Risk Factors for Natural Hearing Evolution in Newborns With Congenital Cytomegalovirus Infection.

Importance: Congenital cytomegalovirus (cCMV) is the major cause of congenital nonhereditary sensorineural hearing loss in children. Currently, criteria to identify infants at increased risk for unfavorable hearing outcome are lacking. Objective: To identify risk factors associated with cCMV-related hearing improvement, hearing deterioration, and late-onset hearing loss. Design, Setting, and Participants: This multicenter cohort study included patients from 6 secondary and tertiary hospitals enrolled in the Flemish CMV registry (Belgium). Newborns with untreated cCMV infection with at least 4-year audiological follow-up were included. Patients who presented with other possible causes of sensorineural hearing loss were excluded. Data were collected for 15 years (January 1, 2007, to February 7, 2022) and analyzed from September 26, 2022, to January 16, 2023. Main Outcomes and Measures: Primary outcome was hearing evolution (per-ear analysis; described as stable hearing, improvement, or deterioration). The association of gestational characteristics, clinical findings, timing of seroconversion, viral load, and hearing status at birth with hearing evolution was investigated using effect sizes (Cramer V, odds ratio [OR], or Hedges g). Results: Of the 387 children, 205 of 385 with nonmissing data were male (53.2%), 113 (29.2%) had a symptomatic infection, and 274 (70.8%) had an asymptomatic infection. Every child was 4 years or older at final hearing evaluation. A total of 701 of 774 ears (90%) showed stable hearing (normal hearing or stable hearing loss since birth) over time. Late-onset hearing loss (normal hearing at birth followed by hearing loss) was present in 43 of 683 ears (6.3%). Among children with hearing loss present at birth, 24 of 34 ears (70.6%) had hearing deterioration, and 6 of 91 ears (6.6%) had hearing improvement. Prematurity was associated with a higher chance of hearing improvement (OR, 12.80; 95% CI, 2.03-80.68). Late-onset hearing loss was more prevalent in a first trimester infection (OR, 10.10; 95% CI, 2.90-34.48). None of the 104 ears of children with a third trimester seroconversion developed late-onset hearing loss. Conclusions and Relevance: Findings of this cohort study support that ongoing audiological follow-up for untreated children with congenital hearing loss is important, as the majority of patients had hearing deterioration. The timing of seroconversion was associated with the risk of developing late-onset hearing loss. These insights can aid in parental counseling, patient stratification, and follow-up. Future research should focus on the effect of treatment, the influence of determined risk factors, and the study of eventual new risk factors in patients at high risk to develop hearing loss.

Cochlear implant electrode array tip-foldover detection by electrode voltage telemetry.

OBJECTIVES: With the introduction of more flexible and thinner electrodes, such as Cochlear's Slim Modiolar Electrode, there is a higher risk of electrode insertion problems, in particular the tip foldover. Timely intraoperative detection of the problem would allow for direct intraoperative correction. This paper describes a non-radiological method for intraoperative tip foldover detection that is applicable in all surgical centers and can quickly deliver accurate results. METHODS: Postoperative radiographs of 118 CI-recipients implanted with Nucleus devices were retrospectively analyzed on the presence of a tip foldover. Electrode Voltage Telemetry (EVT), also called Electric Field Imaging, was performed by means of Cochlear's EVT software tool, which is now integrated into Custom Sound-EP as the Trans-Impedance-Matrix measurement option. Tip foldover detection was automated by using the linear Hough transform for extracting straight-line patterns in the Trans-Impedance Matrix's heatmap. RESULTS: The six cases of electrode tip foldover were accurately identified by the EVT measurements, including two cases with folding location very close to the electrode tip (contact 20). CONCLUSION: Electrode Voltage Telemetry measures the Trans-Impedance Matrix, which can accurately detect tip foldovers of the cochlear implant electrodes within 1 min. This method can be reliably applied in all patients with normal cochlear anatomy and is able to intraoperatively detect foldovers localized even very close to the electrode tip. Application of the linear Hough transform allows for automatic detection of electrode tip foldovers that shows excellent agreement with visual evaluation of the radiological images and the transimpedance matrix's heatmap.

Risk Factors for Hearing Loss at Birth in Newborns With Congenital Cytomegalovirus Infection.

Importance: With a prevalence between 0.2% and 6.1% of all live births, congenital cytomegalovirus (cCMV) infection is a major cause of congenital nonhereditary sensorineural hearing loss. Despite the large amount of research on cCMV-related hearing loss, it is still unclear which newborns are at risk of hearing loss. Objective: To identify independent risk factors for cCMV-related congenital hearing loss and predictors of hearing loss severity at birth. Design, Setting, and Participants: This cross-sectional study of newborns with cCMV infection used data included in the Flemish CMV registry that was collected from 6 secondary and tertiary hospitals in Flanders, Belgium, over 15 years (January 1, 2007, to February 7, 2022). Data were analyzed March 3 to October 19, 2022. Patients were included in the study after confirmed diagnosis of cCMV infection and known hearing status at birth. Patients who presented with other possible causes of sensorineural hearing loss were excluded. Main Outcomes and Measures: Primary outcome was hearing status at birth. Clinical, neurological, and laboratory findings along with the timing of seroconversion and blood viral load were separately considered as risk factors. Binary logistic regression was performed to identify independent risk factors for congenital hearing loss in newborns with cCMV. Effect sizes were measured using Hedges g, odds ratio, or Cramer V. Results: Of the 1033 newborns included in the study (553 of 1024 [54.0%] boys), 416 (40.3%) were diagnosed with symptomatic cCMV infection and 617 (59.7%) with asymptomatic cCMV infection. A total of 15.4% of the patients (n = 159) presented with congenital hearing loss; half of them (n = 80 [50.3%]) had isolated hearing loss. The regression model revealed 3 independent risk factors for congenital hearing loss: petechiae at birth (adjusted odds ratio [aOR], 6.7; 95% CI, 1.9-23.9), periventricular cysts on magnetic resonance imaging (MRI; aOR, 4.6; 95% CI, 1.5-14.1), and seroconversion in the first trimester (aOR, 3.1; 95% CI, 1.1-9.3). Lower viral loads were seen in patients with normal hearing compared with those with congenital hearing loss (median [IQR] viral load, 447.0 [39.3-2345.8] copies per milliliter of sample [copies/mL] vs 1349.5 [234.3-14 393.0] copies/mL; median difference, -397.0 [95% CI, -5058.0 to 174.0] copies/mL). Conclusions and Relevance: Findings of this cross-sectional study suggest that newborns with cCMV infection and petechiae at birth, periventricular cysts on MRI, or a seroconversion in the first trimester had a higher risk of congenital hearing loss. Clinicians may use these risk factors to counsel parents in the prenatal and postnatal periods about the risk of congenital hearing loss. Moreover, linking clinical features to hearing loss may provide new insights into the pathogenesis of cCMV-related hearing loss. The importance of viral load as a risk factor for congenital hearing loss remains unclear.

Novel Impedance Measures as Biomarker for Intracochlear Fibrosis.

Measurement of the complex electrical impedance of the electrode contacts can provide new insights into the factors playing a role in the preservation of residual hearing with cochlear implants (CIs). However, unraveling the contributions related to the different phenomena from impedance data necessitates more advanced measurement and analysis techniques. The present study explores a new impedance measurement option recently included into the cochlear-implant programming software and aims to contribute to a more solid basis for the clinical use of impedance measures as a biomarker for fibrous tissue formation. Twenty adult CI-recipients were followed from surgery until 1 year after implantation by means of Electrode Voltage Telemetry (EVT), also called Electric Field Imaging or TransImpedance-Matrix measurement, and a 4-point technique for probing the voltage between adjacent electrode contacts. The data were compared to the electrode location derived from computed tomography, and to the device usage log. Using our impedance model for electrical stimulation of the cochlea, the polarization impedance related the electrode-tissue interface was determined, and the bulk impedance (access resistance) was split into a near-field and a far-field component. On average, the polarization impedance increased abruptly after surgery, indicating a strong passivation of the electrode contacts before cochlear-implant initiation. Its initial rise resolved almost completely soon after device switchon (2-4 weeks). The gradual increase of the access resistance mainly happened during the first 40 days on a time scale very similar to that observed in a guinea-pig study correlating impedance changes to fibrous tissue growth. The higher increase towards the round window is consistent with the higher amount of tissue observed in histological animal studies close to the electrode entry point. While the initial changes were due to the near-field resistance, the far-field resistance began to rise only after one month for half of the study group, once the near-field component had reached its critical value. This suggests indeed fibrosis initiating near the electrode contacts and spreading thereafter farther away. The near-field resistance positively correlated to device usage. EVT data allow for a further decomposition of the impedance at a cochlear-implant electrode, yielding a more detailed description of the postoperative intracochlear phenomena, such as fibrosis.

Congenital CMV-Associated Hearing Loss: Can Brain Imaging Predict Hearing Outcome?

OBJECTIVES: Congenital cytomegalovirus (cCMV) infection is the leading cause of nonhereditary sensorineural hearing loss in childhood and is also associated with CNS abnormalities. The main objective is to investigate the prognostic value of neonatal cranial ultrasound (cUS) and cranial magnetic resonance imaging (cMRI) in predicting long-term hearing outcome in a large cohort of cCMV-infected symptomatic and asymptomatic patients. DESIGN: Data were prospectively collected from a multicentre Flemish registry of children with cCMV infection born between 2007 and 2016. Neonatal cUS and cMRI scans were examined for lesions related to cCMV infection. Audiometric results at different time points were analyzed. The imaging and audiometric results were linked and diagnostic values of cUS and cMRI were calculated for the different hearing outcomes. RESULTS: We were able to include 411 cCMV patients, of whom 40% was considered symptomatic at birth. Cranial ultrasound abnormalities associated with cCMV infection were found in 76 children (22.2% of the cUS scans), whereas cMRI revealed abnormalities in 74 patients (26.9% of the cMRI scans). A significant relation could be found between the presence of cUS or cMRI abnormalities and hearing loss at baseline and last follow-up. Cranial ultrasound and cMRI findings were not significantly correlated with the development of delayed-onset hearing loss. Specificity and sensitivity of an abnormal cUS to predict hearing loss at final follow-up were 84% and 43%, respectively compared with 78% and 39% for cMRI. Normal cUS and cMRI findings have a negative predictive value of 91% and 92%, respectively, for the development of delayed-onset hearing loss. CONCLUSIONS: Neuroimaging evidence of CNS involvement in the neonatal period is associated with the presence of hearing loss in children with a cCMV infection. Imaging abnormalities are not predictive for the development of delayed-onset hearing loss.

Labyrinthine enhancement on 3D black blood MR images of the brain as an imaging biomarker for cisplatin ototoxicity in (lung) cancer patients.

PURPOSE: Cancer patients treated with platinum-based chemotherapy can present with ototoxicity symptoms. The purpose of this work is to report the imaging features related to cisplatin ototoxicity. METHODS: Between December 2015 and March 2019, a cohort of 96 consecutive patients with lung cancer was selected. Only patients who received cisplatin chemotherapy and underwent an imaging protocol consisting of a Gd-enhanced 3D-BB and 3D-T1W sequence, as well as T2W sequence to exclude metastases, were included. Labyrinthine enhancement was assessed, and all findings regarding the auditory and vestibular function were retrieved from the clinical files. RESULTS: Twenty-one patients met the inclusion criteria. The Gd-enhanced 3D-BB images were used to divide them into the labyrinth enhancement group (LEG) and the labyrinth non-enhancement group (LNEG). None of these patients demonstrated enhancing regions on the 3D-T1W images. The labyrinthine fluid remained high on the T2 images in all patients, excluding metastases. The LEG consisted of 6 patients. The cochlea and semicircular canals were the most frequently affected regions. All the LEG patients that presented with hearing loss (4/6) had cochlear enhancement. Patients with normal hearing had no cochlear enhancement. Five patients (5/6) showed vestibular enhancement. Four of these patients had vestibular symptoms. CONCLUSION: Labyrinthine enhancement as an imaging feature related to cisplatin ototoxicity is unreported. This study demonstrates a correlation between hearing loss and cochlear enhancement and also between vestibular impairment and vestibular/semicircular enhancement on 3D-BB images, which remained invisible on the 3D-T1W images. The labyrinthine enhancement on 3D-BB images in the presence of normal signal intensity of the intralabyrinthine fluid can be used as an imaging biomarker for cisplatin toxicity in daily clinical practice and should not be mistaken for intralabyrinthine metastases.

Long-term results of a modified expansion sphincter pharyngoplasty for sleep-disordered breathing.

Our aim was to evaluate the long-term objective and subjective results of a modified expansion sphincter pharyngoplasty (ESP) technique in patients with sleep-disordered breathing. Single center prospective study of 35 patients underwent an ESP as a primary surgical treatment between June 2012 and September 2015 at the hospital AZ Sint-Jan Bruges-Ostend. Patients were divided into non-OSAS and OSAS (AHI >5). Primary outcome parameters were the Epworth Sleeping Scale (ESS, reduction and score less then 10) and the Visual Analogue Score of snoring (VAS, assessed by partner) evaluated at 3 months and 1 year. In addition, the OSAS group underwent a polysomnography after 6 months to calculate the Apneu-Hypopneu Index (AHI) change. Secondary outcome parameters were possible complications and morbidity rate. The overall Epworth Sleepiness Scale showed a steady total reduction of, respectively, 42 and 48% at the two timepoints. All patients had a post-operative score of less than ten points. The Visual Analogue Score improved in 92% of the patients; of these, the snoring was reduced in 86% and disappeared in 6%. In the OSAS group, we noticed a reduction in AHI of more than 50 in 53% of the patients. A considerable reduction was found in the severe OSAS group, where we found a mean pre-operative average AHI of 41.3/h that was reduced 6 months after the operation to 17.4/h. There were no severe complications or increased morbidity rate observed. This first long-term study shows that the modified ESP seems to be a safe and promising technique in palatal surgery for patients with sleep-disordered breathing. Surgical effectiveness is sustained after 1 year, both in OSAS as in snoring pathology. The technique seems as approachable for the basic ENT surgeon as the uvulopalatopharynoplasty.

Hearing in Children with Congenital Cytomegalovirus Infection: Results of a Longitudinal Study.

OBJECTIVES: To evaluate hearing outcome, to characterize the nature of symptomatic and asymptomatic congenital cytomegalovirus (cCMV) infection and associated hearing loss, and to compare results with data from previous studies. STUDY DESIGN: A prospective multicenter registry was set up in 2007. Six centers participated in the development of a standardized protocol for diagnosis, treatment, and follow-up. Data were gathered in an online registry. Children (n = 379) with a documented cCMV infection and at least 2 separate audiologic evaluations were included. Audiometric results from a multicenter cohort study of children with cCMV infection with longitudinal observation were examined. RESULTS: Results from 123 children with a symptomatic and 256 children with an asymptomatic cCMV infection were analyzed. In the group with symptomatic cCMV, 63% had hearing loss, compared with 8% in the group with asymptomatic cCMV. Delayed-onset hearing loss occurred in 10.6% of symptomatic cCMV and in 7.8% of asymptomatic cCMV. In the group with symptomatic cCMV, 29.3% of children used some kind of hearing amplification; 1.6% in the group with asymptomatic cCMV used hearing amplification. CONCLUSIONS: Symptomatic and asymptomatic cCMV infections are a major cause of hearing loss in childhood. Reliable estimates of the long-term outcome of cCMV infection are mandatory to increase vigilance, especially among pregnant women and to draw attention to preventive measures, vaccine development, and prenatal and postnatal therapy. Universal screening of newborns for cCMV infection should be initiated and combined with longitudinal audiometric follow-up.

Cone beam computed tomography, a low-dose imaging technique in the postoperative assessment of cochlear implantation.

BACKGROUND: Cone beam computed tomography (CBCT) has become an extremely useful technique for dentomaxillofacial imaging because it provides clear images of highly contrasted structures. Previous studies evaluating the applicability of this technique in otologic imaging were very encouraging but were only performed in vitro on temporal bone specimens. The intracochlear positioning of the individual electrodes after cochlear implantation by means of CBCT has not yet been shown in vivo. OBJECTIVE: We describe a protocol for in vivo postoperative imaging of cochlear implants by CBCT. Moreover, the effective dose was measured and compared with the effective dose used on 4- and 16-slice multislice computed tomography (MSCT) by using a RANDO-phantom. MAIN OUTCOME MEASURE: Developing a protocol for in vivo postoperative imaging of cochlear implants by CBCT. RESULTS: CBCT provides high-resolution and almost artifact-free multiplanar reconstruction images allowing assessment of the precise intracochlear position of the electrode and visualization of each of the individual contacts. The calculated effective dose of the used CBCT and MSCT acquisitions is 80 musv for the CBCT, 3,600 musv for the 16-slice computed tomography, and 4,800 musv for the 4-slice computed tomography. CONCLUSION: These preliminary results suggests that, for in vivo postoperative evaluation of cochlear implants, CBCT can provide at least the same information as conventional radiography, digital radiograph, and MSCT but in a more comfortable and a much more safer way.