Vestibular Impairment and Postural Development in Children With Bilateral Profound Hearing Loss.

Importance: Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing. Objectives: To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL. Design, Setting, and Participants: This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023. Main Outcomes and Measures: The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay. Results: A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment. Conclusions and Relevance: This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.

Impact of Hearing Aids on Language Outcomes in Preschool Children With Mild Bilateral Hearing Loss.

This study aimed to investigate the role of hearing aid (HA) usage in language outcomes among preschool children aged 3-5 years with mild bilateral hearing loss (MBHL). The data were retrieved from a total of 52 children with MBHL and 30 children with normal hearing (NH). The association between demographical, audiological factors and language outcomes was examined. Analyses of variance were conducted to compare the language abilities of HA users, non-HA users, and their NH peers. Furthermore, regression analyses were performed to identify significant predictors of language outcomes. Aided better ear pure-tone average (BEPTA) was significantly correlated with language comprehension scores. Among children with MBHL, those who used HA outperformed the ones who did not use HA across all linguistic domains. The language skills of children with MBHL were comparable to those of their peers with NH. The degree of improvement in audibility in terms of aided BEPTA was a significant predictor of language comprehension. It is noteworthy that 50% of the parents expressed reluctance regarding HA use for their children with MBHL. The findings highlight the positive impact of HA usage on language development in this population. Professionals may therefore consider HAs as a viable treatment option for children with MBHL, especially when there is a potential risk of language delay due to hearing loss. It was observed that 25% of the children with MBHL had late-onset hearing loss. Consequently, the implementation of preschool screening or a listening performance checklist is recommended to facilitate early detection.

Differences in music appreciation between bilateral and single-sided cochlear implant recipients.

OBJECTIVE: To compare changes in music appreciation after cochlear implant (CI) surgery for patients with bilateral and single-sided deafness (SSD). METHODS: A retrospective cohort study was performed on all adult CI unilateral or bilateral recipients from November 2019 to March 2023. Musical questionnaire subset data from the Cochlear Implant Quality of Life (CIQOL) - 35 Profile Instrument Score (maximum raw score of 15) was collected. Functional CI assessment was measured with CI-alone speech-in-quiet (SIQ) scores (AzBio and CNC). RESULTS: 22 adults underwent CI surgery for SSD and 21 adults for bilateral deafness (8 sequentially implanted). Every patient group had clinically significant improvements (p < 0.001) in mean SIQ scores in the most recently implanted ear (Azbio (% correct) SSD: 14.23 to 68.48, bilateral: 24.54 to 82.23, sequential: 6.25 to 82.57). SSD adults on average had higher music QOL scores at baseline (SSD: 11.05; bilateral: 7.86, p < 0.001). No group had significant increases in raw score at the first post-operative visit (SSD: 11.45, p = 0.86; bilateral: 8.15, p = 0.15). By the most recent post-implantation evaluation (median 12.8 months for SSD, 12.3 months for bilateral), SSD adults had a significant increase in raw score from baseline (11.05 to 12.45, p = 0.03), whereas bilaterally deafened (7.86 to 9.38, p = 0.12) adults had nonsignificant increases. CONCLUSIONS: SSD patients demonstrate higher baseline music appreciation than bilaterally deafened individuals regardless of unilateral or bilateral implantation and are more likely to demonstrate continued improvement in subjective music appreciation at last follow-up even when speech perception outcomes are similar.

Bilateral, Sequential Sudden Hearing Loss in a Patient With Multiple COVID-19 Infections and Potential Implications on Subsequent Cochlear Implant Performance.

Viruses are often implicated as a cause of sensorineural hearing loss (SNHL), particularly sudden cases, including COVID-19. Determining the viral mechanism that leads to hearing loss is necessary for its future prevention and treatment. The 47-year-old woman who is the subject of this case study presented with sudden SNHL following multiple infections of COVID-19. Following a trial of a contralateral routing of sound device, she received a right cochlear implant (CI). Following a period of high performance, additional cases of COVID-19 infection and device failure issues resulted in the explant/reimplant of 1 ear and implantation of the contralateral ear. Despite extensive rehabilitation after these events, the patient continues to experience difficulties in speech understanding, not reaching her initial high levels of right ear performance. Further research is needed to determine the implications of COVID-19 as it relates to SNHL. This case study aimed to highlight the course of treatment and provide insight into the impact of COVID-19 on sudden hearing loss and its relationship to CI performance.

Pediatric Hearing Aid Daily Wear Time Is Significantly Impacted by Clinician-Family Language Discordance.

PURPOSE: The purpose of this study was to evaluate the potential contribution of limited English proficiency on daily hearing aid wear time for children with hearing loss. METHOD: A retrospective chart review was completed to evaluate hearing aid wear time based on data logging information available at the time of a follow-up visit following an initial hearing aid fitting. Children were included in the study if they had permanent bilateral hearing loss and were less than 60 months of age at the time of their first follow-up visit. Wear time was compared between children who attended an interpreter-mediated appointment and those who did not have an interpreter present. The presence of an interpreter at the appointment was the study indicator that the family had limited English proficiency. RESULTS: Children from families with limited English proficiency exhibited significantly shorter daily wear time (M = 1.3 hr) than their peers whose families were English-proficient speakers, thus, having a shared language with their audiologists (M = 5.2 hr). CONCLUSIONS: Results of this study suggest that family-clinician language discordance might put children at greater risk of shorter hearing aid wear time than children whose caregivers share a common language with their child's audiologist. There can be many linguistic, cultural, and educational factors that contribute to hearing aid wear time in children whose families have limited English proficiency as well as different approaches to improving that wear time. Efforts should ensure that hearing and hearing aid-related information is accessible to all families, especially those with clinician-family language discordance. Such efforts can include, among others, training that improves clinicians' cultural and linguistic responsiveness to the diverse families they serve.

Troubleshooting Cochlear Implant Malfunction Using Neural Response Telemetry and Normal Saline.

Cochlear implantation has become a standard of care for a child diagnosed with bilateral profound sensorineural hearing loss with a structured surgical standard operating procedure. A 3-year-old boy with bilateral profound prelingual sensorineural deafness underwent a Med-EL Sonata Ti100 implant. We faced a peculiar situation intraoperatively after inserting the electrodes and closing the wound. The impedance recording indicated high ground path impedance with short-circuiting of few electrodes. As a bionic implant, its electronic components may at times malfunction both intraoperatively and/or postoperatively; therefore, neural response telemetry (NRT) was invented to check it. By using NRT and a few milliliters of normal saline, we were able to diagnose as well as rectify the malfunctioning of the implant.

Cochleovestibular Phenotype in a Rare Genetic MED13L Mutation.

The gene MED13 participates in transcription. The MED13L gene is a paralog of MED13 that is involved in developmental gene expression. Mutations in the gene have been shown to result in a heterogenous phenotype affecting several physiological systems. Hearing loss has been reported very rarely, and vestibular weakness has never been reported in the condition. In this report, we present a mutation of MED13L in c.1162A > T (p.Arg388Ter), where we detail and describe a cochleovestibular phenotype with objective vestibulometry for the first time. The child showed bilateral sloping sensorineural hearing loss, a bilateral vestibular weakness, and an inner ear vestibular structural abnormality on imaging. Early intervention with hearing aids and vestibular rehabilitation led to a favorable outcome in terms of speech, communication, and balance. We emphasize the importance of comprehensive audiovestibular assessment in children diagnosed with MED13L mutations for effective management of these children.

Computer-based Auditory Training by New Adult Cochlear Implant Recipients Is Associated With Durable Improvements in Cochlear Implant Quality of Life.

OBJECTIVE: The process of adapting to communicate with a cochlear implant (CI) is complex. The use of auditory training after cochlear implantation may help to facilitate improvements in postoperative speech recognition and quality-of-life outcomes in new adult CI recipients. However, the effectiveness of auditory training remains uncertain and long-term effects have not been examined in a large sample of new adult CI users. As such, the objective of this study was to examine the influence of common forms of auditory training on speech recognition and CI-related quality-of-life (CI-related QOL) outcomes at 1 year after cochlear implantation. We hypothesized that patients who reported use of computer-based auditory training (CBAT) would show improved speech and CIQOL-35 Profile scores at 1 year after activation of their implant, compared with their peers. DESIGN: This study was designed as a prospective study and was undertaken at a tertiary academic CI center. Participants included 114 adults undergoing cochlear implantation for bilateral hearing loss. Patients serially self-reported use of the following types of post-CI auditory training over their first-year postactivation: (1) face-to-face training (e.g., speech-language pathologist), (2) passive home-based training (e.g., listening to audiobooks), and (3) CBAT (e.g., self-directed software). Outcomes measures for this study included change in Consonant-Nucleus-Consonant phoneme (CNCp), CNC word (CNCw), AzBio sentences in quiet, and CIQOL-35 Profile global and domain scores from pre-CI to 12-mo post-CI. RESULTS: Of 114 patients, 94 (82.5%) used one or more auditory training resources. Of these, 19.3% used face-to-face training, 67.5% passive home-based training, and 46.5% CBAT. Of 114 patients, 73 had complete CIQOL data. At 12 mo, only CBAT use was associated with significantly greater improvements in global and all domain-specific CIQOL scores ( d -range = 0.72-0.87), compared with those not using CBAT. Controlling for demographics and use of multiple training resources, CBAT remained the strongest positive predictor of CIQOL improvement, with significant associations with global score (ß = 12.019[4.127,19.9]) and all domain scores at 12-mo post-CI: communication (ß = 11.937[2.456,21.318), emotional (ß = 12.293[1.827,22.759), entertainment (ß = 17.014[5.434,28.774), environment (ß = 13.771[1.814,25.727]), listening effort (ß = 12.523[2.798,22.248]), and social (ß = 18.114[7.403,28.826]). No significant benefits were noted with use of CBAT or any other form of auditory training and speech recognition scores at 12-mo post-CI ( d -range = -0.12-0.22). CONCLUSIONS: Auditory training with CBAT was associated with improved CI-related QOL outcomes at 12-mo post-CI. Given its availability and low cost, this study provides evidence to support using CBAT to improve real-world functional abilities in new adult CI recipients.

Adult-onset mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS): a diagnostic challenge.

Rare causes of stroke-like presentations can be difficult to diagnose. We report a case of a man in his 40s who first presented with stroke symptoms, but whose clinical course was not typical for a stroke. A detailed investigation of the patient's medical history revealed bilateral sensorineural hearing loss which prompted a wider diagnostic assessment.Furthermore, lack of vascular risk factors and a normal angiogram strengthened our suspicion of an unusual underlying condition. Raised lactic acid levels and genetic analysis confirmed a diagnosis of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes syndrome.

Association of noise exposure, plasma microRNAs with metabolic syndrome and its components among Chinese adults.

AIMS: We aimed to evaluate the association of occupational noise with metabolic syndrome (MetS) and its components, and to assess the potential role of miRNAs in occupational noise-associated MetS. METHODS: A total of 854 participants were enrolled in our study. Cumulative noise exposure (CNE) was estimated in conjunction with workplace noise test records and research participants' employment histories. Logistic regression models adjusted for potential confounders were used to assess the association of CNE and miRNAs with MetS and its components. RESULTS: We observed linear positive dose-response associations between occupational noise exposure and the prevalence of MetS (OR: 1.031; 95 % CI: 1.008, 1.055). And linear and nonlinear relationship were also found for the association of occupational noise exposure with high blood pressure (OR: 1.024; 95 % CI: 1.007, 1.041) and reduced high-density lipoprotein (OR: 1.051; 95 % CI: 1.031, 1.072), respectively. MiR-200a-3p, miR-92a-3p and miR-21-5p were inversely associated with CNE, or the prevalence of MetS and its components (all P < 0.05). However, we did not find any statistically significant mediation effect of miRNAs in the associations of CNE with MetS. Furthermore, the prevalence of bilateral hearing loss in high-frequency increased (OR: 1.036; 95 % CI: 1.008, 1.067) with CNE level rising, and participants with bilateral hearing loss in high-frequency had a significantly higher risk of MetS (OR: 1.727; 95 % CI: 1.048, 2.819). CONCLUSION: Our study suggests that occupational noise exposure is associated with MetS and its components, and the role of miRNAs in noise-induced increasing MetS risk needs to be confirmed in future studies.

Externalization of Speech When Listening With Hearing Aids.

Subjective reports indicate that hearing aids can disrupt sound externalization and/or reduce the perceived distance of sounds. Here we conducted an experiment to explore this phenomenon and to quantify how frequently it occurs for different hearing-aid styles. Of particular interest were the effects of microphone position (behind the ear vs. in the ear) and dome type (closed vs. open). Participants were young adults with normal hearing or with bilateral hearing loss, who were fitted with hearing aids that allowed variations in the microphone position and the dome type. They were seated in a large sound-treated booth and presented with monosyllabic words from loudspeakers at a distance of 1.5 m. Their task was to rate the perceived externalization of each word using a rating scale that ranged from 10 (at the loudspeaker in front) to 0 (in the head) to -10 (behind the listener). On average, compared to unaided listening, hearing aids tended to reduce perceived distance and lead to more in-the-head responses. This was especially true for closed domes in combination with behind-the-ear microphones. The behavioral data along with acoustical recordings made in the ear canals of a manikin suggest that increased low-frequency ear-canal levels (with closed domes) and ambiguous spatial cues (with behind-the-ear microphones) may both contribute to breakdowns of externalization.

Importance of age at 2nd implantation and interimplant interval to the outcome of bilateral prelingually deafened pediatric cochlear implantation.

BACKGROUND: In Taiwan, the number of cases of sequential bilateral pediatric cochlear implantation (CI) is increasing but data regarding its effectiveness and impact of the reimbursement policy are lacking. We examined the speech perception and quality of life (QOL) of bilateral prelingually deaf children who underwent sequential CI, considering the effects of age at the time of second implantation and interimplant interval. METHODS: We enrolled 124 Mandarin-speaking participants who underwent initial cochlear implant (CI1) in 2001-2019 and a second CI (CI2) in 2015-2020. Patients were followed up for ≥2 years and were categorized into groups based on age at the time of CI2 implantation (<3.5, 3.6-7, 7.1-10, 10.1-13, and 13.1-18 years) and interimplant interval (0.5-3, 3.1-5, 5.1-7, 7.1-10, and >10 years). We evaluated speech perception, device usage rates, and QOL using subjective questionnaires (Speech, Spatial, and Qualities of Hearing and Comprehension Cochlear Implant Questionnaire). RESULTS: Speech perception scores of CI2 were negatively correlated with ages at the time of CI1 and CI2 implantation and interimplant interval. Older age and a longer interimplant interval were associated with higher nonuse rates for CI2 and worse auditory performance and QOL. Among individuals aged >13 years with interimplant intervals >10 years, up to 44% did not use their second ear. Patients aged 7.1 to 10 years had better speech perception and higher questionnaire scores than those aged 10.1 to 13 and 13.1 to 18 years. Furthermore, patients aged 10.1 to 13 years had a lower rate of continuous CI2 usage compared to those aged 7.1 to 10 years. CONCLUSION: Timely implantation of CI2 is essential to achieve optimal outcomes, particularly among sequentially implanted patients with long-term deafness in the second ear and no improvement with hearing aids following CI1 implantation. For CI2 implantation, an upper limit of age of 10 years and interimplant interval of 7 years are essential to prevent suboptimal outcomes. These data can provide useful information to implant recipients, their families, and medical and audiological professionals, enabling a comprehensive understanding of the benefits and potential impacts of the timing of CI2 implantation.

[Genetic characteristic analysis of slight-to-moderate sensorineural hearing loss in children].

Objective:To analyze genetic factors and phenotype characteristics in pediatric population with slight-to-moderate sensorineural hearing loss. Methods:Children with slight-to-moderate sensorineural hearing loss of and their parents, enrolled from the Chinese Deafness Genome Project, were studied. Hearing levels were assessed using pure tone audiometry, behavioral audiometry, auditory steady state response（ASSR）, auditory brainstem response（ABR） thresholds, and deformed partial otoacoustic emission（DPOAE）. Classification of hearing loss is according to the 2022 American College of Medical Genetics and Genomics（ACMG） Clinical Practice Guidelines for Hearing Loss. Whole exome sequencing（WES） and deafness gene Panel testing were performed on peripheral venous blood from probands and validations were performed on their parents by Sanger sequencing. Results:All 134 patients had childhood onset, exhibiting bilateral symmetrical slight-to-moderate sensorineural hearing loss, as indicated by audiological examinations. Of the 134 patients, 29（21.6%） had a family history of hearing loss, and the rest were sporadic patients. Genetic causative genes were identified in 66（49.3%） patients. A total of 11 causative genes were detected, of which GJB2 was causative in 34 cases（51.5%）, STRC in 10 cases（15.1%）, MPZL2 gene in six cases（9.1%）, and USH2A in five cases（7.6%）.The most common gene detected in slight-to-moderate hearing loss was GJB2, with c. 109G>A homozygous mutation found in 16 cases（47.1%） and c. 109G>A compound heterozygous mutation in 9 cases（26.5%）. Conclusion:This study provides a crucial genetic theory reference for early screening and detection of mild to moderate hearing loss in children, highlighting the predominance of recessive inheritance and the significance of gene like GJB2, STRC, MPZL2, USH2A.

Genetic diagnosis of childhood sensorineural hearing loss.

INTRODUCTION: Congenital/early-onset sensorineural hearing loss (SNHL) is one of the most common hereditary disorders in our environment. There is increasing awareness of the importance of an etiologic diagnosis, and genetic testing with next-generation sequencing (NGS) has the highest diagnostic yield. Our study shows the genetic results obtained in a cohort of patients with bilateral congenital/early-onset SNHL. MATERIALS AND METHODS: We included 105 children with bilateral SNHL that received genetic testing between 2019 and 2022. Genetic tests were performed with whole exome sequencing, analyzing genes related to hearing loss (virtual panel with 244 genes). RESULTS: 48% (50/105) of patients were genetically diagnosed. We identified pathogenic and likely pathogenic variants in 26 different genes, and the most frequently mutated genes were GJB2, USH2A and STRC. 52% (26/50) of variants identified produced non-syndromic hearing loss, 40% (20/50) produced syndromic hearing loss, and the resting 8% (4/50) could produce both non-syndromic and syndromic hearing loss. CONCLUSIONS: Genetic testing plays a vital role in the etiologic diagnosis of bilateral SNHL. Our cohort shows that genetic testing with NGS has a high diagnostic yield and can provide useful information for the clinical workup of patients.

Cochlear implantation for progressive hearing loss caused by an A8296G mutation in mitochondrial DNA.

Mitochondrial DNA mutations such as A3243G or A1555G are widely reported to cause hearing loss, but few reports exist on the A8296G mutation, which can also cause hearing loss. This report presents the case of a patient with the A8296G mutation and severe bilateral sensorineural hearing loss (SNHL) that progressed over two decades. The patient had no history of diabetes, but did have a family history of SNHL in her father and maternal grandmother. She was first diagnosed with SNHL at 45 years of age, and an A8296G mutation was found. The hearing threshold in the low-frequency range of the right ear was preserved at diagnosis, but eventually declined resulting in severe bilateral hearing loss by the age of 66 years, and cochlear implantation (CI) was performed in the left ear. The hearing threshold three months after CI was 25-45 dB HL, and the phoneme speech discrimination score in the left ear improved from 20% without CI to 74% with CI. SNHL patients with the A8295G mutation are good candidates for treatment with CI.

The Impact of Patient Factors on Objective Cochlear Implant Verification Using Acoustic Cortical Auditory-Evoked Potentials.

INTRODUCTION: Hearing loss is a major global public health issue that negatively impacts quality of life, communication, cognition, social participation, and mental health. The cochlear implant (CI) is the most efficacious treatment for severe-to-profound sensorineural hearing loss. However, variability in outcomes remains high among CI users. Our previous research demonstrated that the existing subjective methodology of CI programming does not consistently produce optimal stimulation for speech perception, thereby limiting the potential for CI users to derive the maximum device benefit to achieve their peak potential. We demonstrated the benefit of utilising the objective method of measuring auditory-evoked cortical responses to speech stimuli as a reliable tool to guide and verify CI programming and, in turn, significantly improve speech perception performance. The present study was designed to investigate the impact of patient- and device-specific factors on the application of acoustically-evoked cortical auditory-evoked potential (aCAEP) measures as an objective clinical tool to verify CI mapping in adult CI users with bilateral deafness (BD). METHODS: aCAEP responses were elicited using binaural peripheral auditory stimulation for four speech tokens (/m/, /g/, /t/, and /s/) and recorded by HEARLab™ software in adult BD CI users. Participants were classified into groups according to subjective or objective CI mapping procedures to elicit present aCAEP responses to all four speech tokens. The impact of patient- and device-specific factors on the presence of aCAEP responses and speech perception was investigated between participant groups. RESULTS: Participants were categorised based on the presence or absence of the P1-N1-P2 aCAEP response to speech tokens. Out of the total cohort of adult CI users (n = 132), 63 participants demonstrated present responses pre-optimisation, 37 participants exhibited present responses post-optimisation, and the remaining 32 participants either showed an absent response for at least one speech token post-optimisation or did not accept the optimised CI map adjustments. Overall, no significant correlation was shown between patient and device-specific factors and the presence of aCAEP responses or speech perception scores. CONCLUSION: This study reinforces that aCAEP measures offer an objective, non-invasive approach to verify CI mapping, irrespective of patient or device factors. These findings further our understanding of the importance of personalised CI rehabilitation through CI mapping to minimise the degree of speech perception variation post-CI and allow all CI users to achieve maximum device benefit.

Differences in Hearing Devices and Speech Therapy Utilization Between Children With Permanent Unilateral Versus Bilateral Hearing Loss.

OBJECTIVES: In this study, we aimed to describe differences in diagnosis and both auditory and speech/language intervention utilization between children with permanent unilateral hearing loss as compared with bilateral hearing loss. DESIGN: A retrospective cohort study was performed of children evaluated in a multidisciplinary hearing loss clinic at a tertiary care pediatric hospital. Children aged 0 to 18 years with either permanent unilateral or bilateral hearing loss were included. RESULTS: One hundred fourteen children with unilateral hearing loss and 268 children with bilateral hearing loss were studied for a total of 382 children. There were no demographic differences between children with permanent unilateral versus bilateral hearing loss. Rates of newborn hearing screening and referred screening results were similar between those with unilateral and bilateral hearing loss. Despite similar rates of referred newborn hearing screening, those with bilateral hearing loss were diagnosed at a younger age (mean 3.6 years, SD 3.8 years) as compared with those with unilateral hearing loss (mean 5.0 years, SD 4.2 years). Children with unilateral hearing loss had similar severity of hearing loss in their poorer hearing ear as compared with children with bilateral hearing loss, yet they were significantly less likely to be fitted with hearing devices (53% versus 78%) or receive speech/language therapy (36% versus 54%) as compared with children with bilateral hearing loss. Multivariate analysis found that bilateral hearing loss and earlier age of hearing loss diagnosis were associated with hearing device use. CONCLUSIONS: Early diagnosis and intervention for childhood hearing loss have a significant impact on a child's educational success and social relationships. However, little is known about differences in diagnosis and resource utilization between children with permanent unilateral hearing loss versus bilateral hearing loss. Children with unilateral hearing loss were diagnosed at a later age and were less likely to utilize hearing devices or speech/language therapy compared with those with bilateral hearing loss, despite having similar severity of hearing loss in the poorer hearing ear. There is a strong body of evidence that children with unilateral hearing loss have improved hearing outcomes with hearing devices, which suggests there is room for improvement in identifying unilateral hearing loss and providing adequate services to optimize educational success. However, speech therapy is generally implemented in response to language delays. Therefore, children with unilateral loss may have lower rates of language delays as compared with those with bilateral hearing loss, thereby explaining differences in speech therapy utilization.

Speech, Sign, or Both? Factors Influencing Caregivers' Communication Method Decision Making for Deaf/Hard of Hearing Children.

PURPOSE: Caregivers of deaf/hard of hearing infants are faced with challenging decisions regarding their child's communication method. The purpose of the current research note is to characterize the advice that caregivers receive and value as well as the factors that influence caregivers' decision making. METHOD: The current study enrolled 105 caregiver-child dyads, including children between 12 and 18 months of age with bilateral, congenital hearing loss. All children were exposed to spoken language, and 63.81% of children were also exposed to sign language. Caregivers completed the "Making Decisions About Sign, Speech, and Multilingualism Survey" (Crowe et al., 2014). RESULTS: Caregivers most frequently received advice to use both speech and sign and highly valued advice from speech-language pathologists. When considering the use of speech, the factors that caregivers most frequently rated as very important were "My child's future academic success" (96.19%), "My child's future literary success" (95.24%), and "My child's future access to higher education" (95.19%). When considering the use of sign, the factors that caregivers most frequently rated as very important were "My child's ability to form friendships and future relationships" (82.52%), "My child's future literary success" (81.37%), and "My child's future academic success" (81.37%). CONCLUSIONS: Results of the current study suggest that throughout the decision-making process, caregivers' highest priority is understanding how their decisions will influence their child's future access to opportunities and relationships. Providers may consider discussing these factors early in the decision-making process to support caregivers' ability to make an informed choice regarding their child's communication method.