[Recognition of unilateral auditory neuropathy in children].

Objective: This study aimed to compare the audiological characteristics between children with unilateral auditory neuropathy (UAN) and single-sided deafness (SSD) to establish a valid basis for the differential diagnosis of children with UAN. Methods: A retrospective analysis was conducted on audiological and imaging evaluations of children with UAN and SSD who were treated at Beijing Children's Hospital of Capital Medical University between May 2015 and June 2023. There were 17 children with UAN, comprising 10 males and 7 females, with an average age of 4.7 years. Additionally, there were 43 children with SSD, consisting of 27 males and 16 females, with an average age of 6.5 years. Audiological assessments included Auditory brainstem response (ABR), Steady-state auditory evoked potential (ASSR), Behavioural audiometry, Cochlear microphonic potential (CM), Distortino-product otoacoustic emission (DPOAE), and acoustic immittance test. The results of the audiological assessment and imaging phenotypic between the two groups of children were compared and analyzed by applying SPSS 27.0 statistical software. Results: (1) The UAN group (77.8%) had a significantly higher rate of ABR wave IIIL than the SSD group (20.9%) (P<0.01). The PA thresholds at 500 Hz and 1 000 Hz of children with SSD were higher than those of children with UAN, while the ASSR thresholds at 500 Hz, 1000 Hz, 2 000 Hz, and 4 000 Hz of children with SSD were significantly higher than those of children with UAN (P<0.05). (2) The degree of hearing loss in both UAN and SSD children was predominantly complete hearing loss. The percentage of complete hearing loss was significantly higher (χ²=4.353, P=0.037) in the SSD group (93.0%, 40/43) than in the UAN group (63.6%, 7/11). However, the percentage of profound hearing loss was significantly higher in the UAN group (27.3%, 3/11) than in the SSD group (2.3%, 1/43) (Fisher's exact test, P=0.023). In terms of hearing curve configuration, the percentage of flat type was significantly higher in the SSD group (76.7%, 33/43) than in the UAN group (36.4%, 4/11). The proportion of the UAN group (27.3%, 3/11) was significantly higher than that in the SSD group (2.3%, 1/43) in ascending type (P<0.05). There were no statistically significant differences in the hearing curves of the declining type and other types between the two groups (P>0.05). (3) The proportion of imaging assessment without abnormality was significantly more common in the UAN group (81.8%) than in the SSD group (37.1%) (χ²=6.695, P=0.015). Conclusions: Compared to children with SSD, the occurrence of wave IIIL on the ABR test was significantly more common in children with UAN. The percentage of ascending hearing curves was significantly higher in children with UAN than in children with SSD. ASSR thresholds were significantly lower in children with UAN. The normal imaging phenotype was significantly more common in children with UAN than in children with SSD.

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.

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.

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.

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.

Sudden onset hearing loss following intra-abdominal surgery: an unusual association.

We describe the case of a 12-year-old boy who reported unilateral hearing loss following laparoscopic appendicectomy for acute appendicitis under general anaesthesia. He was otherwise fit and well with no previous otological history. Formal audiological assessment by pure tone audiogram demonstrated a unilateral high-frequency sensorineural hearing loss (SNHL).In addition to describing his clinical course, a literature review of SNHL following non-otological surgery was performed. We recommend an awareness of this phenomenon, necessitating its prompt recognition, early audiological assessment and management as per sudden onset SNHL guidelines.

Endoscopic findings and long-term hearing results for pediatric unilateral conductive hearing loss.

OBJECTIVES: Analyze reasons for unilateral conductive hearing loss (CHL) with unknown etiology in children. INTRODUCTION: Unilateral conductive hearing loss (HL) without known etiology can be undiagnosed despite of hearing screening programs. It can be difficult to find the reason for HL and to make a treatment plan. Middle ear endoscopy gives hard-evidence diagnosis and basis for an individual treatment plan. METHODS AND MATERIAL: Prospective clinical follow-up study for a cohort of generally healthy elementary school age children with unilateral conductive HL with unknown etiology. The study population was 192 children, of which 46 had a HL of at least 25 dB with more than 10 dB conductive component. Mean age was 8.7 years. Preoperative tests included otomicroscopy, bone- and air-conduction audiogram, tympanometry, stapes reflex tests, Rinne and Weber test and Otoacoustic emissions. The children underwent endoscopy of the middle ear with an individual treatment plan and long-term follow-up. The aim was to explore etiology and to give a treatment plan for hearing loss. Follow-up included air- and bone conduction hearing tests annually or every other year. Mean follow-up was 5.2 years. RESULTS: A clear etiological finding was found in 36 (78%) ears, stapes anomaly (23) as the most common (64%) finding. Other findings were two cholesteatomas, 2 status after trauma, 5 middle ear anomalies, 5 incus fixations and one incus erosion. Air conduction hearing improved spontaneously during follow-up in 81% (17/21, 2 dropouts) of the stapes anomaly ears (mean 11,3 dB, range 4-32 dB), and none of these ears showed hearing deterioration. In the incus fixation group, one ear showed hearing deterioration. There were no major complications for exploration, and 5 minor postoperative infections. CONCLUSIONS: The most common reason for pediatric unilateral conductive hearing loss was stapes anomaly/fixation. The HL does not deteriorate. Hearing loss in stapes anomalies shows a tendency for spontaneous recovery. Stapes surgery can be postponed or avoided.

Quality of Life in Children with Sensorineural Hearing Loss.

OBJECTIVE: To assess quality of life (QOL) in pediatric patients with sensorineural hearing loss (SNHL) with the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) and the Hearing Environments and Reflection on Quality of Life 26 (HEAR-QL-26) and HEAR-QL-28 surveys. STUDY DESIGN: Prospective longitudinal study. SETTING: Tertiary care center. SUBJECTS AND METHODS: Surveys were administered to patients with SNHL (ages 2-18 years) from July 2016 to December 2018 at a multidisciplinary hearing loss clinic. Patients aged >7 years completed the HEAR-QL-26, HEAR-QL-28, and PedsQL 4.0 self-report tool, while parents completed the PedsQL 4.0 parent proxy report for children aged ≤7 years. Previously published data from children with normal hearing were used for controls. The independent t test was used for analysis. RESULTS: In our cohort of 100 patients, the mean age was 7.7 years (SD, 4.5): 62 participants had bilateral SNHL; 63 had mild to moderate SNHL; and 37 had severe to profound SNHL. Sixty-eight patients used a hearing device. Mean (SD) total survey scores for the PedsQL 4.0 (ages 2-7 and 8-18 years), HEAR-QL-26 (ages 7-12 years), and HEAR-QL-28 (ages 13-18 years) were 83.9 (14.0), 79.2 (11.1), 81.2 (9.8), and 77.5 (11.3), respectively. Mean QOL scores for patients with SNHL were significantly lower than those for controls on the basis of previously published normative data (P < .0001). There was no significant difference in QOL between children with unilateral and bilateral SNHL or between children with SNHL who did and did not require a hearing device. Low statistical power due to small subgroup sizes limited our analysis. CONCLUSION: It is feasible to collect QOL data from children with SNHL in a hearing loss clinic. Children with SNHL had significantly lower scores on validated QOL instruments when compared with peers with normal hearing.

Imaging findings in pediatric single-sided deafness and asymmetric hearing loss.

OBJECTIVE: To examine the imaging findings on computer tomography (CT) and magnetic resonance imaging (MRI) in pediatric single-sided deafness (SSD) and asymmetric hearing loss (ASH). METHODS: The medical records of 189 pediatric patients with SSD and ASH were retrospectively reviewed, and imaging findings were compared. SSD was defined as unilateral profound hearing loss and contralateral normal hearing ear. In the ASH group, ASHw was defined as the worse hearing ear with profound hearing loss, while ASHb was defined as the better hearing ear with mild-moderate hearing loss. RESULTS: There were 170 patients with SSD and 19 patients with ASH. In the SSD group, 83 patients (48.8%) had imaging findings associated with hearing loss. In the ASH group, such imaging findings were found in six (31.6%) of the ASHw and in five (26.3%) of the ASHb ears. The most common finding in the SSD group was cochlear nerve deficiency (50.6%), followed by cochlear dysplasia (39.8%) and enlarged vestibular aqueduct (26.5%). In the ASH groups, cochlear dysplasia was seen in three (50%) of ASHw ears and in two (40%) of the ASHb ears, and enlarged vestibular aqueduct was seen in three (50%) of ASHw ears and in two (40%) of the ASHb ears. CONCLUSION: Imaging studies identified the etiology in half of the cases of SSD and in one-third of ASH patients. Our findings strongly support the use of imaging studies in the evaluation of pediatric SSD and ASH. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:1007-1010, 2020.

Minimal invasive pocket technique for magnet bone implant hearing aid without fixation.

INTRODUCTION: The BAHA (bone-anchored hearing aid) Attract is a magnetic transcutaneous bone conduction device anchored into the temporal bone. The standard surgical technique for BAHA Attract is a multi-tools time-consuming process, which requires a large cutaneous incision. The objective of this study is to describe and test the feasibility of a minimally invasive pocket (MIP) technique for Magnet Bone Implant Hearing Aid (MBIHA) with a modified magnet of BAHA Attract without fixation and without any tissue reduction. We use a 3-cm vertical skin incision and a subperiosteal pocket. METHOD: A study of 10 patients with conductive or mixed hearing loss who benefited from a MBIHA using the MIP technique is presented. The pure tone average (PTA) (dB) for air-conduction thresholds and the speech recognition threshold (SRT) (dB) in speech audiometry in quiet are calculated. The Entific Medical Systems (EMS) questionnaire and the postoperative clinical outcomes are realized. RESULTS: We found a significant improvement of 33.8 dB on average for the PTA and 44.8 dB for the SRT with MBIHA at 3 months, compared with unaided situation. No implant was removed or displaced after 2 years of follow-up. The skin condition remains intact in all the cases. CONCLUSION: The minimally subperiosteal pocket surgical technique MIP without fixation and with tissue preservation for the MBIHA is safe, rapid and effective for patients with conductive or mixed hearing loss. It opens new perspectives of development and modify conventional concept in magnetic coupling of bone-conducted device.

Perda auditiva unilateral e assimétrica na infância / Unilateral and asymmetric hearing loss in childhood

RESUMO Objetivo Descrever a perda auditiva sensorioneural unilateral e bilateral assimétrica em crianças quanto às características etiológicas, audiológicas e demográficas. Método Estudo retrospectivo transversal, desenvolvido na Seção de Implante Coclear do Hospital de Reabilitação de Anomalias Craniofaciais, por meio da análise de prontuários. Resultados Foram analisados os dados de 1152 pacientes, sendo 424 (37%) adolescentes, adultos ou idosos e 728 (63%) crianças, dentre as quais, 691 (95%) apresentavam perda auditiva bilateral simétrica e 37 (5%) perda auditiva sensorioneural unilateral (n=10) ou bilateral assimétrica (n=27). A idade média ao diagnóstico na perda auditiva sensorioneural unilateral foi de 33,58±21,69 meses e na bilateral assimétrica de 33,12±21,69 meses, com prevalência de 1,4% e 3,7%, respectivamente. O indicador de risco para a deficiência auditiva de maior ocorrência para ambos os grupos foi o de antecedente familiar. A maioria dos familiares das crianças com perda auditiva sensorioneural unilateral apresentaram a classificação socioeconômica baixa superior (50%), enquanto que as crianças com perda auditiva sensorioneural bilateral assimétrica se subdividiram igualmente em baixa superior (37%) e média inferior (37%). Conclusão Houve uma maior prevalência da perda auditiva sensorioneural bilateral assimétrica em relação à unilateral, bem como do indicador de risco de hereditariedade, com predomínio do grau profundo na pior orelha e preponderância do sexo feminino, em ambos os grupos. Apesar de a triagem auditiva neonatal propiciar a identificação precoce da perda auditiva sensorioneural unilateral, a idade no diagnóstico audiológico ainda se encontra acima do recomendado. Adicionalmente, a maioria dos familiares das crianças apresentou nível de rendimento baixo.

ABSTRACT Purpose To describe unilateral and bilateral asymmetric sensorineural hearing loss in children and its etiological, audiological and demographic characteristics. Methods Retrospective cross-sectional study developed in the Seção de Implante Coclear of Hospital de Reabilitação de Anomalias Craniofaciais, through the analysis of medical records. Results Data from 1152 patients were analyzed: 424 (37%) adolescents, adults or elderly, and 728 (63%) children, of whom 691 (95%) had bilateral symmetrical hearing loss, and 37 (5%) had unilateral hearing loss (n=10) or bilateral asymmetric (n=27) sensorineural hearing loss. The mean age at diagnosis of unilateral sensorineural hearing loss was 33.58±21.69 months, and for asymmetric bilateral it was 33.12±21.69 months, with a prevalence of 1.4% and 3.7%, respectively. The highest risk indicator for hearing loss for both groups was the family history of permanent deafness, which began in childhood. The majority of the relatives of children with unilateral sensorineural hearing loss presented the highest low socioeconomic classification (50%), while children with bilateral asymmetric sensorineural hearing loss were also be subdivided into upper (37%) and lower (37%). Conclusion We observed a greater occurrence of asymmetric bilateral sensorineural hearing loss compared to unilateral hearing loss, as well as the hereditary risk indicator, with a predominance of the deep ear and female preponderance in both groups. Although neonatal hearing screening provides early identification of unilateral sensorineural hearing loss, the age at the audiological diagnosis is still above the recommended level. In addition, the majority of the children's family members presented a low level of income.

Unilateral hearing loss: benefit of amplification in sound localization, temporal ordering and resolution / Perda auditiva unilateral: benefício da amplificação na ordenação e resolução temporal e na localização sonora

ABSTRACT Purpose To assess the hearing abilities of temporal ordering, temporal resolution and sound localization before and after the fitting of a hearing aid (HA) in individuals with unilateral hearing loss (UHL). Methods There were evaluated 22 subjects, aged 18 to 60 years, diagnosed with sensorineural or mixed UHL, from mild to severe degrees. The study was divided into two stages: the pre and post-adaptation of HA. In both phases, subjects performed an interview, application of Questionnaire for Disabilities Associated with Impaired Auditory Localization, auditory processing screening protocol (APSP) and Random Gap Detection Test (RGDT). Results This study found no statistically significant difference in sound localization and memory evaluations for verbal sounds in sequence, in RGDT and Questionnaire for Disabilities Associated with Impaired Auditory Localization. Conclusion With the effective use of hearing aids, individuals with UHL showed improvement in the auditory abilities of sound localization, ordering and temporal resolution.

RESUMO Objetivo Verificar as habilidades auditivas de ordenação temporal, resolução temporal e localização sonora, antes e após a adaptação do aparelho de amplificação sonora individual (AASI) em indivíduos com perda auditiva unilateral (PAUn). Método Foram avaliados 22 indivíduos, com idades de 18 a 60 anos, com diagnóstico de PAUn sensorioneural ou mista, de graus leve a severo. O estudo foi dividido em duas etapas: a pré- e a pós-adaptação do AASI. Em ambas as etapas, os indivíduos realizaram anamnese, bem como a aplicação do Questionário de Habilidade Auditiva da Localização da Fonte Sonora (QHALFS), avaliação simplificada do processamento auditivo (ASPA) e Random Gap Detection Test (RGDT). Resultados O presente estudo encontrou diferenças estatisticamente significantes nas avaliações de localização sonora e memória para sons verbais em sequência, no RGDT e no QHALFS. Conclusão Com o uso efetivo do AASI, indivíduos com PAUn apresentaram melhora nas habilidades auditivas de localização sonora, ordenação e resolução temporal.

Análise da percepção da fala com uso de dispositivos de amplificação em indivíduos com malformação de orelha e perda auditiva unilateral / Analysis of speech perception with amplification devices in subjects with ear malformation and unilateral hearing loss

RESUMO Objetivo: Verificar a percepção da fala de indivíduos com malformação de orelha e perda auditiva unilateral utilizando dois tipos de amplificação: amplificação sonora individual (AASI) convencional e softband (faixa com vibrador ósseo). Método: Foram selecionados 15 indivíduos, de ambos os sexos, com malformação congênita de orelha externa e/ou orelha média, diagnóstico de perda auditiva unilateral do tipo condutiva ou mista de grau moderado a severo, idade entre 15 e 25 anos, e encaminhamento para amplificação realizado pelo médico otorrinolaringologista. Após a adaptação com AASI e softband, foi realizada avaliação da percepção da fala sem uso da amplificação, com AASI acoplado ao arco e vibrador ósseo (convencional) e com uso do softband (faixa com vibrador). Os indivíduos foram avaliados por meio do Hearing in Noise Test (HINT) nas condições de silêncio e de ruído. Resultados: Foram avaliados sete indivíduos com malformação de orelha unilateral, sendo 57,1% na orelha direita e 42,9% na orelha esquerda. Quanto ao tipo e grau da perda, 71, 4% da amostra possuía perda auditiva condutiva moderada. No teste de percepção de fala na condição de silêncio, ruído frontal e ruído lateral, em três situações: sem amplificação, com o uso do AASI convencional e com o uso do softband, os resultados com uso de dispositivos de amplificação apresentaram-se melhores em todas as condições. Conclusão: Os indivíduos apresentaram melhora sutil, porém não significativa, na percepção de fala tanto em situações de silêncio, ruído frontal e lateral independentemente do tipo de amplificação.

ABSTRACT Purpose: To verify the speech perception in subjects with ear malformation and unilateral hearing loss, fitted with two types of amplification as follows: conventional hearing aids and softband (band with vibrator bone). Method: The study included fifteen subjects of both sexes who presented congenital malformation of the middle or outer ear, diagnosed with unilateral conductive or mixed hearing loss, moderate to severe hearing loss, age range between 15 to 25 years and, prescription from a specialist doctor for hearing device fitting. We performed the speech perception assessment without amplification after the hearing aid and softband fitting, with the hearing aid linked to the bone vibrator (conventional) and the softband (band with the bone vibrator). The subjects were evaluated using the Hearing in Noise Test (HINT), in silence and in noise. Results: Seven subjects with unilateral ear malformation were evaluated, 57.1 % had impairment in the right ear and 42.9 % in the left ear. Regarding the type and the level of hearing loss, 71 % of all subjects included in the sample presented moderate conductive hearing loss. The assessment of speech perception was performed during silence, frontal noise, lateral noise and, during three specifics situations: no amplification, with conventional hearing aid and with the softband. The results with the amplification devices were positive in all evaluated conditions. Conclusion: Evaluated subjects presented improvement in speech perception, in silence, frontal noise and lateral noise situations, regardless of the type of amplification; however, the difference was not statistically significant.

Pigmented Villonodular Synovitis Presenting as Unilateral Hearing Loss: Review of the Literature and Case Report.

BACKGROUND/AIMS: To review the existing literature on pigmented villonodular synovitis (PVNS) of the temporomandibular joint (TMJ) and report a rare case of PVNS of the TMJ presenting with unilateral hearing loss. METHODS: Review of the existing literature and a description of personal experience with PVNS of the TMJ presenting with unilateral hearing loss. RESULTS: Review of the existing literature revealed 76 reported cases of PVNS of the TMJ. The most common presenting symptom was of a slowly enlarging mass or swelling of the preauricular area, with dysfunctional TMJ also frequently reported. All patients underwent surgical excision with some pursuing radiation as adjuvant therapy. Presented Patient: A 46-year-old man presented with several months of unilateral subjective hearing loss and aural fullness. Imaging revealed a mass centered along the superior TMJ with expansion through the squamous temporal bone and extra-axial intracranial extension into the middle cranial fossa. Imaging characteristics and fine-needle aspiration biopsy were consistent with PVNS. INTERVENTION: The patient underwent near-total excision of the mass via frontotemporal craniectomy and lateral temporal bone resection. FOLLOW-UP: At the 16-month follow-up there was no evidence of disease recurrence. CONCLUSION: PVNS of the TMJ represents a rare entity that can present with a variety of symptoms including unilateral hearing loss.

Hearing Loss in Pediatric Septo-Optic Dysplasia.

OBJECTIVE: To identify and characterize hearing loss (HL) in children with septo-optic dysplasia (SOD). METHODS: Otologic and audiometric data for patients less than 18 years of age identified as having SOD who were seen in the Children's Healthcare of Atlanta-Scottish Rite Hospital clinic between 2013 and 2017 were collected and reviewed through a HIPAA-compliant medical record search. Relevant literature was also reviewed with the assistance of Medline. RESULTS: Sixty-four patients with SOD were identified, and 7 of those patients (10.9%) were diagnosed with hearing loss. Type of hearing loss was sensorineural (SNHL) in 5 patients (63%), mixed (MHL) in 1(14%), and conductive (CHL) in 1(14%). Bilateral loss presented in 60% (3/5) of SNHL patients, while the rest demonstrated unilateral loss. Unilateral findings included cochlear nerve deficiency (1) and atresia/microtia (1). Tympanostomy tubes were required in 57% (4/7) of SOD children with hearing loss. Amplification was successfully implemented in 86% (6/7). CONCLUSIONS: Hearing loss was found in nearly 11% of SOD children, and SNHL was identified as (63%) the predominant form of loss. To our knowledge, this is the first retrospective review of hearing loss in a pediatric SOD cohort and the first to report of cochlear nerve deficiency and atresia/microtia in this population. Based on these findings, early identification of hearing loss with imaging when appropriate and treatment of otitis in this population is recommended.

Bilateral Ear Canal Cholesteatoma with Underlying Type I First Branchial Cleft Anomalies.

OBJECTIVES:: To describe a case of bilateral ear canal cholesteatomas in the setting of underlying first branchial cleft cyst anomalies and to review the pathophysiology underlying the development of external auditory canal cholesteatomas from branchial cleft cyst abnormalities. METHODS AND RESULTS:: We present a case study of a 61-year-old man who presented with chronic right-sided hearing loss and left-sided postauricular drainage. Clinical evaluation, radiographic work-up, and pathologic analysis confirmed a diagnosis of bilateral ear canal cholesteatoma in the setting of underlying first branchial cleft cyst anomalies. The patient's clinical course, surgical treatment, and management considerations are discussed here. CONCLUSION:: Ear canal cholesteatoma represents a rare clinical disease entity deserving a thorough initial assessment. Careful consideration of underlying diseases that result in chronic inflammation, such as branchial cleft lesions, should be included in the differential diagnosis of idiopathic canal cholesteatoma in the absence of prior otologic surgery or trauma.

[Transient unilateral sudden hearing loss after spinal anesthesia]. / Spinal anestezi sonrasi gelisen geçici tek tarafli ani isitme kaybi.

Hearing loss after spinal anesthesia is one of the rare complications. It has been suggested that low frequent hearing loss develops after the development of endolymphatic hydrops in the cochlea due to CSF escape after dural puncture. Hearing loss after nonotologic surgical procedures is very rare and usually can only be determined by audiometric evaluation. We aimed to present the sudden unilateral hearing loss and healing process in a patient who underwent spinal anesthesia for elective anterior cruciate ligament surgery in this postoperative period.