Hearing Deterioration Research Articles

Chronic hearing loss turns out being a calcified chondroid mesenchymal neoplasm with FN1::FGFR2 fusion.

A 53 year old female presented with a six-year history of right-sided slow deterioration in hearing and a feeling of pressure in the right ear. The patient had not experienced any pain but reported some paresthesia of the right half of the tongue, whereas no further other cranial nerve deficits were evident. The otoscopy was unremarkable as well as the rest of the clinical ENT examination except for a slight asymptomatic swelling of the right cheek. Imaging findings showed an expansive tumor infiltrating and destroying the right lateral skull base. The tumor was partially composed of cystic/regressive lesions with high contrast media uptake. The tumor had high-signal intensity with water-sensitive sequences (T2w) and was hypointense on T1w images. We performed a tumor resection via a transparotideal-infratemporal approach. Histologically, the tumor was composed of granular variably calcified chondroid matrix with extensive regressive changes and granulation-like tissue reaction associated with calcinosis and crystal deposition. Molecular analysis of the tumor via the TruSight- RNA-Fusion panel detected a fusion involving FN1::FGFR2, consistent with "calcified chondroid mesenchymal neoplasm" (CCMN), a rare tumor entity recently defined by Liu et al 2021. In regular follow-up care no residual tumor has been detected in imaging studies (MRI and CT) within 2 years and 4 months. The biology and consequently the radio sensitivity cannot be defined precisely since long term results are missing due to the first description of this entity in 2021. As a consequence, surgical resection is recommended as the treatment of choice. Thorough clinical and radiological follow-up is mandatory as local recurrences are to be expected due to the infiltrative behavior. In case of a loco regional recurrence the fusion with FGFR2 may represent a therapeutic option for a targeted therapy on molecular level.

Sensorineural hearing loss in post-COVID-19 patients

<b>Background: </b>Hearing loss (HL) is one of the most common chronic conditions after hypertension and arthritis. Mounting indices suggest that sensorineural hearing loss (SNHL) may be one of the adverse effects of severe acute respiratory syndrome coronavirus 2.<br /> <b>Objective: </b>This work aimed at studying SNHL in post-COVID-19 patients, alongside with exploring the relationship between severity of the disease and degree of hearing disability.<br /> <b>Materials and methods: </b>This prospective cross-sectional study was conducted at Al-Azhar University Hospitals, Cairo, Egypt. It included 100 post-COVID-19 patients selected according to the study inclusion and exclusion criteria. Pure tone audiometry was done to confirm the presence of HL and to determine its degree.<br /> <b>Results: </b>Hundred patients with recent oncent hearing symptoms after documented COVID-19 infection were included. Males (71%) were more affected than females (29%). The most of our cases had bilateral moderate HL (45%). Tinnitus was the most frequently associated otological symptom (96.96%). The time of HL onset varied from days to weeks (55% had time onset between 11-30 days post-confirmed infection). Higher thresholds were detected in all frequencies of affected ears with more deterioration of hearing as frequencies increase. All degrees of HL were detected; mild, moderate, severe and profound. There was no significant correlation between severity of COVID-19 infection and severity of HL.<br /> <b>Conclusions: </b>SNHL is one of the extrapulmonary complications of COVID-19. COVID-19 patients may develop hearing affection regardless the severity of the infection. It is recommended to assess hearing functions in patients after COVID-19 for early detection and proper management.

PETAL – LIKE OTOPLASTY IN THE TREATMENT OF PROTRUDING EARS

Introduction. Approximately 5% of humanity suffers from inborn protruding ears, and usually this condition causes marked psychosomatic disorders. Despite years of experience gained in aesthetic surgery, optimal technique of otoplasty is yet to be elaborated. The Aim of the Research. To develop a method of surgical correction of congenital bulging ears in order to optimize the immediate and long-term results of otoplasty. Material and methods. We have analysed the outcomes of 120 patients operated for congenital protruding ears at Lviv City Cosmetologic Hospital between 2020 and 2023. Distance between mastoid process and the concha exceeding 20 mm, and auriculo-cephalic angle over 30° were used as objective criteria of protruding ears. Surgical improvement of tall concha and increased auriculo-cephalic angle were identified as key vectors in elaboration of the original otoplasty technique. Results of the Research. With local anaesthesia, an S-shaped incision is made to the depth of perichondrium of posterior surface of the cartilage of the ear. Once subcutaneous dissection is completed, preoperative markup is transferred with injection needles from anterior surface to posterior surface of the conchal cartilage. A full depth arch-shaped incision of conchal cartilage is made. Perichondrium and skin of anterior surface of the concha are mobilized with a raspatory, from the chondral «petal» to the margin of external meatus. Basis of the chondral «petal» is dissected by 2/3 of its depth along the vertical axis between the pedicle of the helix, and the antitragus. In order to reliably fix the auricula, the cartilage is stitched to the mastoid process with three mattress sutures on the ventral surface of the «petal». Finally, excessive skin is carefully resected, and the wound is closed with continuous suture, with dead space elimination for hematoma prevention. Our technique has the following significant advantages: Unique conchal cartilage is kept entirely, therefore it can be used as auto-transplant in the future. The technique does not significantly affect the rheology of auricula. With this technique correction and modification of antihelical curve and radius is possible; if necessary, width of auricula can be corrected, and auricular height to width ratio can be modified. The proposed «petal» technique significantly reduces the rate of postoperative complications, such as recurrent protruding ears. Postoperatively, the size of external meatus is not reduced, hence no hearing deterioration. The outcomes of the technique we have developed were assessed 6 months after otoplasty as good, satisfactory and unsatisfactory. Good outcome: preservation of symmetry and anatomical «standard» of both auriculas, no recurrence throughout follow-up, and no impairment of sensation in the external ear. Satisfactory outcome: anatomical «standard» of the organ is preserved, with permissible error of up to 3 mm in the symmetrical points on the right and left auriculas, not requiring additional correction. Conclusions. 98 (81,3%) patients operated by the proposed «petal» technique assessed their otoplasty outcome as good, 17 (14,3%) of patients assessed the outcome of surgery as satisfactory. 5 (4,3%) patients were not satisfied with the outcome of surgery. These patients were operated during the early period of development of the technique.

Enhanced Cochlear Coverage and Hearing Preservation in High-Frequency Hearing Loss via Electric Acoustic Stimulation with Longer Electrode.

Electric-acoustic stimulation (EAS) is a promising treatment to improve hearing ability in patients with high-frequency hearing loss (HL). In EAS surgeries, shorter electrodes have been preferred to avoid the presence of an electrode covering the residual hearing region. However, our earlier studies showed that EAS with longer electrodes (28 mm) could preserve acoustic hearing. Additionally, we reported that the hearing preservation (HP) scores were independent of the length of the inserted electrodes, consistent with the systematic review. As most EAS patients gradually lose residual hearing over time due to the natural course of HL, in these cases, providing broader cochlear coverage using longer electrodes was beneficial toward better place-pitch matching. In addition to preparing for the deterioration inhearing inthe future, EAS with longer electrodes could offer various types of map strategies. Herein, we show the pre-, intra-, and post-procedures for EAS surgery. Appropriate preoperative evaluation, less invasive surgery, flexible lateral-wall electrodes, and steroid administration resulted in good HP following EAS with longer electrodes.

Evaluation of Changes in the Patency of the Nasal Cavity and Eustachian Tube Depending on the Phase of the Menstrual Cycle: A Pilot Study.

(1) Background: Estrogen and progesterone, hormones specific to females, undergo fluctuations during the menstrual cycle. The aim of this study was to assess subjective and objective changes in nasal cavity and Eustachian tube patency depending on the phase of the menstrual cycle in two groups of women: those in a follicular phase group and those in a luteal phase group. (2) Materials and Methods: The study group consisted of 25 healthy non-pregnant women aged 24 to 32. Based on the phase of the cycle confirmed in sonography, they were divided into follicular phase (FP) and luteal phase (LP) groups. The Eustachian tube and nasal cavity patency examination was carried out using a SNOT-22 Questionnaire, a rhinomanometer, and a tympanometer. (3) Results: We observed that the incidence of nasal obstruction in SNOT-22 was significantly dependent on the cycle phase (p = 0.012) and was lower in the FP compared to the LP. Similar relationships were noticed between the cycle phase and the rhinomanometry outcome, where the LP was associated with a lower flow. We also revealed that the incidence of ear blockage significantly depended on the cycle phase (p = 0.001) and was lower in the FP compared to the LP. Women whose nasal patency deteriorated during the LP also had more negative pressure values in tympanometry. We observed that patients with negative PEAK L and R levels had a lower flow in rhinomanometry. (4) Conclusions: The results highlight the menstrual cycle's substantial impact on both subjective and objective nasal and Eustachian tube patency measurements. The novel finding in this study is that women whose nasal patency deteriorated during the luteal phase also had more negative pressure values in tympanometry. These results suggest that the deterioration of hearing during the menstrual cycle could be a result of swelling of the nasal mucosa and tubes.

The biphasic impact of apolipoprotein E ε4 allele on age-related hearing loss

Both the ε4 variant of the apolipoprotein E (APOE) gene and hearing loss are well-known risk factors for Alzheimer's disease. However, previous studies have produced inconsistent findings regarding the association between APOE genotypes and hearing levels, necessitating further investigation. The aim of this study was to investigate the relationship between APOE genotypes and hearing levels. This retrospective study analyzed clinical data from a clinical data warehouse of seven affiliated Catholic Medical Center hospitals. The study included 1,162 participants with records of APOE genotypes, audiometric tests, and cognitive function tests. In Generalized linear mixed model analysis, ε4 carriers exhibited lower pure tone audiometry thresholds with an estimate of -0.353 (SE = 0.126, p = 0.005). However, the interaction term for age and APOE ε4 had a coefficient of 0.577 (SE = 0.214 p = 0.006), suggesting that the APOE ε4 gene may accelerate hearing deterioration with age. Subgroup analysis based on an age cut-off of 75 revealed that ε4 carriers had better hearing at younger ages, but showed no significant difference at older ages. These results indicate that the ε4 allele may have a biphasic effect on hearing levels depending on age.

Low-Dose Aspirin and Progression of Age-Related Hearing Loss

Age-related hearing loss is common in an aging population, affecting communication and contributing to a worsened quality of life. It occurs as a result of cochlear degeneration and may be further exacerbated by inflammation and microvascular changes, as observed in animal models. To compare the effect of daily low-dose aspirin vs placebo on the progression of age-related hearing loss in healthy older adults. A prespecified secondary analysis was conducted of the Aspirin in Reducing Events in the Elderly (ASPREE) randomized clinical trial. Participants were 279 healthy community-dwelling individuals living in Australia who were aged 70 years or older and free of overt cardiovascular diseases, dementia, and life-limiting illnesses. Participants were recruited between January 1, 2010, and December 31, 2014, and followed up over 3 years. Statistical analysis was completed from June to December 2023. A 100-mg daily dose of enteric-coated aspirin or matching placebo. Hearing measures were air conduction audiometry and binaural speech perception in noise. Assessments were conducted at baseline, 18 months, and 3 years. The change from baseline hearing measures were analyzed using an intention to treat approach. Aspirin and placebo were compared using mixed linear regression models adjusting for age, sex, diabetes, and smoking. Of 279 participants, 154 (55%) were male, and the median age at baseline was 73.1 years (IQR, 71.5-76.2 years). A total of 98 of 138 participants (71%) in the aspirin group and 94 of 141 participants (67%) in the placebo group reported experiencing hearing loss at baseline. Compared with placebo, aspirin did not affect the changes in mean (SD) 4-frequency average hearing threshold from baseline to year 3 (aspirin: baseline, 27.8 [13.3] dB; year 3, 30.7 [13.7] dB; difference, 3.3 [3.9] dB; placebo: baseline, 27.5 [12.6] dB; year 3, 30.9 [13.8] dB; difference, 3.0 [4.8] dB; P = .55) nor any other tested frequencies. An increase in air conduction threshold indicates a deterioration in hearing. Similarly, for the mean (SD) speech reception threshold, there was no significant difference observed between the aspirin and placebo group at the year 3 follow-up assessment (aspirin: baseline, -9.9 [3.8] dB; year 3, -9.1 [3.8] dB; difference, 0.9 [2.9] dB; placebo: baseline, -10.5 [7.1] dB; year 3, -9.6 [4.1] dB; difference, 0.9 [5.9] dB; P = .86). The findings were consistent across sex, age groups, diabetic and smoking status. In this secondary analysis of the ASPREE randomized clinical trial, low-dose aspirin did not affect the progression of age-related hearing loss. More investigation is warranted on whether a longer follow-up or the use of a more powerful anti-inflammatory agent might prove beneficial. anzctr.org.au Identifier: ACTRN12614000496617.

Modified stapes surgery for otosclerosis: An evaluation of auditory results and postoperative complications.

The senior author Shiao, had introduced a modified version of the traditional stapes surgery, called minimally traumatic stapes surgery (MTSS), and explored its effectiveness in reducing postoperative vertigo. However, MTSS exhibited no significant breakthrough in terms of audiometric results. Building upon the original MTSS through slight modifications and the use of the Medtronic Big Easy ® Piston as the prosthesis, this study evaluates a refined version of the original MTSS technique. In particular, this research sought to investigate the impact of this refined approach on hearing outcomes and postoperative complications among patients diagnosed with otosclerosis. This research comprehensively examined the medical records of individuals diagnosed with otosclerosis who underwent MTSS. The postoperative outcomes assessed encompassed hearing outcomes and the occurrence of any new complications. Overall, 95 patients diagnosed with otosclerosis (comprising 106 affected ears) underwent MTSS. Significant improvements in both the average air conduction threshold (40 ± 16.82 dB after vs 60 ± 14.56 dB before surgery; p < 0.0001) and average air-bone gap (11.99 ± 7.24 dB after vs 29.65 ± 9.47 dB before surgery; p = 0.003) were observed after the surgery. Conversely, no significant change in the average bone conduction threshold was observed after the procedure (28 ± 13.81 dB after vs 29 ± 12.31 dB before surgery; p = 0.149). Among the 106 affected ears, 102 showed postoperative air-bone gap measurements <20 dB, indicating an impressive 96.2% overall hearing improvement. Notably, only a few postoperative complications were observed, including vertigo, chorda tympani injury, facial weakness, and a slight hearing deterioration. The refined MTSS technique promoted significant postoperative hearing improvements with minimal complications. This approach showed potential for addressing the surgical challenges in Asian patients with otosclerosis, emphasizing the importance of further research on this advanced procedural method.

Sialyllactose preserves residual hearing after cochlear implantation

In individuals with hearing loss, protection of residual hearing is essential following cochlear implantation to facilitate acoustic and electric hearing. Hearing preservation requires slow insertion, atraumatic electrode and delivery of the optimal quantity of a pharmacological agent. Several studies have reported variable hearing outcomes with osmotic pump-mediated steroid delivery. New drugs, such as sialyllactose (SL) which have anti-inflammatory effect in many body parts, can prevent tissue overgrowth. In the present study, the positive effects of the pharmacological agent SL against insults were evaluated in vitro using HEI-OC1 cells. An animal model to simulate the damage due to electrode insertion during cochlear implantation was used. SL was delivered using osmotic pumps to prevent loss of the residual hearing in this animal model. Hearing deterioration, tissue fibrosis and ossification were confirmed in this animal model. Increased gene expressions of inflammatory cytokines were identified in the cochleae following dummy electrode insertion. Following the administration of SL, insertion led to a decrease in hearing threshold shifts, tissue reactions, and inflammatory markers. These results emphasize the possible role of SL in hearing preservation and improve our understanding of the mechanism underlying hearing loss after cochlear implantation.

First Clinical Experience with a New Device for the Removal of Cochlear Schwannomas.

Background: In most cases, intralabyrinthine schwannoma (ILS) occurs in patients with unilateral hearing deterioration or neurofibromatosis type II (NF II). The pattern of localization of these tumors varies but mostly affects the cochlea. Extirpation of the cochlear schwannoma, if hidden by the cochlea modiolus, is difficult under the aspect of complete removal. Therefore, a tissue removal device (TRD) was designed and tested in temporal bones. The principle of handling the new device is a pushing and pipe cleaner handling inside the cochlea. This present study aimed to describe the first in vivo experience with the newly developed TRD for removing cochlear intralabyrinthine schwannomas. Methods: In three patients, the TRD was used for the tumor removal of cochlear schwannomas. In two patients with a cochlear schwannoma in combination with a cochlea implantation and one patient suffering from NF II, a cochlear schwannoma was removed with the TRD. The access was performed with a posterior tympanotomy, an enlarged round window approach and an additional second turn access. The device was inserted and extracted gradually from the second turn access until the rings were visible in the second turn access. By pushing and pipe cleaner handling, the tumors were removed. An MRI control was performed on the day postoperatively with a T1 GAD sequence. Results: Tumor removal with the TRD was performed in a 15-min procedure without any complications. An MRI control confirmed complete removal on the postoperative day in all cases. Conclusions: In vivo handling of the device confirmed straightforward handling for the tumor removal. MRI scanning showed complete removal of the tumor by the TRD.

The Impact of Piezoelectric Device in Cholesteatoma Endoscopic Surgery: Retrospective Evaluation of Safety and Functional Results.

Atticotomy represents an essential surgical step within the management of attical cholesteatoma during endoscopic ear surgery. The aim of the present study was to evaluate the safety and functional results of an endoscopic atticotomy performed with piezosurgery, in terms of audiological outcomes and tissue's healing. This is an observational retrospective study on patients with attical cholesteatoma who underwent endoscopic ear surgery with piezoelectric atticotomy and subsequent scutum reconstruction either with tragal cartilage or temporalis muscle fascia. Scutumplasty's status was evaluated via scheduled outpatient controls through an endoscopic check and classified as stable or unstable at least 10 months after surgery. A pre- and postoperative audiometric examination was performed in each patient. Eighty-four patients were enrolled. In 50 out of 84 patients (59.52%), an exclusive endoscopic procedure was performed, whereas in 34 patients (40.48%) a combined microscopic/endoscopicapproach was adopted. In 72 cases (85.71%), scutum's reconstruction appeared to be normally positioned, whereas 12 patients developed a retraction pocket, which was self-cleaning in 8 of them and non-self-cleaning in the remaining 4. In 17 patients, a slight sensorineural hearing loss (between 5 and 15 dBHL) was observed, and in 2 patients, the loss was greater than 15 dBHL. Overall, no significative differences between pre- and postoperative pure-tone average bone thresholds resulted (p = 0.4983), though a mild significant hearing deterioration was detected by the specific analysis at 4000 Hz (p = 0.0291). Piezosurgery represents an extremely useful tool in performing atticotomy during endoscopic tympanoplasties. Our data seem to support the safety of its usage in this specific step, as it did not lead to any significant sensorineural damage on overall pure-tone average. Moreover, satisfactory tissue healing in regard to scutum reconstruction was observed.

"Minimally symptomatic" congenital cytomegalovirus infection: latest data and emerging concepts.

Universal and targeted screening of newborns for congenital cytomegalovirus (CMV) infection is increasing globally. Questions remain concerning the management of infants who have been identified with congenital CMV infection, especially those with "minimally symptomatic" or clinically inapparent infection. Our objective is to discuss current management of CMV-infected neonates with a focus on less affected infants with or without sensorineural hearing loss (SNHL). Valganciclovir is being prescribed increasingly in neonates with congenital CMV infection for improvement in hearing outcomes through 2 years of age. Treatment initiated in the first month of age is recommended for clinically apparent disease. A recent study showed hearing improvement at 18-22 months of age when therapy was initiated at age 1-3 months in infants with clinically inapparent CMV infection and isolated SNHL. Antiviral therapy with either ganciclovir or valganciclovir has shown moderate benefit in prevention of hearing deterioration among infants with clinically apparent CMV infection or isolated SNHL. Sustainability of benefit beyond 2 years of age remains unknown. At present, infants with clinically inapparent CMV infection (normal complete evaluation including hearing) should not receive antiviral therapy. All CMV-infected infants require close audiological and neurodevelopmental follow-up.

Deterioration of Hearing Due to Hearing Aids.

A primary reason for the low adoption of hearing aids (HAs) among the large population with sensorineural hearing loss is the perception that these devices may negatively impact remaining hearing ability. Research addressing this issue has yielded conflicting results. This study examined the long-term effects of HAs on standard audiometric changes in individuals with sensorineural hearing loss. We retrospectively analyzed patients who acquired unilateral HAs between 2015 and 2017 and demonstrated consistent use over a 5-year period. We examined demographics, medical comorbidities, audiometric data, and questionnaire results from the Hearing Handicap Inventory for the Elderly and the International Outcome Inventory for Hearing Aids. Additionally, we reviewed each patient's history of noise exposure and prior HA use. The study included 55 patients who used unilateral HAs, with a mean follow-up period of 5.32 years. Among them, 31 patients (56.4%) used the HA on the right side. Audiometric data from the aided side showed no significant difference from the unaided side in either the pre-fit pure-tone average of air conduction (AC) or word recognition score (WRS) (P =0.73 and P =0.11, respectively). Similarly, no significant differences were noted in the 5-year follow-up audiometry of AC and WRS (P=0.98 and P=0.07, respectively) or in the change from pre-fit to final audiometry for either parameter (AC, P=0.58; WRS, P=0.70). Eleven patients (20%) exhibited a deterioration in hearing (as measured by AC) of 5 dB or greater on the aided side compared with the unaided side, while 23 (53.5%) showed greater WRS deterioration on the aided side. No significant factors were found to contribute to the difference in hearing deterioration between groups for either AC or WRS. No significant factors were identified as contributing to hearing deterioration after prolonged HA use. Overall, the use of HAs did not adversely impact residual hearing.

Click evoked otoacoustic emissions in occupational exposure to lead, concentrations of selected essential elements and markers of oxidative stress

PurposeThis study focused on the selected markers of oxidative stress, impact of elevated lead levels on long-term hearing quality. We investigated whether the presence of certain essential minerals might provide protection to the auditory system against the effects of lead (and cadmium) compounds.MethodsThe research group included 280 male employees of the zinc and lead smelter, which was divided into: L-Pb—low blood lead concentration (PbB) subgroup, H-Pb—high PbB subgroup. Hearing tests were performed using the click evoked otoacoustic emission (CEOAE).ResultsZinc protoporphyrin level was significantly higher in the H-Pb subgroup by 68%. Cd concentration was significantly higher in H-Pb by 33%. The Ca concentration was significantly lower in the H-Pb by − 2%. Selected oxidative stress markers concentration were significantly higher in the H-Pb group: malondialdehyde (MDA) by 4%, and lipofuscin (LPS) by 9%. In the CEOAE results showed statistically significant differences between the L-Pb and H-Pb subgroups. Larger negative changes in otoemission amplitude were observed in H-Pb subgroup. All otoemission results showed a statistically significant negative correlation with age, time of work, MDA concentration, and with PbB. Selected CEOAE parameters showed a significant negative correlation with cadmium blood concentration (CdB), and a positive correlation with Ca and Zn.ConclusionElevated blood lead content in occupational exposure is associated with an increase in MDA and LPS concentration, which negatively correlates with CEOAE parameters. This suggests an important role of oxidative stress in the long-term deterioration of hearing.

Idiopathic sudden sensorineural hearing loss: A trial of modified Stennert’s infusion therapy

Introduction and Aim: Sudden sensorineural hearing loss presents as rapid onset hearing deterioration of more than 30dB within 72 hours at the minimum of three adjacent frequencies. The aim of this research was to determine the effect of Modified Stennert’s Protocol in addressing idiopathic sudden sensorineural hearing loss. Materials and Methods: Retrospective pilot study was done. 14 patients were started on Modified Stennert’s protocol within 72 hours of onset of symptoms and were observed. Our modification included using hydrocortisone instead of prednisolone. Results: All patients presenting with sudden sensorineural hearing loss showed significant improvement in hearing by the end of two weeks and at the end of 3 months. Conclusion: With such obscured pathophysiology, treatment of modified versions of usage of hydrocortisone instead of prednisolone along with dextran and pentoxyfylline has shown prime augmentation towards the patients resulting in near normal hearing.

Valganciclovir in Infants with Hearing Loss and Clinically Inapparent Congenital Cytomegalovirus Infection: A Nonrandomized Controlled Trial

ObjectiveTo assess the efficacy of valganciclovir in infants with hearing loss and clinically inapparent congenital cytomegalovirus infection (cCMV), as there is no consensus on treatment of this group. Study designA nationwide, non-randomized controlled trial, comparing six weeks of oral valganciclovir to no treatment in infants with cCMV, recruited after newborn hearing screening resulted in referral. The choice whether to treat was left to parents of subjects. Eligible subjects were full term infants aged <13 weeks with sensorineural hearing loss and diagnosed with cCMV through dried blood spot testing. The primary outcome, measured by linear and ordinal logistic regression, was change in best-ear hearing from baseline to follow-up at 18-22 months of age. ResultsThirty-seven participants were included in the final analysis, of whom 25 were in the treatment group and 12 in the control group. The majority of subjects in both groups had neuroimaging abnormalities, which were mostly mild. Hearing deterioration was more likely in the control group compared with the treatment group (common odds ratio 0.10, 95% CI 0.02-0.45, P=0.003). Mean best-ear hearing deteriorated by 13.7 dB in the control group, compared with improvement of 3.3 dB in the treatment group (difference 17 dB, 95% CI 2.6 – 31.4, P=0.02). ConclusionsWe investigated treatment in children with hearing loss and clinically inapparent cCMV. Although our study was non-randomized, it is the first prospective and controlled trial in this population. Valganciclovir-treated children with hearing loss and inapparent cCMV had less hearing deterioration at 18 through 22 months of age than control subjects.

Hearing preservation after stereotactic radiosurgery for sporadic intracanalicular vestibular schwannomas classified as Koos grade 1.

The mechanism of hearing loss following stereotactic radiosurgery (SRS) for vestibular schwannomas (VSs) remains unclear. There is conflicting evidence regarding cochlear nerve damage by transient volume expansion of VSs after radiosurgery and radiation-induced cochlear damage. This study aimed to investigate whether there is a specific patient population that can achieve definite hearing preservation after SRS for VSs. A total of 37 consecutive patients with sporadic unilateral intracanalicular VSs and serviceable hearing (Gardner-Roberson [G-R] class I or II) were treated with SRS from 2009 to 2023. This is a retrospective study. Survival analysis with Cox regression for hearing deterioration was performed. The median age was 55 years old. The median tumor volume was 0.089 cm3 , and the median marginal dose was 12.0 Gy. Nonserviceable hearing deterioration occurred in 9 patients (24.3%), with a median onset of 11.9 months after SRS. The actuarial rates of serviceable hearing preservation were 86%, 82%, and 70% at 1, 2, and 3 years after SRS, respectively. In a multivariate analysis, only baseline pure tone average > 30 dB increased the risk of nonserviceable hearing deterioration with significant hazard ratio. There were 13 patients with petit VSs whose tumor volume was smaller than 0.05 cm3 , and 11 of them were treated by a 4-mm single shot with a marginal dose of 12 Gy. None of the 13 patients had nonserviceable hearing deterioration. Petit VSs that can be treated with 4-mm single or double shots with a marginal dose of 12 Gy may achieve hearing preservation after SRS.

Splicing mutations of GSDME cause late-onset non-syndromic hearing loss

Objective:To dentify the genetic and audiological characteristics of families affected by late-onset hearing loss due to GSDMEgene mutations, aiming to explore clinical characteristics and pathogenic mechanisms for providing genetic counseling and intervention guidance. Methods:Six families with late-onset hearing loss from the Chinese Deafness Genome Project were included. Audiological tests, including pure-tone audiometry, acoustic immittance, speech recognition scores, auditory brainstem response, and distortion product otoacoustic emission, were applied to evaluate the hearing levels of patients. Combining with medical history and physical examination to analyze the phenotypic differences between the probands and their family members. Next-generation sequencing was used to identify pathogenic genes in probands, and validations were performed on their relatives by Sanger sequencing. Pathogenicity analysis was performed according to the American College of Medical Genetics and Genomics Guidelines. Meanwhile, the pathogenic mechanisms of GSDME-related hearing loss were explored combining with domestic and international research progress. Results:Among the six families with late-onset hearing loss, a total of 30 individuals performed hearing loss. The onset of hearing loss in these families ranged from 10 to 50 years（mean age: 27.88±9.74 years）. In the study, four splicing mutations of the GSDME were identified, including two novel variants: c. 991－7C>G and c. 1183+1G>T. Significantly, the c. 991－7C>G was a de novo variant. The others were previously reported variants: c. 991-1G>C and c. 991-15_991-13del, the latter was identified in three families. Genotype-phenotype correlation analysis revealed that probands with the c. 991－7C>G and c. 1183+1G>T performed a predominantly high-frequency hearing loss. The three families carrying the same mutation exhibited varying degrees of hearing loss, with an annual rate of hearing deterioration exceeding 0.94 dB HL/year. Furthermore, follow-up of interventions showed that four of six probands received intervention（66.67%）, but the results of intervention varied. Conclusion:The study analyzed six families with late-onset non-syndromic hearing loss linked to GSDME mutations, identifying four splicing variants. Notably, c. 991－7C>G is the first reported de novo variant of GSDME globally. Audiological analysis revealed that the age of onset generally exceeded 10 years,with variable effectiveness of interventions.

Preemptive strategies and lessons learned from complications encountered with microvascular decompression for hemifacial spasm.

Microvascular decompression (MVD) is the only curative treatment modality for hemifacial spasm (HFS). Although generally considered to be safe, this surgical procedure is surrounded by many risks and possible complications. The authors present the spectrum of complications that they met in their case series, the possible causes, and the strategies recommended to minimize them. The authors reviewed a prospectively maintained database for MVDs performed from 2005 until 2021 and extracted relevant data including patient demographics, offending vessel(s), operative technique, outcome, and different complications. Descriptive statistics with uni- and multivariable analyses for the factors that may influence the seventh, eighth, and lower cranial nerves were performed. Data from 420 patients were obtained. Three hundred seventeen of 344 patients (92.2%) with a minimum follow-up of 12 months had a favorable outcome. The mean follow-up (standard deviation) was 51.3 ± 38.7 months. Immediate complications reached 18.8% (79/420). Complications persisted in only 7.14% of patients (30/420) including persistent hearing deficits (5.95%) and residual facial palsy (0.95%). Temporary complications included CSF leakage (3.10%), lower cranial nerve deficits (3.57%), meningitis (0.71%), and brainstem ischemia (0.24%). One patient died because of herpes encephalitis. Statistical analyses showed that the immediate postoperative disappearance of spasms and male gender are correlated with postoperative facial palsy, whereas combined vessel compressions involving the vertebral artery (VA) and anterior inferior cerebellar artery can predict postoperative hearing deterioration. VA compressions could predict postoperative lower cranial nerve deficits. MVD is safe and effective for treating HFS with a low rate of permanent morbidity. Proper patient positioning, sharp arachnoid dissection, and endoscopic visualization under facial and auditory neurophysiological monitoring are the key points to minimize the rate of complications in MVD for HFS.

Efficacy and preservation of hearing with low-dose gentamicin in unilateral meniere's disease: A clinical symptomatology-based study

PurposeMeniere's disease (MD), a disorder of the inner ear, presents numerous therapeutic challenges, and intratympanic (IT) gentamicin has been proposed for intractable cases. However, controversy regarding dosage and method persists. The purpose of this study was to assess the efficacy and safety of low-dose IT gentamicin on vertigo attacks in MD using a clinical symptomatology-based method, wherein administration was repeated only if vertigo attacks recurred, with a 2-week interval between injections. Materials and methodsThis study included 88 patients with unilateral intractable MD. All patients received one to five IT injections with 0.5 ml of 10 mg of gentamicin (80 mg/2 ml) with an interval of 2 weeks between injections. Vertigo attacks were evaluated before and after therapy and categorized into classes A–F according to the 2015 Equilibrium Committee criteria. Audiovestibular assessments, including Pure Tone Audiometry and Vestibulo-Ocular Reflex evaluations, were performed. ResultsBefore treatment, patients had an average of 4.4 vertigo attacks/month; after treatment, this average decreased to 0.52. The majority of patients (57 %) reached Class A or B vertigo control with five or fewer gentamicin injections. VOR gain was slightly affected on the healthy side and significantly reduced on the affected side. No hearing deterioration was found in any of the treated patients. ConclusionsLow-dose IT gentamicin administration based on clinical symptomatology can produce a satisfactory control of vertigo attacks after treatment. This protocol primarily affected the vestibular function, as demonstrated by the significant reduction in VOR gain on the affected side, while avoiding cochlear damage. The lack of adverse events and preservation of hearing underscore the safety and efficacy of this method. These findings have significant clinical implications, suggesting that a low-dose, clinical symptomatology-based gentamicin treatment regimen could be an effective and safe strategy for managing unilateral Meniere's disease in a larger population.