Demographic analysis of hearing impairment based on various parameters in patients with cochlear implant.

Objectives: To analyse the demographic and clinical variables in children having undergone cochlear implant surgery because of deafness. METHODS: The cross-sectional study was conducted from January to November 2022 at the Centre for Research in Experimental and Applied Medicine laboratory of the Department of Biochemistry and Molecular Biology, Army Medical College, Rawalpindi, Pakistan, in collaboration with the Ear, Nose and Throat Department of Combined Military Hospital, Rawalpindi, and comprised children of eith gender aged up to 10 years who had received cochlear implant. Data was collected through questionnaire-based detailed interviews. Syndromic Hearing Loss, Non-Syndromic Hearing Loss, and Acquired Hearing Loss were identified among the subjects. Data was analysed using SPSS 22. RESULTS: Of the 250 cases, 147(58.8%) were boys, 146(58.4%) were aged 0-5 years, 219(87.6%) had prelingual onset of disease, and 202(80.8%) had a non-progressive disease course. In 203(81.2%) cases, normal developmental milestones were seen. Parental consanguinity was observed in 219(87.6%) cases. However, 63(25.2%) patients had a first-degree relative who had a history of deafness. In 170(68%) cases, hearing loss was hereditary, whereas in 80(32%) it was acquired. Meningitis was the most commonly identified risk factor 55(68.75%). Acquired risk factors and family history had significant association with hearing loss (p<0.05). Speech perception significantly improved in all 219(100%) patients with prelingual hearing loss who underwent cochlear implantation. CONCLUSIONS: Majority of the cases were found to be male, had a prelingual disease onset and a non-progressive disease course. Family history was a significant factor, while meningitis was the most common acquired cause of hearing loss.

A Novel PTRH2 Gene Mutation Causing Infantile-onset Multisystem Neurologic, Endocrine, and Pancreatic Disease in a Bahraini Patient.

Infantile-onset multisystem neurologic, endocrine, and pancreatic disease (IMNEPD) is a rare autosomal recessive multisystemic disease with a prevalence of < 1/1 000 000. The wide spectrum of symptoms and associated diseases makes the diagnosis of this disease particularly challenging. Here, we report a 12-year-old Bahraini male who presented with the core clinical features of IMNEPD including intellectual disability, global developmental delay, sensorineural hearing loss, endocrine dysfunction, and exocrine pancreatic insufficiency. The diagnosis was confirmed by genetic testing using whole exome sequencing. This is the first reported case of IMNEPD from Bahrain and was found to have a novel homozygous peptidyl-tRNA hydrolase 2 (PTRH2) gene mutation (NM_001015509.2: c.370del p.(Glu124Lysfs*4)). Moreover, we conducted an extensive literature review with an emphasis on the variable clinical spectrum and genotypes of previously reported patients in comparison to our case.

Three-dimensional heavily T2-weighted FLAIR in the detection of blood-labyrinthine barrier leakage in patients with sudden sensorineural hearing loss: comparison with T1 sequences and application of deep learning-based reconstruction.

OBJECTIVE: Blood-labyrinthine barrier leakage has been reported in sudden sensorineural hearing loss (SSNHL). We compared immediate post-contrast 3D heavily T2-weighted fluid-attenuated inversion recovery (FLAIR), T1 spin echo (SE), and 3D T1 gradient echo (GRE) sequences, and heavily T2-weighted FLAIR (hvT2F) with and without deep learning-based reconstruction (DLR) in detecting perilymphatic enhancement. METHODS: Fifty-four patients with unilateral SSNHL who underwent ear MRI with three sequences were included. We compared asymmetry scores, confidence scores, and detection rates of perilymphatic enhancement among the three sequences and obtained 3D hvT2F with DLR from 35 patients. The above parameters and subjective image quality between 3D hvT2F with and without DLR were compared. RESULTS: Asymmetry scores and detection rate of 3D hvT2F were significantly higher than 3D GRE T1 and SE T1 (respectively, 1.37, 0.11, 0.19; p < 0.001). Asymmetry scores significantly increased with DLR compared to 3D hvT2F for experienced and inexperienced readers (respectively, 1.77 vs. 1.40, p = 0.036; 1.49 vs. 1.03, p = 0.012). The detection rate significantly increased only for the latter (57.1% vs. 31.4%, p = 0.022). Patients with perilymphatic enhancement had significantly higher air conduction thresholds on initial (77.96 vs. 57.79, p = 0.002) and 5 days after presentation (63.38 vs. 41.85, p = 0.019). CONCLUSION: 3D hvT2F significantly increased the detectability of perilymphatic enhancement compared to 3D GRE T1 and SE T1. DLR further improved the conspicuity of perilymphatic enhancement in 3D hvT2F. 3D hvT2F and DLR are useful for evaluating blood-labyrinthine barrier leakage; furthermore, they might provide prognostic value in the early post-treatment period. CLINICAL RELEVANCE STATEMENT: Ten-minute post-contrast 3D heavily T2-weighed FLAIR imaging is a potentially efficacious sequence in demonstrating perilymphatic enhancement in patients with sudden sensorineural hearing loss and may be further improved by deep learning-based reconstruction. KEY POINTS: • 3D heavily T2-weighted FLAIR (3D hvT2F) is a sequence sensitive in detecting low concentrations of contrast in the perilymphatic space. • 3D hvT2F sequences properly demonstrated perilymphatic enhancement in sudden sensorineural hearing loss compared to T1 sequences and were further improved by deep learning-based reconstruction (DLR). • 3D hvT2F and DLR are efficacious sequences in detecting blood-labyrinthine barrier leakage and with potential prognostic information.

Brazilian Society of Otology task force - Vestibular Schwannoma ‒ evaluation and treatment.

OBJECTIVE: To review the literature on the diagnosis and treatment of vestibular schwannoma. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The topics were divided into 2 parts: (1) Diagnosis - audiologic, electrophysiologic tests, and imaging; (2) Treatment - wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy. CONCLUSIONS: Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team.

A Nationwide Study of GATA2 Deficiency in Italy Reveals Novel Symptoms and Genotype-phenotype Association.

GATA2 deficiency is a rare disorder encompassing a broadly variable phenotype and its clinical picture is continuously evolving. Since it was first described in 2011, up to 500 patients have been reported. Here, we describe a cohort of 31 Italian patients (26 families) with molecular diagnosis of GATA2 deficiency. Patients were recruited contacting all the Italian Association of Pediatric Hematology and Oncology (AIEOP) centers, the Hematology Department in their institution and Italian societies involved in the field of vascular anomalies, otorhinolaryngology, dermatology, infectious and respiratory diseases. Median age at the time of first manifestation, molecular diagnosis and last follow-up visit was 12.5 (age-range, 2-52 years), 18 (age-range, 7-64 years) and 22 years (age-range, 3-64), respectively. Infections (39%), hematological malignancies (23%) and undefined cytopenia (16%) were the most frequent symptoms at the onset of the disease. The majority of patients (55%) underwent hematopoietic stem cell transplantation. During the follow-up rarer manifestations emerged. The clinical penetrance was highly variable, with the coexistence of severely affected pediatric patients and asymptomatic adults in the same pedigree. Two individuals remained asymptomatic at the last follow-up visit. Our study highlights new (pilonidal cyst/sacrococcygeal fistula, cholangiocarcinoma and gastric adenocarcinoma) phenotypes and show that lymphedema may be associated with null/regulatory mutations. Countrywide studies providing long prospective follow-up are essential to unveil the exact burden of rarer manifestations and the natural history in GATA2 deficiency.

Sudden sensorineural hearing loss after Covid-19 infection.

Covid-19 pandemic has taken the world by a storm, pushing the boundaries of human endurance and intellect. Caught at the horns of dilemma, humanity is still struggling with the management of the established symptoms not to mention the novel symptoms. In this regard, the novel symptoms must be highlighted to ensure proper and timely management. Viral aetiology has been an established entity for neurological deficits; hence, it would not be a huge leap to consider the correlation between Covid-19 and Sensorineural hearing loss (SNHL). Here, a case is being presented where the patient developed sudden sensorineural hearing loss after Covid-19 infection.

Cochlear implantation in advanced otosclerosis: utility of pre-operative radiological assessment in predicting intra-operative difficulty and final electrode position.

OBJECTIVE: This study aimed to determine if pre-operative radiological scoring can reliably predict intra-operative difficulty and final cochlear electrode position in patients with advanced otosclerosis. METHOD: A retrospective cohort study of advanced otosclerosis patients who underwent cochlear implantation (n = 48, 52 ears) was compared with a larger cohort of post-lingually deaf adult patients (n = 1414) with bilateral hearing loss and normal cochlear anatomy. Pre-operative imaging for advanced otosclerosis patients and final electrode position were scored and correlated with intra-operative difficulty and speech outcomes. RESULTS: Advanced otosclerosis patients benefit significantly from cochlear implantation. Mean duration of deafness was longer in the advanced otosclerosis group (19.5 vs 14.3 years; p < 0.05). CONCLUSION: Anatomical changes in advanced otosclerosis can result in increased difficulty of surgery. Evidence of pre-operative cochlear luminal changes was associated with intra-operative difficult insertion and final non-scala tympani position. Nearly all electrodes implanted in the advanced otosclerosis cohort were peri-modiolar. No reports of facial nerve stimulation were observed.

[Effect of cochlear implantation on speech recognition under different listening modes in children].

Objective:To investigate speech recognition among children with a unilateral cochlear implant（CI）, bilateral CI, and bimodal hearing and identify the benefits of binaural hearing. Methods:A total of 67 children with severe sensorineural hearing loss who received bilateral CI, bimodal hearing, and unilateral CI from 2012 to 2021 were recruited, including 23 cases with unilateral CI, 25 cases of bimodal hearing, and 19 cases of bilateral CI. The aided hearing threshold at 250-4000 Hz and speech recognition performance of two-syllable words and short sentences in quiet and noisy environments were tested, respectively. Results:There was no significant difference in the hearing threshold results of the bilateral CI group, the bimodal hearing group, and the unilateral CI group by ANOVA（P>0.05）. According to the paired t-test within the group, the speech recognition of disyllabic words and short sentences in quiet and noisy environments were significantly different among the three groups（P<0.05）. There was no significant difference in the speech recognition rates of disyllabic words and short sentences among the three groups in a quiet environment（P>0.05）, but there was a statistically significant difference in a noisy environment（P<0.05）. Additional multiple linear regression analysis showed no significant difference in the recognition rates of disyllabic words and short sentences between the bimodal group and the bilateral CI group in quiet and noisy environments（ß=-0.567, P>0.05; ß=-0.643, P>0.05）. There was a statistically significant difference in speech recognition of disyllabic words and short sentences between the bimodal group and the unilateral CI group in the noise environment（ß=-0.124, P<0.05）, but the difference was not statistically significant in a quiet environment（ß=-0.671, P>0.05）; there was a statistically significant difference in the recognition rate of disyllabic words and short sentences between the bilateral CI group and the unilateral CI group in the noise environment（ß=-0.226, P<0.05）, but the difference was not statistically significant in a quiet environment（ß=-0.341, P>0.05）. Conclusion:Clinicians and guardians are encouraged to provide the most comprehensive audiological evaluation for children with hearing loss. Bilateral CI and bimodal hearing have demonstrated enormous advantages over unilateral CI regarding speech recognition in noise. Bimodal hearing deems a strong recommendation if the contralateral ear has sufficient speech recognition capability. Bilateral CI should be warranted if hearing aids deemed to be ineffective.

Comparison of treatment modalities for single-sided deafness in paediatric patients.

OBJECTIVE: Children with single-sided deafness often receive inconsistent clinical recommendations because there is currently no clear best practice in paediatric single-sided deafness. This systematic review of the literature aimed to compare commonly used treatments and attempted to support the use of a particular treatment modality. METHOD: This was a comprehensive literature review from 1 January 2000 to 22 February 2022; the study compared the outcomes of bone conduction devices and cochlear implantation in paediatric patients with single-sided deafness. RESULTS: Fifteen studies consisting of 202 patients were examined. Variables including speech reception in quiet and noise, as well as quality of life measures were compared. Both cochlear implantation and bone-anchored hearing aids demonstrated benefits in sound perception. Quality of life measures improved with both modalities. CONCLUSION: Although both bone-anchored hearing aids and cochlear implantation appear to provide significant improvements, additional research with more direct comparisons is needed to provide more decisive results.

Townes-Brocks syndrome caused by de novo mutation in spalt-like transcription factor 1 gene: a case report and literature review / 中华围产医学杂志

Objective:To summarize the clinical features and gene variations in children with Townes-Brocks syndrome (TBS).Methods:The clinical data of a female infant diagnosed with TBS caused by human spalt-like transcription factor 1 ( SALL1) gene mutation in Gansu Maternal and Child Health Hospital in May 2022 were analyzed retrospectively. Relevant articles up to July 2022 were retrieved from several databases including CNKI, VIP, Wanfang, Chinese Medical Journal Network and PubMed with the terms of " SALL1 gene" and "Townes-Brocks syndrome". Patients diagnosed with TBS caused by SALL1 gene mutation were retrieved and the clinical phenotype-genotype correlations in patients with TBS caused by frameshift mutation in SALL1 gene were analyzed and summarized. Descriptive statistical analysis was applied. Results:(1) Clinical data: The index patient was a 40-day-old girl exhibiting major clinical manifestations of polycystic kidney dysplasia, congenital external ear deformity, preaxial polydactyly and recto-perineal fistula. Whole exome sequencing and Sanger sequencing revealed a heterozygous variation of c.420delC (p.S141fs*42) in the SALL1 gene, while the same gene was found to be wild type in her parents and sister. The variant was predicted to be pathogenic (PVS1+PS2+PM2). (2) Literature review retrieved 161 cases of TBS, of which 71 were attributable to a frameshift mutation in SALL1 gene. Clinical phenotypes of the 71 cases and the index case were summarized. TBS was mainly characterized by external ear, hand and anal deformities, sometimes accompanied by hearing loss, abnormal kidney development and foot deformity. A small number of affected cases presented with rare clinical phenotypes such as abnormal eyes, hypothyroidism and abnormal development. At present, the human gene mutation database records 110 variations in the SALL1 gene, with a majority located in exon 2. The most common mutation type was frameshift variation, accounting for 52%, followed by missense variation and nonsense variation. Conclusion:TBS should be considered in children with ear, hand and anal malformations, accompanied by renal dysfunction and hearing loss, and genetic testing is recommended for timely diagnosis.

Correlation between cerebral small vessel disease and sudden sensorineural hearing loss / 国际脑血管病杂志

Objective:To investigate the relationship between the overall burden score of cerebral small vessel disease (CSVD) and sudden sensorineural hearing loss (SSNHL) and its severity.Methods:Patients with SSNHL admitted to Zhengzhou People’s Hospital from January 2019 to June 2022 were used as the case group, and age- and gender-matched patients with tension headache or benign positional vertigo were used as the control group. MRI was used to evaluate the phenotype of CSVD, including white matter hyperintensities (WMHs), cerebral microbleeds (CMBs), enlarged perivascular spaces (EPVS), and lacune of presumed vascular origin, and then the total burden score of CSVD was calculated. Multivariate logistic regression analysis was used to determine the independent factors of SSNHL. Ordinal logistic regression analysis was used to investigate the relationship between the overall burden of CSVD and the severity of hearing loss in patients with SSNHL. Results:A total of 86 patients with SSNHL and 90 age- and gender-matched controls were included. There were statistically significant differences in triglycerides, high-density lipoprotein cholesterol, fasting blood glucose levels, WMHs overall score classification, periventricular WMHs score classification, deep WMHs score classification, CMBs, moderate to severe EPVS, CSVD overall burden score and classification between the two groups (all P<0.05). Multivariate logistic regression analysis showed that after adjusting for high-density lipoprotein cholesterol, fasting blood glucose ≥6.1 mmol/L (odds ratio [ OR] 2.149, 95% confidence interval [ CI] 1.042-4.432; P=0.038), triglycerides ≥1.7 mmol/L ( OR 7.012, 95% CI 2.962-16.597; P=0.000), CSVD overall burden score >1 (compared to 0 point, 2 points: OR 4.095, 95% CI 1.364-12.291, P=0.012; 3 points: OR 5.776, 95% CI 1.888-17.614, P=0.002; 4 points: OR 7.900, 95% CI 2.526-24.706; P=0.000) were significantly independently correlated with SSNHL. Ordinal logistic regression analysis showed that after adjusting for age and fasting blood glucose, the flat decline type hearing loss ( OR 1.276, 95% CI 1.131-1.618; P=0.044) and total deafness type hearing loss ( OR 1.029, 95% CI 1.002-1.058; P=0.038), and the overall burden of CSVD being moderate ( OR 1.318, 95% CI 1.036-1.677; P=0.025) and severe ( OR 2.330, 95% CI 1.232-4.406; P=0.009) were significantly independent associated with the degree of hearing loss in patients with SSNHL. Conclusion:The CSVD total burden score of moderate to severe degree is independently associated with SSNHL and the degree of hearing loss.

Application value of auditory steady-state response combined with auditory brainstem response for hearing assessment in children with sensorineural hearing loss / 中国基层医药

Objective:To investigate the application value of auditory steady-state response (ASSR) combined with short sound auditory brainstem response (ABR) for hearing assessment in children with sensorineural hearing impairment, providing evidence for clinical diagnosis of the disorder.Methods:A total of 90 children with sensorineural hearing impairment who received treatment at Jiaxing Maternity and Child Health Care Hospital from January 2020 to June 2021 were included in this prospective study. These children were randomly divided into a control group and an observation group ( n = 45 per group). The children in the control group underwent ABR testing, while those in the observation group underwent ASSR testing in addition to ABR thresholds testing. The hearing impairment was compared between the two groups. The ABR and ASSR thresholds were compared among children with different degrees of hearing impairment. The correlation between ABR and ASSR at 2 and 4 kHz carrier frequencies was analyzed in the observation group. Results:The abnormal rate of hearing in the observation group was 68.89% (31/45), which was significantly higher than 44.44% (20/45) in the control group ( χ2 = 5.48, P = 0.019). At a carrier frequency of 0.5 kHz, the ASSR thresholds of children with moderate and severe hearing impairment in the observation group were (63.11 ± 6.82) dB nHL and (84.65 ± 5.31) dB nHL, respectively, which were significantly higher than the ABS thresholds (56.12 ± 4.63) dB nHL and (76.87 ± 5.15) dB nHL ( t = 2.94, 2.78, both P < 0.05). There was a positive correlation between ABR and ASSR thresholds at 2 and 4 kHz carrier frequencies in children with different degrees of hearing impairment in the observation group ( r = 0.896, 0.901, both P < 0.05). Conclusion:The combination of ABR and ASSR testings is more accurate in judging the degree of hearing impairment in children with sensorineural hearing impairment than the ABR testing, and can provide a reliable basis for later clinical treatment.

Brazilian Society of Otology task force - Vestibular Schwannoma - evaluation and treatment

Abstract Objective: To review the literature on the diagnosis and treatment of vestibular schwannoma. Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results: The topics were divided into 2 parts: (1) Diagnosis - audiologic, electrophysiologic tests, and imaging; (2) Treatment - wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy. Conclusions: Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team.

Bilateral symmetrical maculopathy and heterochromia in Waardenburg syndrome / Maculopatia simétrica bilateral e heterocromia na síndrome de Waardenburg

ABSTRACT Waardenburg syndrome is a rare congenital genetic disorder characterized by sensorineural hearing loss and pigmentary abnormalities of the hair, skin, and eyes. Based on the different clinical presentations, it is divided into four subtypes as in WS1 to WS4. This report describes a 15-year-old boy who presented with low vision and bilateral hearing loss. His visual acuity was 20/200 in both eyes. Slit-lamp examination revealed complete iris heterochromia, with one blue iris and one brown iris. Fundus examination showed symmetrical pigmentation of the retina and choroid, with atrophy of the pigment epithelium in the macular region, notably also in the eye with normal iris pigment illustrating the broad spectrum of the iris and fundus pigmentation as part of this syndrome. A carefully clinical and ophthalmological evaluation should be done to differentiate various types of Waardenburg syndrome and other associated auditory-pigmentary syndrome. Early diagnosis in some cases may be crucial for the adequate development of patients affected with this condition.

RESUMO A síndrome de Waardenburg é uma doença genética congênita rara caracterizada por perda auditiva neurossensorial e anormalidades pigmentares do cabelo, da pele e dos olhos. Com base nas diferentes apresentações clínicas, é dividida em quatro subtipos (WS1 a WS4). Este relato descreve o caso de um menino de 15 anos que apresentava baixa visão e perda auditiva bilateral. Sua acuidade visual era de 20/200 em ambos os olhos. O exame em lâmpada de fenda revelou heterocromia completa da íris, com uma íris azul e uma íris marrom. A fundoscopia mostrou pigmentação simétrica da retina e coroide, com atrofia do epitélio pigmentar na região macular, notadamente também no olho com pigmento de íris normal, ilustrando o amplo espectro de pigmentação de íris e fundo como parte dessa síndrome. Uma avaliação clínica e oftalmológica criteriosa deve ser feita para diferenciar os vários tipos de síndrome de Waardenburg e outras síndromes auditivo-pigmentares associadas. O diagnóstico precoce em alguns casos pode ser crucial para o desenvolvimento adequado dos pacientes acometidos por essa condição.

Satisfaction and quality of life in cochlear implant users with long sensory deprivation / Satisfação e qualidade de vida em usuários de implante coclear com longo tempo de privação sensorial

ABSTRACT Purpose The aim of the study was to verify the level of satisfaction of CI users with long periods of hearing deprivation, highlighting the positive and negative aspects of the use of the device and their quality of life. Methods This is a analytical research, of the type observational cross-sectional study. The study was performed with 24 patients from a private Institute of Otorhinolaryngology. Three surveys were applied: Satisfaction with Amplification in Daily Life (SADL), International Outcome Inventory - Cochlear Implant (IOI - CI) to assess cochlear implant satisfaction and WHOQOL- bref to assess the quality of life. The results in the pre and post lingual groups were compared. Results The highest degree of satisfaction was reported with regard to personal image, positive effects, and how the users feel about their CI. The lowest degree of satisfaction was reported regarding the cost-benefit of the CI and the competitive noise. In the WHOQOL-bref assessment, the highest scores were found in physical, psychological and social relations domains. When comparing the results of the surveys, the pre and post-lingual groups showed no difference in relation to the achieved scores. Conclusion The participants had a high level of satisfaction with the use of cochlear implants. The longer the sensory deprivation time, the greater the degree of satisfaction with the device. The use of the CI electronic device reflects on the individual's quality of life.

RESUMO Objetivo Verificar o nível de satisfação de usuários de IC com longos períodos de privação auditiva, destacando os aspectos positivos e negativos do uso do dispositivo e avaliar a qualidade de vida desses indivíduos. Método Trata-se de uma pesquisa analítica, do tipo estudo observacional transversal. O estudo foi realizado com 24 pacientes de um instituto privado de Otorrinolaringologia. Foram aplicados três questionários: Satisfaction with Amplification in Daily Life (SADL), International Outcome Inventory- Implante Coclear (IOI - IC) para avaliar a satisfação com o implante coclear e WHOQOL-bref para avaliar a qualidade de vida. Os resultados nos grupos pré e pós-lingual foram comparados. Resultados O maior grau de satisfação foi relatado em relação à imagem pessoal, efeitos positivos e como os usuários se sentem em relação ao seu IC. O menor grau de satisfação ocorreu em relação ao custo-benefício do IC e ao ruído competitivo. Na avaliação do WHOQOL-bref, os maiores escores foram encontrados nos domínios físico, psicológico e relações sociais. Os grupos pré e pós-lingual não apresentaram diferença em relação aos escores alcançados. Conclusão Os participantes apresentaram alto nível de satisfação com o uso do implante coclear. Quanto maior o tempo de privação sensorial maior o grau de satisfação com o dispositivo. O uso do dispositivo eletrônico de IC reflete melhora na qualidade de vida do indivíduo.

[Cochlear nerve canal stenosis: a review of recent research].

Some patients with severe-profound sensorineural hearing loss （SNHL） with normal cochlear anatomical structure received cochlear implantation （CI） and the hearing and speech rehabilitation effect was not ideal. Through retrospective analysis, it was found that some of these patients had cochlear never canal （CNC） stenosis, or atresia in severe cases.This article reviews the development of the CNC, the diagnostic criteria of CNC stenosis and the results of hearing and speech rehabilitation in these patients after CI.

Granulomatosis with polyangiitis in a patient with polydipsia, facial nerve paralysis, and severe otologic complaints: a case report and review of the literature.

BACKGROUND: Granulomatosis with polyangiitis, formerly known as Wegener granulomatosis, is a necrotizing vasculitis with granulomatous inflammation that belongs to the class of antineutrophil cytoplasmic antibodies-positive diseases. It occurs in a localized and a systemic form and may present with a variety of symptoms. Involvement of the upper respiratory tract is very common, while neurologic, endocrinological, and nephrological dysfunction may occur. CASE PRESENTATION: We describe the case of a 29-year-old Central European male patient presenting with severe bilateral sensorineural hearing loss, otorrhea, and one-sided facial nerve paralysis. The patient was unsuccessfully treated with i.v. antibiotics at another hospital in Berlin, and tympanic tubes were inserted. After presentation to our emergency room, he was hospitalized and further diagnostics started. Increased fluid intake and 12 kg weight gain over the last months were reported. The patient was diagnosed with granulomatosis with polyangiitis and diabetes insipidus. The patient's condition improved after treatment with rituximab. DISCUSSION: A comprehensive PubMed search of all articles with granulomatosis with polyangiitis and diabetes insipidus was conducted to assess which combination of symptoms occurs simultaneously and whether other parts of the pituitary are commonly involved. The 39 selected articles, describing 61 patients, showed that ear-nose-throat involvement occurred most commonly, in 71% of cases. Of patients, 59% had involvement of the anterior pituitary gland, while true panhypopituitarism occurred in 13% of cases. Only one case report featured the same set of symptoms as described herein. CONCLUSION: Granulomatosis with polyangiitis is a highly variable disease, commonly involving the upper airways, but that may present with symptoms solely related to the pituitary gland. Clinicians should have a low threshold to investigate for granulomatosis with polyangiitis in patients with therapy-resistant otorrhea. Patients may present with a complex set of symptoms, and integrating different specialists when additional symptoms occur may lead to faster diagnosis.

Probability of clinically significant hearing recovery following salvage intratympanic steroids for sudden sensorineural hearing loss in the 'real world'.

OBJECTIVE: This study aimed to determine the probability of hearing recovery in patients with idiopathic sudden sensorineural hearing loss following salvage intratympanic steroids. METHOD: A retrospective review of all patients receiving salvage intratympanic steroid injections for idiopathic sudden sensorineural hearing loss was performed (January 2014 to December 2019). Twenty-two patients were identified, of whom 15 met inclusion criteria. Pre- and post-treatment audiograms were compared with the unaffected ear. Hearing recovery was categorised based on American Academy of Otolaryngology Head and Neck Surgery criteria. RESULTS: Only 1 patient out of 15 (6.7 per cent) made a partial recovery, and the remainder were non-responders. The median duration of time between symptom onset and first salvage intratympanic steroid treatment was 52 days (range, 14-81 days). No adverse reactions were observed. CONCLUSION: 'Real world' patients with idiopathic sudden sensorineural hearing loss present differently to those in the literature. Sudden sensorineural hearing loss should be diagnosed with care and intratympanic steroid injections initiated early if considered appropriate. Patients should make an informed decision on treatment based on prognostic factors and local success rates.

[Tinnitus and depression after cochlear implantation in adult sensorineural deafness].

Objective:The aim of this study is to investigate the changes and correlation of tinnitus and depression in adult patients with severe deafness after cochlear implantation. Methods:A total of 166 adult patients who underwent cochlear implantation（CI） were retrospectively selected as the research objects. All patients were investigated by Chinese Version of the Tinnitus Handicap Inventory（THI-C） and Chinese Version of the Beck Depression Inventory-Ⅱ（BDI-Ⅱ-C） before and after operation, and the improvement of tinnitus after operation was observed THI-C and BDI-Ⅱ-C scores before and after operation and the correlation between them. Results:Re-examination at 6 months after CI showed that the evaluation grade of tinnitus disability before and after the operation decreased significantly（Z=-9.478, P<0.001）, and the THI-C score （t=69.128, P<0.001）, and BDI-Ⅱ-C score （t=58.531，P<0.01）were significantly reduced. Spearman correlation analysis showed that there was a significant positive correlation between THI-C and BDI-Ⅱ-C scores before and after operation, aswell as the improvement of THI-C score and BDI-Ⅱ-C score（rpreoperative=0.763, rpostoperative=0.741, rdifference=0.741, all P<0.001）. Conclusion:Cochlear implantation can significantly improve tinnitus and depression in adult patients with sensorineural hearing loss. With the improvement of tinnitus, the depressive state of patients can be alleviated accordingly.

[Effects of cytomegalovirus infection on infants' hearing and speech development].

Objective:To investigate the effects of cytomegalovirus（CMV） infection on infants' hearing and speech development. Methods:A total of 192 infants with cytomegalovirus infection were selected as research objects（CMV group）. Among 320 normal infants who received physical examinations in the Second Affiliated Hospital of Zhengzhou University during the same period were selected as the control group. Using transiently evoked otoacoustic emission to conduct initial hearing screening. Jointing automatic auditory brainstem response screening method to follow up for infants infected with cytomegalovirus. Those who failed to pass the screening were diagnosed with auditory brainstem response and acoustic immittance examination. The two groups of infants were evaluated for follow-up at the age of 12, 24, 36 months using the Gesell Development scale. Results:Hearing screening（initial hearing screening and 42 d hearing re-screening）: CMV group retrospectively failed rates 28.65%（55/192）, 31.77%（61/192）, normal control group retrospectively failed rates 9.06%（29/320）, 4.06%（13/320）, the results of the two groups' hearing screening showed statistically significant differences（P<0.05）. 48 cases of diagnostic ABR were abnormal in CMV group in 3 month's hearing diagnosis, including 11 cases of secretory otitis media, 37 cases of sensorineural hearing loss. Follow-up for 36 months, 192 infants with CMV infection were confirmed congenital SNHL 37 cases（19.27%）, 21 cases of delayed SNHL（10.94%）, a total of diagnosis with SNHL 58 cases（30.21%）. The development quotient（DQ） of CMV group were respectively 92.05±4.68, 86.53±4.46, 85.92±4.82 in 12, 24, 36 months, and the DQ value of the normal control group were respectively 93.10±4.56, 94.35±4.52, 95.03±4.16. At the age of 24, 36 months, the DQ value of two groups' differences were statistically significant（P<0.05）. Conclusion:CMV infection is hearing loss' risk factors. It had the characteristics of volatility, delay and progressive decline. Follow-up should be strengthened for hearing and speech development.