Significant Hearing Loss Research Articles

Aging effects on neural processing of rhythm and meter.

When listening to musical rhythm, humans can perceive and move to beat-like metrical pulses. Recently, it has been hypothesized that meter perception is related to brain activity responding to the acoustic fluctuation of the rhythmic input, with selective enhancement of the brain response elicited at meter-related frequencies. In the current study, electroencephalography (EEG) was recorded while younger (<35) and older (>60) adults listened to rhythmic patterns presented at two different tempi while intermittently performing a tapping task. Despite significant hearing loss compared to younger adults, older adults showed preserved brain activity to the rhythms. However, age effects were observed in the distribution of amplitude across frequencies. Specifically, in contrast with younger adults, older adults showed relatively larger amplitude at the frequency corresponding to the rate of individual events making up the rhythms as compared to lower meter-related frequencies. This difference is compatible with larger N1-P2 potentials as generally observed in older adults in response to acoustic onsets, irrespective of meter perception. These larger low-level responses to sounds have been linked to processes by which age-related hearing loss would be compensated by cortical sensory mechanisms. Importantly, this low-level effect would be associated here with relatively reduced neural activity at lower frequencies corresponding to higher-level metrical grouping of the acoustic events, as compared to younger adults.

Extended high-frequency audiometric analyses of tinnitus patients exhibiting normal hearing on conventional pure-tone audiometry

Background While hearing loss is the greatest risk factor associated with developing tinnitus, some tinnitus patients exhibit no hearing loss on conventional pure-tone audiometry (PTA). Objectives This study was developed to assess whether tinnitus patients with normal hearing as measured via conventional PTA would exhibit differences from normal controls upon extended high-frequency (EHF) audiometric evaluation. Methods In total, 102 tinnitus patients were separated into unilateral and bilateral tinnitus groups. Age- and sex-matched controls without tinnitus were enrolled. Results No significant differences were observed when comparing EHF audiometry results in the 9–14 kHz range between controls and tinnitus patients, with only left-sided tinnitus ears exhibiting higher hearing thresholds than contralateral ears at 9, 10, and 14 kHz. Relative to normal controls, the hearing thresholds in the 2–8 kHz range for tinnitus ears were significantly increased. Conclusions and significance Relative to controls, tinnitus patients with normal hearing did not exhibit any significant hearing loss in the EHF range. Unexpectedly, tinnitus patients with normal hearing exhibited significant hidden hearing loss in the conventional frequency range rather than in the EHF range. For patients with normal hearing, it appears to be unnecessary to conduct EHF examinations to detect hearing loss in the EHF range.

How to Improve Audiology Services: The Patient Perspective

How to Improve Audiology Services: The Patient Perspective

Therapeutic effect of NLRP3 inhibition on hearing loss induced by systemic inflammation in a CAPS-associated mouse model.

SummaryBackgroundCryopyrin-associated periodic syndrome (CAPS) is an inherited autoinflammatory disease caused by a gain-of-function mutation in NLRP3. Although CAPS patients frequently suffer from sensorineural hearing loss, it remains unclear whether CAPS-associated mutation in NLRP3 is associated with the progression of hearing loss.MethodsWe generated a mice with conditional expression of CAPS-associated NLRP3 mutant (D301N) in cochlea-resident CX3CR1 macrophages and examined the susceptibility of CAPS mice to inflammation-mediated hearing loss in a local and systemic inflammation context.FindingsUpon lipopolysaccharide (LPS) injection into middle ear cavity, NLRP3 mutant mice exhibited severe cochlear inflammation, inflammasome activation and hearing loss. However, this middle ear injection model induced a considerable hearing loss in control mice and inevitably caused an inflammation-independent hearing loss possibly due to ear tissue damages by injection procedure. Subsequently, we optimized a systemic LPS injection model, which induced a significant hearing loss in NLRP3 mutant mice but not in control mice. Peripheral inflammation induced by a repetitive low dose of LPS injection caused a blood-labyrinth barrier disruption, macrophage infiltration into cochlea and cochlear inflammasome activation in an NLRP3-dependent manner. Interestingly, both cochlea-infiltrating and -resident macrophages contribute to peripheral inflammation-mediated hearing loss of CAPS mice. Furthermore, NLRP3-specific inhibitor, MCC950, as well as an interleukin-1 receptor antagonist significantly alleviated systemic LPS-induced hearing loss and inflammatory phenotypes in NLRP3 mutant mice.InterpretationOur findings reveal that CAPS-associated NLRP3 mutation is critical for peripheral inflammation-induced hearing loss in our CAPS mice model, and an NLRP3-specific inhibitor can be used to treat inflammation-mediated sensorineural hearing loss.FundingNational Research Foundation of Korea Grant funded by the Korean Government and the Team Science Award of Yonsei University College of Medicine.

Magnetic Targeting of Gadolinium Contrast to Enhance MRI of the Inner Ear in Endolymphatic Hydrops.

1. Determine the feasibility and efficiency of local magnetic targeting delivery of gadolinium (Gad) contrast to the inner ear in rodents. 2. Assess any potential ototoxicity of magnetic targeting delivery of Gad in the inner ear. 3. Study the utility of magnetic targeting delivery of Gad to visualize and quantify endolymphatic hydrops (EH) in a transgenic mouse model. Controlled in vivo animal model study. Paramagnetic Gad was locally delivered to the inner ear using the magnetic targeting technique in both rat and mouse models. Efficiency of contrast delivery was assessed using magnetic resonance imaging (MRI). Ototoxicity of Gad was examined with histology of the cochlea and functional audiological tests. The Phex mouse model was used to study EH, hearing loss, and balance dysfunction. Magnetic targeting delivery of Gad contrast was used in the Phex mouse model to visualize the effects of EH using MRI. Magnetic targeting improved the delivery of Gad to the inner ear and the technique was reproducible in both rat and mouse models. The delivery method did not result in microstructural damage or any significant hearing loss in a normal animal. Magnetic targeting of Gad in the Phex mouse model allowed detailed visualization and quantification of EH. This study provided the first evidence of the effectiveness and efficiency of the local magnetic targeting delivery of gadolinium contrast to the inner ear and its application to the visualization and quantification of EH. Laryngoscope, 133:914-923, 2023.

OTHR-03. Oxaliplatin as a hearing-sparing alternative to carboplatin in tandem autologous stem cell transplants in pediatric CNS malignancy

Abstract BACKGROUND: Intensive chemotherapy with tandem autologous stem cell transplants (autoSCT) is shown to improve survival for children with CNS malignancy. Platinum-based chemotherapeutic agents in these regimens, mainly cisplatin and carboplatin, have resulted in significant sensorineural hearing loss. Oxaliplatin, a newer platinum-based agent, is considered less ototoxic. Empiric substitution of oxaliplatin for carboplatin in preparative regimens for autoSCT have been tried. However, the survival and ototoxicity outcomes have not been studied. OBJECTIVE: To compare the overall survival and ototoxicity of oxaliplatin versus carboplatin preparatory chemotherapy regimens in children who received tandem autoSCT for treatment of CNS malignancy. METHODS: We performed a retrospective chart review of all pediatric patients with primary CNS tumors who received tandem autoSCT from 2011 to 2018 at Children’s Hospital Colorado. Demographics, clinical outcomes, and medication administration records were extracted from electronic medical records. Hearing evaluations, performed at pre-transplant, after each transplant episode, and at follow-up visits, were reviewed and graded by an audiologist. Comparisons were performed using Fisher’s exact tests and log rank test statistics. RESULTS: 32 pediatric patients with CNS tumors met inclusion criteria. Seven patients received oxaliplatin in place of carboplatin in one or more preparatory regimens. There was no statistically significant difference in overall survival between the two groups (p=0.99). A total of 85 follow-up audiograms were available for assessment, including long-term follow up. Of the 13 audiograms that showed hearing loss, one (8%) had prior oxaliplatin exposure, compared to 18/72 (25%) audiograms without hearing loss had prior oxaliplatin exposure (p=0.28). CONCLUSIONS: Oxaliplatin is effective and well-tolerated when used in lieu of carboplatin in preparatory regimen for autoSCT for pediatric CNS malignancy. This study is limited by its small size. A larger, multi-center study is warranted to confirm oxaliplatin’s safety and effect on survival and ototoxicity in pediatric autoHSCT.

RELATIONSHIP OF THE SIZE AND LOCATION OF THE TYMPANIC MEMBRANE PERFORATION IN CHRONIC SUPPURATIVE OTITIS MEDIA WITH THE MAGNITUDE OF HEARING LOSS

Chronic suppurative otitis media is a severe health condition that affects people worldwide. Perforation in the tympanic membrane reduces the surface area of the membrane available for sound transmission. It’s important to identify and treat tympanic membrane perforation as soon as possible, since untreated tympanic membrane perforation contributes to chronic disruptive changes in the middle ear, resulting in further hearing loss, which is a major physical and psychological issue that affects people’s lives. The study has been conducted to compare the relationship between the location and size of perforation of the tympanic membrane in chronic suppurative otitis media with the magnitude of hearing loss. The cross-sectional prospective study was conducted among 67 patients who visited the Otorhinolaryngology department for myringoplasty. The study period was from January to April 2021, the age group of patients was between 8 and 43 years old. The number of female participants was 35 (52.2%) and males – 32(47.8%) respectively. Average age ±SD was 21.93±9.47 years. Fifty (74.6%) patients had right ear perforation and seventeen (25.4%) with left ear perforation/ It was not statistically significant (χ2=22.891; p-value=0.290). Most of the patients were with subtotal of (all quadrants) 30 (44.77%), 2-3 quadrants, and one quadrant perforation were 23 (34.32%) and 14 (20.89%) respectively. Most of the patients, i.e.37 (55.22%), had hearing loss between 26-40dB. There was a significant connection between the perforation size and degree of hearing, (χ2=22.891; p-value &lt;0.0001). In one quadrant perforation, greatest hearing loss was on the post inferior quadrant 29.97±4.32 dB. For 2-3 quadrants perforation the hearing loss was greatest inpostero-superior combined with postero-inferior perforation 37.42±9,59dB. The most significant hearing loss was in subtotal perforation mean ±SD was 42.13±7.07dB. Hearing loss increases in proportion to the extent of the perforation, post inferior quadrant alone and combined with the post-super were observed with the greatest hearing loss according to the findings of this study.

Screening detects clinically significant hearing loss in patients with GU malignancies.

e18624 Background: Four in 10 people aged 60-69 have hearing loss in frequencies that mediate speech comprehension. Untreated losses undermine medication adherence, increase risk for accidental injury, and generate higher total healthcare expenditures. Genitourinary (GU) malignancies are most frequently diagnosed in people over 60, and these patients are commonly exposed to ototoxic treatments like platinum-based chemotherapy. We hypothesized that a significant number of GU patients would fail hearing screenings at levels reflecting clinically significant hearing loss. Methods: This is a prospective, two-cohort pilot study conducted at Tulane Medical Center. Consecutive patients with active GU malignancies who did not use hearing aids were enrolled in cohort A. The primary objective of cohort A (screening) was to determine the prevalence of hearing loss among GU patients. Cohort B (interventional) will investigate the efficacy of a non-custom amplifier in improving communication for patients with hearing loss. The hearing screening consisted of a 9-item Self-Assessment of Communication (SAC) and a 25 decibel (dB) pure tone hearing screening at 1 kHz, 2 kHz, 4 kHz via an Earscan3 portable audiometer with headphones in a quiet exam room. The primary endpoint was the proportion of patients who failed the hearing screening (missed ≥ 2/6 tones). The SAC generates a global handicap score (none, slight, mild-to-moderate, severe). Surveys identifying any amount of handicap were considered positive. Patients who failed screenings were offered referrals to audiology. Here, we present results from cohort A. Results: From 8/21 to 1/22, 66 patients were invited to participate. Nine patients screen-failed (n = 8 hearing aids; n = 1 uninterested). Cohort A enrolled 57 patients [median age 68 (33-86); 91% men; 60% prostate, 24% kidney and 14% bladder cancer]. Most patients (82%) had evidence of metastatic disease and 12%, 19%, 36%, 21% of patients were receiving chemotherapy, immunotherapy, hormonal therapy, and no active therapy, respectively. Twenty (35%) patients had prior exposure to neurotoxic chemotherapy. Forty patients (70%) met the primary endpoint. Compared to the pure tone screening, the SAC had a specificity of 94%, and a sensitivity of 40%. The SAC asks patients for a situation where they want to hear better; 61% of patients who volunteered a scenario (17/28) identified a complex listening environment like an outpatient encounter. A minority of patients (n = 3) accepted audiology referrals after failing screenings. Cohort B is actively enrolling. Conclusions: Clinically significant hearing loss was prevalent among patients with GU malignancies. Patients reported struggling in listening situations with auditory demands like outpatient encounters but were unable to self-identify losses and did not pursue audiology referrals after failed screenings. Interventional studies addressing this unmet need are warranted.

Hearing Loss After Cisplatin-based Chemoradiotherapy for Locally Advanced Head and Neck Cancer: A Prospective Single-institution Study.

A single-institution prospective study was conducted to evaluate hearing loss rate after intensity modulated radiotherapy with concomitant cisplatin-based chemotherapy (CRT) for locally advanced head and neck cancer and identify cochlear dosimetric parameters associated with hearing loss risk. Hearing assessment, patients' characteristics, tumor-related variables, and cochlear quantitative dosimetric factors for adults with locally advanced head and neck cancer treated with CRT were prospectively collected. Each patient repeated audiometry at baseline (pre-CRT), 1 month after CRT, and then every 3 to 6 months. For each cochlea minimum dose (Dmin), mean dose (Dmean), and maximum dose (Dmax) were extracted from treatment plans. Logistic analysis was used for multivariate modeling. The relation between cochlear dosimetric factors and significant hearing loss was also analyzed with receiver operating characteristic (ROC) curves. Between January 2016 and December 2018, 35 patients (70 cochleae) were included. Most patients (n=29; 82.9%) had primary cancer in a low-risk region (oral cavity, oropharynx, larynx). All patients completed the programmed CRT. During follow-up, significant hearing loss was recorded in 13 cases (37.1%). The ROC areas for significant hearing loss in relation to Dmin, Dmean, and Dmax were 0.70, 0.66, and 0.66, respectively. A dose-dependent relationship was noted between cochlear Dmin and significant hearing loss. Dmin <14.4 Gy is associated with reduced rates of significant hearing loss after concomitant cisplatin-based CRT in patients with locally advanced head and neck cancer.

Otitis Media and Its Association With Hearing Loss in Chinese Adults: A Population Based Study of 4 Provinces in China.

ObjectiveOtitis media is a recognized cause of significant hearing loss, particularly in developing countries. This study aimed to investigate the relationship between otitis media and hearing loss in Chinese adults aged 18 years and older.MethodsThe survey was based on WHO Ear and Hearing Disorders Survey Protocol and 36,783 adults at the ages between 18 years and above were selected in this study. Trained local examiners performed pure tone audiometry to screen people with hearing loss, and those who were screened positively for hearing loss were referred to audiologists to make final diagnosis. All participants underwent clinical ENT check-up and otoscopic examination by doctors trained in ENT. Each participant was assigned a single middle ear diagnosis. Diagnoses were assigned as per the WHO classification of ear and hearing disorders.ResultsLogistic regressions showed that higher prevalence of hearing loss was found in participants with otitis media, with an unadjusted odds ratio of 5.67 (95%CI: 4.66, 6.90). The next two models (Model 2–3) had slight impact on ORs. The interaction of residency and otitis media was statistically significant (OR = 1.70, 95%CI = 1.15, 2.53); otitis media patients in rural areas had higher risk of hearing loss. However, this interaction became not significant in 65 years old and above participants.ConclusionsOtitis media was associated with the risk of hearing loss. Compared with urban patients with otitis media, rural patients have the higher risk of hearing loss. Action to reduce the risk of hearing loss in otitis media will require attention to rural-urban disparities.

Personalized prediction of the results of reconstructive surgery for chronic otitis media

Introduction. The goal of the surgical treatment of patients with chronic otitis media is to eliminate the consequences of the inflammatory and destructive disease while preserving the anatomy of the middle ear and improving auditory function. According to the literature, the outcome of middle ear reconstructive surgery for the surgeon and the patient in most cases remains unpredictable. The development of an integrated approach to predicting the results of reconstructive sanitizing surgery will make it possible to select the most effective surgical tactics in terms of treatment time and type of surgical intervention.Aim.To develop a methodology for personalized prediction of the result of operations on the temporal bone in patients with chronic otitis media and substantiate the choice of optimal surgical tactics, taking into account clinical and functional parameters.Materials and methods.A retrospective study was carried out to compare the data of patients with the use of the author’s personalized prognosis method: “A method for predicting the result of tympanoplasty” and “A method for predicting the degree of hearing loss in otosurgery for chronic otitis media” with the results of surgical treatment of 263 patients with chronic otitis media, operated on in hospitals in Moscow. Chelyabinsk in 2018–2020.Results and discussion. In the observation, the patients were divided into two groups according to the sum of points, which determined the volume, type and timing of the operation. The sum of points less than or equal to 20 was assessed as a favorable prognosis for reconstructive debridement surgery. The sum of points in the range from 21–40 was assessed as the possibility of carrying out onestage surgery for the purpose of sanitation and reconstruction, but the patient should be informed about the unfavorable prognosis for the restoration of hearing. A score greater than 40 is assessed as an unfavorable prognosis, with an indication for separate-stage surgery, indicating a socially significant hearing loss in the postoperative period in patients with chronic otitis media.Conclusion. A personalized approach to predicting the results of surgical treatment forms groups of patients with chronic suppurative otitis media and helps in choosing surgical tactics in terms of the type, volume and timing of one-stage or separatestage reconstructive surgery and the need for reoperation for a period of one year.

IMPACT OF GENDER AND DEGREE OF HEARING LOSS ON SELF ESTEEM IN CHILDREN AND ADOLESCENTS

Background: Hearing impairment is considered as one of the major contributor of drop in low self-esteem of adolescents. Children and adolescents with significant hearing loss have difficulties like communication and social skills in different aspects of their quality of life which later on lead to anxiety, depression and social isolation. Objective: To find out the impact of gender and degree of hearing loss in children and adolescents not using hearing aid. Methodology: A cross sectional survey was conducted in Khyber Pakhtunkhwa. Total participants were 551 selected between 9-15 years age , with mild and moderate degree of hearing loss with no history of using hearing aid. The Rosenberg self-esteem scale was used to measure the self-esteem level. The data was analysed through multiple linear regression testing SPSS version 21. Results: The mean age of the study participants was 12.38±1.773 year. The degree of hearing loss and the gender were statistically significant predictor {F (df) =157.636(2,548), p&lt;0.001, r2 = .365} of the self-esteem in hearing impaired children. Hence, the age was not significant predictor on the self-esteem (β=.367, p=.228) Conclusion: The self-esteem in the children and adolescents with hearing impairment was affected by gender and degree of hearing loss. Whereas age have no impact on the self-esteem. Keywords: Adolescent, children, Hearing impairment, hearing loss, self-esteem.

Pathophysiological to Clinical Aspects of Chronic Suppurative Otitis Media (CSOM): Narrative Literature Review

Chronic suppurative Otitis Media (CSOM) is inflammation of the middle ear mucosa and mastoid space that lasts more than 2 months characterized by perforation of the tympanic membrane and continuous or intermittent discharge from the ear canal. Secretion in the form of watery or thick clear or pus. CSOM with cholesteatoma is characterized by a perforation that is located marginally or can be tampered with the bone and often causes dangerous complications. CSOM is still a major health problem, especially in developing countries. The incidence of CSOM cases is estimated at more than 20 million people worldwide. Of these, approximately 5 million patients suffer from chronic otitis media with cholesteatoma, although the overall number of cases of chronic otitis media with cholesteatoma appears to be decreasing. The prevalence of CSOM worldwide is around 65-330 million people with complaints of watery ears, 60% of them (39-200 million) suffer from significant hearing loss. The incidence of chronic otitis media with cholesteatoma is 3 in 100,000 in children and 9.2 in 100,000 adults.

Hearing loss in fabry disease: A 16 year follow-up study of the Danish nationwide cohort

BackgroundFabry disease (FD) is a lysosomal storage disorder resulting in systemic accumulation of globotriaosylceramide (Gb3) causing multi-organ dysfunction. The audiologic involvement in FD has been neglected in previous studies; while not a lethal aspect of the disease, hearing loss can have a significantly negative impact on quality of life. ObjectiveTo investigate hearing loss from baseline through 16 years follow-up of the Danish FD cohort and to compare audiometric data to other clinical variables. MethodsData was collected prospectively and assessed retrospectively during a period of 16 years from 83 patients (age: 9–72 years; sex: 29 males and 54 females). 55 patients underwent treatment. Air conduction thresholds was assessed at six frequencies between 0.25 and 8 kHz bilaterally. Data was analyzed using multilinear models. ResultsMean follow-up period for patients undergoing a FD specific treatment was 7.8 years (0–12.8 years, SD 3.8 years, n = 55). Hearing thresholds for FD patients deviated from healthy individuals at all frequencies for both sexes (p < 0.001). Males had more profound hearing loss than females at high frequencies (4,8 kHz) (p = 0.025). There was no improvement in hearing with treatment (p = 0.343♂, p = 0.256♀). No associations between hearing loss and measured glomerular filtration rate, left ventricular wall thickness or cerebral white matter lesions were found. Lower plasma Gb3 concentration correlated with better hearing (p = 0.046) in males. ConclusionOur findings demonstrated significant hearing loss in FD patients compared to audiologically healthy individuals at all frequencies, and no change in hearing during treatment. Lower plasma Gb3 concentrations correlated with better hearing in males.

Extensive hearing loss induced by low‐frequency noise exposure

BackgroundWith little attention given to low‐frequency traffic noise and our understanding that cochlear function may be highly susceptible to low‐frequency noise, there is an urgent need to determine traffic noise‐induced hearing loss (NIHL), not only the hearing loss at low frequency but also the possible high‐frequency hearing loss.MethodsThe current study aims to investigate the potential for extensive hearing loss induced by exposure to 0.063 kHz octave band noise (OBN), which is an important component of low‐frequency traffic noise. The threshold of auditory brainstem response (ABR) was used to evaluate hearing function before and after noise exposure. Chinchillas were randomly assigned into seven different groups. Group 63‐3 h/6 h, Group 2 k‐3 h/6 h, and group 4 k‐3 h/6 h were exposed for either 3 or 6 h to 0.063, 2, and 4 kHz OBN at 90 dB SPL, respectively. The control group was not exposed to noise.ResultsSignificant ABR threshold‐shifts (TS) were observed at 0.88, 2, 4, and 5.7 kHz in Group 63‐6 h, and at 2.8 and 4 kHz in Group 2 k‐6 h, and at 5.7 kHz in Group 4 k‐6 h. ABR‐TS were consistent with outer hair cell (OHC) losses, exposure to 0.063 kHz OBN at 90 dB SPL for 6 h induced large‐scale losses of OHC both in low‐ and high‐frequency region.ConclusionsExposure to 0.063 kHz low‐frequency OBN at 90 dB SPL for 6 h leads to significant hearing loss over an extensive range from low to high frequencies.

Executive Summary of Clinical Practice Guideline on Tympanostomy Tubes in Children (Update).

Executive Summary of Clinical Practice Guideline on Tympanostomy Tubes in Children (Update).

Cochlear implantation in South Africa (part 2)

Cochlear implantation is a timeous and cost-effective solution for severe-to-profound sensorineural hearing loss (SNHL) and has transformed the lives of many individuals with significant hearing loss. The advent of cochlear implantation has meant that, for the first time, one of the senses (hearing), having been entirely lost, can be restored. The previous article in this series sketched the problem of severe-toprofound SNHL, and how cochlear implantation can overcome this, how a cochlear implant (CI) works, the history of cochlear implantation and the principles of the multidisciplinary CI team. The current status of cochlear implantation in South Africa (SA) is the subject of discussion in this article, the second of this two-part series on cochlear implantation. It comprises a study of the best available current data on the status of CI in SA.

Differences Between Young and Older Adults in Working Memory and Performance on the Test of Basic Auditory Capabilities†.

The Test of Basic Auditory Capabilities (TBAC) is a battery of auditory-discrimination tasks and speech-identification tasks that has been normed on several hundred young normal-hearing adults. Previous research with the TBAC suggested that cognitive function may impact the performance of older adults. Here, we examined differences in performance on several TBAC tasks between a group of 34 young adults with a mean age of 22.5 years (SD = 3.1 years) and a group of 115 older adults with a mean age of 69.2 years (SD = 6.2 years) recruited from the local community. Performance of the young adults was consistent with prior norms for this age group. Not surprisingly, the two groups differed significantly in hearing loss and working memory with the older adults having more hearing loss and poorer working memory than the young adults. The two age groups also differed significantly in performance on six of the nine measures extracted from the TBAC (eight test scores and one average test score) with the older adults consistently performing worse than the young adults. However, when these age-group comparisons were repeated with working memory and hearing loss as covariates, the groups differed in performance on only one of the nine auditory measures from the TBAC. For eight of the nine TBAC measures, working memory was a significant covariate and hearing loss never emerged as a significant factor. Thus, the age-group deficits observed initially on the TBAC most often appeared to be mediated by age-related differences in working memory rather than deficits in auditory processing. The results of these analyses of age-group differences were supported further by linear-regression analyses with each of the 9 TBAC scores serving as the dependent measure and age, hearing loss, and working memory as the predictors. Regression analyses were conducted for the full set of 149 adults and for just the 115 older adults. Working memory again emerged as the predominant factor impacting TBAC performance. It is concluded that working memory should be considered when comparing the performance of young and older adults on auditory tasks, including the TBAC.

Biallelic p.V37I variant in GJB2 is associated with increasing incidence of hearing loss with age

PurposeThis study aimed to quantitatively assess the incidence of hearing loss in relation to age in individuals with biallelic p.V37I variant in GJB2. MethodsPopulation screening of the biallelic p.V37I variant was performed in 30,122 individuals aged between 0 and 97 years in Shanghai. Hearing thresholds of the biallelic p.V37I individuals and the controls were determined by click auditory brainstem response or pure tone audiometry. ResultsBiallelic p.V37I was detected in 0.528% (159/30,122) of the subjects. Of the biallelic p.V37I newborns, 43.91% (18/41) passed their distortion-product otoacoustic emissions–based newborn hearing screening or had hearing thresholds lower than 20 decible above normal hearing level. The older newborns had elevated hearing thresholds, with increasing incidence of 9.52%, 23.08%, 59.38%, and 80.00% for moderate or higher grade of hearing loss in age groups of 7 to 15 years, 20 to 40 years, 40 to 60 years, and 60 to 85 years, respectively. Their hearing deteriorated at a rate of 0.40 dB hearing level per year on average; males were more susceptible, and deterioration occurred preferentially at higher sound frequencies. ConclusionThe biallelic p.V37I variant is associated with steadily progressive hearing loss with increasing incidence over the course of life. Most of the biallelic p.V37I individuals may develop significant hearing loss in adulthood and, can benefit from early diagnosis and intervention through wide-spread genetic screening.

Ototoxicity and long-term hearing outcome in pediatric patients receiving cisplatin

Hearing is essential in child development. Cisplatin which is a common chemotherapy used in many pediatric solid-tumor protocols cause various degrees of ototoxicity. Several risk factors for cisplatininduced ototoxicity have been reported, including race and age. This study aimed to evaluate the incidence of ototoxicity and its long-term outcome in Thai pediatric solid-tumor patients receiving cisplatin and to determine the risk factors associated with hearing impairment. A retrospective study was conducted in solid-tumor patients < 15 years old from 2007 to 2019 at Siriraj Hospital, Bangkok, Thailand. Hearing was evaluated by an audiogram and/or auditory steady-state response and the impairment was graded according to the Common Terminology Criteria for Adverse Events version 5. Grade 2 and above was considered significant hearing loss. In total, the hearing of 47 patients was evaluated. At the end of treatment, hearing impairment and significant hearing loss were found in 66% and 48.9% of patients, respectively. A high median cumulative cisplatin dose was significantly associated with worse hearing impairment (p = 0.039) and a more progressive grading of ototoxicity (p = 0.005). A risk factor for significant hearing loss was a cumulative dose ≥400 mg/m2 (p = 0.014). All 9 patients who received a cumulative dose > 600 mg/m2 and 5 patients who received aminoglycoside developed significant hearing loss. One patient had progressive hearing impairment at 8 months after the end of treatment and 1 patient developed grade 3 ototoxicity which required a hearing aid after bone marrow transplantation. The latter patient received a total cisplatin dose of 708.2 mg/m < sup > 2 < /sup > and carboplatin 1400 mg/m < sup > 2 < /sup > . The incidence of hearing impairment in pediatric patients receiving cisplatin is high. Regular hearing evaluation is essential for the early detection of ototoxicity. Long-term follow-up is recommended, especially in patients who have a combination of other risk factors for hearing loss.