The Use of Electronic Health Records for Behavioral Phenotyping of School-Age Children With Unilateral Hearing Loss: A Methodological Approach.

PURPOSE: This methodological study describes a technique for extracting information from de-identified electronic health records (EHRs) to identify occurrences of permanent unilateral hearing loss (UHL) and associated educational comorbidities. METHOD: This was an exploratory methodological study utilizing approximately 3.3 million de-identified medical records. Structured and unstructured data were extracted using both automated and manual methods. When both methods were available, positive and negative predictive values were calculated to evaluate the utility of using automated methods. RESULTS: We defined a cohort of 471 records that met our criteria of school-age children with permanent UHL and no additional significant disabilities/diagnoses. Fifty-one percent of the children reflected in this cohort had indicators of adverse educational progress, defined as documentation of receiving educational services, speech-language therapy, and/or parental/teacher concern, with 12% of records reflecting overlapping services/concerns. Negative predictive values were generally high and positive predictive values were generally low, suggesting automated searches are useful for excluding factors of interest, but not finding them. CONCLUSIONS: This study demonstrates the feasibility of using EHRs in examining UHL in school-age children. By restricting our cohort to individuals who were seen in audiology clinic, we were able to capture variables such as educational difficulty that are not routinely ascertained in medical contexts. The proportion of children in this cohort demonstrating a marker of adverse educational progress is consistent with numerous prior observational studies, thus providing validity to this ascertainment approach. We describe challenges encountered in creating this cohort and detail our hybrid approach to ascertaining key variables accurately.

Auditory capacity of the better-hearing ear in asymmetric hearing loss.

PURPOSE: Our aim was to investigate the course of the hearing capacity of the better-hearing ear in single-sided deafness (SSD) and asymmetric hearing loss (AHL) over time, in a multicenter study. METHODS: We included 2086 pure-tone audiograms from 323 patients with SSD and AHL from four hospitals and 156 private practice otorhinolaryngologists. We collected: age, gender, etiology, duration of deafness, treatment with CI, number and monosyllabic speech recognition, numerical rating scale (NRS) of tinnitus intensity, and the tinnitus questionnaire according to Goebel and Hiller. We compared the pure tone audiogram of the better-hearing ear in patients with SSD with age- and gender-controlled hearing thresholds from ISO 7029:2017. RESULTS: First, individuals with SSD showed a significantly higher hearing threshold from 0.125 to 8 kHz in the better-hearing ear compared to the ISO 7029:2017. The duration of deafness of the poorer-hearing ear showed no relationship with the hearing threshold of the better-hearing ear. The hearing threshold was significantly higher in typically bilaterally presenting etiologies (chronic otitis media, otosclerosis, and congenital hearing loss), except for Menière's disease. Second, subjects that developed AHL did so in 5.19 ± 5.91 years and showed significant reduction in monosyllabic word and number recognition. CONCLUSIONS: Individuals with SSD show significantly poorer hearing in the better-hearing ear than individuals with NH from the ISO 7029:2017. In clinical practice, we should, therefore, inform our SSD patients that their disease is accompanied by a reduced hearing capacity on the contralateral side, especially in certain etiologies.

Response to OKN-007 and NAC in a Patient with Unilateral Hearing Loss and Chronic Tinnitus from Vestibular Schwannoma.

BACKGROUND: Tinnitus is the perception of sound in the absence of external acoustic stimulation. Being one of the most common diseases of the ear, it has a global prevalence ranging from 4.1 to 37.2%. To date, it has been difficult to treat tinnitus as its pathophysiology is poorly understood and there are limited treatment options. OBJECTIVE: To investigate the effect of OKN-007 (also known as HPN-07), a nitrone-based investigational drug, in combination with oral N-acetylcycsteine (NAC), for the treatment of hearing loss and chronic tinnitus under an individual expanded access protocol. PATIENT CASE: We report the case of a patient who presented with left-sided ear fullness, mild tinnitus, and mild high frequency sensorineural hearing loss with 100% word recognition. A large enhancing mass seen on MRI revealed a vestibular schwannoma. He underwent subtotal resection of the tumor resulting in a moderate-to-profound sensorineural hearing loss and catastrophic tinnitus. The patient was treated with intravenous OKN-007 at 60 mg/kg dosed three times per week and oral NAC 2500 mg twice daily. RESULTS: Post-treatment audiometric testing revealed an average of 16.66 dB in hearing threshold improvement in three frequencies (125, 250 and 500 Hz) with residual hearing in the affected left ear. His tinnitus loudness matching improved from 90 dB to 19 dB post-treatment. His Tinnitus Handicap Inventory improved from 86/100 (Catastrophic) to 40/100 (Moderate). He also experienced improvements in sleep, concentration, hearing, and emotional well-being, and reported significantly decreased levels of tinnitusrelated distress. CONCLUSIONS: This case report highlights the feasibility and therapeutic potential of the combination of OKN-007 and NAC in treating hearing loss and tinnitus that warrants further investigation.

[Prospective comparative analysis of cochlear implant patients with single-sided deafness and asymmetric hearing loss with regard to health-related quality of life, tinnitus distress and psychological comorbidities]. / Prospektive Vergleichsanalyse von CI-Patienten mit einseitiger Taubheit und asymmetrischem Hörverlust hinsichtlich der gesundheitsbezogenen Lebensqualität, Tinnitusbelastung und psychischen Komorbiditäten.

BACKGROUND: Patients with single-sided deafness (SSD) and asymmetric hearing loss (AHL) are increasingly being treated with cochlear implants (CI) due to the demonstrated improvements in auditory abilities and quality of life. To date, there are few published studies in which these two groups are comparatively studied. The aim of the current study was to examine which factors differ between those two patient groups, especially preoperatively. METHODS: A secondary analysis of the previously published raw data of 66 prospectively recruited CI patients (21 SSD/45 AHL) was performed. In addition to the hearing outcome, tinnitus distress (tinnitus questionnaire), health-related quality of life (Nijmegen Cochlear Implant Questionnaire, NCIQ), stress (Perceived Stress Questionnaire, PSQ), and psychological comorbidities (General Depression Scale, ADS­L and Generalized Anxiety Disorder scale, GAD-7) were assessed in SSD and AHL patients pre- and postoperatively. RESULTS: Preoperatively, SSD patients showed significantly higher scores in the NCIQ subdomains "elementary" and "advanced sound perception" than the AHL group. Stress (PSQ) and anxiety symptoms (GAD-7) were significantly higher preoperatively in SSD patients than in AHL patients. After CI, these differences were strongly reduced, with minimal differences being detectable between the groups in the investigated domains postoperatively. CONCLUSION: SSD and AHL patients differ significantly preoperatively in terms of their subjective hearing assessment and psychosocial parameters. In SSD patients, psychological stress factors may have a stronger impact on the quality of life than in AHL patients. These aspects should be taken into account in the preoperative counseling and postoperative rehabilitation.

Normative Values for the Inner EAR Scale.

OBJECTIVE: To determine normative values for the Inner Effectiveness of Auditory Rehabilitation (Inner EAR) scale, a validated instrument utilized to study the impact of hearing loss and potential treatments. STUDY DESIGN: Observational outcomes study. SETTING: Academic medical center and community care sites. METHODS: We included patients who were at least 18 years of age and completed the Inner EAR scale, pure-tone audiometry, and word recognition score assessment. Based on audiometry results, patients were categorized as having: (1) normal bilateral hearing, (2) unilateral hearing loss, and (3) bilateral hearing loss. The distributions of Inner EAR scale scores were assessed within each category. Fisher's exact test was utilized to determine whether data-driven threshold values could discriminate among the 3 clinical groups. RESULTS: Two hundred and twenty-two consecutive patients with hearing-related complaints met inclusion criteria. Mean Inner EAR scores for patients with bilateral hearing loss (29.2, interquartile range [IQR] 10-41.5), unilateral hearing loss (38.9, IQR 23-49), and normal hearing (46.6, IQR 31-62) were significantly different (analysis of variance F < 0.0001). An Inner EAR score threshold of 50 supported the ability to statistically significantly discriminate between bilateral hearing loss and normal hearing (p = .003), as well as between unilateral hearing loss and normal hearing (p = .015). CONCLUSION: An Inner EAR score normative threshold value of 50 provides significant discriminatory ability between normal hearing and unilateral or bilateral hearing loss on audiometry. Normative values provide useful, frequently referenced data when assessing responses to treatment. Based on these data, this threshold may help distinguish patients with and without perceived functional impact from hearing loss.

Prevalence of Cochlear Nerve Deficiency and Hearing Device Use in Children With Single-Sided Deafness.

OBJECTIVE: This study aimed to assess the prevalence of cochlear nerve deficiency (CND) in a cohort of pediatric patients with single-sided deafness (SSD). A secondary objective was to investigate trends in intervention and hearing device use in these children. STUDY DESIGN: Case series with chart review. SETTING: Pediatric tertiary care center. METHODS: Children ages 0 to 21 years with SSD (N = 190) who underwent computerized tomography (CT) and/or magnetic resonance imaging (MRI) were included. Diagnostic criteria for SSD included unilateral severe-to-profound sensorineural hearing loss with normal hearing sensitivity in the contralateral ear. Diagnostic criteria for CND included neuroradiologist report of an "aplastic or hypoplastic nerve" on MRI or a "stenotic cochlear aperture" on CT. RESULTS: The prevalence of CND was 42% for children with CT only, 76% for children with MRI only, and 63% for children with both MRI and CT. Of the children with MRI and CT, there was a 90% concordance across imaging modalities. About 36% of children with SSD had hearing devices that routed sound to the normal hearing ear (ie, bone conduction hearing device/contralateral routing of signal), while only 3% received a cochlear implant. Approximately 40% did not have a hearing device. Hearing device wear time averaged 2.9 hours per day and did not differ based on cochlear nerve status. CONCLUSION: There is a high prevalence of CND in children with SSD. Cochlear nerve status should be confirmed via MRI in children with SSD. The limited implementation and use of hearing devices observed for children with SSD reinforce the need for increased support for early and continuous intervention.

[Analysis of audiological features in infants with unilateral hearing loss].

Objective:To investigate the audiological characteristics and possible causes of unilateral hearing loss in infants and young children. Methods:105 infants from Beijing Maternal and Child Health Care Institution who failed the newborn hearing screening and were referred to the Children's Hearing Diagnosis Center of PLA General Hospital for hearing diagnosis. They were diagnosed with unilateral hearing loss and underwent clinical data collection. A full set of audiological examinations included ABR, 40 Hz auditory event related potential, ASSR, DPOAE, tympanometry. Results:①In initial diagnosis, 45 cases（42.86%） had mild hearing loss, 19 cases（18.10%） had moderate hearing loss, 14 cases（13.33%） had severe hearing loss, and 27 cases（25.71%） had severe hearing loss; Among them, 65 cases（61.90%） were conductive hearing loss or mixed hearing loss, and 40 cases（38.10%） were sensorineural hearing loss. ②83 of 105 cases had follow-up visits: 24 cases were normal, 15 cases with mild hearing loss, 4 cases with moderate hearing loss, 12 cases with severe hearing loss, and 26 cases with extremely severe hearing loss, 2 cases of hearing loss in both ears. ③From the initial diagnosis to the follow-up diagnosis, the change of mild hearing loss was the largest, followed by moderate hearing loss, severe and extremely severe hearing loss basically did not change; the number of mild and severe conductive hearing loss which recovered to normal hearing was most, the number of sensorineural hearing loss changed little. Conclusion:The infants who failed the newborn hearing screening and were diagnosed with unilateral hearing loss were mainly mild to moderate conductive hearing loss and severe to extremely severe sensorineural hearing loss. The hearing of children with hearing loss gradually improved, and severe and extremely severe sensorineural hearing loss remained unchanged.

Pediatric Single-Sided Deafness.

The purpose of this article is to outline the current state of evaluating children with unilateral hearing loss, with significant focus on cochlear implantation, in terms of reviewing the key points of the history, including the duration of deafness, outlining the recommended audiometric testing battery, and discussing issues related to imaging of the auditory system and related anatomy. In addition, a comprehensive and up-to-date summary of outcomes in terms of speech perception, sound localization, and quality of life for both the child with unilateral hearing loss as well as their parent(s) is reported.

Children With Unilateral Hearing Loss After Newborn Hearing Screening in Taiwan.

PURPOSE: We explored the intervention characteristics and language outcomes of children with unilateral hearing loss (UHL) in Taiwan after the implementation of universal newborn hearing screening (UNHS) to highlight changes in attitudes and actions toward hearing-related treatments. METHOD: Data of 132 children with UHL in birth cohorts from 2012 to 2019 were included. This retrospective study examined differences in age at identification, hearing aid (HA) fitting, and seeking supportive services. Commonly requested attributes of services, reasons for HA rejection, and children's language performance were investigated. RESULTS: The age at identification decreased from 50.3 months in 2012 to 2.6 months in 2019. Similar trends of declining age were obtained for the age at HA fitting and age at first service contact. In addition, 40% of the parents did not seek support until the child became older (M = 30.5 months) and showed more noticeable behaviors related to hearing loss, and only 64% of the children were consistent HA users. Children with UHL enrolled in the intervention programs approximated the average language performance of the assessment norm; however, they showed depressed language levels when compared to the norm group at the 75th percentile. CONCLUSIONS: UNHS had a positive impact on early identification and intervention in children with UHL. Compared to parents of children with all types of hearing loss, parents of children with UHL seemed to be more uncertain about aural habilitation at the early stage.

Are children with unilateral hearing loss more tired?

OBJECTIVE: To determine whether children with unilateral sensorineural hearing loss (USNHL) and unilateral conductive hearing loss (UCHL) have higher levels of fatigue than literature reported normal hearing (LRNH) children. METHODS: This was a cross-sectional survey utilizing the PedsQL™ Multidimensional Fatigue Scale administered to children with unilateral hearing loss (UHL) and their parents at two tertiary care academic medical centers and a nationwide microtia/atresia conference. The PedsQL™ Multidimensional Fatigue Scale was used to compare child and parental proxy reports of fatigue among USNHL, UCHL, and LRNH children. ANOVA and post-hoc Tukey Honest Significant Difference testing were used for statistical analysis. RESULTS: Of 69 children included in the study, 42 had UCHL (61%) and 27 (39%) had USNHL. Children with USNHL reported more total fatigue (mean 69.1, SD 19.3) than LRNH children (mean 80.5, SD 13.3; difference -11.4; 95% CI: -19.98 to -2.84) and children with UCHL (mean 78.0, SD 14.5; difference -8.95; 95% CI: -17.86 to 0.04). Children with UCHL reported similar levels of fatigue compared to LRNH children (difference -2.5; 95% CI: -9.95 to 5.03). Parents of children with USNHL reported greater levels of fatigue (mean 67.6, SD 22.6) in their children than parents of LRNH children (mean 89.6, SD 11.4; difference -22.0; 95% CI: -29.8 to -14.3) and parents of children with UCHL (mean 76.2, SD 17.3; difference -8.6; 95% CI: -17.5 to 0.21). Parents of children with UCHL also report higher levels of fatigue than parents of LRNH children (difference -13.4; 95% CI: -19.98 to -6.84). CONCLUSIONS: Children with USNHL reported greater levels of fatigue than LRNH children and children with UCHL. Results implicate cognitive load as an important consideration in children with hearing loss. The measurement of fatigue may be a useful indicator to determine the benefit of intervention (e.g., amplification) for these children.

Pediatric Single-Sided Deafness: A Review of Prevalence, Radiologic Findings, and Cochlear Implant Candidacy.

OBJECTIVE: To characterize the prevalence, imaging characteristics, and cochlear implant candidacy of pediatric patients with single-sided deafness (SSD). METHODS: An audiometric database of patients evaluated at a large tertiary academic medical center was retrospectively queried to identify pediatric patients (<18 years old) with SSD, defined as severe to profound sensorineural hearing loss in one ear and normal hearing in the other. Medical records of identified patients were reviewed to characterize the prevalence, etiology, and cochlear implant candidacy of pediatric patients with SSD. RESULTS: We reviewed audiometric data obtained from 1993 to 2018 for 52,878 children at our institution. 191 (0.36%) had the diagnosis of SSD. Cochlear nerve deficiency (either hypoplasia or aplasia) diagnosed on MRI and/or CT was the most common etiology of SSD and was present in 22 of 88 (25%) pediatric SSD patients with available imaging data. 70 of 106 (66%) pediatric SSD patients with available imaging had anatomy amenable to cochlear implantation. CONCLUSIONS: Pediatric SSD is a rare condition and the most common etiology based on radiology is cochlear nerve deficiency. High resolution imaging of the temporal bone is essential to determine cochlear nerve morphology prior to consideration of cochlear implantation.

Bone-anchored hearing system, contralateral routing of signals hearing aid or cochlear implant: what is best in single-sided deafness?

PURPOSE: The aim of the study was to compare long-term results after 1 year in patients with single-sided deafness (SSD) who were fitted with different hearing aids. The participants tested contralateral routing of signals (CROS) hearing aids and bone-anchored hearing systems (BAHS). They were also informed about the possibility of a cochlear implant (CI) and chose one of the three devices. We also investigated which factors influenced the choice of device. METHODS: Prospective study with 89 SSD participants who were divided into three groups by choosing BAHS, CROS, or CI. All participants received test batteries with both objective hearing tests (speech perception in noise and sound localisation) and subjective questionnaires. RESULTS: 16 participants opted for BAHS-, 13 for CROS- and 30 for CI-treatment. The greater the subjective impairment caused by SSD, the more likely patients were to opt for surgical treatment (BAHS or CI). The best results in terms of speech perception in noise (especially when sound reaches the deaf ear and noise the hearing ear), sound localization, and subjective results were achieved with CI. CONCLUSION: The best results regarding the therapy of SSD are achieved with a CI, followed by BAHS. This was evident both in objective tests and in the subjective questionnaires. Nevertheless, an individual decision is required in each case as to which SSD therapy option is best for the patient. Above all, the patient's subjective impairment and expectations should be included in the decision-making process.

Functional Consequences of Poor Binaural Hearing in Development: Evidence From Children With Unilateral Hearing Loss and Children Receiving Bilateral Cochlear Implants.

Poor binaural hearing in children was hypothesized to contribute to related cognitive and academic deficits. Children with unilateral hearing have normal hearing in one ear but no access to binaural cues. Their cognitive and academic deficits could be unique from children receiving bilateral cochlear implants (CIs) at young ages who have poor access to spectral cues and impaired binaural sensitivity. Both groups are at risk for vestibular/balance deficits which could further contribute to memory and learning challenges. Eighty-eight children (43 male:45 female, aged 9.89 ± 3.40 years), grouped by unilateral hearing loss (n = 20), bilateral CI (n = 32), and typically developing (n = 36), completed a battery of sensory, cognitive, and academic tests. Analyses revealed that children in both hearing loss groups had significantly poorer skills (accounting for age) on most tests than their normal hearing peers. Children with unilateral hearing loss had more asymmetric speech perception than children with bilateral CIs (p < .0001) but balance and language deficits (p = .0004, p < .0001, respectively) were similar in the two hearing loss groups (p > .05). Visuospatial memory deficits occurred in both hearing loss groups (p = .02) but more consistently across tests in children with unilateral hearing loss. Verbal memory was not significantly different than normal (p > .05). Principal component analyses revealed deficits in a main cluster of visuospatial memory, oral language, mathematics, and reading measures (explaining 46.8% data variability). The remaining components revealed clusters of self-reported hearing, balance and vestibular function, and speech perception deficits. The findings indicate significant developmental impacts of poor binaural hearing in children.

Overview of Medical Evaluation of Unilateral and Bilateral Hearing Loss in Children.

Early identification, treatment, and medical evaluation of childhood hearing loss are essential to promoting language and social development, regardless of their age of presentation. Evaluation of hearing loss in children should prioritize reversible and treatable causes. Multiple algorithms have been established to address the changing prevalence of genetic or infectious contributions to hearing loss and include recommendations on laboratory testing, imaging, and genetic testing. Despite these recommendations, significant practice variation remains on assessing the etiology of hearing loss in children.

Decision-making following identification of an infant's unilateral hearing loss: Parent and professional perspectives.

BACKGROUND: Parents of infants identified with unilateral hearing loss (UHL) make decisions about managing their infant's hearing loss based on limited evidence and before knowing whether their infant will require additional support. OBJECTIVES: The decision-making processes of parents and clinicians regarding the management of UHL following newborn hearing screening were examined. PROCEDURE: Two convenience samples were recruited: 15 parents of children with permanent UHL aged under 4 years, and 14 clinicians. Applied thematic analysis of the semi-structured interviews elicited insight into the complexities surrounding decision-making from a parent and clinician perspective. RESULTS: Three main themes captured the decision-making process: motivation for decision-making, limited evidence creates uncertainty, and available evidence builds certainty. The diverse experiences and opinions of parents and clinicians highlight the complexity of decision-making when there are contested opinions and no clear best management option. CONCLUSIONS: The choices parents make about the management of their child's UHL can have lifelong implications for their child. Many questions need answering before parents can effectively evaluate the short- and long-term consequences of their options and whether the advantages outweigh the disadvantages in the long-term. This uncertainty is challenging for professionals and parents and risks cognitive biases influencing clinical and parental decision-making.

Factors Associated With Unilateral Hearing Loss and Impact on Communication in US Adults.

OBJECTIVE: To investigate the factors associated with unilateral hearing loss (UHL) and its impact on communication in US adults. STUDY DESIGN: Cross-sectional study. SETTING: Nationally representative sample of US adults. METHODS: We analyzed data from the 2011-2012 and 2015-2016 National Health and Nutritional Examination Survey, in which participants aged 20 to 69 years completed an audiometric evaluation (n = 8138). UHL was defined as a speech frequency pure-tone average ≥25 dB in the worse hearing ear and <25 dB in the better hearing ear. Logistic regression was used to examine the association between UHL and relevant factors. RESULTS: The prevalence of UHL was 8.1% (95% CI, 7.3%-9.0%) in US adults. Factors associated with UHL included older age, male sex, white race, lower level of education, diabetes, cardiovascular disease, and off-work noise exposure. Among adults with UHL, 40% (95% CI, 32%-48%) reported subjective trouble with hearing, a rate higher than the 12% (95% CI, 11%-14%) among normal-hearing adults. After adjusting for relevant factors, adults with UHL were more likely to report difficulties with following conversations with noise (odds ratio [OR], 1.7; 95% CI, 1.2-2.5) and frustration when talking to family and friends (OR, 3.0; 95% CI, 1.9-4.6). Higher levels of communication difficulties were observed with worsening level of UHL. CONCLUSIONS: Adults with UHL report significant communication difficulties in comparison to normal-hearing adults. Further research is needed to understand the psychosocial impact of UHL on adults and ways to improve communication support for adults with UHL.

Another 'BEE'? - Brain-Eye-Ear (BEE) Disease Secondary to HbSC Disease Masquerading as Multiple Sclerosis.

Recurrent episodes of neurological dysfunction and white matter lesions in a young adult raise suspicion for multiple sclerosis (MS). However, occlusive retinopathy, hearing loss and absence of CSF oligoclonal bands are atypical for MS and should make the clinician consider an alternative diagnosis. We describe a man with hearing loss, visual signs and symptoms, and an accumulating burden of brain lesions, who was treated for a clinical diagnosis of MS for nearly two decades. Genetic testing revealed a unifying diagnosis.

Expanding the indications for the bone anchored hearing system (BAHS) in patients with single sided deafness.

PURPOSE: Provide data to support expansion of FDA indications for the Bone anchored hearing system (BAHS). MATERIALS AND METHODS: This retrospective study in a tertiary otologic referral center included106 consecutive subjects who were implanted with a Bone Anchored Hearing System (BAHS) between January 2009 and January 2015 for single sided deafness. Subjects were divided into three groups by bone conduction pure tone average (PTA) of the better hearing ear: 0-20 dB (group 1), 21-40 dB (group 2) and 41-55 dB (group 3). All patients underwent BAHS implantation. Speech perception data (Hearing In Noise Test and Consonant-Nucleus-Consonant testing) was collected before and after surgical intervention. Patient-reported quality of life measures were obtained at least 6 months after activation. These included the Abbreviated Profile of Hearing Aid Benefit and Glasgow Benefit Inventory. RESULTS: All three groups of subjects demonstrated statistically significant improvement in outcome measures following BAHS. Subject reported quality of life outcome measures demonstrated significant improvement in disability from hearing loss and in quality of life. CONCLUSIONS: Patients with single sided deafness who have bone conduction thresholds worse than 20 dB in their contralateral ear are still able to benefit significantly from BAHS.