Comportamento Auditivo e Validação no Diagnóstico Audiológico e Intervenção em Bebês e Crianças com Deficiência Auditiva / Auditory Behavior and Validation in Audiological Diagnosis and Intervention in Infants and Children with Hearing Impairment / Comportamiento auditivo y validación en el diagnóstico e intervención audiológicos en bebés y niños con deficiencia auditiva

Introdução: A atuação profissional com bebês e crianças pequenas com deficiência auditiva exige conhecimento e técnica específica no que diz respeito à prescrição e adaptação do aparelho de amplificação sonora individual (AASI) e ao processo de desenvolvimento de linguagem. Limitações e imprecisões ao longo do processo diagnóstico poderão comprometer todos os procedimentos subsequentes do processo de intervenção. Objetivo: Analisar a validação do processo de diagnóstico audiológico e intervenção em bebês e crianças com deficiência auditiva a partir da análise comparativa de exames audiológicos, comportamento auditivo e aplicação do princípio de verificação cruzada após adaptação de AASI. Método: Foram sujeitos da pesquisa 12 crianças de até 36 meses de idade, com diagnóstico de perda auditiva neurossensorial bilateral, selecionados a partir da disponibilidade de acesso ao serviço para a avaliação e agrupados em G1 (sujeitos com Índice de Inteligibilidade de Fala - SII 65 dB até 35%) e G2 (sujeitos com Índice de Inteligibilidade de Fala - SII 65 dB acima de 54%). Resultados: A média de idade do diagnóstico audiológico foi de 4,33 meses. Os resultados audiológicos de todas as crianças tiveram correspondência entre si, com exceção de dois sujeitos do G2. Conclusão: O comportamento auditivo não só permitiu a validação dos processos de diagnóstico e intervenção auditiva dos sujeitos da pesquisa, como também permitiu a identificação de comportamentos não compatíveis com a audibilidade devido ao uso inconsistente dos AASI. A aplicação dos instrumentos de acompanhamento de desenvolvimento mostrou-se adequada para o monitoramento do desenvolvimento de habilidades de audição e linguagem em crianças pequenas. (AU)

Introduction: Professional work with infants and young children with hearing impairment requires specific knowledge and technique regarding the prescription and adaptation of the individual sound amplification device (PSAD) and the language development process. Limitations and inaccuracies throughout the diagnostic process may compromise all subsequent procedures of the intervention process. Purpose: To analyze the validation of the process of audiological diagnosis and intervention in infants and children with hearing impairment based on the comparative analysis of audiological tests, auditory behavior and application of the cross-checking principle after adaptation of hearing aids. Method: The research subjects were 12 children aged up to 36 months, with a diagnosis of bilateral sensorineural hearing loss, selected from the availability of access to the service for the evaluation and grouped into G1 (subjects with Speech Intelligibility Index - SII 65 dB up to 35%) and G2 (subjects with Speech Intelligibility Index - SII 65 dB above 54%). Results: The average age of the audiological diagnosis was 4.33 months. The audiological results of all children corresponded to each other, except for two subjects from G2. Conclusion: The auditory behavior not only allowed the validation of the processes of diagnosis and auditory intervention of the research subjects, but also allowed the identification of behaviors that are not compatible with audibility due to the inconsistent use of hearing aids. The application of developmental monitoring instruments proved to be adequate for monitoring the development of hearing and language skills in young children. (AU)

Introducción: El trabajo profesional con lactantes y niños pequeños con discapacidad auditiva requiere conocimientos y técnica específicos respecto a la prescripción y adaptación del dispositivo individual de amplificación del sonido (PSAD) y el proceso de desarrollo del lenguaje. Las limitaciones e imprecisiones a lo largo del proceso de diagnóstico pueden comprometer todos los procedimientos posteriores del proceso de intervención. Propósito: Analizar la validación del proceso de diagnóstico e intervención audiológica en lactantes y niños con discapacidad auditiva a partir del análisis comparativo de pruebas audiológicas, conducta auditiva y aplicación del principio de cruce tras adaptación de audífonos. Método: Los sujetos de investigación fueron 12 niños de hasta 36 meses, con diagnóstico de hipoacusia neurosensorial bilateral, seleccionados de la disponibilidad de acceso al servicio para la evaluación y agrupados en G1 (sujetos con Índice de inteligibilidade del Habla - SII 65 dB hasta 35%) y G2 (sujetos com Índice de inteligibilidade del Habla - SII 65 dB por encima del 54%). Resultados:La edad promedio del diagnóstico audiológico fue de 4,33 meses. Los resultados audiológicos de todos los niños se correspondieron entre sí, a excepción de dos sujetos del G2. Conclusión: La conducta auditiva no sólo permitió validar los procesos de diagnóstico e intervención auditiva de los sujetos de investigación, sino que también permitió identificar conductas no compatibles con la audibilidad debido al uso inconsistente de audífonos. La aplicación de instrumentos de seguimiento del desarrollo demostró ser adecuada para controlar el desarrollo de las habilidades auditivas y lingüísticas en niños pequeños. (AU)

Cogan Syndrome: A Case Study and Review of the Literature.

Cogan syndrome is an autoimmune disease characterized by vestibular symptoms, bilateral sensorineural hearing loss, and inflammatory ocular manifestations, which may be accompanied by systemic vasculitis. We herein present the case of a patient with bilateral sensorineural hearing loss who presented with pain over her cochlear implantation incision site. She was later found to have evidence of ocular disease and underlying vasculitis leading to a diagnosis of Cogan syndrome.

Contribuição do potencial evocado auditivo de estado estável na escolha do implante coclear ou aparelho de amplificação sonora em crianças com perda auditiva / Contribution of auditory steady state evoked potential to the election of cochlear implantation or hearing aid for children with hearing impairment / Contribución del potencial evocado auditivo de estado estable en la elección de implantación coclear o audífono en niños con deficiencia auditiva

Introdução: A deficiência auditiva em crianças prejudica a aquisição e o desenvolvimento da linguagem oral, o que pode ser minimizado com o diagnóstico e a confirmação da surdez nos primeiros meses de vida. O Potencial Evocado auditivo de estado estável (PEAEE) destaca-se diante dos demais potenciais evocados auditivos devido à facilidade de registro, à objetividade das respostas, à estimulação de várias frequências simultaneamente, em ambas as orelhas, além da identificação da audição residual. Objetivo: Verificar a contribuição do PEAEE na definição terapêutica (escolha do implante coclear ou aparelho de amplificação sonora) para a reabilitação auditiva de crianças. Método: Foram analisados os registros de 20 crianças de um mês a três anos de idade com perda auditiva neurossensorial de grau severo ou profundo bilateral e que foram submetidas ao PEAEE e ao potencial evocado auditivo de tronco encefálico frequência específica (PEATE-FE). Ambos realizados nas frequências de 500 Hz e 2000 Hz no equipamento Smart-EP Intelligent Hearing Systems®. Resultados: Houve diferença entre os exames quanto à ocorrência de resíduo auditivo, pois, um número significativo de indivíduos apresentou respostas ausentes no PEATE-FE e respostas presentes no PEAEE. Não ocorreu associação entre a presença de resíduo auditivo, o grau da perda e a idade da criança com o tipo de intervenção terapêutica. Conclusão: A presença de resíduo auditivo, a classificação do grau da perda e a idade da criança não influenciaram na conduta terapêutica final. (AU)

Introduction: Hearing impairment in children debilitates the acquisition and development of oral language, which can be minimized with diagnosis and confirmation of deafness in the first months of life. Auditory Steady State Evoked Potential (ASSEP) analysis stands out from others auditory evoked potentials due to the ease of recording, objectivity of the answers, stimulation of several frequencies simultaneously, in both ears, besides the identification of residual hearing. Purpose: Determine the contribution of the ASSEP for the therapeutic definition (election of cochlear implantation or hearing aid device) in hearing rehabilitation of children. Methods: The records of 20 children aged one month to three years with severe or profound bilateral neurosensory hearing loss, who were submitted to ASSEP and specific frequency brainstem auditory evoked potential (BAEP) analysis were analyzed. Both tests performed at frequencies of 500 Hz and 2000 Hz using the equipment Smart-EP Intelligent Hearing Systems®. Results: There was difference between the exams regarding the occurrence of residual hearing, since a significant number of individuals had absent responses on the BAEP and present responses on the ASSEP. There was no association between the presence of residual hearing, degree of hearing loss and the child's age with the type of therapeutic intervention. Conclusion: The presence of residual hearing, classification of the degree of loss and child's age exerted no influence on the final conduct. (AU)

Introducción: La deficiencia auditiva en niños hace daño a la adquisición y el desarrollo del lenguaje oral, que se puede minimizar con el diagnóstico y confirmación de la sordera en los primeros meses de vida. El Potencial Evocado de Estado Estable (PEAEE) se destaca de los demás potenciales evocados auditivos por la facilidad de registro, objetividad de las respuestas, estimulación de varias frecuencias simultáneamente, en ambos oídos, además de la identificación de audición residual. Objetivo: Verificar la contribución del PEAEE para la definición de las terapéuticas adoptadas (elección de implantación coclear o audífono) en la rehabilitación auditiva de niños. Método: Fueron analizados los registros de 20 niños de un mes a tres años de edad con pérdida auditiva sensorineural de grado severo o profundo bilateral y que fueron sometidas al PEAEE y al potencial evocado auditivo de tronco encefálico por frecuencia específica (PEATE-FE). Ambos se realizaron en las frecuencias de 500 Hz y 2000 Hz en el equipo Smart-EP Intelligent Hearing Systems®. Resultados: Hubo diferencia entre los exámenes con respecto a la ocurrencia de residuo auditivo, dado que, un número significativo de sujetos presentaron respuestas ausentes en PEATE-FE y respuestas presentes en PEAEE. No hubo asociación entre la presencia de residuo auditivo, el grado de pérdida y la edad del niño con el tipo de intervención terapéutica. Conclusión: La presencia de residuo auditivo, clasificación del grado de pérdida y edad del niño no influyeron en la conducta final. (AU)

Universal neonatal hearing screening and delayed hearing loss or late-developmental hearing loss.

OBJECTIVE: To determine the percentage of children with permanent bilateral postnatal hearing loss in order to study its incidence, related risk factors, diagnosis and treatment. METHODS: Retrospective study to collect data on children diagnosed with hearing loss outside the neonatal period in the Hearing Loss Unit of the Hospital Universitario Central de Asturias, from April 2014 to April 2021. RESULTS: 52 cases met the inclusion criteria. The detection rate of congenital hearing loss in the neonatal screening programme in the same study period was 1.5 children per thousand newborns per year, adding postnatal hearing loss results in a rate of infant bilateral hearing loss of 2.7 children per thousand (55.5% and 44.4% respectively). Thirty-five children presented risk factors for hearing loss, of which 23 were at retrocochlear risk. The mean age at referral was 91.9 (18-185) months. Hearing aid fitting was indicated in 44 cases (84.6%). Cochlear implantation was indicated in eight cases (15.4%). DISCUSSION: Although congenital hearing loss accounts for the majority of childhood deafness, postnatal hearing loss has a significant incidence. This may be mainly due to: (1) that hearing impairment may arise in the first years of life, (2) that mild hearing loss as well as hearing loss in severe frequencies are undetectable by neonatal screening in some cases, (3) that some children may have false negative results. CONCLUSION: postnatal hearing loss requires identification of risk factors and long-term follow-up of children with hearing loss, as it needs to be detected and treated early.

Targeted Next-Generation Sequencing in Children With Bilateral Sensorineural Hearing Loss: Diagnostic Yield and Predictors of a Genetic Cause.

OBJECTIVE: To investigate the diagnostic yield of targeted next-generation sequencing using hearing loss panels and to identify patient-related factors that are associated with a definite genetic cause. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Children with congenital or late-onset, bilateral sensorineural hearing loss. INTERVENTIONS: Diagnostic. MAIN OUTCOME MEASURES: The number of patients with a definite genetic diagnosis. RESULTS: We report on 238 patients with hearing loss: 130 were male and 108 were female. About 55% had congenital hearing loss. A genetic cause was identified in 94 of the patients (39.5%), with 72.3% of these showing nonsyndromic and 27.6% showing syndromic hearing loss. The diagnostic yield was highest among North African patients (66.7%). A multiple linear regression model shows that profound hearing loss, family history of hearing loss, congenital hearing loss, and North African ethnicity are significantly related to identifying a genetic cause. CONCLUSIONS: Targeted next-generation sequencing using a panel of hearing loss genes identified a genetic diagnosis in almost 40% of children with bilateral sensorineural hearing loss. We describe the predictors of a genetic diagnosis, and this information may be used during genetic counseling.

Association of Genetic Diagnoses for Childhood-Onset Hearing Loss With Cochlear Implant Outcomes.

Importance: In the US, most childhood-onset bilateral sensorineural hearing loss is genetic, with more than 120 genes and thousands of different alleles known. Primary treatments are hearing aids and cochlear implants. Genetic diagnosis can inform progression of hearing loss, indicate potential syndromic features, and suggest best timing for individualized treatment. Objective: To identify the genetic causes of childhood-onset hearing loss and characterize severity, progression, and cochlear implant success associated with genotype in a single large clinical cohort. Design, Setting, and Participants: This cross-sectional analysis (genomics) and retrospective cohort analysis (audiological measures) were conducted from 2019 to 2022 at the otolaryngology and audiology clinics of Seattle Children's Hospital and the University of Washington and included 449 children from 406 families with bilateral sensorineural hearing loss with an onset younger than 18 years. Data were analyzed between January and June 2022. Main Outcomes and Measures: Genetic diagnoses based on genomic sequencing and structural variant analysis of the DNA of participants; severity and progression of hearing loss as measured by audiologic testing; and cochlear implant success as measured by pediatric and adult speech perception tests. Hearing thresholds and speech perception scores were evaluated with respect to age at implant, months since implant, and genotype using a multivariate analysis of variance and covariance. Results: Of 406 participants, 208 (51%) were female, 17 (4%) were African/African American, 32 (8%) were East Asian, 219 (54%) were European, 53 (13%) were Latino/Admixed American, and 16 (4%) were South Asian. Genomic analysis yielded genetic diagnoses for 210 of 406 families (52%), including 55 of 82 multiplex families (67%) and 155 of 324 singleton families (48%). Rates of genetic diagnosis were similar for children of all ancestries. Causal variants occurred in 43 different genes, with each child (with 1 exception) having causative variant(s) in only 1 gene. Hearing loss severity, affected frequencies, and progression varied by gene and, for some genes, by genotype within gene. For children with causative mutations in MYO6, OTOA, SLC26A4, TMPRSS3, or severe loss-of-function variants in GJB2, hearing loss was progressive, with losses of more than 10 dB per decade. For all children with cochlear implants, outcomes of adult speech perception tests were greater than preimplanted levels. Yet the degree of success varied substantially by genotype. Adjusting for age at implant and interval since implant, speech perception was highest for children with hearing loss due to MITF or TMPRSS3. Conclusions and Relevance: The results of this cross-sectional study suggest that genetic diagnosis is now sufficiently advanced to enable its integration into precision medical care for childhood-onset hearing loss.

Characteristics and prognosis analysis of bilateral sudden sensorineural hearing loss: A retrospective case-control study.

OBJECTIVES: To evaluate the clinical characteristics and prognostic factors of simultaneous and sequential bilateral sudden sensorineural hearing loss (Si-BSSNHL and Se-BSSNHL, respectively). DESIGN: Retrospective case-control study. SETTING: A single tertiary referral centre. PARTICIPANTS: Patients diagnosed with unilateral sudden sensorineural hearing loss (USSNHL), Si-BSSNHL, or Se-BSSNHL between September 2018 and November 2019. MAIN OUTCOME MEASURES: Demographic and clinical characteristics, audiological features, laboratory results and hearing recovery were analysed for intergroup comparisons. Prognostic factors for BSSNHL were analysed using univariate and multivariate logistic analyses between the overall and no-recovery groups. RESULTS: Compared to the USSNHL group, a larger final pure-tone average (PTA) (H = 38.0 and 53.8, respectively, both adjusted p-value (p adj) <.05), lower hearing gain (H = -70.8 and - 74.6, respectively, both p adj <.001) and higher homocysteine levels (H = 46.8, 58.8, respectively, both p adj <.05) were observed in the Si-BSSNHL and Se-BSSNHL groups, while the rate of positive vestibular tests and proportion of tinnitus were lower in the Se-BSSNHL group (χ2  = 8.5 and 38.1, respectively, both p adj <0.05). The USSNHL group showed a significant difference in the degree of deafness and therapeutic outcome in the Se-BSSNHL and Si-BSSNHL groups, respectively (χ2  = 12.4, p adj <.05; χ2  = 13.6; p adj <.05). Hypertension (95% confidence interval, 1.014-28.623, p < .05) and onset days (95% confidence interval, 0.007-0.626, p < .05) were associated with the therapeutic effects of BSSNHL. CONCLUSIONS: Higher homocysteine levels in BSSNHL may implicate microvascular disorders as a causative factor of BSSNHL. Hypertension and onset days were associated with the prognosis of BSSNHL.

Expressive vocabulary of school-age children with mild to moderately severe hearing loss.

OBJECTIVES: The main goal of this study was to describe the expressive vocabulary of school-age children with mild to moderately severe hearing loss (CHL group) and to compare their performance with children with normal hearing (CNH group) of the same age. Another aim was to examine the interaction between nonword repetition and expressive vocabulary size. Furthermore, the interaction between results on vocabulary tests and background factors, such as the age of diagnosis, age of hearing aid fitting, and amount of hearing aid use, was explored. METHOD: School-aged children with mild to moderately severe, permanent bilateral hearing loss and children with normal hearing were included in this cross-sectional study. The children participated in assessments of naming pictures, defining words, and repetition of nonwords and sentences. Results of the CHL group and the CNH group were compared. The analysis also included the degree of hearing loss, the age of diagnosis, amount of hearing aid use, and level of parental education. RESULTS: The CHL group performed significantly below the CNH group on all measures: picture naming, defining words, nonword repetition, and repetition of sentences. The proportion of words pronounced with correct phonological structure when picture naming was more limited in the CHL group than in the CNH group. There was a significant positive correlation between the amount of hearing aid use and nonword repetition ability in the CHL group. Age of diagnosis and age of hearing aid fitting was not significantly correlated with the outcomes of the vocabulary assessments in this study. CONCLUSION: Despite the technological advancement of hearing aids, the expressive vocabulary in school-aged children with mild to moderately severe, permanent bilateral, hearing impairment does not reach the same level as for children with normal hearing, although there is a variation in performance within the group. The variation in the CHL group was not uniquely impacted by either age, degree of hearing loss, or the age of diagnosis. The amount of hearing aid use seems to impact the perception of new words. More studies of expressive vocabulary are needed, because they capture a dimension of word learning that seems particularly sensitive to hearing loss and hearing aid use.

Hearing aid benefits in children with mild bilateral hearing loss: AUDIO-INFANS study.

OBJECTIVES: There is a lack of consensus regarding the definition, consequences, and management of mild bilateral hearing loss in children. The objective of this study is to analyze the benefit of hearing aids in children with mild bilateral hearing loss by evaluating their functional hearing. METHODS: This retrospective study included 57 children with mild bilateral hearing loss between 20 dB HL and 40 dB HL. Pure tone and speech audiometry thresholds were assessed with and without hearing aids. Two groups were subsequently formed: group E with an effective use of hearing aids (>10 h/day), and group IE whose use of hearing aids was deemed ineffective (<10 h/day). RESULTS: Without hearing aids, the initial median of hearing level was 35 dB HL and 36 dB HL in the right and left ears, respectively, compared to 23 dB HL and 25 dB HL with hearing aids. The Lafon test performed on 25 children at 55 dB HL and 65 dB HL showed results ranging from 0% to 100% without hearing aids and from 90% to 100% with hearing aids. Scores obtained with hearing aids were significantly higher than those without them at an average speech level. Median age at diagnosis and at prescription were found to significantly influence the daily use of hearing aids. CONCLUSIONS: Our results show that in the case of mild bilateral hearing loss, hearing aids have positive effects on the functional hearing of children and help them to no longer be disadvantaged. This study highlights the need to provide regular support to these children to ensure their optimal care in the event of hearing-related problems. Coordination between the different professionals working with these children is also necessary for their follow-up and appropriate management.

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.

Factors affecting phoneme discrimination in children with sequential bilateral cochlear implants.

OBJECTIVES: To investigate the effects of a number of variables on phoneme discrimination (PD) performance in children with sequential bilateral cochlear implants (SeqBiCIs) and compare PD performance between the 2 implantation sides and between children with bilateral cochlear implants (BiCIs) and their age-matched peers with normal hearing (NH). DESIGN: All participants completed the Auditory Speech Sound Evaluation Phoneme Discrimination Test. STUDY SAMPLE: The sample included 23 children with SeqBiCIs as the study group and 23 with NH as the control group. RESULTS: A significant difference was found between the scores of the two groups under the CI1 and CI2 conditions (p = 0.001), CI1 and BiCI conditions (p = 0.002), and CI2 and BiCI conditions (p = 0.001). PD scores with CI1 significantly depend on age at CI1 and duration of bilateral use. PD scores with CI1 were significant predictors of PD performance with CI2. Duration of BiCI use was a significant predictor of PD scores with BiCI. CONCLUSIONS: The age at CI1 and the duration of bilateral cochlear implant use were found to improve phoneme discrimination performance in children with a sequential bilateral cochlear implant. According to the success of the CI1, it is possible to predict the success of CI2 use.

Cryptococcal meningitis presenting with acute hearing loss.

Reversible sensorineural hearing loss is a recognised complication of cryptococcal meningitis. Cryptococcal meningitis typically presents with usual symptoms of fever, headache and neck stiffness. This case highlights acute, profound, bilateral hearing loss as the initial symptom and presentation of cryptococcal meningitis in a young woman, who was later diagnosed with AIDS.

Aetiologies of profound bilateral sensorineural hearing loss among children in Ekiti State, South Western Nigeria.

INTRODUCTION: a strong need exists for the knowledge of aetiologies of diseases as this will guide the clinicians on the strategies for their treatment and prevention. In this study, we determined the aetiologies of profound bilateral sensorineural hearing loss (pbSNHL) with a view to provide the relevant data needed for preventive and therapeutic intervention among children in Ekiti State, South Western Nigeria. METHODS: medical records of children with pbSNHL seen over a ten-year period were analysed. RESULTS: in all, records of 142 children with pbSNHL were analysed. The results showed spectrum of aetiologies that were similar to those reported decades ago with the 'unknown' assuming a recurring decimal. Of the known (acquired) cases, measles takes up the largest 'chunk' with a prevalence of 45.8%. Twenty-eight (32.2%) of those with febrile illnesses had treated their fever with ototoxic antibiotics. Late diagnosis was characteristic of the pbSNHL. CONCLUSION: this study shows that there hasn't been any significant shift in the common causes of pbSNHL. Of great concern is the persistence of the 'unknown' causes which assumes a recurring decimal in this and previous studies. Also worrisome is the high prevalence of measles-induced pbSNHL despite the availability of anti-measles vaccine. We therefore suggest effective immunization against measles and other vaccine-preventable causes of pbSNHL. The need for intensive research on the unknown causes of pbSNHL is hereby stressed. Also recommended is routine hearing assessment for survivors of febrile conditions so as to detect, if any, hearing loss promptly.

Localizing Thalamomesencephalic Afferent and Efferent Pupillary Defects.

ABSTRACT: A 42-year-old Algerian man presented for binocular oblique diplopia, hypersomnolence with drop attacks, bilateral hearing loss, and thoracic pain. He had a right thalamomesencephalic hemorrhage due to an underlying cavernous malformation treated with subtotal surgical resection. On neuro-ophthalmic examination, the patient had a left relative afferent pupillary defect and a right oculosympathetic efferent pupillary defect (i.e., Horner syndrome) in addition to other thalamomesencephalic eye and neurologic signs (right fourth nerve palsy, hearing loss, hemiparesis, and thalamic pain). Clinicians should recognize the localizing value of this unique constellation of mesencephalic afferent and efferent pupillary defects.

Delayed-Onset NOG Gene-Related Syndromic Conductive Deafness: A Case Report.

We report a 6-year-old girl with progressive bilateral conductive hearing loss for 2 years. She passed the newborn hearing screening conducted with otoacoustic emissions testing and had a normal development of speech and language, which indicated that her deafness was delayed-onset. She also had congenital proximal interphalangeal joints. Proximal symphalangism was confirmed by genetic testing (NOG gene: c.406C > T, p.R136C). Bilateral stapes ankyloses were proved by surgery and her hearing was improved after stapedotomy by over 30 dB. Besides, this case should remind clinicians to carefully distinguish NOG gene-related deafness from congenital ossicular malformation and pediatric otosclerosis.

Comparison of Speech Performance in Bimodal versus Bilateral Cochlear Implant Users.

OBJECTIVE: To assess whether listening with two cochlear implants (bilateral) offers significant benefits in terms of speech perception over listening with one cochlear implant and one hearing aid (bimodal). METHODS: Retrospective review of bilateral cochlear implant recipients (24 pediatric and 26 adult). Bimodal listening was compared to bilateral listening in terms of speech perception performance at 1-year post second implant under three listening conditions: 50 dBHL, 35 dBHL, and 50 dBHL+5 SNR. Changes in speech performance from bimodal (before second implant) to bilateral (after second implant) listening were determined within subjects and compared to a separate control group of bimodal users matched for age of first implantation who never received a second implant (10 pediatric and 20 adult). RESULTS: In the pediatric group, compared to bimodal listening prior to a second implant, speech perception scores with bilateral implants increased significantly when measured at 50 dBHL, 35 dBHL, and 50 dBHL+5 SNR. By contrast, pediatric bimodal controls who never received a second implant failed to demonstrate similar improvement over 1 year's time. In the adult group, compared to bimodal listening prior to a second implant, speech perception scores with bilateral implants increased when measured at 50 dBHL, but were not significantly different at 35 dBHL and 50 dBHL + 5 SNR. Adult bimodal controls who never received a second implant failed to demonstrate significant improvement in all conditions over 1 year's time. CONCLUSION: Bilateral listening with two cochlear implants improved speech perception performance relative to bimodal listening in the pediatric population. Improvement in the adult population was not as significant. LEVEL OF EVIDENCE: 4, Retrospective Chart Review. Laryngoscope, 131:E1322-E1327, 2021.

Determining unilateral or bilateral hearing aid preference in adults: a prospective study.

OBJECTIVE: Despite high rates of bilateral hearing aid fitting globally, a number of adults continue to reject one hearing aid. The current study aimed to identify a clinically suitable tool for determining, pre-fitting, which clients might prefer one hearing aid.Study Sample: Ninety-five new adult hearing aid candidates, aged 49-87 years, were assessed prior to a first hearing aid fitting. Sixty-eight participants adhered to the prescribed protocol for both bilateral and unilateral hearing aid use. DESIGN: Performance was assessed on a modified version of the Listening in Spatialised Noise - Sentences test (LiSN-S), the Dichotic Digits difference Test, the Experiential Hearing Aid simulator, and the Grooved Pegboard Test. All participants were fitted bilaterally, but were instructed to alternate between unilateral and bilateral hearing aid use over fourteen weeks post-fitting. Participants' wearing preferences were assessed via a short questionnaire. RESULTS: 78% of participants expressed an overall preference for bilateral hearing aid use. Only the LiSN-S bilateral advantage test outcomes significantly correlated with overall wearing preference. CONCLUSIONS: Although the LiSN-S bilateral advantage score related to overall wearing preference, the accuracy of the predictor was too low to warrant implementation of this test prior to hearing aid fitting. The current practice of recommending bilateral hearing aid use continues to be the best option for clinicians.