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.

Concurrent chemoradiotherapy using weekly cisplatin remains standard of care for locally advanced cervical cancer in Sub-Saharan Africa. While cisplatin remains a popular cancer chemotherapeutic, it has an irreversible ototoxic effect on patients\' auditory system. However, there is a paucity of epidemiological information on its extent and severity during cervical cancer treatment. In a region with a high burden of cervical cancer, this has serious consequences for aural intervention and rehabilitation.
Using a prospective cohort study design, 82 patients with incident cervical cancer, receiving weekly cisplatin chemotherapy (50 mg/m2 body surface) at a tertiary level hospital in KwaZulu-Natal Province of South Africa, underwent audiological assessments at various intervals. We describe the temporal impact of cisplatin exposure on hearing loss, its combined effect with HIV-infection, and estimate ototoxicity incidence in this cohort. The median age was 52 years with Stages IIB (45%) and IIIB (35.4%) cancers being most common. Complaints of reduced hearing sensitivity increased significantly (p<0.0001). Bilateral, asymmetrical sensorineural hearing loss, with greater effect in the extended high-frequency range, was evident. Cisplatin dosage was significantly associated with ototoxicity severity at one- (p = 0.017), three- (p = 0.010), and six-month (p = 0.015) post-treatment follow-up. HIV-seropositivity (53.7%) was significantly associated with NCI-CTCAE Grading Scale at three- (p = 0.022) and six-months (p = 0.023) post-treatment. Multiple Tobit regression revealed a cumulative dose effect bilaterally, after adjustment for age and HIV status, evident from 9000Hz and above in the right ear, while a plateau effect was observed at 250mg/m2 in the left ear. The incidence was ototoxicity was 98% at a cumulative dose of 150mg/m2.
The findings of this epidemiologic study highlight the temporal course and severity of ototoxicity experienced by cervical cancer patients treated with cisplatin, with greater impact in HIV-positive subgroup, thus underscores the need for audiological monitoring and timely interventions in this cohort.

OBJECTIVE: Some studies have directly compared the National Acoustic Laboratories\' prescription for non-linear hearing aids (HAs) version 2 (NAL-NL2) and Desired Sensation Level for non-linear HAs version 5 (DSLv5), although none were performed in Japan. As the Japanese language is a tonal language that has different linguistic characteristics than those of the studied languages, we compared the outcomes of the NAL-NL2 and DSLv5 in hearing-impaired Japanese participants.
METHODS: A crossover-controlled trial was conducted on 18 first-time HA users with bilateral moderate sensorineural hearing loss. Participants wore HAs adjusted with each prescriptive method for four weeks. The prescriptions were assessed using speech discrimination testing and the abbreviated profile of hearing aid benefit (APHAB). Consequently, participants were asked to select their preferred prescription and determine which was better for \"listening to a conversation\" and when \"noisy.\"
RESULTS: The mean DSLv5 real ear insertion gain for an input level of 65 dB sound pressure level (SPL) was higher than that of the NAL-NL2 at 250 and 500 Hz. The average speech discrimination score was 78 ± 14% at a 65-dB SPL and 75 ± 17% at an 80-dB SPL for the NAL-NL2, and 79 ± 11% at a 65-dB SPL and 77 ± 17% at an 80-dB SPL for the DSLv5. These differences were not significant. No significant differences were observed in APHAB subscale scores between the two prescription methods. Ultimately, 11 (61%) and 7 (39%) participants preferred the NAL-NL2 and DSLv5, respectively, with no significant differences.
CONCLUSIONS: Although the gain of the NAL-NL2 is smaller than that of the DSLv5, both had the same hearing effect. Therefore, the NAL-NL2 may be more useful than the DSLv5 in Japanese.

To determine the benefit of sequential cochlear implantation after a long inter-implantation interval in children with bilateral deafness receiving their second implant between 5 and 18 years of age.
Prospective cohort-study.
Tertiary multicenter.
85 children with bilateral deafness and unilateral implantation receiving a contralateral cochlear implant at the age of 5 to 18 years.
The primary outcomes were speech recognition in quiet and noise (CVC) scores. The secondary outcomes were language outcomes and subjective hearing abilities, all measured before and 12 months after sequential bilateral cochlear implantation. Medians of the paired data were compared using the Wilcoxon signed-rank test. Univariable linear regression analyses was used to analyze associations between variables and performance outcomes.
A significant benefit was found for speech recognition in quiet (96% [89-98] vs 91% [85-96]; p < 0.01) and noise (65% [57-75] vs 54% [47-71]; p = 0.01) in the bilateral CI condition compared to unilateral (n = 75, excluded 10 non-users). No benefit was seen for language outcomes. The subjective sound quality score was statistically significant higher in bilateral compared to the unilateral CI condition. Pre-operative residual hearing level in the ear of the second implant, the inter-implant interval and age at time of second implantation was not significantly associated with performance scores.
After 12 months of use, sequential bilateral cochlear implantation showed improved speech perception in quiet and noise and improved subjective sound quality outcomes in children despite a great inter-implantation interval (median of 8 years [range 1-16 years]).

OBJECTIVE: 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.

OBJECTIVE: This investigation was to ascertain the performance of the UNHS in Taiwan.
METHODS: The predefined questionnaire was delivered on the phone in 2016. The descriptive analysis was applied to the research data. 941 neonates in birth cohorts 2013-2014 who were documented as a bilateral referral in the national UNHS tracking system were targeted. The respondents were either caregivers or family members.
RESULTS: 40.3% of 941 children were lost to follow-up, and 66.24% of 363 children were diagnosed with SNHL. 45.15% of 163 children used hearing amplification device(s). 77.46% of hearing amplification device users and 7.51% of non-users participated in the auditory training courses. By six months of age, 38.51% and 22.58% of children diagnosed with bilateral SNHL commenced the hearing amplification device fitting and the auditory training courses, respectively.
CONCLUSIONS: More efforts are needed to enhance the performance of the UNHS to achieve national goals stated in the 2014 Taiwan UNHS Revised Guidelines and the well-known benchmarks set by the JCIH in 2007. The development of an electronic tracking system for storing and sharing information on the follow-up on children with congenital hearing loss was as significant as the improvements in the understanding of early hearing detection and intervention of the public and stakeholders.

Mitochondrial myopathy is caused by the absence and/or insufficiency of L-carnitine, a quaternary enzyme responsible for transporting free fatty acids into the mitochondria. The primary function of the mitochondria is to produce energy, contributing to proper cell functioning. Muscular lipidosis causes abnormalities in enzymes that metabolize fat, resulting in the accumulation of harmful amounts of fats in tissues. The aim of this study was to present the case study of patient B.D., a 37-year-old woman diagnosed with muscular lipidosis with L-carnitine deficiency at 6 years old, and describe the speech-language follow-up performed at a hearing care clinic. The first entry in the patient\'s medical chart was on 03/05/1989, with continuous use of 2g/day of L-carnitine prescribed by a neurologist. The mother reported that B.D. had difficulty hearing and was inattentive, which became more evident when she started school. In 1988 the patient was diagnosed with moderate bilateral sensorineural hearing loss and began using behind-the-ear (BTE) hearing aids in 1989, after which her academic performance and communication improved. In 1998 she switched to Completely in Canal (CIC) hearing aids, which are more discreet, provided better sound localization and greater high frequency gain, although her hearing thresholds worsened slightly. She completed her graduate studies and currently works at a large financial institution. It was concluded that early neurological diagnosis and speech-language intervention enabled adequate language development in the patient.
A miopatia mitocondrial é causada pela ausência e/ou insuficiência de uma enzina quaternária, L-carnitina, responsável por transportar ácidos graxos livres para a parte interna da mitocôndria. A função da mitocôndria é produzir energia, contribuindo para o bom funcionamento das células. A Lipidose Muscular é uma doença que provoca anomalias em enzimas que metabolizam gordura e por consequência causa acúmulo de toxinas de subprodutos com gordura nos tecidos. O objetivo deste trabalho é apresentar o estudo de caso da paciente B.D., 37 anos, diagnosticada com Lipidose Muscular aos seis anos, com deficiência de L-Carnitina e relatar o acompanhamento fonoaudiológico realizado em um serviço de saúde auditiva. A abertura de prontuário da paciente foi realizada em 05/03/1989. Foi prescrito pelo neurologista o uso contínuo de 2g/dia de L-carnitina. A mãe relatou que B.D. apresentava dificuldades em ouvir, pois era muito desatenta, o que foi mais evidente quando começou a frequentar a escola. Em 1988, a paciente foi diagnosticada com perda auditiva neurossensorial de grau moderado bilateral e começou a fazer uso de aparelhos de amplificação sonora individual retroauriculares em 1989. O desempenho escolar e comunicação melhoraram. Em 1998, passou a utilizar aparelhos tipo micro canal, o que a favoreceu esteticamente, promoveu melhora da localização sonora e maior ganho em altas frequências. Os limiares de audibilidade apresentaram uma leve piora e a paciente atualmente é pós-graduada e trabalha em uma grande instituição financeira. Conclui-se que o diagnostico neurológico e a intervenção fonoaudiológica precoces possibilitaram o adequado desenvolvimento de linguagem da paciente.

Tinnitus is the perception of sound without an external stimulus, often experienced as a ringing or buzzing sound. Subjective tinnitus is assumed to origin from changes in neural activity caused by reduced or lack of auditory input, for instance due to hearing loss. Since auditory deprivation is thought to be one of the causes of tinnitus, increasing the auditory input by cochlear implantation might be a possible treatment. In studies assessing cochlear implantation for patients with hearing loss, tinnitus relief was seen as a secondary outcome. Therefore, we will assess the effect of cochlear implantation in patients with primarily tinnitus complaints.
In this randomised controlled trial starting in January 2021 at the ENT department of the UMC Utrecht (the Netherlands), patients with a primary complaint of tinnitus will be included. Fifty patients (Tinnitus Functional Index (TFI) >32, Beck\'s Depression Index <19, pure tone average at 0.5, 1, 2 and 4 kHz: bilateral threshold between 50 and ≤75 dB) will be randomised towards cochlear implantation or no intervention. Primary outcome of the study is tinnitus burden as measured by the TFI. Outcomes of interest are tinnitus severity, hearing performances (tinnitus pitch and loudness, speech perception), quality of life, depression and patient-related changes. Outcomes will be evaluated prior to implantation and at 3 and 6 months after the surgery. The control group will receive questionnaires at 3 and 6 months after randomisation. We expect a significant difference between the cochlear implant recipients and the control group for tinnitus burden.
This research protocol was approved by the Institutional Review Board of the University Medical Center (UMC) Utrecht (NL70319.041.19, V5.0, January 2021). The trial results will be made accessible to the public in a peer-review journal.
Trial registration number NL8693; Pre-results.

OBJECTIVE: To assess the effect on hearing of non-functioning ventilation tubes due to blockage during the first six months post-operatively, using UK national guidelines.
METHODS: A prospective, observational study was conducted on 37 children who underwent bilateral ventilation tube insertion. Air and bone conduction thresholds were measured before and following surgery, and at one, three and six months post-operatively. Tube non-function was assessed by tympanometry supported by otoscopy.
RESULTS: Post-operatively, an average of 21 per cent of ventilation tubes were non-functioning. Ears with non-functioning tubes had significantly (p = 0.0001) poorer mean air conduction thresholds than functioning tubes, with a magnitude of 6 dB HL. Ears with otorrhoea were most affected (15 per cent). At any one visit, the air-bone gap was closed to 10 dB or less in 76 per cent of ears. Non-functioning tubes reduced this to 56 per cent. Compared with tympanometry, otoscopy underdiagnosed tube non-function due to blockage by 22 per cent.
CONCLUSIONS: Non-functioning of ventilation tubes occurs frequently and can be missed on otoscopy. Although it is associated with poorer air conduction thresholds, the magnitude of this difference is unlikely to warrant further intervention unless there is otorrhoea or recurrence of bilateral hearing impairment.

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.
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.
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.
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.