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Globally around 34 million children present disabling hearing loss. If unaddressed, hearing loss can negatively impact several aspects such as communication, cognition, language, and speech. Most needy people with hearing impairment do not have access to treatments and it’s a national and international public health problem. Meningococcal disease is caused by the bacterium Neisseria meningitides, affects mainly children, can cause hearing loss, and is a global public health challenge. This study aimed to present the case report of a child who acquired meningococcal disease and bilateral profound sensorineural hearing loss in primary infancy. At the age of six months, the child acquired meningococcal meningitis and performed antibiotic therapeutics. Four years later, the audiometric evaluation detected hearing loss. At the age of seven years, the patient started speech language therapy sessions. At the age of nine years, hearing prostheses were provided by a hospital. At the age of ten, the child began to deny the use of hearing aids at home and school. Two years later, she started her studies at a special school where the deaf community is clearly present. At the age of fourteen, the patient became completely fluent in Sign language, stopping speech therapy sessions. The analyzed case demonstrated a lack of children’s surveillance by a multidisciplinary team and the relevance of following children’s guidance vaccination. It is required to provide adequate interventions for children with hearing loss, which can potentially aid them to develop their skills and to achieve a better quality of life. A nivel mundial, alrededor de 34 millones de niños presentan pérdida auditiva discapacitante. Si no se aborda, la pérdida auditiva puede tener un impacto negativo en varios aspectos, como la comunicación, la cognición, el lenguaje y el habla. La mayoría de las personas necesitadas con discapacidad auditiva no tienen acceso a tratamientos y es un problema de salud pública nacional e internacional. La enfermedad meningocócica es causada por la bacteria Neisseria meningitides, afecta principalmente a niños, puede causar pérdida de audición y es un desafío de salud pública mundial. Este estudio tuvo como objetivo presentar el caso clínico de un niño que adquirió enfermedad meningocócica e hipoacusia neurosensorial profunda bilateral en la primera infancia. A la edad de seis meses, el niño contrajo una meningitis meningocócica y se le practicó una terapia antibiótica. Cuatro años después, la evaluación audiométrica detectó hipoacusia. A la edad de siete años, la paciente inició sesiones de logopedia. A la edad de nueve años, un hospital le proporcionó prótesis auditivas. A la edad de diez años, el niño comenzó a negar el uso de audífonos en el hogar y la escuela. Dos años más tarde, inició sus estudios en una escuela especial donde la comunidad sorda está claramente presente. A la edad de catorce años, el paciente domina completamente el lenguaje de señas, interrumpiendo las sesiones de terapia del habla. El caso analizado demostró la falta de vigilancia de los niños por un equipo multidisciplinario y la pertinencia de seguir las orientaciones de vacunación de los niños. Se requiere brindar intervenciones adecuadas para los niños con pérdida auditiva, que potencialmente puedan ayudarlos a desarrollar sus habilidades y lograr una mejor calidad de vida. Globalmente, cerca de 34 milhões de crianças apresentam perda auditiva incapacitante. Se não tratada, a perda auditiva pode impactar negativamente em diversos aspectos, como comunicação, cognição, linguagem e fala. A maioria das pessoas carentes com deficiência auditiva não tem acesso a tratamentos, sendo este um problema de saúde pública nacional e internacional. A doença meningocócica é causada pela bactéria Neisseria meningitides, acomete principalmente crianças, pode causar perda auditiva, e é um desafio de saúde pública global. Este estudo teve como objetivo apresentar o relato de caso de uma criança que adquiriu doença meningocócica e perda auditiva sensorioneural bilateral profunda na primeira infância. Aos seis meses, a criança adquiriu meningite meningocócica e realizou antibioticoterapia. Quatro anos depois, a audiometria detectou a perda auditiva. Aos sete anos de idade, a paciente iniciou sessões de terapia fonoaudiológica. Aos nove anos, as próteses auditivas foram fornecidas por um hospital. Aos dez anos, a criança começou a não utilizar mais os aparelhos auditivos em casa e na escola. Dois anos depois, iniciou seus estudos em uma escola especial onde a comunidade surda está claramente presente. Aos quatorze anos, a paciente tornou-se completamente fluente em Língua de Sinais, interrompendo as sessões de terapia fonoaudiológica. O caso analisado demonstrou a falta de vigilância à criança pela equipe multidisciplinar e a relevância de seguir as orientações de vacinação infantil. É necessário fornecer intervenções adequadas para crianças com deficiência auditiva, que potencialmente podem auxiliá-las a desenvolver suas habilidades e alcançar uma melhor qualidade de vida.

Previous research has demonstrated differences in the acoustic features of crying signals between deaf and typical hearing (TH) infants. This study aims at comparing the acoustic parameters of cries of infants with different degrees of deafness versus TH infants. About 110 infants aged 6–12 months (61 TH infants, 34 infants with bilateral deafness of variable degrees and 15 infants with unilateral deafness) were enrolled in the study. Results indicated that the most important acoustic parameters to demonstrate the difference between the crying of TH infants and deaf and hard-of-hearing (DHH) infants as well as between the crying of infants with different degrees of deafness are F0, cry duration, intensity, F2, and F4. In terms of accuracy, the parameter that showed the highest accuracy to differentiate between TH infants and DHH infants was F0 (74.5%). The accuracy of the combined acoustic parameters (F0, cry duration, intensity, and F4) was 70%. F0 was considered the best predictor and F4 the second best predictor of severity of deafness.

This article presents a case of sudden bilateral deafness in the context of a severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) infection and resultant coronavirus disease 2019 (COVID-19). After treatment in the intensive care unit for acute respiratory distress syndrome and acute kidney failure, hearing ability had drastically changed. While hearing had been subjectively normal before the infection, deafness was now measured on the left and profound hearing loss on the right ear. The patient was treated with cochlea implants on the left and a hearing aid in the right ear. The hearing loss is most likely a complication of COVID-19.

This article presents a case of sudden bilateral deafness in the context of a severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) infection and resultant coronavirus disease 2019 (COVID-19). After treatment in the intensive care unit for acute respiratory distress syndrome and acute kidney failure, hearing ability had drastically changed. While hearing had been subjectively normal before the infection, deafness was now measured on the left and profound hearing loss on the right ear. The patient was treated with cochlea implants on the left and a hearing aid in the right ear. The hearing loss is most likely a complication of COVID-19.

Sudden-onset bilateral cortical deafness is a very rare symptom of stroke, but must be recognized as stroke, as it is a treatable condition, and the treatment is highly time dependent. We report a 53-year-old man with an acute onset of complete bilateral hearing loss that gradually improved spontaneously over 4 h. The hearing loss was explained by an infarction visualized on magnetic resonance imaging, which showed a subacute temporoparietal ischemic lesion in the left cerebral hemisphere involving the insular cortex and an older infarction in the right temporoparietal region. The location of these kinds of lesions may typically not cause motor deficits, but sensory and cognitive (e.g., aphasia) symptoms, which can be challenging to recognize in a suddenly deaf patient. Taking the possible differential diagnoses into account, immediate stroke workup should always be prioritized in patients with sudden bilateral deafness, as acute revascularizing treatment is possible.

PURPOSE OF REVIEW Cochlear implants have been used to treat bilateral deafness for over 40 years. A more recent development has been the application of this technology toward single-sided deafness (SSD) and asymmetric hearing loss (AHL), culminating in the Food and Drug Administration (FDA) approval in the United States for this indication in July of 2019. This review examines the recent literature on cochlear implants for SSD and AHL, focusing on speech perception, sound localization, tinnitus, and quality of life. RECENT FINDINGS There is an expanding body of literature supporting the use of cochlear implant in SSD/AHL patients. The cochlear implant offers a unique rehabilitation option for this subset of patients as it potentially restores binaural hearing (direct stimulation of both ears). A review of the literature reveals significant improvements with the cochlear implant in speech perception in quiet and noise, sound localization, tinnitus, and quality of life. In addition, these improvements appear to be greater than what conventional options such as a contralateral routing of signal (CROS) hearing aid and bone conduction device (BCD) are able to achieve. SUMMARY Cochlear implants offer additional benefits over previously available options of CROS and BCDs and have recently been approved by the FDA for use in patient with SSD/AHL. Given the growing body of literature demonstrating the benefit of cochlear implant over other devices, it is important to offer cochlear implant as an option to these patients.

Objective: To determine the audiometric profile of deafness in our practice. Materials and Methods: Longitudinal prospective study was conducted out in the ENT department and cervicofacial surgery of Gabriel Toure University Hospital in Bamako, we made an exhaustive sampling of all the patients who consulted for hearing loss, tinnitus, vertigo, hiring report, medical expertise and whose deafness was confirmed to tonal audiometry with an age greater than or equal to 15 years. It was spread over 10 months (June 2016 to March 2017). A total of 200 patients were collected. Exclusion criteria were all patients under 15 years of age as well as deafness related to earwax or foreign bodies, and refusal to participate in the study Results: During our study period, 6055 outpatients were admitted out of which 734 patients underwent audiometric testing and 200 patients (3.30%) met our criteria. The male sex was the most represented with a rate of 60%. The sex ratio was 1.5 or 3 men for 2 women. The 15 to 25 age group was the most represented at 37.5%. The average age was 37.18 years old with extremes ranging from 15 to 83 years old. Pupils/students were the most represented with a rate of 29.5%, followed by housewives in 23%, farmers in 10.5% and military in 10%. As antecedent 26% of our patients had a chronic otitis media (OMC), against 23% who had no antecedent otological and 17% had a traumatic antecedent. Bilateral deafness was the most common with a rate of 64.5%. The mode of progressive appearance was the most frequent in 74.5%. As functional signs 46.22% of our patients had a hearing loss associated with tinnitus. Otoscopic examination was pathological in 34.5% of our patients. Mixed deafness was the most common in 43.35%, followed by perception deafness in 32.19% and transmission deafness 24.46%. Mean deafness was the most common with a rate of 48.91%, was severe in 20.22%, mild in 18.31%, deep in 11.47% and cophotic in 01.09%. Asymmetrical curves were the most found in 65.89% of our patients. Conclusion: Deafness is a sensory disability responsible for communication disorder, sometimes disabling. Audiometry, although subjective, remains essential in the diagnosis of deafness.

OBJECTIVES Our primary objective was to investigate the long-term usage of cochlear implants (CIs) in children who underwent sequential bilateral CI (SeqBCI), and to study factors that impact usage. Our secondary objective was to understand the difference in subjective benefit and educational/employment status, between users and non-users of the second CI (CI2). STUDY DESIGN Prospective case series. SETTING Tertiary academic centers. PATIENTS Sixty-eight young adults who underwent SeqBCI as children. INTERVENTIONS Pediatric SeqBCI. MAIN OUTCOME MEASURES The main outcome measures were the current usage of the first CI (CI1) and CI2, factors that determine usage, current perceptions of their CIs, educational/employment status, and Speech, Spatial and Qualities of Hearing scale (SSQ12) scores. RESULTS Sixty five (95.6%) participants were using CI1 for over 8 h/d and the rest were using CI1 for 4 to 8 h/d. Fourty four (64.7%) participants used CI2 for at least 4 h/d, 10 (15%) indicated that they rarely used CI2 (

Hearing loss is a rare complication of cardiac surgery; bilateral profound deafness has never been reported in this setting. A 45-year-old male presented with profound bilateral sudden deafness following arch surgery and frozen elephant trunk. Patient’s presentation, surgery details and aetiological mechanisms are discussed.

We report the case of a 46-year old African American woman who presented to the emergency department with one week of progressive bilateral deafness associated with worsening gait abnormalities, visual changes, and confusion. She was diagnosed with Wernicke encephalopathy (WE) attributed to alcohol abuse; her symptoms, including hearing loss, improved with thiamine replacement. WE, a condition due to thiamine deficiency, commonly affects those with alcohol use disorder or gastric bypass history. Though traditionally associated with a triad of encephalopathy, ophthalmoplegia, and ataxia, it can be more rarely associated with auditory deficits or other neurologic findings. Though hearing loss has previously been reported as a rare symptom of WE, it has not been described in WE due to alcohol abuse. We performed a review of the literature to determine if WE associated with hearing loss had been previously reported.

We describe a rare case of meningeal carcinomatosis (MC) in a 66-year-old man who presented with bilateral deafness and vertigo, initially presumed to be neurofibromatosis type-2. Brain magnetic resonance imaging (MRI) of the patient revealed bilateral gadolinium enhanced masses at the cerebellopontine angle. However, multiple central nervous system symptoms, including loss of consciousness, gradually appeared. He had a history of gastric cancer; therefore, a lumbar puncture was performed. Cytological examination of the cerebrospinal fluid confirmed the presence of adenocarcinoma cells. The general condition of this patient worsened, and he died 46 days after the first onset of hearing loss. An autopsy was performed, and multiple infiltrations of adenocarcinoma cells in the brain were confirmed, though undetected by MRI. The prognosis of MC is extremely poor; therefore, rapid diagnosis is important to prevent mortality. Retrospectively, a lumbar puncture could have been conducted earlier to identify MC, especially in consideration of the clinical history of this patient.

The auditory cortex has been shown to participate in visual processing in individuals with complete auditory deprivation. However, it remains unclear whether partial hearing deprivation like single-sided deafness (SSD) leads to similar cross-modal plasticity. To investigate this, we enrolled individuals with long-term SSD, into functional MRI scans under resting-state and a visuo-spatial working memory task. Contrary to previous findings in bilateral deafness, our study revealed decreased activation in the auditory cortex in both left (LSSD) and right (RSSD) single-sided deafness compared to normal hearing controls, with statistical significance in RSSD. The degree of involvement was correlated with residual hearing ability in RSSD. These observations suggest that SSD can lead to a downward cross-modal plasticity: the more hearing ability lost, the fewer brain resources in the auditory cortex can be applied to visual tasks. In addition, the fronto-parietal cortex was observed to be less activated during the visual task in RSSD while the resting-state fMRI revealed increased functional connectivity between the fronto-parietal cortex and the auditory cortex, suggesting fronto-parietal resources may be recruited less by vision but more by hearing. The LSSD showed a similar alteration trend with RSSD, but without statistical significance. Together these findings may indicate that when hearing is partially deprived in SSD, there may be redistribution for brain resources between hearing and vision, and vision tends to allocate less resources. Our findings in this pilot study of unilateral auditory-deprived individuals enrich the understanding of cross-modal plasticity in the brain.

Background Most sudden-onset hearing loss is due to otolaryngologic- and very rarely to cerebrovascular disease. We report a woman with sudden bilateral sensorineural hearing loss. This case suggests that even in the absence of brainstem or cerebellar signs, magnetic resonance imaging (MRI) and MR angiography (MRA) should be performed since such studies may reveal signs of life-threatening vertebrobasilar artery occlusion. Case presentation A 73-year-old Japanese woman with a history of hypertension, hyperlipidemia, and atrial fibrillation who suffered bilateral deafness with vertigo and vomiting was transferred from a local hospital to our department. On admission her consciousness was clear and vertigo was absent. Neurological examination revealed only bilateral sensorineural hearing loss. Head computed tomography (CT) returned no significant findings. The next morning she gradually developed severe drowsiness. Diffusion-weighted MRI demonstrated acute cerebral infarction in the brainstem and bilateral cerebellum; MRA showed basilar artery occlusion due to a cardioembolic thrombus. Revascularization was obtained by endovascular treatment. However, her condition worsened progressively during the following hours. CT revealed new brainstem lesions, massive cerebellar swelling, and obstructive hydrocephalus. She died on the second day after her admission. Conclusions When hearing loss is due to vertebrobasilar occlusive disease, the prognosis is very poor. We suggest that vertebrobasilar stroke be suspected in patients with bilateral sensorineural hearing loss who present with risk factors for stroke such as atrial fibrillation and other neurologic signs.

Background Meningeal carcinomatosis is a very rare metastatic site of gastric cancer and meningeal carcinomatosis without other metastatic sites is much extremely rare. Herein, we report our experience with a very rare case of meningeal carcinomatosis which was difficult to diagnose the recurrence by general systemic examination and was found due to the deafness despite the sustained high tumor markers. Case presentation A 68-year-old man consulted a hospital with vomiting and hematemesis. Laboratory tests revealed severe anemia. He was referred to our hospital and underwent an emergency gastroscopy, which revealed Borrman type 3 tumor and oozing of blood. Biopsy specimen showed gastric cancer. After several examinations, total gastrectomy was performed and tegafur-gimeracil-oteracil potassium (S-1) was initiated as adjuvant chemotherapy one month after surgery. Tumor marker levels (CEA and CA19-9) remained high for three months after surgery. S-1 was continued while shortening the imaging study follow-up period. Nine months after surgery, he noticed difficulty in hearing with facial paralysis, dizziness, tinnitus, and appetite loss. He was diagnosed with meningeal carcinomatosis and bilateral internal auditory canal metastasis. He died approximately two months later. Conclusion Meningeal carcinomatosis should be considered if bilateral deafness and vestibulopathy develop after gastrectomy, even if no recurrence is apparent in the abdominal cavity.

OBJECTIVE Meniere's disease (MD) is characterized by episodes of vertigo, tinnitus, and sensorineural hearing loss. In the setting of bilateral deafness due to MD alone or contralateral pathology, cochlear implantation (CI) improves hearing. Active MD is characterized by fluctuating auditory symptoms and vertigo; whereas remittance of vertiginous symptoms and severe, permanent sensorineural hearing loss characterizes the inactive disease state. This study evaluates outcomes for MD patients compared with the general CI population and assesses if disease activity affects implant outcomes. STUDY DESIGN Retrospective chart review. SETTING Tertiary referral center. PATIENTS Twenty-four patients with MD that received CI (7 active, 16 inactive, and 1 Probable Meniere's), and 24 age-matched controls. INTERVENTIONS Cochlear implantation. MAIN OUTCOME MEASURES Word Recognition Score, Sentence Recognition Score (SRS), and Speech Reception Threshold. RESULTS Best-aided preoperative and postoperative audiometric data were compared per ear between MD patients and controls and stratified by disease status using descriptive statistics with mixed-effects modeling. Patients with MD derived significantly more benefit from CI than controls when comparing differences between preoperative and postoperative levels for Word Recognition Score (12.2%, p = 0.0236), SRS (12.8%, p = 0.0375), and Speech Reception Threshold (-14.4 dB, p = 0.0188). Active disease status does not negatively impact CI outcomes and patients with active MD may benefit from greater gains in SRS (23.5%, p = 0.0107). CONCLUSIONS CI provides greater gains in functional hearing for patients with MD compared with age-matched controls. Patients with active MD seem to perform better with respect to SRS following CI than patients with inactive status.

Neuroplasticity following bilateral deafness and auditory restoration has been repeatedly investigated. In clinical practice, however, a significant number of patients present a severe-to-profound unilateral hearing loss (UHL). To date, less is known about the neuroplasticity following monaural hearing deprivation and auditory input restoration. This article provides an overview of the current research insights on the impact of UHL on the brain and the effect of auditory input restoration with a cochlear implant (CI). An exhaustive systematic review of the literature was performed selecting 38 studies that apply different neural analyses techniques. The main results show that the hearing ear becomes functionally dominant after monaural deprivation, reshaping the lateralization of the neural network for auditory processing, a process that can be considered to influence auditory restoration. Furthermore, animal models predict that the onset time of UHL impacts auditory restoration. Hence, the results seem to advocate for early restoration of UHL, although further research is required to disambiguate the effects of duration and onset of UHL on auditory restoration and on structural neuroplasticity following UHL deprivation and restoration. Ongoing developments on CI devices compatible with Magnetic Resonance Imaging (MRI) examinations will provide a unique opportunity to investigate structural and functional neuroplasticity following CI restoration more directly. ispartof: JOURNAL OF CLINICAL MEDICINE vol:9 issue:3 ispartof: location:Switzerland status: published

Simultaneous bilateral cochlear implantation is often seen as the gold-standard intervention for many people with bilateral deafness. However, this intervention is not as commonly performed in China. This paper presents the surgical technique and the peri- and postoperative safety results from simultaneous bilateral implantation and compares them with those from sequential bilateral implantation. Twenty nine children (aged 12‒18 months) met the inclusion/exclusion criteria and took part in this study. 10 participants received a unilateral cochlear implantation; 19 participants received simultaneous bilateral cochlear implantation. The same standard surgical technique (transmastoid facial recess approach with round window insertion) was used in all implantations. Safety was assessed via monitoring peri- and postoperative adverse events. The helpfulness of select surgical tools was assessed via questionnaire. No adverse events were reported for any participants. All queried surgical tools were helpful in all 244 recorded responses. Simultaneous bilateral cochlear implantation can be performed using the same surgical technique as unilateral implantation and poses no increased safety risk for children aged 1‒2 years.

Hearing loss is a common consequence of the strong acidosis induced by bromate poisoning. Partial hearing recovery has been achieved through medical or rehabilitative therapy but reports of surgical otology treatment for this condition are rare. We report the case of a 48-year-old female patient who underwent cochlear implantation after bromate intoxication had induced bilateral deafness. In cases with life-threatening renal damage, the diagnosis of hearing loss is sometimes delayed, but in our case, hearing impairment was unavoidable despite early detection of symptoms and early disruption of the use of diuretics that could cause hearing damage. Hearing loss 12 hours after bromate ingestion was successfully reversed through cochlear implantation (CI) six months after completing acute phase treatment, including dialysis for acute kidney injury. The benefit of CI for deafness by bromate intoxication is highlighted by this case.

Neonatal conjugated hyperbilirubinemia is a diagnostic challenge. A full term, small for gestational age boy presented with cholestasis, hypoglycemia, hyperferritinemia and severe bilateral deafness. Diagnostic work-up revealed two hereditary diseases: alpha-1-antitrypsin deficiency (PI*ZZ genotype) and autosomal recessive deafness type 3 (compound heterozygous MYO15A gene mutation). In addition, we found late hypoglycemia on full enteral feeding which complicated this case. Hyperferritinemia is an uncommon finding in newborn cholestasis without liver failure.

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) without systemic symptoms but with initial symptoms related to the ear, such as hearing loss, otalgia, and dizziness, has recently been reported. We have categorized this condition as otitis media with AAV (OMAAV), and have recently proposed its diagnostic criteria. Methods: To determine the effectiveness of cochlear implantation (CI) in patients with profound hearing loss due to OMAAV. We examined the language understanding ability of four patients with bilateral profound or total deafness due to OMAAV, who underwent CI. Results: In three of the four patients, the language understanding ability with CI was poor. These three patients with poor performance had characteristic features, including a short interval from the onset of ear symptoms to total deafness and clear enhancement of the cochlea on magnetic resonance imaging (MRI). Conclusion: The poor results observed in patients with a rapidly progressive history of hearing loss were attributed to possible severe and profuse intracochlear bleeding and/or destruction of structures, including the spiral ganglion. All the three patients showed contrast enhancement in the inner ear on MRI. We believe that preoperative evaluation of the history of hearing loss as well as the findings of contrast-enhanced MRI is important for predicting the prognosis after CI., Auris Nasus Larynx, 45(5), pp.922-928; 2018

Objective To present qualification criteria, surgery, and results of cochlear implantation via the middle fossa approach. Study design A retrospective analysis of medical charts of patients. Setting Cochlear implant program in academic medical center. Patients Six adults after middle ear surgery due to chronic otitis media with bilateral deafness or profound hearing loss. Intervention Analysis aimed to identify patients qualified for cochlear implantation via middle fossa approach and to present surgery and result. Main outcome measure Qualification criteria, details of surgery and surgical, functional and audiological results. Results Surgery was completed in four patients and in the remaining two it was stopped because of adhesions and bleeding from the dura. In three patients, there were no postoperative complications and in one individual intracranial hematoma occurred. Postoperative computed tomography showed the intracochlear position of the electrode in all the implanted patients. All of the patients use their implant for 14 to 16 hours daily. One year after the implantation, they had hearing thresholds in the sound-free field of between 25 and 45 dB sound pressure level, number recognition scores between 80 and 100% and monosyllabic words recognition between 30 and 90%. Conclusion Successful cochlear implantation via middle fossa approach results in satisfactory hearing, bringing deaf people into a hearing environment. However, this approach is technically difficult and provides the risk of intracranial complications. In our opinion this approach should be used in special cases only (e.g., postmeningitis or otosclerotis patients with obliteration of basal turn of cochlea). In analysed group it should not be considered the primary solution.

ObjectivesSodium bromate is a strong oxidant, and bromate intoxication can cause irreversible severe-to-profound sensorineural hearing loss. This paper reports the first case in the English literature of bromate-induced hearing loss with hearing recovery measured by formal audiological assessment.Case reportA 72-year-old woman was admitted to hospital with complaints of profound hearing loss, nausea, diarrhoea and anuria after bromate ingestion in a suicide attempt. On admission, pure tone audiometry and auditory brainstem responses showed profound bilateral deafness. Under the diagnosis of bromate-induced acute renal failure and sensorineural hearing loss, continuous haemodiafiltration was performed. When dialysis was discontinued, pure tone audiometry and auditory brainstem responses showed partial threshold recovery from profound deafness.ConclusionSevere-to-profound sensorineural hearing loss is a common symptom of bromate intoxication. Bromate-induced hearing loss may be partially treated, and early application of continuous haemodiafiltration might be useful as a treatment for this intractable condition.

Background:Sudden sensorineural hearing loss is a rare otological condition with potential for dire outcomes including permanent hearing loss. Although the majority of cases are deemed idiopathic, bilateral sudden sensorineural hearing loss represents a rare subset typically related to systemic conditions, with higher morbidity and mortality. A controversial association with acute otitis media has been reported, with few bilateral cases published in the literature.Case report:A very rare case of bilateral sudden sensorineural hearing loss associated with acute otitis media is described, with a review of the literature.Conclusion:The limited evidence available suggests that acute otitis media with tinnitus and/or bacterial pathology may have an increased risk of sudden sensorineural hearing loss, which is consistent with the case described. Although there is no sufficiently powered published evidence to provide definitive treatment guidelines, the literature reviewed suggests that early myringotomy and antibiotics may greatly improve treatment outcomes.

Wolfram syndrome (WS) is an autosomal recessive disorder caused by mutations in WFS1 gene. The clinical features include diabetes insipidus, diabetes mellitus (DM), optic atrophy, deafness, and other variable clinical manifestations. In this paper, we present the clinical and genetic characteristics of 3 WS patients from 3 unrelated Turkish families. Clinical characteristics of the patients and the age of onset of symptoms were quite different in each pedigree. The first two cases developed all symptoms of the disease in their first decade of life. The heterozygous father of case 2 was symptomatic with bilateral deafness. The first ocular finding of one patient (patient 3) was bilateral cataract which was accompanying DM as a first feature of the syndrome. In this patient's family, there were two members with features suggestive of WS. Previously known homozygous mutations, c.460+1G>A in intron 4 and c.1885C>T in exon 8, were identified in these cases. A novel homozygous c.2534T>A mutation was also detected in the exon 8 of WFS1 gene. Because of the rarity and heterogeneity of WS, detection of specific and nonspecific clinical signs including ocular findings and family history in non-autoimmune, insulinopenic diabetes cases should lead to a tentative diagnosis of WS. Genetic testing is required to confirm the diagnosis.

Takayasu arteritis (TAK) is a rare type of noninfectious, systemic vasculitis of unknown etiology. A 45-year-old female diagnosed with TAK complained of acute onset bilateral deafness. Auditory tests revealed complete deafness in both ears. Magnetic resonance imaging with fluid-attenuation inversion recovery of her temporal bone demonstrated high signal intensity in the whole cochlea and vestibule in both ears. Computed tomography carried out 10 months after the onset of bilateral deafness revealed prominent ossification localized in the area around the modiolus in both cochleae. The patient underwent cochlear implantation in both ears. The cochlear lumens were narrowed and filled with scar tissue, which hindered the insertion of an electrode in both ears. In TAK, cases associated with bilateral severe to profound SNHL where corticosteroid therapy is ineffective in improving hearing, cochlear implantation should be considered within a reasonable period to avoid the difficulty of electrode insertion associated with cochlear ossification.

BACKGROUND Data on neurodevelopmental outcomes of children surviving after fetoscopic laser surgery (FLS) for twin-to-twin transfusion syndrome (TTTS) are scarce. METHODS We retrospectively investigated children surviving after FLS for TTTS at 16 to 26 weeks' gestation between 2003 and 2014. Children were evaluated by standardized neurologic examinations using the Kyoto Scale 2001 at a corrected age of 3 years ± 6 months. Neurodevelopmental impairment (NDI) was defined as cerebral palsy (CP), bilateral blindness, bilateral deafness or a developmental quotient (DQ)

Purpose: This study analyses data logs in order to investigate the usage pattern of cochlear implant (CI) recipients with single-sided deafness (SSD-CI) and bilaterally deaf, uni- or bilaterally implanted CI recipients (Uni-CI and Bil-CI). Data logging is available from SCAN, an automated auditory scene classifier which categorizes auditory input into 6 listening environments. Methods: CI usage data were retrospectively available from data logs of 206 CI recipients using the Nucleus 6 system obtained between January 2013 and June 2015. For all recipients, we analysed time on air and time spent in the listening environments. For statistical analysis, we matched the CI recipients according to age and duration of CI experience and classified them into 4 age groups. Results: SSD-CI showed a similar time on air compared to Uni- and Bil-CI. Usage behaviour of SSD-CI was comparable to Uni- and Bil-CI regarding exposure to music, speech in quiet and speech in noise. With increasing age, exposure to quiet increased and exposure to music decreased across all CI recipient groups in relation to time on air. Conclusion: In total, the CI usage pattern of SSD-CI is comparable for the majority of listening environments and age groups to that of Uni- and Bil-CI. The results of our study show that SSD-CI benefit equally from CI implantation.

International audience; PURPOSE : Many bilaterally deaf adults are only able to receive one cochlear implant (CI), resulting in suboptimal listening performance, especially in challenging listening environments. Adding a contralateral routing of signal (CROS) device to a unilateral CI is one possibility to alleviate these challenges. This study examined the benefit of such a CROS device.METHODS : Thirteen adult subjects with at least 6 months of CI use, and no or limited benefit of a hearing instrument in the contralateral ear were included in the study. The perceived benefit of a CROS device in everyday listening environments was evaluated up to 1 year after initial fitting using several questionnaires. Speech intelligibility performance was determined using the French matrix sentence test in quiet and in two speech-in-noise setups and was followed for 3 months after CROS fitting.RESULTS : Subjects indicated high satisfaction with the practical usability of the CROS device and long-term device retention was high. Perceived benefits in everyday listening environments were reported. Formal speech intelligibility tests revealed statistically significant median improvements of 6.93 dB SPL (Wilcoxon Z = 2.380, p = 0.017) in quiet and up to 8.00 dB SNR (Wilcoxon Z = 2.366, p = 0.018) in noise. These benefits were accessible immediately without a need for prolonged acclimatization.CONCLUSIONS : Subjective satisfaction and device retention as well as speech intelligibility benefits in quiet and in noise prove the CROS device to be a valuable addition to a unilateral CI in cases of bilateral deafness where bilateral implantation is not an option.

The advent of cochlear implants has enormously improved the quality of sensory perception in deaf children. Notwithstanding these advantages, the current literature shows a substantial variability in language proficiency among implanted children. This case series explores the variability of language acquisition in congenitally deaf children with cochlear implants. We report 4 prelingually deaf children (mean age=10.5; SD=1.08), affected by a genetically determined bilateral deafness, due to GJB2 gene mutation Cx26. Each implanted child underwent a systematic assessment of speech perception and production, as well as of lexical, morphologic, and syntactic skills in both comprehension and production. Notwithstanding similar clinical histories and similarly good postimplant pure-tone audiometry, two of the four children fared very poorly in speech audiometry, whereas the other two children gained very good results. We suggest that the language impairment detected in (some) implanted children may not be fully accounted for by pure auditory thresholds and that may be the outcome of concomitant damage to core components of the child’s linguistic brain.

Background. Rickettsial illnesses are a group of arthropod-borne remerging diseases. They are subdivided into three groups as typhus, spotted fever, and scrub typhus group. Complications such as reversible bilateral deafness due to spotted fever rickettsioses are infrequently reported in the literature. Case Presentation. We present a young pregnant female who developed bilateral sensorineural deafness on the fifth day of an acute febrile illness with a maculopapular rash. Rickettsia conorii IgG (>1/450) became highly positive, and she received oral azithromycin for ten days with complete resolution of fever and rash. The sensorineural deafness slowly improved over several months. Conclusion. Sensorineural deafness is a rare complication of spotted fever group rickettsioses. Since usage of doxycycline is controversial in pregnancy, azithromycin gave a favourable recovery of fever. Sensorineural deafness took several months to resolve after completion of antibiotics.

Background There have been many reports on the treatment effect of cochlear implantation and hearing aids in the treatment of tinnitus in patients with severe hearing loss. However, as far as we are aware, there are no reports of investigation of treatment approaches for the tinnitus of deaf patients whose communication is solely carried out in sign language due to a long duration of deafness. Case presentation We experienced a case of severe tinnitus with bilateral deafness for more than 50 years. The patient is a 69-year-old woman who communicates with her family solely in sign language. Family stress triggered the onset of tinnitus, accompanied by sleep disorder and palpitations. At the initial visit, she suffered from severe tinnitus (THI 94) as well as strong tendencies toward depression and anxiety. Because neither the patient nor her family was willing to use cochlear implantation, the administration of an antidepressant and a sleep-inducing agent was started, which resulted in improvement of the psychological conditions. Tinnitus distress, synchronized with the heartbeat, was relieved by the addition of autogenic training. At four and half years after the initial visit, the THI score had dropped to 0, and the subjective tinnitus and palpitation had almost disappeared, with only a low dose of antidepressant necessary. Conclusion A deaf patient with severe tinnitus was successfully treated with drug and psychotherapy.

Objective: To report clinical presentation, management and outcomes of a rare complication of cochlear implant surgery. Patient: A 68-year-old man, affected by profound bilateral deafness because of superficial cerebral hemosiderosis, presented to Authors’ Department 8 days after cochlear implant surgery with vomiting, fever, and mental confusion. Brain computed tomographic (CT) scan showed a massive collection of intracranial air from an osteodural defect in the right tegmen mastoideum because of repeated nose blowing in the postoperative period. Intervention: A multilayer reconstruction of the tegmen with obliteration of the mastoid cavity using abdominal subcutaneous adipose tissue was performed, preserving the cochlear implant in place. Main Outcome and Results: Following surgery the patient showed rapid neurological improvement and CT scan performed 2 days later showed complete resolution of the intracranial air collection. He is currently using the cochlear implant with open set performances. Conclusion: Pneumocephalus is a rare complication of cochlear implant surgery. In patients with severe neurological signs following cochlear implantation (CI), pneumocephalus should be suspected. Drilling of mastoid air cells may expose dura mater and positive high pressure events may break meningeal layers and force air into the cranial cavity.

Deafness induces many plastic changes in the auditory neural system. For instance, dendritic changes cause synaptic changes in neural cells. SMI-32, a monoclonal antibody reveals auditory areas and recognizes non-phosphorylated epitopes on medium- and high-molecular-weight subunits of neurofilament proteins in cortical pyramidal neuron dendrites. We investigated SMI-32-immunoreactive (-ir) protein levels in the auditory cortices of rats with induced unilateral and bilateral deafness. Adult male Sprague-Dawley rats were divided into unilateral deafness (UD), bilateral deafness (BD), and control groups. Deafness was induced by cochlear ablation. All rats were sacrificed, and the auditory cortices were harvested for real-time quantitative polymerase chain reaction (RT-qPCR) and western blot analyses at 2, 4, 6, and 12 weeks after deafness was induced. Immunohistochemical staining was performed to evaluate the location of SMI-32-ir neurons. Neurofilament heavy chain (NEFH) mRNA expression and SMI-32-ir protein levels were increased in the BD group. In particular, SMI-32-ir protein levels increased significantly 6 and 12 weeks after deafness was induced. In contrast, no significant changes in protein level were detected in the right or left auditory cortices at any time point in the UD group. NEFH mRNA level decreased at 4 weeks after deafness was induced in the UD group, but recovered thereafter. Taken together, BD induced plastic changes in the auditory cortex, whereas UD did not affect the auditory neural system sufficiently to show plastic changes, as measured by neurofilament protein level.

The risk factors that contribute to the development of hearing loss in the children with chronic kidney failure (CRF) are complications and/or manifestations of the disease itself as well as the side effects of its treatment. There are many similarities between the anatomical, physiological, and pharmacological features of the nephron and the striavascularis of the cochlea. The objective of the present study was the evaluation of the status of the auditory function in the children presenting with CRF.A total of 62 children with CRF were examined. 40 patients were on peritoneal dialysis and 9 on hemodialysis whereas 13 children did not receive hemodialysis. Kidney was transplanted in 7 patients. The registration of transient evoked otoacoustic emission (TEOAE) was performed in all the children. For patients without TEOAE complex underwent the audiological examination.The TEOAE was not registered in 17 of the 62 examined children. According to the results of the comprehensive audiological examinations, 13 children suffered from the hearing loss of different forms and severity while 5 children presented with bilateral deafness. As the duration of CRF progressed, the patients became more prone to develop hearing impairment. Hearing loss is possibly genetically determined. Among the etiological factors underlying the development of the hearing loss the high doses of immunosuppressants and aminoglycosides are considered to be the principal ones.Цель исследования - изучить состояние слуховой функции у детей с хронической болезнью почек (ХБП). В исследование включены 62 ребенка. 40 больных находились на перитонеальном диализе, 9 - на гемодиализе, 13 детям диализ не проводился, 7 пациентам пересажена почка. Пациентам проводилась регистрация задержанной вызванной отоакустической эмиссии (ЗВОАЭ). Детям с незарегистрированной ЗВОАЭ проводилось расширенное аудиологическое исследование слуха, которое включало тональную пороговую аудиометрию, акустическую импедансометрию, регистрацию коротколатентных слуховых вызванных потенциалов. Из 62 детей у 17 ЗВОАЭ не зарегистрирована. При расширенном аудиологическом обследовании у 13 детей диагностирована тугоухость разных форм и степеней, у 5 из 13 - двусторонняя глухота. Нарушение слуха у больных ХПН обусловлено тяжестью и длительностью заболевания и, вероятно, генетически детерминировано - проявилось у пациентов, находящихся на перитонеальном диализе и детей с пересаженной почкой, пороками развития почек, врожденной аномалией развития мочевыделительной системы и множественными дисфункциями со стороны других органов и систем.

Labyrinthitis with vestibulocochlear dysfunction in chronic inflammatory bowl disease is a rare but well described manifestation or complication. The diagnostic and therapeutic possibilities and limitations of this inflammatory otopathy are presented and discussed in the context of a case report. A bilateral loss of vestibular function and sensorineural hearing loss occurred in a 26-year-old male patient with previously diagnosed Crohn's disease. He was treated with immune suppressive therapy during the onset and development of cochleovestibular symptoms. Diagnostic tests included pure tone audiograms, speech audiometry, caloric tests, VEMPs, and MRI. Therapy included azathioprine, corticosteroids (systemic and intratympanic), monoclonal antibodies, and cochlear implants. Despite immunosuppressive therapy including monoclonal antibodies, the patient progressively lost his hearing. Finally, bilateral cochlear implantation was carried out with good results. The treatment of patients with systemic inflammatory or autoimmune disease affecting the cochlear-vestibular organ is challenging. An interdisciplinary approach is needed. In cases of bilateral deafness, cochlear implantation is a satisfactory treatment and should not be delayed unnecessarily.