and bilateral hearing loss and 5 of the 28 children with CND and unilateral hearing loss also had other inner ear abnormalities. CONCLUSION: Our results suggest differences in the causes and mechanisms of CND in children with bilateral versus unilateral hearing loss.

Hearing preservation after cochlear implantation using deeply inserted flex atraumatic electrode arrays.

The aim of this study was to investigate hearing preservation in adults receiving cochlear implants with Flex arrays. Fifteen adults (19 ears) implanted with Flex EAS® (n = 4) or Flex Soft® (n = 15; MedEL, Innsbruck, Austria) were included in this retrospective study. The mean array insertion length was 25 ± 0.9 mm (n = 19), and the mean cochlear coverage was 435 ± 14.5° (n = 19), with no difference between EAS and Soft arrays. Residual low-frequency hearing was preserved in all implanted ears but deteriorated [pure-tone average (125-1000 Hz) 55.1 ± 2.90 dB before vs. 81.0 ± 3.02 dB after surgery (n = 19, p < 0.01)]. Both Flex arrays allow deep insertion with reproducible hearing preservation.

Cochlear ossification in patients with profound hearing loss following bacterial meningitis.

CONCLUSION: Cochlear ossification following bacterial meningitis is related to causative pathogen, but not age at disease or time point of evaluation. However, progression may occur over time, especially in case of primary signs of ossification. OBJECTIVE: To investigate the occurrence and degree of cochlear ossification on CT and MRI in patients with bilateral profound hearing loss following bacterial meningitis, in relation to causative pathogen, age at disease, and time point of evaluation. Progression of ossification in cases that underwent more than one scan was evaluated. METHODS: In the period 1982-2008, 47 cochlear implantations were performed in 34 consecutive candidates suffering from bilateral profound hearing loss following bacterial meningitis. A retrospective review of patient files and preoperative CT and MR images was performed. RESULTS: Cochlear ossification was observed in 35% of patients and 26% of ears on CT. The corresponding values for MRI were 44 and 30% (difference not significant). Streptococcus pneumoniae infection caused ossification more frequently than Neisseria meningitidis. No difference was found between pediatric and adult cases, and the occurrence of ossification was not related to the time point of evaluation. Signs of progressive ossification were found in cases with two CT scans, especially if ossification was present at the first scan.

Audiovestibular findings in a branchio-oto syndrome patient with a SIX1 mutation.

CONCLUSION: A reported mutation in SIX1 was identified in a patient with familial hearing loss (HL), a left preauricular pit, and bilateral enlarged vestibular aqueducts (EVA). Although the characteristic symptoms of EVA including fluctuating HL and repetitive vertigo were not seen in the patient, further studies are needed to clarify the association between EVA and such symptoms. OBJECTIVES: To study the audiovestibular functions, and to identify the causative gene in a patient with branchio-oto syndrome. METHODS: We enrolled a 30-year-old female in whom HL was pointed out at the age of 6 years. She visited our department at the age of 21 years, and had not experienced any progression of her HL, tinnitus, or vertigo. Pure-tone audiograms showed bilateral moderate mixed HL with no apparent progression during a 9-year follow-up period. Audiovestibular examinations included distortion product otoacoustic emissions (DPOAEs), electrocochleography (ECochG), and electronystagmography (ENG). Direct sequencing was utilized to screen for SIX1, EYA1, SLC26A4, GJB2, and mitochondrial DNA MTRNR1 including 1555 position. RESULTS: The findings of DPOAEs, ECochG, and ENG indicated cochlear HL with no vestibular dysfunction. A previously reported mutation of a heterozygous c.386A > G (p.Y129C) in SIX1 was detected. No mutation was identified in EYA1, SLC26A4, GJB2, or MTRNR1.

Electric acoustic stimulation of the auditory system: experience and results of ten patients using MED-EL's M and FlexEAS electrodes.

OBJECTIVE: To evaluate the hearing preservation rate and speech perception scores in patients with profound high frequency hearing loss and acoustically aidable low frequency hearing, managed with the MED-EL electric acoustic stimulation system referenced to the insertion depth of the electrode array. STUDY DESIGN: Retrospective data analysis. PARTICIPANTS AND SETTING: Ten patients implanted at the Auditory Implant Centre, Guy's and St Thomas's Hospital, London, UK. MAIN OUTCOME MEASURES: Pure tone audiometry, speech perception tests and electrode insertion depth angle. RESULTS: Postoperatively, functional hearing preservation allowing electric acoustic stimulation was achieved in eight patients and total preservation of residual hearing in five patients with follow-up periods of more than 12 months. Three of four (75%) patients with an insertion depth of >360 degrees had a threshold shift of >25 dB, and all four patients had a threshold shift of >10 dB. All patients with total hearing preservation had the electrode inserted up to 360 degrees at maximum. Overall, speech perception outcomes increased significantly and hearing impairment was significantly reduced after electric acoustic stimulation or electric stimulation alone as compared with the preoperative scores. CONCLUSION: Electric acoustic stimulation provides significant benefit to individuals with profound high frequency hearing loss. Studies with larger number of patients are needed to establish the optimal electrode insertion angle as well as to further analyse the benefit of electric acoustic stimulation.

Computed tomography findings in large vestibular aqueduct syndrome.

CONCLUSIONS: Patients with large vestibular aqueduct syndrome (LVAS) have disturbed morphogenesis of bony labyrinth. Semicircular canal anomalies are common in LVAS. OBJECTIVE: To describe the additional inner ear anomalies on CT imaging in pediatric patients with LVAS, and to investigate the lateral semicircular canal (LSCC) anomalies associated with LVAS by measurement of the LSCC bony island width. PATIENTS AND METHODS: We retrospectively reviewed the digitally stored temporal bone CT imaging obtained for 23 patients with LVAS, additional inner ear anomalies were noted, and measurements of the LSCC bony island width were made on axial CT scans on the workstation. Measurements were compared to the normative data obtained from 20 patients without sensorineural hearing loss. RESULTS: Of the 23 patients (bilateral in 22 and unilateral in 1), additional inner ear malformations were identified in 21 cases presenting either singly or in combination. A small LSCC bony island (<3 mm in diameter) appeared highly typical; vestibule and LSCC anomalies were identified in 13 cases (26 ears) based on visual inspection combined with abnormal measurements. Dehiscence of the superior and/or posterior semicircular canal was identified in 19 ears, and Mondini deformity was identified in 6 ears.

Rubinstein-Taybi syndrome and mixed bilateral hypoacousia case report.

OBJECTIVE: To describe a case of Rubinstein-Taybi syndrome (RTS) in association with mixed bilateral hypoacousia. PATIENT: A 10-year-old boy presented at a private otology practice center in Bogotá, Colombia, with signs and symptoms of RTS. In addition, the patient presented with mixed bilateral conductive hypoacousia, predominantly in the left ear with a 40- to 50-dB conductive component, caused by middle ear malformations and fixation of the ossicular chain and stapes footplate. INTERVENTION: The patient underwent a left exploratory tympanotomy and then a stapedotomy. MAIN OUTCOME MEASURES: Computed tomography, genetic evaluation, and audiologic testing. RESULTS: Postoperative recovery was normal. There was closure of the conductive gap (< or =10 dB) with improvement of subjective hearing. CONCLUSION: We presented a case of a patient with RTS and bilateral mixed hypoacousia who underwent a stapedotomy with a final improvement in his hearing performance. We consider it important to report and describe the simultaneous presence of these pathologic findings and to be able to analyzeif these otologic findings are coincidental or truly associated with RTS.