Background: The cause of sudden sensorineural hearing loss (SSNHL) is uncertain in a significant number of patients. A vascular etiology has been proposed because SSNHL increases the risk of... Show moreBackground: The cause of sudden sensorineural hearing loss (SSNHL) is uncertain in a significant number of patients. A vascular etiology has been proposed because SSNHL increases the risk of developing a stroke. Cardiovascular risk factors can cause cerebral small vessel disease (CSVD). The presence of CSVD in turn raises the risk of stroke. Aim: The aim of this study was to compare the presence of CSVD and cardiovascular risk factors in elderly patients with idiopathic SSNHL (iSSNHL) to a control cohort. Method: Patients with iSSNHL of 50 years and older were compared with a control cohort with patients suspected of trigeminal neuralgia or vestibular paroxysmia. The primary outcome was the difference in the number of white matter hyperintensities using the ordinal Fazekas scale. Secondary outcomes were the presence of brain infarctions on MRI and the difference in cardiovascular risk factors. Results: In the SSNHL cohort, Fazekas score 2 was most frequently seen compared with Fazekas 1 in the control cohort. The distribution of Fazekas scores did not differ significantly. The sum of the Fazekas scores were 13,925 and 14,042 for iSSNHL and controls, respectively (p = 0.908). Brain infarctions were seen in 8 patients with iSSNHL (n = 118) and in 13 patients in the control cohort (n = 118) (p = 0.361). None of the cardiovascular risk factors were more frequently seen in the iSSNHL cohort. Conclusion: Patients with iSSNHL did not exhibit more CSVD on MRI than controls. This result is in contrast with previous literature demonstrating a higher risk of stroke in patients with iSSNHL than in controls. A prospective analysis with a larger study population is therefore warranted. Show less
Objective: To analyze the effect of dizziness-related symptoms on the long-term quality of life (QoL) of patients with unilateral vestibular schwannoma. Methods: In this cross-sectional study,... Show moreObjective: To analyze the effect of dizziness-related symptoms on the long-term quality of life (QoL) of patients with unilateral vestibular schwannoma. Methods: In this cross-sectional study, patients with a unilateral vestibular schwannoma diagnosed between 2004 and 2013 completed a disease-specific QoL questionnaire (Penn Acoustic Neuroma Quality of Life [PANQOL]) and the Dizziness Handicap Inventory (DHI) in 2020. Linear regression was performed to assess the correlation between QoL and the DHI total score, and the scores of the DHI functional, emotional, and physical subdomains. Potential confounders such as age, sex, tumor size at baseline, and treatment modality (active surveillance, surgery, or radiotherapy) were included in the model. Results: In total, 287 of 479 patients (59%) experienced dizziness with a median follow-up of 10 years. The DHI total score was significantly associated with the PANQOL total score. On average, we found a reduction of 0.7 points on the PANQOL for each additional point on the DHI. The DHI emotional subdomain was the most prominent determinant of poorer QoL. Each point on the DHI emotional subscale was associated with a reduction of 1.3 on the PANQOL score. Treatment modality did not have a clinically relevant effect on dizziness-related QoL. Conclusions: Even mild dizziness can have a significant and clinically relevant effect on the QoL of patients with unilateral vestibular schwannoma in the long term. This holds true for all treatment modalities. Addressing the vestibular problems may improve QoL in vestibular schwannoma patients, and DHI subscale analysis may help tailor the optimal vestibular intervention. Show less
Objective: In vestibular schwannoma patients, a loss of signal intensity (SI) on T2-weighted magnetic resonance imaging (MRI) has been reported within the ipsilateral labyrinth. The purpose of this... Show moreObjective: In vestibular schwannoma patients, a loss of signal intensity (SI) on T2-weighted magnetic resonance imaging (MRI) has been reported within the ipsilateral labyrinth. The purpose of this study was to quantitatively evaluate the occurrence and course of this intensity loss in relation to proton radiotherapy and its possible association with hearing loss. Study Design: Retrospective chart review. Setting: Tertiary referral center. Patients: Patients who received proton therapy for a vestibular schwannoma and underwent at least two high-resolution T2-weighted cisternographic sequence (constructive interference in steady state/fast imaging employing steady-state acquisition/DRIVE) MRIs and audiometry assessments. Main Outcome Measures: Relative T2 SIs from the vestibules and basal/apical cochlear turns of the labyrinth, bilaterally.ResultsNinety-five MRI scans from 34 patients were included. The apical turn of the ipsilateral cochlea showed a lower mean cochlear SI than on the contralateral side (+/- 3.5 versus 5.0). The mean relative cochlear SI did not significantly change after proton radiotherapy. The ipsilateral vestibule showed a higher SI than the cochlea. The relative mean cochlear SI was not directly correlated to (the degree of) hearing loss before or after proton radiotherapy, nor did it predict future hearing loss. Conclusion: The relative mean cochlear SI on cisternographic T2-MRI in vestibular schwannoma patients is diminished on the treated side, when compared with the ipsilateral vestibule and the contralateral cochlea/vestibule. The SI of the ipsilateral cochlea does not further decrease after proton radiotherapy and seems to be related to the tumor rather than the therapy. The diminished cochlear SI does not correlate with subsequent loss of hearing. Show less
Hypothesis: Insertion speed during cochlear implantation determines the risk of cochlear trauma. By slowing down insertion speed tactile feedback is improved. This is highly conducive to control... Show moreHypothesis: Insertion speed during cochlear implantation determines the risk of cochlear trauma. By slowing down insertion speed tactile feedback is improved. This is highly conducive to control the course of the electrode array along the cochlear contour and prevent translocation from the scala tympani to the scala vestibuli. Background: Limiting insertion trauma is a dedicated goal in cochlear implantation to maintain the most favorable situation for electrical stimulation of the remaining stimulable neural components of the cochlea. Surgical technique is one of the potential influencers on translocation behavior of the electrode array. Methods: The intrascalar position of 226 patients, all implanted with a precurved electrode array, aiming a mid-scalar position, was evaluated. One group (n = 113) represented implantation with an insertion time less than 25 seconds (fast insertion) and the other group (n = 113) was implanted in 25 or more seconds (slow insertion). A logistic regression analysis studied the effect of insertion speed on insertion trauma, controlled for surgical approach, cochlear size, and angular insertion depth. Furthermore, the effect of translocation on speech performance was evaluated using a linear mixed model. Results: The translocation rate within the fast and slow insertion groups were respectively 27 and 10%. A logistic regression analysis showed that the odds of dislocation increases by 2.527 times with a fast insertion, controlled for surgical approach, cochlear size, and angular insertion depth (95% CI = 1.135, 5.625). We failed to find a difference in speech recognition between patients with and without translocated electrode arrays. Conclusion: Slowing down insertion speed till 25 seconds or longer reduces the incidence of translocation. Show less
Koppelaar-van Eijsden, H.M.; Schermer, T.R.; Bruintjes, T.D. 2022
Objective: To critically appraise and summarize the quality of the measurement properties of the Dizziness Handicap Inventory's (DHI) in adult patients with complaints of dizziness. Databases... Show moreObjective: To critically appraise and summarize the quality of the measurement properties of the Dizziness Handicap Inventory's (DHI) in adult patients with complaints of dizziness. Databases Reviewed: Pubmed, Embase, and CINAHL. Methods: The selected literature databases were systematically searched to identify studies investigating one or more measurement properties of the DHI. From the included studies, relevant data were extracted, their methodological quality was assessed, the results were synthesized and the evidence was graded and summarized according the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) methodology. Results: The search strategy resulted in 768 eligible publications, 42 of which were included in the review. Overall, evidence on the DHI's content validity was either lacking or limited and of low quality. Moderate evidence was found for inconsistent structural validity, sufficient construct validity and borderline sufficient responsiveness. Based on the studies included, low evidence was found for sufficient reliability of the DHI total score. No evidence synthesis could be done for the DHI's internal consistency due to multidimensionality (i.e., lack of support of the original subscales) and for its measurement error due to a lack of published information on the minimal important change. Conclusions: The current evidence for a number of measurement properties of the DHI is suboptimal. Because of its widespread use and the current lack of a better alternative, researchers can use the DHI when assessing handicapping effects imposed by dizziness, but they should be aware of its limitations. Moreover, we recommend using the DHI total score only and also to consider adding an instrument with more favorable measurement properties when assessing self-perceived handicap in patients with dizziness. Show less
Objective: To assess the efficacy and toxicity of proton radiotherapy in vestibular schwannoma. Study Design: Retrospective chart review and volumetric MRI-analyses. Setting: Tertiary referral... Show moreObjective: To assess the efficacy and toxicity of proton radiotherapy in vestibular schwannoma. Study Design: Retrospective chart review and volumetric MRI-analyses. Setting: Tertiary referral center. Patients: Vestibular schwannoma patients treated with protons between 2003 and 2018. Intervention: Proton radiotherapy. Main Outcome Measures: Tumor control was defined as not requiring salvage treatment. Progressive hearing loss was defined as a decrease in maximum speech discrimination score below the 95% critical difference in reference to the pretreatment score. Hearing assessment includes contralateral hearing and duration of follow-up. Dizziness and/or unsteadiness and facial and trigeminal nerve function were scored. Patients who had surgery prior to proton radiotherapy were separately assessed. Results: Of 221 included patients, 136 received single fraction and 85 fractionated proton radiotherapy. Actuarial 5-year local control rate was 96% (95% CI 90-98%). The median radiological follow-up was 4.5 years. Progressive postirradiation speech discrimination score loss occurred in 42% of patients with audiometric follow-up within a year. Facial paresis was found in 5% (usually mild), severe dizziness in 5%, and trigeminal neuralgia in 5% of patients receiving protons as primary treatment. Conclusions: Proton radiotherapy achieves high tumor control with modest side effects aside from hearing loss in vestibular schwannoma patients. Limited and heterogeneous outcome reporting hamper comparisons to the literature. Potential sequelae of radiation therapy impacting vestibular function, cognitive function, and quality of life warrant further evaluation. Subgroups that benefit most from proton radiotherapy should be identified to optimize allocation and counterbalance its costs. Show less
Duinkerken, C.W.; Weger, V.A. de; Dreschler, W.A.; Molen, L. van der; Pluim, D.; Rosing, H.; ... ; Zuur, C.L. 2021
Objectives: To determine safety, feasibility, and preliminary activity of transtympanic injection of sodium thiosulfate (STS) against cisplatin-induced hearing loss (CIHL). DESIGN Randomized... Show moreObjectives: To determine safety, feasibility, and preliminary activity of transtympanic injection of sodium thiosulfate (STS) against cisplatin-induced hearing loss (CIHL). DESIGN Randomized controlled trial. SETTING Tertiary cancer hospital. PATIENTS Adults to be treated with high-dose cisplatin (>= 75 mg/m(2)). INTERVENTION Selected by randomization, 0.1 M STS gel on one side and placebo gel on the other side was transtympanically applied to the middle ear 3 hours before cisplatin administration. After amendment, the placebo ear was left untreated. Main Outcome Measure: Primary outcome was safety and feasibility. Secondary outcomes included pharmacokinetic analysis of systemic cisplatin and preliminary activity of STS. Clinically relevant CIHL was defined as a >= 10 dB threshold shift at pure-tone average 8-10-12.5 kHz (PTA(8-12.5)). Response to STS was defined as a threshold shift at PTA(8-12.5) in the STS-treated ear of >= 10 dB smaller than the untreated ear. Results: Twelve patients were treated. Average CIHL at PTA(8-12.5) was 12.7 dB in untreated ears and 8.8 dB SPL in STS-treated ears (p = 0.403). Four patients did not develop CIHL. Four out of eight patients with CIHL responded to STS: CIHL at PTA(8-12.5) in STS-treated ears was 18.4 dB less compared to untreated ears (p = 0.068). Grade 1 adverse events were reported. Pharmacokinetic results were available for 11 patients. Conclusion: Transtympanic application of STS was safe and feasible. Based on our pharmacokinetic analysis, we postulate that transtympanic STS does not interfere with the systemically available cisplatin. Our results provide a preliminary proof of concept for transtympanic application of STS in preventing CIHL and warrants further evaluation on a larger scale. Show less
Objective: To identify predictors of tumor recurrence and postoperative facial nerve function after translabyrinthine surgery for unilateral vestibular schwannomas. Study Design: Retrospective... Show moreObjective: To identify predictors of tumor recurrence and postoperative facial nerve function after translabyrinthine surgery for unilateral vestibular schwannomas. Study Design: Retrospective study. Setting: Tertiary referral center. Patients: Between 1996 and 2017 a total of 596 patients with unilateral vestibular schwannoma underwent translabyrinthine surgery. Pre- and postoperative clinical status, radiological, and surgical findings were evaluated. Intervention(s): Translabyrinthine surgery. Main Outcome Measure(s): Potential predictors for tumor recurrence and facial nerve outcome were analyzed using Cox regression and ordinal logistic regression, respectively. Results: The extent of tumor removal was total in 32%, near-total in 58%, and subtotal in 10%. In 5.5% (33/596) of patients the tumor recurred. Subtotal tumor resection (p = 0.004, hazard ratios [HR] = 10.66), a young age (p = 0.008, HR = 0.96), and tumor progression preoperatively (p = 0.042, HR = 2.32) significantly increased the risk of recurrence, whereas tumor size or histologic composition did not. A good postoperative facial nerve function (House-Brackmann grade 1-2) was achieved in 85%. The risk of postoperative facial nerve paresis or paralysis increased with tumor size (p < 0.001, OR = 1.52), but was not associated with the extent of tumor removal, histologic composition, or patient demographics. Conclusions: Translabyrinthine surgery is an effective treatment for vestibular schwannoma, with a good local control rate and facial nerve outcome. The extent of tumor removal is a clinically relevant predictor for tumor recurrence, as are young patient age and preoperative tumor progression. A large preoperative tumor size is associated with a higher risk of postoperative facial nerve paresis or paralysis. Show less
Objectives: The aim of this study is to report on our preliminary experience with Transimpedance Matrix (TIM)-measurement for the detection of cochlear implant electrode tip foldovers compared with... Show moreObjectives: The aim of this study is to report on our preliminary experience with Transimpedance Matrix (TIM)-measurement for the detection of cochlear implant electrode tip foldovers compared with intraoperative imaging in patients implanted with the slim modiolar electrode (SME). Study Design: Proof of concept study. Setting: Tertiary university referral center. Patients: Twenty five ears (in 22 patients) implanted consecutively with the SME. Intervention(s): Following cochlear implantation, intraoperative TIM-measurement and fluoroscopy were performed. One week postoperatively, the electrode position was evaluated using Computed Tomography (CT)-imaging. Main Outcome Measures: Electrode array tip foldover. Results: Electrode array tip foldover occurred in three of the 25 cochlear implantations performed (12%). In each case, the foldover was detected by both TIM and fluoroscopy, leading to reposition and correct intracochlear placement of the array. Conclusions: TIM-measurement is a promising method for the intraoperative detection of an electrode array tip foldover. The TIM-tool with intuitive heatmap display is easy to use, fast, and readily available to clinics using TIM-software in the operating theatre. Show less
Background: To coordinate and align the content for registration of cholesteatoma care. Methods: Systematic Delphi consensus procedure, consisting three rounds: two written sessions followed by a... Show moreBackground: To coordinate and align the content for registration of cholesteatoma care. Methods: Systematic Delphi consensus procedure, consisting three rounds: two written sessions followed by a face-to-face meeting. Before this procedure, input on important patient outcomes was obtained. Consensus was defined as at least 80% agreement by participants. Hundred-thirty-six adult patients who had undergone cholesteatoma surgery and all ENT surgeons of the Dutch ENT Society were invited. The consensus rounds were attended by ENT surgeons with cholesteatoma surgery experience. Feasibility and acceptability of outcome measures and reporting agreements were assessed in round 1 by 150 ENT surgeons. In round 2 definitions were narrowed and context information to interpret outcome measure were questioned. In round 3, the results, amendments, and the open-ended points were discussed to reach agreement. Results: Most important outcome measures are: 1) the presence or absence of a cholesteatoma in the first 5 years after surgical removal of cholesteatoma, 2) hearing level after surgical removal of cholesteatoma, and 3) the documented assessment of patient's complaints with a validated patient reported outcome measures questionnaire (PROM). Furthermore, consensus was reached on the registration of cholesteatoma type (residual/recurrent), localization of cholesteatoma, and reporting of the presence of cholesteatoma in the follow-up. Conclusion: Consensus was reached on the content and method of registration of cholesteatoma care based on patient's and ENT surgeons input. Three outcome measures were defined. National agreements on the method and content of registration will facilitate monitoring and feedback to the ENT surgeon about the cholesteatoma care. Show less
Maas, B.D.P.J.; Bruintjes, T.D.; Zaag-Loonen, H.J. van der; Winters, S.M.; Masius-Olthof, S.; Colijn, C.; ... ; Leeuwen, R.B. van 2019
Objective: To analyze and compare experiences of anxiety and discomfort caused by the Epley maneuver and the willingness for a potential retreatment between younger (≤70 yr) and older (>70 yr)... Show moreObjective: To analyze and compare experiences of anxiety and discomfort caused by the Epley maneuver and the willingness for a potential retreatment between younger (≤70 yr) and older (>70 yr) patients.Study design: Prospective, observational cohort study.Setting: Tertiary referral center.Methods: Dutch adults, diagnosed with posterior canal benign paroxysmal positional vertigo, were included. Patients with dementia or subjected to an Epley maneuver ≤4 months before the hospital visit were excluded. Patients were asked about their experiences with the Epley maneuver directly after the maneuver, after 2 weeks and after 2 months. Differences in experiences between the age groups were tested with the Fisher's exact test and subgroup analyses were performed using multivariable logistic regression.Results: Out of 179 included patients, 115 (64%) were aged ≤70 and 134 (75%) were female. In both groups, 25% reported anxiety directly after the Epley maneuver. Two weeks later, 19 and 27%, respectively (p = 0.26) reported anxiety. In the young group, 30% expressed discomfort directly after treatment compared with 45% in the elderly (OR 1.98, 95% CI 1.05-3.75, p = 0.04). Two weeks later, this was 36 and 38%, respectively (p = 0.87). This decreased to 24 and 33%, respectively (p = 0.37), after 2 months. There was no significant difference in willingness for potential retreatment between both groups after 2 weeks (93 and 95%, respectively, p = 0.75) nor after 2 months (97 and 92%, respectively, p = 0.13).Conclusion: The Epley maneuver results in little anxiety and discomfort both in young and in elderly patients. Most patients show willingness for potential retreatment in case symptoms recur. Show less
Maas, B.D.P.J.; Bruintjes, T.D.; Zaag-Loonen, H.J. van der; Winters, S.M.; Masius-Olthof, S.; Colijn, C.; ... ; Leeuwen, R.B. van 2019
Objective: To analyze and compare experiences of anxiety and discomfort caused by the Epley maneuver and the willingness for a potential retreatment between younger (<= 70 yr) and older (>70... Show moreObjective: To analyze and compare experiences of anxiety and discomfort caused by the Epley maneuver and the willingness for a potential retreatment between younger (<= 70 yr) and older (>70 yr) patients. Study Design: Prospective, observational cohort study. Setting: Tertiary referral center. Methods: Dutch adults, diagnosed with posterior canal benign paroxysmal positional vertigo, were included. Patients with dementia or subjected to an Epley maneuver <= 4 months before the hospital visit were excluded. Patients were asked about their experiences with the Epley maneuver directly after the maneuver, after 2 weeks and after 2 months. Differences in experiences between the age groups were tested with the Fisher's exact test and subgroup analyses were performed using multivariable logistic regression. Results: Out of 179 included patients, 115 (64%) were aged <= 70 and 134 (75%) were female. In both groups, 25% reported anxiety directly after the Epley maneuver. Two weeks later, 19 and 27%, respectively (p = 0.26) reported anxiety. In the young group, 30% expressed discomfort directly after treatment compared with 45% in the elderly (OR 1.98, 95% CI 1.05-3.75, p = 0.04). Two weeks later, this was 36 and 38%, respectively (p = 0.87). This decreased to 24 and 33%, respectively (p = 0.37), after 2 months. There was no significant difference in willingness for potential retreatment between both groups after 2 weeks (93 and 95%, respectively, p = 0.75) nor after 2 months (97 and 92%, respectively, p = 0.13). Conclusion: The Epley maneuver results in little anxiety and discomfort both in young and in elderly patients. Most patients show willingness for potential retreatment in case symptoms recur. Show less
Objectives: To evaluate non echo-planar diffusion weighted magnetic resonance imaging (non-EP DW MRI) at 9 months after primary surgery to rule out residual cholesteatoma in patients scheduled... Show moreObjectives: To evaluate non echo-planar diffusion weighted magnetic resonance imaging (non-EP DW MRI) at 9 months after primary surgery to rule out residual cholesteatoma in patients scheduled before second-look-surgical exploration.Study Design: Prospective observational study.Setting: Secondary teaching hospital.Patients/Interventions: Patients who were scheduled for second-look-surgery after primary canal wall up repair of cholesteatoma underwent 1.5 T MRI including non-EP DWI and high-resolution coronal T1 and T2-FS SE sequences.Main Outcome Measures: Imaging studies were evaluated for the presence of cholesteatoma by three independent observers. Intraoperative observations were regarded the standard of reference. Ear, nose, throat (ENT) surgeons were blinded for imaging findings. The primary outcome was the negative predictive value (NPV) of MR imaging, secondary outcomes were sensitivity, specificity, and positive predictive value.Results: Thirty-three patients underwent both MRI and surgery, among whom 22 had a cholesteatoma. Mean time between primary surgery and MRI was 259 days (standard deviation [SD] 108). NPV of non-EP DW MRI in detecting recurrent cholesteatoma was 53% (95% CI: 32-73%). Sensitivity and specificity were 59% (39-77%) and 91% (62-98%), respectively. The positive predictive value was 93% (69-99%). In five out of nine false-negative cases, recurrent cholesteatoma measured 3mm or less. Using a 3mm detection threshold, NPV increased to 79%.Conclusion: Non-EP DW MRI cannot replace second look surgery in ruling-out residual cholesteatoma at 9 months after primary surgery. It could be used in a follow-up strategy in low risk patients. Further research is needed which types of residual cholesteatoma are not revealed by MRI. Show less
Objective: By discussing the design, findings, strengths, and weaknesses of available studies investigating the influence of angular insertion depth on speech perception, we intend to summarize the... Show moreObjective: By discussing the design, findings, strengths, and weaknesses of available studies investigating the influence of angular insertion depth on speech perception, we intend to summarize the current status of evidence; and using evidence based conclusions, possibly contribute to the determination of the optimal cochlear implant (CI) electrode position.Data Sources: Our search strategy yielded 10,877 papers. PubMed, Ovid EMBASE, Web of Science, and the Cochrane Library were searched up to June 1, 2018. Both keywords and free-text terms, related to patient population, predictive factor, and outcome measurements were used. There were no restrictions in languages or year of publication.Study Selection: Seven articles were included in this systematic review. Articles eligible for inclusion: (a) investigated cochlear implantation of any CI system in adults with post-lingual onset of deafness and normal cochlear anatomy; (b) investigated the relationship between angular insertion depth and speech perception; (c) measured angular insertion depth on imaging; and (d) measured speech perception at, or beyond 1-year post-activation.Data Extraction and Synthesis: In included studies; quality was judged low-to-moderate and risk of bias, evaluated using a Quality-in-Prognostic-Studies-tool (QUIPS), was high. Included studies were too heterogeneous to perform meta-analyses, therefore, effect estimates of the individual studies are presented. Six out of seven included studies found no effect of angular insertion depth on speech perception.Conclusion: All included studies are characterized by methodological flaws, and therefore, evidence-based conclusions regarding the influence of angular insertion depth cannot be drawn to date. Show less
Objective: To illustrate a case of sensorineural hearing loss (SNHL) after immunotherapy based on T cell receptor (TCR) gene therapy using modified T cells recognizing melanoma antigen recognized... Show moreObjective: To illustrate a case of sensorineural hearing loss (SNHL) after immunotherapy based on T cell receptor (TCR) gene therapy using modified T cells recognizing melanoma antigen recognized by T cells 1 for disseminated melanoma.Patient: We present a 59-year-old woman with profound subacute bilateral SNHL including unilateral deafness after immunotherapy based on TCR gene therapy using modified T cells recognizing melanoma antigen recognized by T cells 1 for disseminated melanoma. Ten days after treatment, the patient developed hearing loss of 57 dB hearing loss air conduction at pure-tone average 0.5-1-2-4 kHz in the right ear, and >100 dB hearing loss air conduction at pure-tone average 0.5-1-2-4 in the left ear. The right ear recovered partially, while the left ear remained deaf, despite oral prednisolone (1.0 mg/kg) and salvage treatment with three transtympanic injections of 0.5 ml dexamethasone (4.0 mg/ml).Conclusion: Based on our presented case and a vast amount of literature there is circumstantial evidence that TCR gene therapy for melanoma targets the perivascular macrophage-like melanocytes in the stria vascularis, resulting in SNHL. We suggest that SNHL after TCR gene therapy may be caused by a disruption of the blood-labyrinth-barrier and the endolymphatic potential and/or a sterile inflammation of the stria vascularis. In severe cases like our subject, we posit that endolymphatic hydrops or hair cell loss may cause irreversible and asymmetrical deafness. Steroid prophylaxis via transtympanic application is debatable. Show less