Severe Sensorineural Hearing Loss Research Articles

[Analysis of clinical manifestations and imaging features of facial nerve schwannomas].

Objective:To explore the clinical manifestations and imaging characteristics, and to clarify the imaging value in the diagnosis of facial nerve schwannomas. Methods:Retrospectively analyze the data of 23 patients with facial nerve schwannomas confirmed by surgery and pathology in the Department of Otorhinolaryngology of the First Affiliated Hospital of the Air Force Military Medical University from September 2020 to September 2022, including 8 males and 15 females, aged 18-66 years old. Summarize and analyze their clinical symptoms, specialized examinations, and imaging findings. Results:The clinical manifestations were facial nerve paralysis in 15 cases（2 cases of HB Ⅳ, 6 cases of HB Ⅴ, 7 cases of HB Ⅵ）, hearing loss in 14 cases（5 cases of conductive deafness, 2 cases of mixed deafness, and 7 cases of severe sensorineural hearing loss）, 8 cases tinnitus, 7 cases ear pain, 4 cases dizziness, 4 cases headache, 2 cases ear pus, and parotid gland tumors in 6 cases presenting as local masses. Endoscopic examination revealed 8 cases of external ear canal tumors and 3 cases of intratympanic tumors. Combining temporal bone HRCT, MRI enhanced scanning, and CPR imaging techniques, 1 case involved the internal auditory canal segment, 2 cases in the tympanic segment, 6 cases in the parotid gland area. A total of 14 cases involved two or more segments of the internal auditory canal segment, the labyrinthine segment, geniculate ganglion, the tympanic segment, and the mastoid segment. When the tumors were large, adjacent structures were involved. It was found that 8 cases invaded the external auditory canal and tympanic cavity, ossicles were displaced or bony destruction; 3 cases invaded the jugular foramen area, and 1 case grew to the middle cranial fossa region with temporal lobe brain parenchymal compression. Conclusion:The clinical manifestations of facial nerve schwannomas are diverse. The combination of various imaging techniques will be conducive to topical and qualitative diagnosis and provide an important basis for treatment strategies.

Speech perception outcomes in hearing-impaired individuals with microphone & receiver in the ear (M&RIE) hearing aids.

The Microphone and Receiver in the ear (M&RIE) hearing aid is designed to consider the pinna cues by placing an additional microphone along with a standard receiver at the entrance of the ear canal. There is a need to clinically validate the technology using speech identification measures, as a literature shortage exists. The objective of the study was to evaluate speech identification and quality rating with hearing aids fitted with standard and M&RIE receivers. Twenty individuals (mean ± SD age being 34.10 ± 11.9years) with mild-to-moderately severe sensorineural hearing loss were divided into two groups: naïve and experienced. Their speech identification obtained using the receiver in the ear hearing aid compatible with standard and M&RIE receivers were compared. The study assessed speech identification in quiet using ten sentences, calculated SNR-50 using the Spearman-Karber equation, and used an 11-point scale for self-perceived quality rating. The study analyzed data using SPSS and found no significant difference between naive and experienced hearing aid users in quiet and SNR-50 conditions (p > 0.05). However, naive users rated higher in clarity, naturalness, pleasantness, and overall impression of standard receiver settings. Across different receiver settings, both naïve and experienced groups revealed better speech identification with the M&RIE receiver for both SIS (p < 0.05) and SNR-50 (p < 0.05) measures. The study found that placing a third microphone in the ear canal improves speech identification and quality rating in both naïve and experienced users, with pinna and ear canal resonance playing a crucial role in prescribing hearing aid gain.

[Clinical features of congenitally enlarged bony portion of Eustachian tube].

Objective:To investigate the clinical features of patients with congenitally enlarged bony portions of the Eustachian tube（ET）. Methods:The medical history, physical examination, hearing test, temporal bone high resolution computed tomography（HRCT） of six patients（nine ears） with congenitally enlarged bony portion of the ET were retrospectively analyzed. Results:Four patients were men and two were women. The minimum, maximum, and average ages were 5, 21, and（14.7±6.4） years, respectively. Three malformations were bilateral and three were left-sided. Three ears had conductive hearing loss（average bone and air conduction thresholds were 13.7 dB and 71.3 dB）, three had mixed hearing loss（average bone and air conduction thresholds were 27.7 dB and 83.7 dB）, and one had extremely severe sensorineural hearing loss. The average maximum length and width of the enlarged bony ET on temporal bone HRCT were（22.61±2.94） mm and（6.50±2.33） mm, respectively. The enlargement was combined with an external auditory canal malformation in six ears, narrow tympanic cavity in six, tympanic antrum malformation in five, ossicular chain malformation in seven, cochlear malformation in six, helicotrema malformation in three, vestibule widening in two, semicircular canal malformation in three, vestibular window malformation in six, facial nerve abnormality in five, internal auditory meatus malformation in two, low middle cranial fossa in eight, and severe internal carotid artery malformation in one. Conclusion:Bony ET enlargement is a rare congenital middle ear malformation which could combined with other ear malformations. Patients can have no ET dysfunction but different patterns of hearing loss. The defect is usually found unintentionally during imaging, and the HRCT of temporal bone is significant.

The VertiGO! Trial protocol: A prospective, single-center, patient-blinded study to evaluate efficacy and safety of prolonged daily stimulation with a multichannel vestibulocochlear implant prototype in bilateral vestibulopathy patients.

A combined vestibular (VI) and cochlear implant (CI) device, also known as the vestibulocochlear implant (VCI), was previously developed to restore both vestibular and auditory function. A new refined prototype is currently being investigated. This prototype allows for concurrent multichannel vestibular and cochlear stimulation. Although recent studies showed that VCI stimulation enables compensatory eye, body and neck movements, the constraints in these acute study designs prevent them from creating more general statements over time. Moreover, the clinical relevance of potential VI and CI interactions is not yet studied. The VertiGO! Trial aims to investigate the safety and efficacy of prolonged daily motion modulated stimulation with a multichannel VCI prototype. A single-center clinical trial will be carried out to evaluate prolonged VCI stimulation, assess general safety and explore interactions between the CI and VI. A single-blind randomized controlled crossover design will be implemented to evaluate the efficacy of three types of stimulation. Furthermore, this study will provide a proof-of-concept for a VI rehabilitation program. A total of minimum eight, with a maximum of 13, participants suffering from bilateral vestibulopathy and severe sensorineural hearing loss in the ear to implant will be included and followed over a five-year period. Efficacy will be evaluated by collecting functional (i.e. image stabilization) and more fundamental (i.e. vestibulo-ocular reflexes, self-motion perception) outcomes. Hearing performance with a VCI and patient-reported outcomes will be included as well. The proposed schedule of fitting, stimulation and outcome testing allows for a comprehensive evaluation of the feasibility and long-term safety of a multichannel VCI prototype. This design will give insights into vestibular and hearing performance during VCI stimulation. Results will also provide insights into the expected daily benefit of prolonged VCI stimulation, paving the way for cost-effectiveness analyses and a more comprehensive clinical implementation of vestibulocochlear stimulation in the future. ClinicalTrials.gov: NCT04918745. Registered 28 April 2021.

Heimler syndrome with a complaint of blurred vision caused by compound heterozygous variants in PEX1.

Heimler syndrome (HS) is a rare disorder that includes sensorineural hearing loss (SNHL), nail abnormalities, and enamel hypoplasia. Patients with this syndrome can also exhibit ocular manifestations. At present, only a few cases of HS have been reported, existing knowledge of this syndrome is limited, and many cases have been misdiagnosed or even missed. This is the first report of Heimler syndrome with blurred vision as the first complaint, which was diagnosed by genetic analysis in the ophthalmology department. An 8-year-old girl complained of bilateral visual blur and night blindness from birth. Ophthalmic examinations revealed bilateral retinitis pigmentosa with cystoid macular edema, visual impairment with hyperopia and astigmatism. Hearing test revealed bilateral severe sensorineural hearing loss. Dental examinations revealed enamel hypoplasia. In addition, whole-exome sequencing (WES) identified two pathogenic variants in PEX1: the previously reported missense variant c.2966T > C (p.I989 T), and the novel frameshift variant c.1671_1672del (p.G558Sfs*33). Heimler syndrome is caused by compound heterozygous PEX1 pathogenic variants, c.2966T > C (p.I989 T) and c.1671_1672del (p.G558Sfs*33), which contributed to the diversity of clinical and genetic profiles in this patient. The main clinical manifestations include bilateral retinitis pigmentosa with cystoid macular edema, sensorineural hearing loss, and enamel hypoplasia. Systemic examinations are suggested for patients suspected of having pigmentary retinal dystrophy, especially combined with hearing-related impairments. Genetic testing can help us to make a definitive diagnosis.

Auditory brainstem implantation: surgical experience and audiometric outcomes in the pediatric population.

Pediatric data regarding treatment via an auditory brainstem implant (ABI) remains sparse. The authors aimed to describe their experience at their institution and to delineate associated demographic data, audiometric outcomes, and surgical parameters. An IRB-approved, retrospective chart review was conducted among the authors' pediatric patients who had undergone auditory brainstem implantation between 2012 and 2021. Demographic information including sex, age, race, coexisting syndrome(s), history of cochlear implant placement, average duration of implant use, and follow-up outcomes were collected. Surgical parameters collected included approach, intraoperative findings, number of electrodes activated, and complications. A total of 19 pediatric patients had an ABI placed at the authors' institution, with a mean age at surgery of 4.7 years (range 1.5-17.8 years). A total of 17 patients (89.5%) had bilateral cochlear nerve aplasia/dysplasia, 1 (5.3%) had unilateral cochlear nerve aplasia/dysplasia, and 1 (5.3%) had a hypoplastic cochlea with ossification. A total of 11 patients (57.9%) had a history of cochlear implants that were ineffective and required removal. The mean length of implant use was 5.31 years (0.25-10 years). Two patients (10.5%) experienced CSF-related complications requiring further surgical intervention. The most recent audiometric outcomes demonstrated that 15 patients (78.9%) showed improvement in their hearing ability: 5 with sound/speech awareness, 5 able to discriminate among speech and environmental sounds, and 5 able to understand common phrases/conversation without lip reading. Nine patients (47.4%) are in a school for the deaf and 7 (36.8%) are in a mainstream school with support. The authors' surgical experience with a multidisciplinary team demonstrates that the retrosigmoid approach for ABI placement in children with inner ear pathologies and severe sensorineural hearing loss is a safe and effective treatment modality. Audiometric outcome data showed that nearly 79% of these patients had an improvement in their environmental and speech awareness. Further multicenter collaborations are necessary to improve these outcomes and potentially standardize/enhance electrode placement.

PKHD1L1, a gene involved in the stereocilia coat, causes autosomal recessive nonsyndromic hearing loss

Identification of genes associated with nonsyndromic hearing loss is a crucial endeavor given the substantial number of individuals who remain without a diagnosis after even the most advanced genetic testing. PKHD1L1 was established as necessary for the formation of the cochlear hair-cell stereociliary coat and causes hearing loss in mice and zebrafish when mutated. We sought to determine if biallelic variants in PKHD1L1 also cause hearing loss in humans. Exome sequencing was performed on DNA of four families segregating autosomal recessive nonsyndromic sensorineural hearing loss. Compound heterozygous p.[(Gly129Ser)];p.[(Gly1314Val)] and p.[(Gly605Arg)];p[(Leu2818TyrfsTer5)], homozygous missense p.(His2479Gln) and nonsense p.(Arg3381Ter) variants were identified in PKHD1L1 that were predicted to be damaging using in silico pathogenicity prediction methods. In vitro functional analysis of two missense variants was performed using purified recombinant PKHD1L1 protein fragments. We then evaluated protein thermodynamic stability with and without the missense variants found in one of the families and performed a minigene splicing assay for another variant. In silico molecular modeling using AlphaFold2 and protein sequence alignment analysis were carried out to further explore potential variant effects on structure. In vitro functional assessment indicated that both engineered PKHD1L1 p.(Gly129Ser) and p.(Gly1314Val) mutant constructs significantly reduced the folding and structural stabilities of the expressed protein fragments, providing further evidence to support pathogenicity of these variants. Minigene assay of the c.1813G>A p.(Gly605Arg) variant, located at the boundary of exon 17, revealed exon skipping leading to an in-frame deletion of 48 amino acids. In silico molecular modeling exposed key structural features that might suggest PKHD1L1 protein destabilization. Multiple lines of evidence collectively associate PKHD1L1 with nonsyndromic mild–moderate to severe sensorineural hearing loss. PKHD1L1 testing in individuals with mild–moderate hearing loss may identify further affected families.

Relationship of Serum Prestin Levels to the Severity of Sensorineural Hearing Loss.

Prestin is an outer hair cell (OHC) protein responsible for increasing cochlear sensitivity and has been proposed as a biomarker.We aimed to evaluate whether the serum prestin level is related to the severity of chronic sensorineural hearing loss (SNHL). Ninetysubjects were recruited from the patient base at Samarra public hospitals and clinics in Iraq from January to October of 2022. They were divided into three groups equally: agroup of healthy people without hearing loss (G0), a group with moderate SNHL (G1), and a group with severe SNHL (G2). The subjects ranged from 20 to 80 years of age and included 51 males and 39 females. Blood samples were collected, then serum was separated, and enzyme-linked immunosorbent assays were performed to quantify the levels of prestin. Hearing thresholds were sequentially statistically higher across the three groups. While prestin levels were significantly higher in G1 and G2 than that in G0, there were no differences between the G1 and G2 levels. Serum prestin levels were positively correlated with hearing thresholds in G1, but not G2. Our results suggest that in the clinical setting, prestin is sensitive to chronic mild to moderate SNHL (i.e., up to 40-60 dB), not more severe loss.This range is consistent with the added sensitivity provided by OHCs in the cochlea and provides support for prestin as a biomarker of OHC-mediated SNHL.

Recurrent missense variant identified in two unrelated families with MPZL2-related hearing loss, expanding the variant spectrum associated with DFNB111.

MPZL2-related hearing loss is a rare form of autosomal recessive hearing loss characterized by progressive, mild sloping to severe sensorineural hearing loss. Thirty-five previously reported patients had biallelic truncating variants in MPZL2, with the exception of one patient with a missense variant of uncertain significance and a truncating variant. Here, we describe the clinical characteristics and genotypes of five patients from four families with confirmed MPZL2-related hearing loss. A rare missense likely pathogenic variant [NM_005797.4(MPZL2):c.280C>T,p.(Arg94Trp)] located in exon 3 was confirmed to be in trans with a recurrent pathogenic truncating variant that segregated with hearing loss in three of the patients from two unrelated families. This is the first recurrent likely pathogenic missense variant identified in MPZL2. Apparently milder or later-onset hearing loss associated with rare missense variants in MPZL2 indicates that some missense variants in this gene may cause a milder phenotype than that resulting from homozygous or compound heterozygous truncating variants. This study, along with the identification of truncating loss of function and missense MPZL2 variants in several diverse populations, suggests that MPZL2-related hearing loss may be more common than previously appreciated and demonstrates the need for MPZL2 inclusion in hearing loss testing panels.

Cochlear Implantation in Inner Ear Malformations

Introduction: Cochlear Implantation (CI) has proven to be an effective treatment for severe bilateral Sensorineural Hearing Loss (SNHL). Inner ear malformation is a rare anomaly and occurs in approximately 20% of cases with congenital SNHL. There are particular challenges in the implantation of malformed cochleae, such as in cases of facial nerve anomalies, Cerebrospinal Fluid (CSF) leaks or facial stimulation, and the outcomes may differ depending on the severity of the malformation. The aim of this study was to assess the impact of Inner Ear Malformations (IEMs) on surgical complications and outcomes of cochlear implantation. Methods: Between 2018 and 2022, 9 patients with inner ear malformations were implanted in our department and completed at least 1 year of follow-up. The age range was between 2 years and 4 months and 5 years (average, 3.36 yr). Auditory performance, receptive and expressive language skills, and production and use of speech were evaluated preoperatively and at least 1 year after implantation. Results: In the study group, the most common malformation was an isolated Enlarged Vestibular Aqueduct (EVA) (55,5%). Overall, the patients with IEMs showed significant improvement in auditory-verbal skills. In general, the patients who had normal cochleae scored significantly better compared to patients with IEMs. Conclusion: Based on these findings, cochlear implantation is surgically feasible in patients with common cavity, IP types I and II, and EVA. The surgeon should be ready to make modifications in the surgical approach because of the abnormal course of the facial nerve and be ready to produce special precautions to cerebrospinal fluid gusher. Patients with EVA were the best performers in terms of auditory-verbal skills.

A Fused Deep Denoising Sound Coding Strategy for Bilateral Cochlear Implants.

Cochlear implants (CIs) provide a solution for individuals with severe sensorineural hearing loss to regain their hearing abilities. When someone experiences this form of hearing impairment in both ears, they may be equipped with two separate CI devices, which will typically further improve the CI benefits. This spatial hearing is particularly crucial when tackling the challenge of understanding speech in noisy environments, a common issue CI users face. Currently, extensive research is dedicated to developing algorithms that can autonomously filter out undesired background noises from desired speech signals. At present, some research focuses on achieving end-to-end denoising, either as an integral component of the initial CI signal processing or by fully integrating the denoising process into the CI sound coding strategy. This work is presented in the context of bilateral CI (BiCI) systems, where we propose a deep-learning-based bilateral speech enhancement model that shares information between both hearing sides. Specifically, we connect two monaural end-to-end deep denoising sound coding techniques through intermediary latent fusion layers. These layers amalgamate the latent representations generated by these techniques by multiplying them together, resulting in an enhanced ability to reduce noise and improve learning generalization. The objective instrumental results demonstrate that the proposed fused BiCI sound coding strategy achieves higher interaural coherence, superior noise reduction, and enhanced predicted speech intelligibility scores compared to the baseline methods. Furthermore, our speech-in-noise intelligibility results in BiCI users reveal that the deep denoising sound coding strategy can attain scores similar to those achieved in quiet conditions.

Treatment of Meniere's disease with simultaneous triple semicircular canal occlusion and cochlear implantation.

Report three cases of simultaneous triple semicircular canal occlusion (TSCO) and cochlear implantation (CI) as the treatment of intractable Meniere's disease (MD). Patients with MD can present occasionally with intractable vertigo and profound sensorineural hearing loss (SNHL). TSCO and CI have been proposed to control vertigo and restore profound deafness in patients with MD separately. However, a few studies have reported simultaneous TSCO and CI in the same surgical procedure for the treatment of MD. In the present study, we described three patients with MD showing incapacitating vertigo and severe SNHL who underwent simultaneous TSCO and CI after examinations of auditory system, vestibular system, and imaging. Their symptoms were significantly alleviated during the follow-up period. The combined TSCO and CI remains a viable treatment option which is effective for the control of vertigo as well as the restoring of hearing in patients with MD.

A Descriptive Study on Imperiled Cochlear Implant Salvage Using Double Flap Cover-What We Learned in 6 Years.

Cochlear implant surgery is the standard of care for severe sensorineural hearing loss. Infection followed by implant extrusion is an infrequent complication of this surgery. The ideal treatment is explantation of the implant. However, implant removal and reimplantation is a challenging surgery and may have poor speech reception outcomes. The cost of a new implant especially in developing countries is also a deterrent. Our study dwells on the feasibility of salvaging exposed cochlear implants by a combination of pericranial flaps followed by a scalp flap cover. The study was done in a tertiary care hospital over a period of six years. Out of 303 cochlear implant surgeries, 12 patients had implant exposure and extrusion. Patients having meningitis and sepsis were excluded from the study. All patients underwent debridement and cover with double flap (Pericranial flaps and scalp rotation flap). The average operating time was 2.17h. The surgery is technically simple with a short learning curve. It brings in rich blood supply and there is fair amount of tissue mobility. In 11 patients we were able to salvage the implant. Patients were followed for a period of 01year post operatively. Our study suggests that salvage of infected implant should be attempted as it is feasible, durable and effective in appropriate patients.

Efficacy of intratympanic steroid therapy for severe and profound sudden sensorineural hearing loss

BackgroundIntratympanic steroids (ITS) in treating sudden sensorineural hearing loss (SSNHL) have become more widespread. AimThis study investigates whether ITS treatment provides additional benefits when combined with systemic steroids (SS) in patients with severe and profound SSNHL. Materials and methodsPatients diagnosed with severe and profound SSNHL were divided into two groups: SS group and SS combined with ITS group. Pure-tone audiometry was performed before and three months after treatment completion. The pure-tone average (PTA), frequency-specific hearing gains, and average values were compared between the groups. ResultsThe study included 57 patients (27 SS, 30 ITS), with a mean age of 50.09 ± 15.56. Before treatment: SS PTA 84.40 ± 15 dB HL, ITS 87.50 ± 9.38 dB HL (p = 0.36). After treatment: SS 62.2 ± 23.13 dB HL, ITS 65.17 ± 12.19 dB HL (p = 0.55). Average hearing gain: SS 22.19 ± 13.81 dB HL, ITS 22.33 ± 12.24 dB HL (p = 0.96). Frequency-specific gains were similar (p > 0.05). SS group: 12 slight improvement, 10 no improvement, 3 partial, 2 complete recovery. ITS group: 23 slight improvement, 6 no improvement, 1 partial. ConclusionIn our study, combining ITS with SS treatment did not provide additional benefits in treating severe and profound SSNHL.

Outcomes of a targeted congenital cytomegalovirus infection screening approach among infants born ≥35 weeks gestation

Cytomegalovirus (CMV) is a common cause of congenital viral infection. The estimated incidence of congenital CMV (CCMV) is about 1–6% worldwide, although reliable estimates from Singapore and surrounding countries are unavailable.1,2 Previous local serologic studies among pregnant women reported high rates of CMV seropositivity (&gt;80%),3 highlighting the possible high burden of CCMV. With the establishment of oral valganciclovir as an effective treatment for infants with CCMV, prompt diagnosis and treatment of infants with symptomatic disease is important to reduce the risk of moderate to severe sensorineural hearing loss (SNHL) and neurodevelopmental impairment.4 We report the findings of a targeted CCMV screening approach using failed newborn hearing screen (NHS) or small-for-gestational-age (SGA) status as triggers for screening among infants born ≥35 weeks gestation.

Test-retest reliability of the urban outdoor situated phoneme (SiP) test*

Objective To introduce the urban outdoor version of the Situated Phoneme (SiP) test and investigate its test-retest reliability. Design Phonemic discrimination scores in matched-spectrum real-world (MSRW) maskers from an urban outdoor environment were measured using a three-alternative forced choice test paradigm at different phoneme-to-noise ratios (PNR). Each measurement was repeated twice. Test-retest scores for the full 84-trial SiP-test, as well as for four types of contrasting phonemes, were analysed and compared to critical difference scores based on binomial confidence intervals. Study sample Seventy-two adult native speakers of Swedish (26–83 years) with symmetric hearing threshold levels ranging from normal hearing to severe sensorineural hearing loss. Results Test-retest scores did not differ significantly for the whole test, or for the subtests analysed. A lower amount of test-retest score difference than expected exceeded the bounds of the corresponding critical difference intervals. Conclusions The urban outdoor SiP-test has high test-retest reliability. This information can help audiologists to interpret test scores attained with the urban outdoor SiP-test.

Evaluation of Hearing Status in Various Type of Cerebral Palsy Children in a Tertiary Hospital

Background: Cerebral palsy (CP) in children are frequently associated with sensorineural pathology.&#x0D; Objectives: To assess the severity of hearing impairment in children with CP and correlate the auditory impairment with different types of CP.&#x0D; Materials &amp; Methods: This cross-sectional study was conducted in the Department of Pediatric neurology and Autolaryngiology- Head and Neck Surgery BSMMU, during March to September 2011. Diagnosed CP patients were included consecutively. After taking informed written consent, proper history and clinical examinations was done and severity of hearing loss was assessed.&#x0D; Results: Among the 50 children with CP, Tympanometry result revealed that negative middle ear pressure was present in 32% in spastic quadriplegia(SQ), 24% in spastic hemiplegia (SH), 16% in spastic diplegia (SD) and 24% in mixed type. Eustachian tube dysfunction was present in 62.5% in spastic hemiplegia. In TOAE result 52% were pass and 48% were referred and ABR were suggested. On the basis of result of TOAE, those were referred, initially they were encountered as hearing impairment in this study. Among them, in SQ were 18%, SH were 12% and mixed. Follow up auditory brain stem response revealed mild, moderate and severe sensorineural hearing loss were 8% in spastic quadriplegic type and the spastic diplegia and athetoid group were 2% only mild, Moderate sensorineural hearing were 2% in spastic hemiplegia group.&#x0D; Conclusion: Spastic Quadriplegic cerebral palsy patients had the maximum incidence of hearing impairment. Perinatal asphyxia and prematurity were the main risk factor of hearing impairment.&#x0D; BANGLADESH J CHILD HEALTH 2022; VOL 46 (1) : 19-22

Characteristics of vestibular-evoked myogenic potentials in children with vestibular malformation and severe sensorineural hearing loss

BackgroundWith the increasing clinical focus on the safety of bilateral cochlear implantation (CI) and the potential risk of bilateral vestibular dysfunction, evaluating vestibular end-organ function in patients with vestibular malformations with accompanying abnormalities has been strongly recommended. ObjectivesTo identify the vestibular-evoked myogenic potential (VEMP) values among children with sensorineural hearing loss (SNHL) with vestibular malformation and assess the effectiveness of VEMP testing for inner ear malformations (IEM) diagnosis. MethodsThis study included 96 children (192 ears), including those with vestibular malformations (48 ears), large vestibular aqueduct syndrome (LVAS) (50 ears), and SNHL without IEM (94 ears; control group). All groups underwent ocular and cervical VEMP (oVEMP and cVEMP, respectively) testing. The response rates, VEMP parameters, and wave characteristics were compared. ResultsThe cVEMP response rates were 37.5 %, 64 %, and 58.51 % and the oVEMP response rates were 42.86 %, 78.95 %, and 77.27 % in the vestibular malformation, LVAS, and control groups, respectively, and significantly differed between groups (cVEMP: X2 = 18.228, P<0.001) (oVEMP: X2 = 7.528, P = 0.023). Significant inter-group differences were observed for the cVEMP and oVEMP latency and amplitude (P < 0.05). The LVAS group's waveform exhibited a prolonged latency and increased amplitude compared with that of the other groups. ConclusionPatients with SNHL were highly susceptible to otolith dysfunction, regardless of comorbid vestibular malformation presence. Measuring VEMPs is an effective and rapid evaluation technique for vestibular function and could provide a basis for vestibular rehabilitation training.

Digital Health Literacy: Evaluating the Readability and Reliability of Cochlear Implant Patient Information on the Web.

Hearing aids and implants are used to treat hearing loss, with cochlear implants being the most successful option for severe sensorineural hearing loss. Patients frequently use the internet as a trusted source of clinical information before committing to any therapeutic procedure, including receiving a cochlear implant. A health resource's readability and dependability influence its value to patients. Readability refers to how easily language can be understood, whereas reliability refers to the correctness and consistency of the information presented. JAMA standards and the DISCERN tool were used to assess the reliability of the websites listed. For readability analysis, the FRE, FKG and GFI were chosen. The acceptable readability level was set to < 7 for the FKG, GF score over 17 as the equivalent of college-level education and ≥ 80.0 for the FRE. The readability scores vary across the sources, suggesting a range of comprehension levels required for understanding the cochlear implant patient information found on Google. There was a statistical difference detected in Discern score between the groups (p = 0.008). The mean discern score was significantly higher in hospital generated sources when compared to industry (3.13±0.69 vs. 2.11±0.78, p = 0.03).

AUDITORY ACUITY IN DIABETES MELLITUS TYPE II: A CASE–CONTROL STUDY

Objectives: The objective of the study was to analyze the status of auditory acuity in patients with Type II diabetes mellitus (DM) as compared to healthy individuals of comparable age groups using pure tone audiometry. Methods: This was a case–control study in which 80 known cases of DM were enrolled as cases (Group D) and a similar number of age-matched healthy individuals were included as the control group (Group N). Demographic details such as age and gender were compared. A detailed history was taken and a general and systemic examination was done. Pure tone audiometry (250Hz, 500Hz, 1000Hz, 2000Hz, 4000Hz, 6000Hz, and 8000 Hertz frequencies) was done in all the patients, and air conduction and bone conduction of both the ears were determined. SSPE 21 software was used for statistical analysis. p&lt;0.05 was considered to be statistically significant. Results: Both groups were found to be comparable in terms of gender and age distribution with no statistically significant difference (p&lt;0.05). Mean fasting and postprandial blood sugar levels as well as HbA1c were found to be higher in Group D as compared to Group N and the difference was highly significant (p&lt;0.0001). In Group D (Diabetic patients), mild, moderate, and severe sensorineural hearing loss (SNHL) at speech frequency was seen in 31 (38.75%) whereas moderate and severe SNHL was seen in 22 (27.50%) and 18 (22.50%) patients. In Group N (Non-diabetic), 5 (6.25%) patients had mild SNHL and moderate and severe hearing loss was not seen in any of the patients. The mean hearing threshold (Both Ears) for bone as well as air conduction was found to be more in Group D as compared to Group N at all frequencies and the difference was statistically significant (p&lt;0.05). Conclusion: Individuals with Type II DM are found to have an increased incidence of subclinical hearing loss. This subtle hearing loss may go undetected for a considerable period of time and hence regular audiometric evaluation is required for early diagnosis of subclinical hearing loss in patients of DM.