Neurocognitive outcome in children with sensorineural hearing loss after treatment of malignant embryonal brain tumors.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 2029-2029
Author(s):  
Christine Dahl ◽  
Iska Moxon-Emre ◽  
Vijay Ramaswamy ◽  
Ute Katharina Bartels ◽  
Uri Tabori ◽  
...  

2029 Background: Neurological side effects associated with childhood brain tumors and their treatments contribute to long term neurocognitive morbidity. The aims of this study were to identify the incidence of sensorineural hearing loss (SNHL) in a large sample of children treated for malignant brain tumors, and to evaluate the potential relationship between SNHL and intellectual functioning following the completion of treatment. Methods: We conducted a prospective follow-up study at a single center with review of 119 patients treated for embryonal brain tumors at the Hospital for Sick Children, between 1996-2015, to analyze the impact of significant SNHL (Chang > 2b) on intellectual function. Hearing was assessed post-treatment (median age: 13.5y (+4.5)) and the median age for neurocognitive testing was 12.8y (+ 4.1). The median interval from time of diagnosis was 5.8y (+ 3.7). Results: Severe SNHL was identified in half the patients (50.4%, n = 60/119). We identified a subset of patients (n = 61) who had assessments of intellectual function. In this cohort, intellectual function was significantly poorer in the group with severe SNHL, even after controlling for the effect of craniospinal radiation (severe SNHL 22.4 Gy + 13.3, no or mild hearing loss 20.4 Gy +12.8) and boost dose and volume. Children experiencing severe SNHL had lower overall IQ (severe SNHL 72.4 + 16.6; no/mild hearing loss 92.0 + 20.5) p < 0.001 and in significantly lower verbal comprehension (severe SNHL 78.7 + 15.9; no/mild hearing loss 94.7 + 13.8) p < 0.001, and working memory (severe SNHL 78.2+ 17.6; no/mild hearing loss 94.8 + 16.4) p < 0.001, scores. Conclusions: Hearing loss is a much more significant complication in children with embryonal brain tumors than previously estimated. We show the profound impact of hearing loss on intellectual deficit in children. Namely, patients with severe SNHL have difficulty using and understanding verbal language, and they have a reduced ability to concentrate and manipulate information in short-term memory. Our results have implications on future trial designs and follow-up of children treated for embryonal brain tumors.

2017 ◽  
Vol 19 (suppl_4) ◽  
pp. iv44-iv45 ◽  
Author(s):  
Christine Dahl ◽  
Iska Moxon-Emre ◽  
Vijay Ramaswamy ◽  
Ute Bartels ◽  
Uri Tabori ◽  
...  

2017 ◽  
Vol 19 (suppl_6) ◽  
pp. vi140-vi140
Author(s):  
Iska Moxon-Emre ◽  
Christine Dahl ◽  
Vijay Ramaswamy ◽  
Ute Bartels ◽  
Uri Tabori ◽  
...  

Author(s):  
Gaelle Vofo ◽  
Marrigje Aagje de Jong ◽  
Michal Kaufman ◽  
Julia Meyler ◽  
Ron Eliashar ◽  
...  

Abstract Objectives Idiopathic sudden sensorineural hearing loss (SSNHL) represents a frequently encountered otological entity, of various types and severity, with an array of associated symptoms including vertigo. This is a devastating life-changing condition with a blurry prognosis. The objective of this study was to determine the clinical association of vestibular impairment by electronystagmography (ENG) and caloric tests, and their ability to predict prognosis. Methods An observational, crossectional study was carried out amongst patients admitted with SSNHL. Each consenting patient had an audiometry test performed on admission as well as ENG and caloric tests. Treatment included oral steroids and carbogen with intratympanic steroids used only as salvage treatment. Follow-up was completed after 6 months when hearing gains were evaluated. Finally, an association was sought between the rate of recovery and ENG and caloric test results. Results Of 35 patients included, marked recovery was seen in patients without vertigo when compared to those with vertigo (p=0.003). A statistically significant association was found between the presence of vertigo and hearing deterioration (p=0.008). More so, normal electronystagmography results were associated with marked recovery (p=0.04). Conclusions The vestibular end organs are both subjectively and objectively affected in SSNHL as demonstrated by the abnormal ENG and caloric tests in our study despite the small sample size. Concomitant vestibular involvement carries poorer prognosis and routine identification may help foresee the recovery of patients with SSNHL and as such, aid in patient counseling. ENG and caloric tests are easily available and may be recommended for all patients with SSNHL.


2019 ◽  
Vol 46 (3) ◽  
pp. 353-359 ◽  
Author(s):  
So Young Kim ◽  
Mi Kyung Kim ◽  
Jae-Sung Lim ◽  
Il Gyu Kong ◽  
Hyo Geun Choi

1993 ◽  
Vol 107 (3) ◽  
pp. 230-232
Author(s):  
Jos P. P. M. van Leeuwen ◽  
Cor W. R. J. Cremers ◽  
Henk O. M. Thijssen ◽  
Henk E. Meyer

Progressive sensorineural hearing loss is the most important early symptom of a cerebellopontine angle process. A case report is presented of a 42-year-old woman who was referred to our department in 1979. Oil cistemography showed non filling of the left internal acoustic canal. Audiometry was planned as the method of control, but she did not return until nine- years later. In 1988, an acoustic neurionoma of 4 cm diameter was found in the left CPA. Pure tone audiometry and speech audiometry showed that during the nine-year interval, her 60 dB flat sensorineural hearing loss and speech perception thresholds had remained almost unchanged. A follow-up with only tone and speech audiometry can lead to a false negative diagnosis in some of these cases. Calculation of the growth in tumour volume over nine years in this patient showed a tumour volume doubling time of about 15 months.


2017 ◽  
Vol 138 (1) ◽  
pp. 31-35 ◽  
Author(s):  
Maurizio Barbara ◽  
Luigi Volpini ◽  
Chiara Filippi ◽  
Francesca Atturo ◽  
Simonetta Monini

2021 ◽  
Vol 11 ◽  
Author(s):  
Miranda L. Camet ◽  
Anne Spence ◽  
Susan S. Hayashi ◽  
Ningying Wu ◽  
Jennifer Henry ◽  
...  

BackgroundSensorineural hearing loss is a well-known side effect of cisplatin (CDDP). There is limited research on the effect of dosing, infusion times, and schedules of cisplatin administration and their impact on hearing loss.MethodsA retrospective review of 993 pediatric patients’ medical and audiological charts from August 1990 to March 2015 was conducted using stringent inclusion criteria to characterize patients with hearing loss. 248 of these patients received CDDP. Of these, 216 patients had sufficient CDDP infusion data to assess for sensorineural hearing loss attributable to CDDP and its associated risk factors. Chart reviews were performed to extract clinical data including CDDP dosing information. Demographic and clinical characteristics were summarized by descriptive statistics, and univariate and multivariate logistic regressions were performed to examine the relationship between hearing loss and specific parameters of cisplatin administration (amount infused per dose, prescribed infusion time, total number of doses, number of doses per cycle, number of cycles, cumulative cisplatin exposure). Stepwise variable selection procedure was performed in the multivariate model building to extract the best subset of risk factors for the prediction of hearing loss and worsening ototoxicity grade using an established ototoxicity grading scale from the International Society of Pediatric Oncology (SIOP).ResultsA total of 153 patients with complete medical and audiologic data were evaluable for analysis. Hearing loss was identified in 72.6% of the patients. Multivariate analysis revealed that age [OR=0.90 (0.84-0.97), p-value=0.0086], radiation to any part of the body, [OR=3.20 (1.29-7.93), p-value=0.012], amount infused per dose (mg/m2) [OR=1.018 (1.002-1.033), p-value=0.029], and cumulative cisplatin exposure (mg/m 2) [OR=1.004 (1-1.008), p-value=0.027] were associated with hearing loss. Similar associations were also found between these risk factors and worsening SIOP grade.ConclusionIn one of the largest studies examining the influence of CDDP dosing and schedules on hearing loss, we found the amount of CDDP infused per dose is a significant risk factor. Considerations in designing regimens that reduce the amount of CDDP infused per dose may reduce the risk of hearing loss. Randomized prospective trials are needed.


2017 ◽  
Vol 6 (2) ◽  
Author(s):  
Mirko Tessari ◽  
Andrea Ciorba ◽  
Lucas Omar Mueller ◽  
Qinghui Zhang ◽  
Mariapaola Cristini ◽  
...  

Reports of extra-cranial venous outflow disturbances have recently been linked to sudden sensorineural hearing loss (SSNHL). Aims of the present study are: i) to quantify, with mathematical model, the impact of jugular valve function on the pressure of the superior and inferior petrosal sinuses (SPS, IPS) and the main auricolar veins; ii) to verify the feasibility of the application of mathematical model in the clinical setting in terms of consistency respect to the usual measures of SSNHL outcome. Extra-cranial venous outflow and post analysis were respectively blindly assessed by echo colour-Doppler (ECD) and a validated mathematical model for the human circulation. The pilot study was conducted on 1 healthy control and in a group of 4 patients with different outcome of SSNHL. The main finding was the significant increased pressure calculated in the SPS and IPS of patients with ipsilateral jugular obstruction due to not mobile valve leaflets (6.55 mmHg), respect to the other subjects without extracranial complete obstruction (6.01 mmHg), P=0.0006. Moreover, we demonstrated an inverted correlation between the extrapolated pressure values in the SPS/IPS and the mean flow measured in the correspondent internal jugular vein (r= –0.87773; r-squared= 0.7697; P=0.0009). The proposed mathematical model can be applied to venous extra-cranial ECD investigation in order to derive novel clinical information on the drainage of the inner ear. Such clinical information seems to provide coherent parameters potentially capable to drive the prognosis. This innovative approach was proven to be feasible by the present pilot investigation and warrants further studies with an increased sample of patients.


2017 ◽  
Vol 2 (5) ◽  
pp. 262-268 ◽  
Author(s):  
Taha A. Jan ◽  
Aaron K. Remenschneider ◽  
Christopher Halpin ◽  
Margaret Seton ◽  
Michael J. McKenna ◽  
...  

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