Enlarged vestibular aqueduct as a cause of postneonatal deafness.

INTRODUCTION: The enlarged vestibular aqueduct (EVA) is the most frequent malformation of the inner ear associated with sensorineural hearing loss (5-15%). It exists when the diameter in imaging tests is greater than 1.5 mm at its midpoint. The association between hearing loss and EVA has been described in a syndromic and non-syndromic manner. It can appear as a familial or isolated form and the audiological profile is highly variable. The gene responsible for sensorineural hearing loss associated with EVA is located in the same region described for Pendred syndrome, where the SCL26A4 gene is located. OBJECTIVE: To describe a series of children diagnosed with EVA in order to study their clinical and audiological characteristics, as well as the associated genetic and vestibular alterations. METHOD: Retrospective study of data collection of children diagnosed with EVA, from April 2014 to February 2023. RESULTS: Of the 17 cases, 12 were male and 5 were female. 5 of them were unilateral and 12 bilateral. In 5 cases, a cranial traumatism triggered the hearing loss. Genetic alterations were detected in 3 cases: 2 mutations in the SCL26A4 gene and 1 mutation in the MCT1 gene. 13 patients (76.5%) were rehabilitated with hearing aids and 9 of them required cochlear implantation. DISCUSSION: The clinical importance of AVD lies in the fact that it is a frequent finding in the context of postneonatal hearing loss. It is convenient to have a high suspicion to diagnose it with imaging tests, to monitor its evolution, and to rehabilitate early.

[Clinical analysis of 11 cases of otogenic intracranial complications treated by multidisciplinary collaboration].

Objective:To analyze the clinical diagnosis, treatment ，and surgical timing of otogenic intracranial complications. Methods:The clinical data of 11 patients with intracranial complications with ear symptoms as the first manifestation in Department of Otorhinolaryngology Head and Neck Surgery, Qilu Hospital of Shandong University（Qingdao） from December 2014 to June 2022 were collected, including 8 males and 3 females, aged from 4 to 69 years. All patients had complete otoendoscopy, audiology, imaging and etiology examination, and the diagnosis and treatment plan was jointly developed through multidisciplinary consultation according to the critical degree of clinical symptoms and imaging changes. Among the 11 patients, 5 cases were treated with intracranial lesions first in neurosurgery department and middle ear lesions later in otolaryngology, 3 cases of meningitis, were treated with middle ear surgery after intracranial infection control, 1 case was treated with middle ear lesions and intracranial infection simultaneously, and 2 cases were treated with sigmoid sinus and transverse sinus thrombosis conservatively. They were followed up for 1-6 years. Descriptive statistical methods were used for analysis. Results:All the 11 patients had ear varying symptoms, including ear pain, pus discharge and hearing loss, etc, and then fever appeared, headache, disturbance of consciousness, facial paralysis and other intracranial complication. Otoendoscopy showed perforation of the relaxation of the tympanic membrane in 5 cases, major perforation of the tension in 3 cases, neoplasia in the ear canal in 1 case, bulging of the tympanic membrane in 1 case, and turbidity of the tympanic membrane in 1 case. There were 4 cases of conductive hearing loss, 4 cases of mixed hearing loss and 3 cases of total deafness. Imaging examination showed cholesteatoma of the middle ear complicated with temporal lobe brain abscess in 4 cases, cerebellar abscess in 2 cases, cholesteatoma of the middle ear complicated with intracranial infection in 3 cases, and sigmoid sinus thrombophlebitis in 2 cases. In the etiological examination, 2 cases of Streptococcus pneumoniae were cultured in the pus of brain abscess and cerebrospinal fluid, and 1 case was cultured in streptococcus vestibularis, Bacteroides uniformis and Proteus mirabilis respectively. During the follow-up, 1 patient died of cardiovascular disease 3 years after discharge, and the remaining 10 patients survived. There was no recurrence of intracranial and middle ear lesions. Sigmoid sinus and transverse sinus thrombosis were significantly improved. Conclusion:Brain abscess, intracranial infection and thrombophlebitis are the most common otogenic intracranial complications, and cholesteatoma of middle ear is the most common primary disease. Timely diagnosis, multidisciplinary collaboration, accurate grasp of the timing in the treatment of primary focal and complications have improved the cure rate of the disease.

[Hearing rehabilitation in postmeningitis deafness in the context of the time factor]. / Agyhártyagyulladás okozta súlyos fokú sensorineuralis halláscsökkenés rehabilitációs esélyei az idofaktor tekintetében.

INTRODUCTION: Postmeningitis deafness appears in 0-11% of the meningitis cases. Cochlear ossification can develop in these patients, which may make the hearing rehabilitation impossible with cochlear implantation. Due to ossification, it is critical to refer patients to the implant centre without any delay. OBJECTIVE: The aim of this study was to examine the time factor between the appearance of deafness and the first examination in a cochlear implant centre, the possibilities and effectivity of hearing rehabilitation. METHOD: In our tertial referral centre, postmeningitis deafened patients were examined between 2014 and 2022 retrospectively. Hearing results, imaging, possibilities of rehabilitation, complications of cochlear implantations and the hearing results were investigated. RESULTS: 8 patients (3 children, 5 adults) were investigated. The time between the start of deafness and the first appearance varied between 3 weeks to 9 years. Bilateral profound hearing loss was measured in all patients. In 6 cases, cochlear ossification was observed (4 patients bilateral). Cochlear implantation was conducted in 5 patients (4 bilateral, 1 unilateral). In 3 cases, implantation was impossible due to severe ossification. Hearing results showed good hearing levels with poor speech perception in all cases. DISCUSSION: The rehabilitation of severe hearing loss caused by meningitis can present many challenges to clinicians. A critical point in the care is the urgent referral of patients to a cochlear implantation centre as soon as possible after the life-threatening condition has passed. The implementation of further diagnostic and the earliest possible implantation is the responsibility of the implantation centre itself. CONCLUSION: It is recommended to develop a new protocol with the involvement of allied professions to clear patient pathways for an effective treatment strategy. Orv Hetil. 2023; 164(19): 729-738.

Recurrent Swelling in Pediatric Cochlear Implant Patients.

OBJECTIVE: To determine the prevalence, characteristics, and outcomes of pediatric patients with recurrent swelling over their cochlear implant receiver package. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: A total of 332 bilateral cochlear implant patients under the age of 18 years were reviewed. Twelve patients with more than one episode of swelling around their cochlear implant receiver package were isolated. Patients with clinical evidence of infection were excluded from the study. The etiology of hearing loss was heterogeneous. INTERVENTION: Three patients underwent ultrasound, and three patients underwent bedside aspiration. Most patients were treated with 7 days of oral broad-spectrum antibiotics. MAIN OUTCOME MEASURE: Incidence, frequency, and course of recurrent swelling around cochlear implant receiver packages. RESULTS: The first swelling appeared between 0.86 and 9.95 years after surgery (mean, 3.38), and the occurrence of the last episode ranged from 0.06 to 3.42 years from the current date (mean, 1.04). The total number of episodes ranged from 2 to 18 (mean, 6). Seven patients had unilateral swellings, and five had bilateral swellings. Swellings were associated with upper respiratory tract infection or minor trauma, or had no identifiable cause. Aspiration in three cases showed evidence of altered blood. CONCLUSIONS: Recurrent otherwise asymptomatic swelling over cochlear implant receiver packages in children is more common than initially thought. Possible causes include hematoma and seroma secondary to upper respiratory tract infection. The incidence and the timing of swelling are variable. There were no swelling-related device failures or reimplantations so patients and parents can be reassured about the long-term outcome.

Clinical analysis of 11 cases of otogenic intracranial complications treated by multidisciplinary collaboration / 临床耳鼻咽喉头颈外科杂志

Objective:To analyze the clinical diagnosis, treatment ，and surgical timing of otogenic intracranial complications. Methods:The clinical data of 11 patients with intracranial complications with ear symptoms as the first manifestation in Department of Otorhinolaryngology Head and Neck Surgery, Qilu Hospital of Shandong University（Qingdao） from December 2014 to June 2022 were collected, including 8 males and 3 females, aged from 4 to 69 years. All patients had complete otoendoscopy, audiology, imaging and etiology examination, and the diagnosis and treatment plan was jointly developed through multidisciplinary consultation according to the critical degree of clinical symptoms and imaging changes. Among the 11 patients, 5 cases were treated with intracranial lesions first in neurosurgery department and middle ear lesions later in otolaryngology, 3 cases of meningitis, were treated with middle ear surgery after intracranial infection control, 1 case was treated with middle ear lesions and intracranial infection simultaneously, and 2 cases were treated with sigmoid sinus and transverse sinus thrombosis conservatively. They were followed up for 1-6 years. Descriptive statistical methods were used for analysis. Results:All the 11 patients had ear varying symptoms, including ear pain, pus discharge and hearing loss, etc, and then fever appeared, headache, disturbance of consciousness, facial paralysis and other intracranial complication. Otoendoscopy showed perforation of the relaxation of the tympanic membrane in 5 cases, major perforation of the tension in 3 cases, neoplasia in the ear canal in 1 case, bulging of the tympanic membrane in 1 case, and turbidity of the tympanic membrane in 1 case. There were 4 cases of conductive hearing loss, 4 cases of mixed hearing loss and 3 cases of total deafness. Imaging examination showed cholesteatoma of the middle ear complicated with temporal lobe brain abscess in 4 cases, cerebellar abscess in 2 cases, cholesteatoma of the middle ear complicated with intracranial infection in 3 cases, and sigmoid sinus thrombophlebitis in 2 cases. In the etiological examination, 2 cases of Streptococcus pneumoniae were cultured in the pus of brain abscess and cerebrospinal fluid, and 1 case was cultured in streptococcus vestibularis, Bacteroides uniformis and Proteus mirabilis respectively. During the follow-up, 1 patient died of cardiovascular disease 3 years after discharge, and the remaining 10 patients survived. There was no recurrence of intracranial and middle ear lesions. Sigmoid sinus and transverse sinus thrombosis were significantly improved. Conclusion:Brain abscess, intracranial infection and thrombophlebitis are the most common otogenic intracranial complications, and cholesteatoma of middle ear is the most common primary disease. Timely diagnosis, multidisciplinary collaboration, accurate grasp of the timing in the treatment of primary focal and complications have improved the cure rate of the disease.

Management of children with co-occurring sleep disordered breathing and hearing loss.

OBJECTIVES: To examine hearing loss incidence in a pediatric otolaryngology patients with sleep disordered breathing, and secondarily, identify possible disparities in management. METHODS: A retrospective case-control study of pediatric patients with sleep-disordered breathing or obstructive sleep apnea from 2012 to 2019 was conducted at a private, not-for-profit, academic, tertiary care center. Study parameters were extracted from the electronic medical record and compared in study groups of patients with sleep-disordered breathing generated based on presence of hearing loss (cases) versus absence (controls). Study parameters were also compared in study groups based on timing of hearing loss onset. RESULTS: 14.8% of patients with sleep disordered breathing had hearing loss. Hearing loss was associated with an increased risk of undergoing adenotonsillectomy (OR 1.632 [1.294-2.058], p < 0.001, adjusted for age). In patients who underwent polysomnographic testing, 9.8% patients had pre-existing hearing loss and 12% patients developed hearing loss over the study period. Patients with pre-existing hearing loss and those who developed hearing loss had significantly more visits with otolaryngology compared to controls (p < 0.001). Hearing loss did not significantly delay adenotonsillectomy. More patients who developed hearing loss had adenotonsillectomies (OR 2.475 [1.672-3.663], p < 0.001, adjusted for age) versus controls. This difference was not identified in patients with pre-existing hearing loss. CONCLUSION: Patients with evidence of hearing loss in addition to sleep disordered breathing had more adenotonsillectomies performed and more clinic visits. Further work must be done to understand the associations and implications of hearing loss in this population.

[The cochlear implantation in patients with syndromic deafness. Clinical examples]. / Osobennosti provedeniya kokhlearnoi implantatsii u patsientov s sindromal'noi glukhotoi.

OBJECTIVE: To describe clinical observations of patients with syndromic deafness. RESULTS: Deaf patients with CHARGE, Crouzon, and Wildervanck syndromes were monitored at the Russian Research Clinical Center for Audiology and Hearing Rehabilitation (Moscow) in different years. All of them were diagnosed having bilateral congenital deafness. After collecting anamnesis, evaluating the results of computed tomography of the temporal bones, and audiological examination, it was decided to conduct the cochlear implantation. CONCLUSION: The only method that allows patients with bilateral congenital deafness to gain hearing is the cochlear implantation. The malformations of the tympanic cavity structures, an abnormal course of the facial nerve canal lead to technical difficulties during the surgical stage of cochlear implantation. The navigation equipment, monitoring of the facial nerve makes it easier to find anatomical structures, as well as to avoid injuries.

[Hearing loss following the use of topical otitis therapeutics in dogs]. / Hörverlust nach Anwendung topischer Otitispräparate bei Hunden.

OBJECTIVE: Hearing loss occurring in temporal association with the topical application of otic medications is regularly reported to the Federal Office of Consumer Protection and Food Safety (Bundesamt für Verbraucherschutz und Lebensmittelsicherheit - BVL) in the form of adverse event (AE) reports. Although deafness or impaired hearing are listed as possible adverse reactions in the Summary of Product Characteristics of the otic medications approved in Germany little information about the underlying causes is available to date. MATERIAL AND METHODS: A search for cases reporting impaired hearing following the use of otic medication was conducted in the national AE database. Subsequently, descriptive analysis was performed. Due to their small number, cases involving cats were excluded. Possible risk factors and causes of hearing loss were considered against the background of current literature. RESULTS: While dogs of all age groups were affected, the majority of reports referred to dogs older than 10 years of age. Besides crossbreds, dogs of the breeds West Highland White Terrier, Dalmatian, Miniature Poodle and French Bulldog were most frequently involved. The analysis of the available data does not point to specific products or active substances that could be associated with a more frequent occurrence of hearing loss. CONCLUSION AND CLINICAL RELEVANCE: In addition to possible ototoxicity of a product, other causes of hearing loss should be considered. These include the underlying otitis itself, age-related hearing loss, previously undetected unilateral congenital deafness, or conductive deafness due to obstruction of the ear canal. Treatment options include discontinuation of potentially ototoxic substances or treatment of conductive deafness, e. g. by removal of drug residues and exudate or treatment of otitis media. In the case of hearing loss subsequent to the use of otic medication, the BVL should be notified of this event in as much detail as possible in order to further improve the data situation concerning this topic.

Brain MRI findings of prelingually deaf children and cochlear implant outcome: Preliminary results.

OBJECTIVE: To estimate the incidence of brain abnormalities in a cohort of prelingually deaf children and whether these abnormalities can impact the hearing outcomes of patients eligible for cochlear implantation (CI). METHODS: We performed a retrospective review of consecutive medical charts of prelingually deaf children under 12 years of age who underwent brain magnetic resonance imaging (MRI) during their preoperative workup for CI surgery. We used the category of auditory performance (CAP) test and the speech intelligibility rating (SIR) test to assess the hearing and speech performance of the children, respectively. RESULTS: The MRIs of 285 patients, 174 boys and 111 girls with a mean age of 36.4 (±16) months, were evaluated for this study. We identified 31 patients with abnormal findings (10.88%): (17/31) (54.8%) had MRI brain abnormalities, (9/31) (29%) had inner ear anomalies, and (5/31) (16.1%) had both inner ear and brain abnormalities. The most frequent inner ear anomaly was an enlarged vestibular aqueduct, while white matter lesions were the most common brain abnormality. The CAP and SIR mean score of patients with inner ear anomalies was slightly, but not significantly, higher than those of patients with brain abnormalities. CONCLUSION: CAP and SIR scores were not significantly different in children with brain abnormalities than inner ear anomalies. These patients can still benefit from CI to improve their overall hearing and speech performance.

Toward Personalized Diagnosis and Therapy for Hearing Loss: Insights From Cochlear Implants.

ABSTRACT: Sensorineural hearing loss (SNHL) is the most common sensory deficit, disabling nearly half a billion people worldwide. The cochlear implant (CI) has transformed the treatment of patients with SNHL, having restored hearing to more than 800,000 people. The success of CIs has inspired multidisciplinary efforts to address the unmet need for personalized, cellular-level diagnosis, and treatment of patients with SNHL. Current limitations include an inability to safely and accurately image at high resolution and biopsy the inner ear, precluding the use of key structural and molecular information during diagnostic and treatment decisions. Furthermore, there remains a lack of pharmacological therapies for hearing loss, which can partially be attributed to challenges associated with new drug development. We highlight advances in diagnostic and therapeutic strategies for SNHL that will help accelerate the push toward precision medicine. In addition, we discuss technological improvements for the CI that will further enhance its functionality for future patients. This report highlights work that was originally presented by Dr. Stankovic as part of the Dr. John Niparko Memorial Lecture during the 2021 American Cochlear Implant Alliance annual meeting.

[Otologic disorders and management strategies in Turner syndrome].

Objective: To analyze the incidence and risk factors of otologic disorders in patients with Turner syndrome (TS), so as to provide management strategies for ear health. Methods: This study is a prospective study based on questionnaires and a cross-sectional study. The TS patients who visited our hospital from 2010 January to 2021 March were included (A total of 71 patients with TS were included in this study. the age of TS diagnosed was 3- to 11-year-old, age of visiting ENT department was 4- to 27-year-old) and the incidence of otologic diseases in different age groups was investigated by questionnaires. The cross-sectional study included ear morphology and auditory function assessment, and further analysis of the risk factors that related to ear disease. Prism was used for data analysis. Results: The investigation found that the incidence of acute otitis media in patients aged 3-6 and 7-12 years was higher than that of patients over 12 years old, which was 33.8%(24/71), 42.9%(30/70)and 23.5%(8/34), respectively; 21.1% (15/71) of patients were recurrent acute otitis media in patients aged 3-6 years, and about 46.6% (7/15)of them persisted beyond 6-year. The prevalence of otitis media with effusion in the three groups was 32.4%(23/71), 34.3%(24/70)and 38.2%(13/34), respectively; the recurrence rate of tympanocentesis was 100%(7/7), 42.9%(3/7)and 50.0%(1/2), which was significantly higher than that of grommet insertion. For age groups of 3-6 and 7-12 years, the prevalence of acute otitis media and secretory otitis media was lower in the X chromosome structure abnormal patients; while for patients older than 12 years, otitis media with effusion was the highest prevalence in Y-chromosome-containing karyotypes. In addition, the prevalence of acute otitis media and otitis media with effusion in patients with other system diseases were increased significantly. A cross-sectional study found that 7.0% (5/71)of the lower auricular, 4.2% (3/71)of the external auditory canal narrow, and 38.0% (27/71)of the tympanic membrane abnormality. 35.2%(25/71) had abnormal hearing, including 17 cases of conductive deafness, 6 cases of sensorineural hearing loss, and 2 cases of mixed deafness. The rest of the patients had normal hearing, but 6 of them had abnormalities in otoacoustic emission. Eustachian tube function assessment found that the eustachian tube dysfunction accounted for 38%(27/71). Hearing loss and abnormal Eustachian tube function were not significantly related to karyotype(Chi-square 2.83 and 2.84,P value 0.418 and 0.417), but significantly related to other system diseases(Chi-square 13.43 and 7.53,P value<0.001). Conclusions: The incidence of TS-related otitis media and auditory dysfunction is significantly higher than that of the general population. It not only occurs in preschool girls, but also persists or develops after school age. Accompanied by other system diseases are risk factors for ear diseases. Clinicians should raise their awareness of TS-related ear diseases and incorporate ear health monitoring into routine diagnosis and treatment.

Auditory and speech outcomes of cochlear implantation in post-meningitis deafness.

PURPOSE: This study aims to identify the auditory, speech, and surgical outcomes of cochlear implantation in patients with profound SNHL following bacterial meningitis. METHODS: Subjects with bilateral severe to profound SNHL who underwent unilateral cochlear implantation from 2003 to 2020 were included in this historical cohort study. The main outcomes were assessed using Categories of Auditory Performance (CAP) and Speech Intelligibility Ratings (SIR) scores. The CAP and SIR outcomes were collected as three-time points after surgery: 6, 12, and 24 months. In order to achieve the strength of the relationship and for computing the Risk Ratio (RR) by log-binominal regression method, we used two binary categorizations of CAP and SIR in our analyses. RESULTS: The mean of age at implantation of the study and control group were 144.30 (156.90) and 121.10 (133.70) months, respectively. In the study group, 19 of 35 (54.3%) patients were male, and 16 (45.7%) were female. In the control group, 34 of 81 (42.0%) patients were male and 47 (58.0%) were female. The mean scores of CAP and SIR in our study improved significantly during the time in both groups. All p-values (p) were significant in both groups (T2 vs T1, T3 vs T1, and T3 vs T2). Our analysis by log-binomial regression and computing the RR based on the first and second categorization of CAP and SIR showed moderate to strong relationships between the presence of a history of meningitis and inappropriate CAP and SIR outcomes in these patients. CONCLUSIONS: Although subjects who were deafened due to meningitis benefit significantly from cochlear implantation, we found moderate to strong relationships between the history of meningitis and inappropriate CAP and SIR outcomes in these patients.

Determination risk factors for severe and profound hearing loss in child candidates for cochlear implantation in southeast of Iran during 2014-2020.

BACKGROUND: Hearing loss can have a major impact on children's language development, academic success and hearing comprehension. The aim of the present study was to determinate risk factors for severe and profound hearing loss in child candidates for cochlear implantation in southeast of Iran during 2014-2020. MATERIALS AND METHODS: This case-control study consisted of 400 children referring to a cochlear implant center (in southeastern Iran) from Bandar Abbas, Zahedan and Kerman during the years 2014-2020 as cases. The subjects were selected using the random sampling method; 200 children hospitalized in Shafa and Afzalipour hospitals were selected as controls. RESULTS: Based on the results of the multivariate logistic regression, weight less than 1500 g (OR = 4.40: p < 0.05), hospitalization in NICU (OR = 7.21: p < 0.05), family history of hearing loss (OR = 11.47: p < 0.05), Gestational age over 35 (OR = 9.63: p < 0.05), intracranial hemorrhage (OR = 5.18: p < 0.05), consanguineous marriage (OR = 12.48: p < 0.05) and high fever and seizures (OR = 3.02: p < 0.05) were recognized as risk factors for sensorineural deafness in children. CONCLUSION: Most of the risk factors for deafness are preventable, and hereditary factors play an important role in congenital deafness in children. Therefore, genetic counseling before consanguineous marriage, early diagnosis, timely intervention can prevent many cases of hearing loss in children.

Incidence of Mumps Deafness in Japan, 2005-2017: Analysis of Japanese Insurance Claims Database.

BACKGROUND: Mumps deafness causes serious problems, and incidence data are needed to identify its disease burden. However, such data are limited, and the reported incidence is highly variable. Nationwide studies in Japan with a large age range are lacking. METHODS: This was a retrospective observational investigation of the 2005-2017 mumps burden using employment-based health insurance claims data. Data were analyzed for 5,190,326 people aged 0-64 years to estimate the incidence of mumps deafness. RESULTS: Of 68,112 patients with mumps (36,423 males; 31,689 females), 102 (48 males; 54 females) developed mumps deafness-an incidence of 15.0 per 10,000 patients (1 in 668 patients). Fifty-four (52.9%) patients had mumps deafness in childhood (0-15 years), and 48 (47.1%) had mumps deafness in adolescence and adulthood (16-64 years); most cases occurred in childhood, the peak period for mumps onset. The incidence of mumps deafness per 10,000 patients was 73.6 in adolescence and adulthood, 8.4 times higher than the incidence of 8.8 in childhood (P < 0.001). In childhood, the incidence of mumps deafness was 7.2 times higher among 6-15-year-olds (13.8; 95% CI, 10.2-18.2) than among 0-5-year-olds (1.9; 95% CI, 0.6-4.5), and this difference was statistically significant (P < 0.001). No sex difference was observed. CONCLUSIONS: The incidence of mumps deafness per 10,000 patients aged 0-64 years was 15.0 (1 in 668 patients). A secondary risk of deafness following mumps virus infection was identified not only for children, but also for adolescents and adults.