The Merits of Bilateral Application of Middle Ear Implants in Patients With Bilateral Conductive and/or Mixed Hearing Loss.

This study investigated sound localization abilities in patients with bilateral conductive and/or mixed hearing loss (BCHL) when listening with either one or two middle ear implants (MEIs). Sound localization was measured by asking patients to point as quickly and accurately as possible with a head-mounted LED in the perceived sound direction. Loudspeakers, positioned around the listener within a range of +73°/-73° in the horizontal plane, were not visible to the patients. Broadband (500 Hz-20 kHz) noise bursts (150 ms), roved over a 20-dB range in 10 dB steps was presented. MEIs stimulate the ipsilateral cochlea only and therefore the localization response was not affected by crosstalk. Sound localization was better with bilateral MEIs compared with the unilateral left and unilateral right conditions. Good sound localization performance was found in the bilaterally aided hearing condition in four patients. In two patients, localization abilities equaled normal hearing performance. Interestingly, in the unaided condition, when both devices were turned off, subjects could still localize the stimuli presented at the highest sound level. Comparison with data of patients implanted bilaterally with bone-conduction devices, demonstrated that localization abilities with MEIs were superior. The measurements demonstrate that patients with BCHL, using remnant binaural cues in the unaided condition, are able to process binaural cues when listening with bilateral MEIs. We conclude that implantation with two MEIs, each stimulating only the ipsilateral cochlea, without crosstalk to the contralateral cochlea, can result in good sound localization abilities, and that this topic needs further investigation.

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.

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.

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.

Younger Age at Cochlear Implant Activation Results in Improved Auditory Skill Development for Children With Congenital Deafness.

PURPOSE: The U.S. Food and Drug Administration indications for cochlear implantation in children is currently 9 months of age and older for children with bilateral profound sensorineural hearing loss (SNHL). Studies have shown that earlier activation of a cochlear implant (CI) can lead to better spoken language outcomes. As auditory skills are a precursor to the development of spoken language, this study was developed to investigate the influence of age at CI activation on auditory skill acquisition in young children. A secondary aim was to describe the auditory skills of children implanted prior to 9 months of age as compared to children with older ages of activation. METHOD: Functional Listening Index (FLI) scores obtained during routine clinical visits were reviewed for 78 pediatric CI recipients with congenital bilateral profound hearing loss who were activated before 2 years of age. A linear mixed-effects model assessed the effect of age at CI activation on cumulative FLI scores over time. RESULTS: There was a significant interaction between age at activation and chronological age at the time of evaluation, indicating that children with earlier access to sound achieved a greater number of auditory skills than those with later CI activations when measured at the same chronological age. Children activated before the age of 9 months approximated scores expected of children with typical hearing, whereas children activated between 9 and 24 months of age did not. CONCLUSIONS: Younger age at CI activation is associated with increased auditory skills over time. Children who undergo cochlear implantation and CI activation before 9 months achieve more auditory skills by 4 years of age than children who are activated at later ages. These data suggest that reducing the approved age at cochlear implantation for children with congenital bilateral profound SNHL may support optimal auditory skill acquisition.

Hearing aid use in 11-year-old children with mild bilateral hearing loss: Associations between parent and child ratings and datalogging.

OBJECTIVE: This study examined the hearing aid use in older school-aged children with mild bilateral hearing loss. More specifically, it investigated children's and parents' estimation of use in comparison to datalogging as well as explored the situations children used their hearing aids. METHODS AND MATERIALS: Sixteen children with mild bilateral hearing loss and their parents participated. Of those, 14 children used hearing aids. Children and parents completed a questionnaire on hours of hearing aid use and situations hearing aids were used. Datalogging of the hearing aids was recorded and compared to the outcome of the questionnaires. RESULTS: Datalogging indicated average hearing aid use time was 6.6 h. Children significantly overestimated their use of their hearing aids while approximately half the parents overestimated their child's use. Children used their hearing aids most often at school and in the car. CONCLUSION: Children with mild bilateral hearing loss overestimate the amount of time they are wearing their hearing aids. This may impact counselling and intervention on the use of hearing aids. Therefore, school-aged children should be included in the discussions around potentially increasing use of hearing aids.

Mild matters: parental insights into the conundrums of managing mild congenital hearing loss.

OBJECTIVE: To explore and describe parental experiences related to the management of mild bilateral congenital hearing loss in children. DESIGN: Using qualitative methods, we conducted semi-structured interviews with parents/caregivers until saturation of themes was achieved. We analysed transcripts using inductive content analysis. STUDY SAMPLE: Caregivers of children under 3-years-old with mild bilateral sensorineural hearing loss. RESULTS: We interviewed 12 parents. Parental perception of advice regarding hearing aid fitting was varied; almost all children were offered hearing aids. Perceived positives related to hearing aids: feeling empowered that action has been taken; improvements in the child's hearing perception and; facilitation of behavioural management. Perceived negatives of hearing aid use: difficulties with compliance resulting in parental frustration and guilt, damage/loss of equipment, discomfort, parental discord, altered quality of natural sound and potential bullying/stigma. Some parents were ambivalent about the effect of the hearing aids. Where hearing aids were offered and not fitted, there was significant ongoing uncertainty, and the family carried the burden of their decision. CONCLUSIONS: There was a wide variation in perceived advice regarding early hearing aid fitting in children with mild bilateral hearing loss. We identified parental perceptions of positive/negative impacts of hearing aid fitting and potential perceived harms from not fitting.

The Influence of Forced Social Isolation on the Auditory Ecology and Psychosocial Functions of Listeners With Cochlear Implants During COVID-19 Mitigation Efforts.

OBJECTIVES: The impact of social distancing on communication and psychosocial variables among individuals with hearing impairment during COVID-19 pandemic. It was our concern that patients who already found themselves socially isolated (Wie et al. 2010) as a result of their hearing loss would be perhaps more susceptible to changes in their communication habits resulting in further social isolation, anxiety, and depression. We wanted to better understand how forced social isolation (as part of COVID-19 mitigation) effected a group of individuals with hearing impairment from an auditory ecology and psychosocial perspective. We hypothesized that the listening environments would be different as a result of social isolation when comparing subject's responses regarding activities and participation before COVID-19 and during the COVID-19 pandemic. This change would lead to an increase in experienced and perceived social isolation, anxiety, and depression. DESIGN: A total of 48 adults with at least 12 months of cochlear implant (CI) experience reported their listening contexts and experiences pre-COVID and during-COVID using Ecological Momentary Assessment (EMA; methodology collecting a respondent's self-reports in their natural environments) through a smartphone-based app, and six paper and pencil questionnaires. The Smartphone app and paper-pencil questionnaires address topics related to their listening environment, social isolation, depression, anxiety, lifestyle and demand, loneliness, and satisfaction with amplification. Data from these two-time points were compared to better understand the effects of social distancing on the CI recipients' communication abilities. RESULTS: EMA demonstrated that during-COVID CI recipients were more likely to stay home or be outdoors. CI recipients reported that they were less likely to stay indoors outside of their home relative to the pre-COVID condition. Social distancing also had a significant effect on the overall signal-to-noise ratio of the environments indicating that the listening environments had better signal-to-noise ratios. CI recipients also reported better speech understanding, less listening effort, less activity limitation due to hearing loss, less social isolation due to hearing loss, and less anxiety due to hearing loss. Retrospective questionnaires indicated that social distancing had a significant effect on the social network size, participant's personal image of themselves, and overall loneliness. CONCLUSIONS: Overall, EMA provided us with a glimpse of the effect that forced social isolation has had on the listening environments and psychosocial perspectives of a select number of CI listeners. CI participants in this study reported that they were spending more time at home in a quieter environments during-COVID. Contrary to our hypothesis, CI recipients overall felt less socially isolated and reported less anxiety resulting from their hearing difficulties during-COVID in comparison to pre-COVID. This, perhaps, implies that having a more controlled environment with fewer speakers provided a more relaxing listening experience.

Early Intervention, Parent Talk, and Pragmatic Language in Children With Hearing Loss.

BACKGROUND AND OBJECTIVES: Pragmatic language skills form the foundation for conversational competence, whereas deficits in this area are associated with behavioral problems and low literacy skills. Children who are deaf or hard of hearing demonstrate significant delays in this critical area of language. Our purpose with this research was to identify variables associated with pragmatic language ability in children who are deaf or hard of hearing. METHODS: This was a longitudinal study of 124 children with bilateral hearing loss between 4 and 7 years of age living in Colorado. As part of a comprehensive speech and language assessment, pragmatic language skills were evaluated annually by using the Pragmatics Checklist. RESULTS: The children's pragmatic skills increased significantly with age. Higher levels of pragmatic language ability at 7 years of age were predicted by (1) meeting Early Hearing Detection and Intervention 1-3-6 guidelines (hearing screening by 1 month, identification of hearing loss by 3 months, and receiving intervention by 6 months of age), (2) greater quantity of parent talk, (3) higher nonverbal intelligence, (4) lesser degrees of hearing loss, and (5) higher maternal education. CONCLUSIONS: With the findings of this study, we underscore the importance of pediatricians and other health care professionals counseling parents about the value of adherence to the Early Hearing Detection and Intervention 1-3-6 guidelines with regard to intervention outcomes. The strong association between amount of child-directed parent talk in the first 4 years of life and pragmatic language outcomes at 7 years of age emphasizes the need for professionals to encourage parents to talk to their children as much as possible.

Haptic sound-localisation for use in cochlear implant and hearing-aid users.

Users of hearing-assistive devices often struggle to locate and segregate sounds, which can make listening in schools, cafes, and busy workplaces extremely challenging. A recent study in unilaterally implanted CI users showed that sound-localisation was improved when the audio received by behind-the-ear devices was converted to haptic stimulation on each wrist. We built on this work, using a new signal-processing approach to improve localisation accuracy and increase generalisability to a wide range of stimuli. We aimed to: (1) improve haptic sound-localisation accuracy using a varied stimulus set and (2) assess whether accuracy improved with prolonged training. Thirty-two adults with normal touch perception were randomly assigned to an experimental or control group. The experimental group completed a 5-h training regime and the control group were not trained. Without training, haptic sound-localisation was substantially better than in previous work on haptic sound-localisation. It was also markedly better than sound-localisation by either unilaterally or bilaterally implanted CI users. After training, accuracy improved, becoming better than for sound-localisation by bilateral hearing-aid users. These findings suggest that a wrist-worn haptic device could be effective for improving spatial hearing for a range of hearing-impaired listeners.

Hearing Aid Use, Auditory Development, and Auditory Functional Performance in Swedish Children With Moderate Hearing Loss During the First 3 Years.

Purpose This study investigated longitudinal hearing aid (HA) use in a cohort of children with moderate hearing loss (CHL), fitted with amplification before the age of 6 months. Additionally, the relationship of HA use and aided audibility on outcomes of parental questionnaires of auditory skills was examined, and these outcomes were compared to a group of children with normal hearing (CNH). Method Nine CHL and 29 CNH and their parents participated in the study. Measures were collected at initial fitting, 10, 18, 24, 30, and 36 months of age. Parents reported hours of HA use and situations the HAs were used. Datalogging and speech intelligibility index were also collected. Auditory skills were measured through parental questionnaires. Results The mean hours of HA use/day for this cohort increased from 7.55 at the ages of 10 months to 10.15 at 36 months according to datalogging. Parental estimations of hours of HA use and in which situations varied between subjects. Correlations between HA use from datalogging and speech intelligibility index to measures of auditory skills were weak. CHL showed similar results to CNH on auditory development at the ages of 10, 18, and 24 months but presented with significantly lower scores on auditory functional performance in noise at 30 and 36 months of age. Conclusions Longitudinal monitoring of HA use from fitting of amplification with the combination of objective and subjective tools may have a positive impact on HA use in CHL. The lower scores on listening in noise compared to CNH call for further attention.

Using Contextual and Visual Cues to Improve Sung Word Recognition in Hearing Aid Users.

Older adults commonly experience hearing loss that negatively affects the quality of life and creates barriers to effective therapeutic interactions as well as music listening. Music therapists have the potential to address some needs of older adults, but the effectiveness of music interventions is dependent on the perception of spoken and musical stimuli. Nonauditory information, such as contextual (e.g., keywords, picture related to song) and visual cues (e.g., clear view of singer's face), can improve speech perception. The purpose of this study was to examine the benefit of contextual and visual cues on sung word recognition in the presence of guitar accompaniment. The researcher tested 24 community-dwelling older adult hearing aid (HA) users recruited through a university HA clinic and laboratory under 3 study conditions: (a) auditory stimuli only, (b) auditory stimuli with contextual cues, and (c) auditory stimuli with visual cues. Both visual and contextual nonauditory cues benefited participants on sung word recognition. Participants' music background and training were predictive of success without nonauditory cues, and visual cues provided greater benefit than contextual cues. Based on the results of this study, it is recommended that music therapists increase the accessibility of music interventions reliant upon lyric recognition through the incorporation of clear visual and contextual cues.

Progression of hearing loss and cochlear implantation in large vestibular aqueduct syndrome.

OBJECTIVES: Large vestibular aqueduct syndrome (LVAS) is a congenital inner ear malformation that commonly results in progressive sensorineural hearing loss (SNHL) and cochlear implantation (CI). Though LVAS accounts for approximately 15% of pediatric SNHL, little is known regarding the rate and severity of SNHL in these patients. We sought to characterize the timing of SNHL progression to CI in patients with LVAS. METHODS: We performed a retrospective chart review at our institution from 2000 to 2018 using ICD-10 "large vestibular aqueduct syndrome," and through identifying patients with CI who had LVAS. Demographic, surgical, and audiometric data were collected. Theoretical CI candidacy was approximated using a pure tone average (PTA) HL threshold of 70 dB. RESULTS: Of 103 patients, 96 had bilateral LVAS, and 7 had unilateral LVAS. Forty-one patients had bilateral implants, 52 had unilateral implants, and 10 were not implanted. The mean age at first implant was 8.62 years old [95%CI = 6.75,10.49], the mean age at second implant was 12.24 years old [95%CI = 8.33,16.15], and the mean time between implants was 4.37 years [95%CI = 3.02,5.73]. LVAS patients reached HL threshold of 70 dB at a mean age of 5.16 years old (SD = 3.04) for the "worse ear" and 9.08 years old (SD = 4.96) for the "better ear." CONCLUSIONS: LVAS patients are a heterogenous population of patients, in which some may undergo progression of HL and some may not. Further, there may be a discrepancy in the timing between patients' theoretical CI candidacy and when they undergo CI. In order to optimize timing of CI, individual monitoring and close observation of LVAS patients is recommended.

The influence of age and language on developmental trajectory of theory of mind in children with cochlear implants.

OBJECTIVE: The present study investigates if children with cochlear implants (CI) are associated with delayed development of Theory of Mind (ToM) and the differences in gain of ToM ability with respect to age, language and other aspects of CI. METHODS: One-hundred-eleven children with participation of their hearing parents, aged between 36 months and 132 months, who had congenital profound bilateral deafness and were unilateral CI users; plus 99 healthy children underwent 'Peabody Picture Vocabulary' and 'Sally-Anne' tests. A total of 77 children with CI (mean age: 76.51 months, 31 girls and 46 boys) and 82 healthy children (mean age: 72.41 months, 47 girls and 35 boys) were included in the analyses. Analytic comparisons were created between the controls and children with CI and between subgroups of CI users by univariate and multivariate analysis. The effects of age of hearing aid use, age of CI surgery, duration of CI use, language scores and the presence of risk factors, early rehabilitation with hearing aid and CI on the ToM development were analyzed. RESULTS: The Sally-Anne test success rates of 67.1% were significantly higher in controls than that of children with CI (49.4%) (p < 0.05). The mean age difference of the children, who were successful in Sally-Anne test was 5.33 months in favor of healthy controls. The ToM task success rates were 57.1% (24/42) and 40% (14/35) in children with early CI and late CI respectively. The children, who were good at language, were also better in Sally-Anne tests, but the mean Peabody Picture Vocabulary test scores were indifferent in the same age group with respect to the presence of CI use (p > 0.05). However, multivariate analysis presented the higher language scores as the only significant independent variable that has impact on the success in Sally-Anne test (p < 0.05). CONCLUSION: The results revealed that among all children, who did better in ToM were older in age and better in language skills. Children with CI also performed better in false-belief test depending on their language scores. Early CI surgery, older age, and hence longer CI use can provide a well-developed ToM for children with CI.

Speech perception with simultaneous bilateral cochlear implants: Is there a unilateral predominance?

OBJECTIVES: To assess the audiological outcomes and speech performance among children with simultaneous bilateral cochlear implants (CIs). METHODS: This was a retrospective case review of 41 patients with prelingual deafness who failed a hearing aid trial and received simultaneous bilateral CIs. Ear-specific responses in terms of pure tone average (PTA), speech reception thresholds (SRTs), and speech discrimination score (SDS) were recorded for both ears of all patients. RESULTS: The PTA and SRT for the right and left CIs were comparable (P-value = 0.861 and P-value = 0.524, respectively). The SDS was slightly higher for the right ear, although it was not significantly different from that for the left ear (P-value = 0.375), yielding only a 2.42% difference. CONCLUSION: We found no significant side preference in all assessment scores for children with simultaneous bilateral CIs. Therefore, we cautiously advise implanting the CI in the right ear in case of symmetrical hearing loss when other prognostic factors do not favor the left ear.

Cochlear implantation after traumatic brain injury without otic capsule fracture: A case report and literature review.

OBJECTIVE: The aim of this study was to report a case of cochlear implantation (CI) for a patient with an otic capsule-sparing traumatic brain injury (TBI) and to review the relevant literature. METHODS: A patient with history of TBI received a CI for bilateral profound hearing loss. A systematic review of the literature was performed to identify and compare similar cases. RESULTS: A 36-year-old male with a history of hearing loss from right acute labyrinthitis was referred for bilateral profound sensorineural hearing loss (SNHL) after a fall with associated injury to the central auditory nervous system (CANS) including the brainstem. On the right, behavioral acoustic threshold measurements were in the profound range with absent OAEs. On the left, testing revealed no measurable behavioral acoustic thresholds and variable physiologic measures. A right unilateral cochlear implant was performed with most recent follow-up demonstrating speech awareness thresholds of 25 dB HL with excellent detection of all 6 Ling sounds. However, the patient also continues to suffer from other neurologic sequelae related to his TBI, which challenge his ability to demonstrate objective and subjective benefit. A systematic review of the literature demonstrates variable outcomes for patients with TBI and SNHL. CONCLUSIONS: Patients with profound SNHL and TBI present a distinct rehabilitative challenge for clinicians. CI may provide meaningful benefit in this population, though care should be taken in patient selection and counseling.

Cortical deafness of following bilateral temporal lobe stroke.

Cortical deafness is an extremely rare clinical manifestation that originates mainly from bilateral cortical lesions in the primary auditory cortex. Its main clinical manifestation is the bilateral sudden loss of hearing. Diagnosis is difficulty due to its rarity and similarity with other language and communication disorders, such as Wernicke's aphasia, auditory agnosia or verbal deafness. Herein, we present a case report of a young woman with a sudden bilateral loss of auditory comprehension. Initially, a psychiatric nature of the disorder was considered, but the persistence of the symptoms, lead to the diagnosis of cortical deafness secondary to bilateral ischemic lesions in both temporal lobes. Progressive improvement occurred and three months after the initial manifestations she manifested pure verbal deafness. Cortical deafness usually has a poor functional prognosis, with limited therapeutic options. Rehabilitation and speech therapy is recommended to improve the chance of patients achieving communication skills.

Cochlear implantation and mastoid obliteration in a patient with Alström Syndrome.

OBJECTIVES: To describe the outcomes of cochlear implantation with mastoid obliteration in a patient with Alström Syndrome and chronic otorrhea. METHODS: This is a single case discussion of a patient with Alström Syndrome and chronic otorrhea who underwent unilateral cochlear implantation and mastoid obliteration in January 2019. Subsequent contralateral procedure was performed 4 months later. Implantation was pursued due to the progressive sensorineural hearing-loss that is characteristic of Alström Syndrome. Serial Audiograms were obtained before and after procedure. RESULTS: Following implantation, audiological reports improved to near normal thresholds from the previous 60-85 dB sloping hearing loss. The patient's language skills rapidly improved as well as the ability to express her personality. Mastoid obliteration effectively resolved the chronic otorrhea that further complicated this case. CONCLUSION: Cochlear implantation with mastoid obliteration was successful in improving hearing thresholds and resolving chronic otorrhea in a patient with Alström Syndrome.

Audiological Considerations for Managing Mild Bilateral or Unilateral Hearing Loss in Infants and Young Children.

Purpose This clinical focus article describes considerations for recommending assistive hearing technology to infants and young children who have mild bilateral or unilateral hearing loss. These conditions present special challenges compared to bilateral permanent hearing losses that are moderate to profound in their degree in that the recommendation to proceed with technology is not as clear. Conclusion Current clinical practice guidelines and protocols for pediatric hearing aid fitting recommend managing these conditions on a case-by-case basis. Descriptions of key considerations for recommending assistive hearing technology for infants and young children with mild bilateral hearing loss or unilateral hearing loss are offered herein.