Pneumococcal Meningitis Induces Hearing Loss and Cochlear Ossification Modulated by Chemokine Receptors CX3CR1 and CCR2.

PURPOSE: Pneumococcal meningitis is a major cause of hearing loss and permanent neurological impairment despite widely available antimicrobial therapies to control infection. Methods to improve hearing outcomes for those who survive bacterial meningitis remains elusive. We used a mouse model of pneumococcal meningitis to evaluate the impact of mononuclear phagocytes on hearing outcomes and cochlear ossification by altering the expression of CX3CR1 and CCR2 in these infected mice. METHODS: We induced pneumococcal meningitis in approximately 500 C57Bl6 adult mice using live Streptococcus pneumoniae (serotype 3, 1 × 105 colony forming units (cfu) in 10 µl) injected directly into the cisterna magna of anesthetized mice and treated these mice with ceftriaxone daily until recovered. We evaluated hearing thresholds over time, characterized the cochlear inflammatory response, and quantified the amount of new bone formation during meningitis recovery. We used microcomputed tomography (microCT) scans to quantify cochlear volume loss caused by neo-ossification. We also performed perilymph sampling in live mice to assess the integrity of the blood-perilymph barrier during various time intervals after meningitis. We then evaluated the effect of CX3CR1 or CCR2 deletion in meningitis symptoms, hearing loss, macrophage/monocyte recruitment, neo-ossification, and blood labyrinth barrier function. RESULTS: Sixty percent of mice with pneumococcal meningitis developed hearing loss. Cochlear fibrosis could be detected within 4 days of infection, and neo-ossification by 14 days. Loss of spiral ganglion neurons was common, and inner ear anatomy was distorted by scarring caused by new soft tissue and bone deposited within the scalae. The blood-perilymph barrier was disrupted at 3 days post infection (DPI) and was restored by seven DPI. Both CCR2 and CX3CR1 monocytes and macrophages were present in the cochlea in large numbers after infection. Neither chemokine receptor was necessary for the induction of hearing loss, cochlear fibrosis, ossification, or disruption of the blood-perilymph barrier. CCR2 knockout (KO) mice suffered the most severe hearing loss. CX3CR1 KO mice demonstrated an intermediate phenotype with greater susceptibility to hearing loss compared to control mice. Elimination of CX3CR1 mononuclear phagocytes during the first 2 weeks after meningitis in CX3CR1-DTR transgenic mice did not protect mice from any of the systemic or hearing sequelae of pneumococcal meningitis. CONCLUSIONS: Pneumococcal meningitis can have devastating effects on cochlear structure and function, although not all mice experienced hearing loss or cochlear damage. Meningitis can result in rapid progression of hearing loss with fibrosis starting at four DPI and ossification within 2 weeks of infection detectable by light microscopy. The inflammatory response to bacterial meningitis is robust and can affect all three scalae. Our results suggest that CCR2 may assist in controlling infection and maintaining cochlear patency, as CCR2 knockout mice experienced more severe disease, more rapid hearing loss, and more advanced cochlear ossification after pneumococcal meningitis. CX3CR1 also may play an important role in the maintenance of cochlear patency.

Cochlear Implantation in a Case of Relapsing Polychondritis With Profound Hearing Loss and Labyrinthine Ossification.

Relapsing polychondritis is an autoimmune disorder causing inflammation of cartilaginous structures, sensory epithelium, and cardiovascular system. Hearing loss is a rare and dreadful complication of this pathology. We report a case of relapsing polychondritis in a 38-year-old female who developed gradually progressive bilateral profound hearing loss. She did not have any improvement with medical management. Cochlear implantation was performed to rehabilitate her hearing. As the scala tympani was obliterated, a scala vestibuli insertion was performed. A complete insertion was possible with a compressed electrode, and she had good evoked compound action potential scores. Her categories of auditory performance scores were 6 at the end of one year. Patients with relapsing polychondritis can progress to profound hearing loss in rare cases and should be carefully followed up to identify early labyrinthine ossification. A scala vestibuli insertion can be performed with good outcomes in cases with ossification involving scala tympani. The surgeon should be ready for a middle-turn cochleostomy or a drill-out procedure in patients with advanced ossification.

Outcomes and Review in Surgery of Lateral Semicircular Canal Dehiscence in Cholesteatomatous Otitis Media: A Case Series.

Labyrinthine fistula is one of the most frequent complications of cholesteatoma otitis. We report two cases where a canal wall down mastoidectomy is made due to the characteristics of the cholesteatoma, achieving good outcomes in relation to bone conductive hearing. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03973-1.

Non-Syndromic Sensorineural Hearing Loss in Children.

Pediatric hearing loss is common with significant consequences in terms of language, communication, social and emotional development, and academic advancement. Radiological imaging provides useful information regarding hearing loss etiology, prognosis, therapeutic options, and potential surgical pitfalls. This review provides an overview of temporal bone imaging protocols, an outline of the classification of inner ear anomalies associated with sensorineural hearing loss and illustrates some of the more frequently encountered and/or important causes of non-syndromic hearing loss.

Caracterización de complicaciones por otitis media aguda en el Hospital Clínico de la Pontificia Universidad Católica de Chile

Introducción: las complicaciones por otitis media aguda tienen una incidencia menor a un 1%. Aun así, se describe en literatura una mortalidad de un 5%. Actualmente existe escasa evidencia sobre estas complicaciones en nuestra realidad local. Objetivo: describir las de complicaciones de otitis media aguda según tipo, sexo, síntomas de ingreso, exámenes solicitados y tratamiento. Material y Métodos: estudio descriptivo retrospectivo de revisión de fichas clínicas que incluyó pacientes que ingresaron al Hospital Clínico de la Pontificia Universidad Católica de Chile con diagnóstico de otitis media aguda entre el 1 enero de 2000 hasta el 30 de julio de 2022. Resultados: se obtuvieron 71 pacientes ingresados por complicación de otitis media aguda. La edad media fue de 26,79 años, con 46 mujeres y 25 hombres. Se observaron complicaciones extracraneales, intracraneales y complicaciones simultáneas. Las complicaciones más frecuentes fueron la laberintitis y mastoiditis sin reportar casos de mortalidad. Discusión: actualmente existe escasa información sobre complicaciones de otitis media aguda y su epidemiología a nivel local. Las complicaciones extracraneales fueron más frecuentes: de ellas, la laberintitis y la mastoiditis agudas. El diagnóstico es clínico con apoyo de exámenes tanto de laboratorio, audiovestibulares e imágenes. Con respecto al tratamiento quirúrgico, se debe evaluar caso a caso y debe incluir miringotomía con o sin instalación de tubo de ventilación y mastoidectomía en casos refractarios. Es importante la sospecha y la derivación precoz

Introduction: Complications due to acute otitis media have an incidence of less than 1%. Nevertheless, it is described in the literature as having a 5% mortality rate. Currently, there is little evidence of these complications in our local reality. Objective: Describe the complications of acute otitis media based on its type, patient gender, admission symptoms, requested exams, and treatment. Material and Methods: Retrospective and descriptive study of patients' medical records admitted at the "Hospital Clínico de la Pontificia Universidad Católica de Chile" who were diagnosed with acute otitis media from January 1st, 2000, to July 30th, 2022. Results: There were 71 patients admitted due to complications of acute otitis media. On average, they were 26.29 years old; 46 were women, and 25 were men. Extracranial, intracranial, and simultaneous complications were observed. The most frequent ones were labyrinthitis and mastoiditis, without any mortality cases reported. Discussion: Currently, there is scarce information about the complications of acute otitis media and its local epidemiology. Extracranial complications were the most frequent: acute labyrinthitis and mastoiditis. The clinical diagnosis is supported by laboratory, audiovestibular, and image tests. Regarding surgical treatment, each patient must be evaluated independently. It should include myringotomy with or without a ventilation tube and mastoidectomy in refractory cases. It is crucial to detect it early and proceed with a medical referral.

Differences in vestibulo-ocular reflexes between vestibular neuritis and labyrinthitis.

Objective: To expand our understanding of the pathophysiological mechanisms underlying vestibular neuritis and labyrinthitis by identifying any difference in the vestibulo-ocular reflex for each semicircular canal. Study Design: Retrospective analysis. Setting: The Department of Otorhinolaryngology - Head and Neck Surgery, Chosun University Hospital, from January 2015 to December 2021. Methods: We included 23 vestibular neuritis and 27 labyrinthitis patients who had been hospitalized. Pure-tone audiometry, a bithermal caloric test, and a video head-impulse test were performed within 5 days of symptom onset. Results: In the vestibular neuritis group, mean vestibulo-ocular reflex gains were decreased to 0.51 in the ipsilesional horizontal canal and 0.55 in anterior canal, leading to marked asymmetry, whereas the gain of the ipsilesional posterior canal was relatively preserved at 0.85. In the labyrinthitis group, the mean vestibulo-ocular reflex gain was 0.72 in the ipsilesional horizontal canal, 0.73 in the ipsilesional anterior canal, and 0.55 in the ipsilesional posterior canal. We observed statistical differences in the vestibulo-ocular reflex gain and incidence of corrective saccades on the ipsilesional side in three semicircular canals between the groups (p = .002 for horizontal canal, p = .003 for anterior canal, and p < .001 for posterior canal). The receiver operating characteristic curve showed that pure-tone audiometry, ipsilesional posterior canal gain, and gain asymmetry of posterior canal were excellent parameters for distinguishing labyrinthitis from vestibular neuritis. Conclusion: Vestibular neuritis and labyrinthitis patients have different degrees and patterns of video head-impulse test involvement in the three semicircular canals, suggesting that the two distinct disorders may have different etiologies.

Infliximab for the Treatment of Inflammatory Labyrinthitis: A Retrospective Cohort Study.

Inflammatory labyrinthitis is defined as a fluctuant vestibulo-cochlear syndrome associated with an impairment of the blood-labyrinthine barrier (BLB) on delayed FLAIR MRI sequences. Systemic and intratympanic corticosteroids are the gold standard treatment but their effect is frequently insufficient. The objective is here to determine whether infliximab could be of value in the treatment of bilateral inflammatory labyrinthitis. A retrospective monocentric study was conducted between January 2013 and December 2021. All patients included in the study were affected with a bilateral vestibulo-cochlear syndrome associated with bilateral blood-labyrinthine barrier impairment. Patients were administered infliximab at the dose of 5 mg/kg every 6 weeks for 6 months. Audiometry, MRI with delayed FLAIR sequences on the labyrinth, and corticosteroid doses still required were assessed both before and after treatment with infliximab was completed. Pure-tone average (PTA) was the primary outcome. The secondary outcomes were the speech recognition threshold (SRT), the Dizziness Handicap Inventory (DHI) score, and the corticosteroid (CS) dose. A total of nine patients including five men and four women were enrolled in the study. Thirteen ears were analyzed. After a 6-month period of treatment, the mean PTA (54 ± 24 db versus 66 ± 22 db; p = 0.027), SRT (54 ± 37 db versus 66 ± 32 db; p = 0.041) and DHI score (27 ± 15 versus 9 ± 2; p = 0.032) significantly improved. After the 6-month treatment period, the mean CS dose decreased from 38 ± 33 to 6 ± 5 mg/day (p = 0.003). We conclude that infliximab substantially improves the vestibulo-cochlear function in patients with bilateral inflammatory labyrinthitis and could be of value in corticosteroid-dependent cases.

Protocol for Evaluation and Management of Hearing Loss After Meningitis.

Bacterial meningitis is the most common cause of post-natal acquired hearing loss in children. Although cochlear implantation helps in improving the hearing in these patients, the fibrosis and ossification of the cochlear lumen that occurs as a result of bacterial meningitis, limits the chances of successful implantation. In developing countries like India, the reduced awareness, limited resources, and financial constraints warrant judicial use of radiological and audiological tests to increase the rate of successful cochlear implantation. The present paper is a review of the literature and a proposed protocol for follow-up of post-meningitis patients to help clinicians diagnose and hence, intervene early when profound hearing loss occurs. Every patient who has had an episode of bacterial meningitis must be followed up for atleast 2 years for possible hearing loss with frequent audiological and radiological evaluation, as required. Cochlear implantation must be done as early as possible when profound hearing loss is detected.

Labyrinthitis Ossificans in a Post-Splenectomy Patient With Meningitis: A Case Report and Review of Literature.

This report describes a case of cochlear implantation to treat profound deafness three months after a diagnosis of bacterial meningitis in a patient with a remote history of splenectomy. A 71-year-old woman with a remote history of a splenectomy over 20 years before presented with bilateral profound deafness that occurred as sequela from pneumococcal meningitis three months prior. The patient had been vaccinated against the 23-valent polysaccharide pneumococcal vaccine (PPV-23). The audiometric evaluation revealed no response in either ear. Imaging was suggestive of complete ossification of the right cochlea with partial ossification of the basal turn of the left cochlea. She underwent successful left-sided cochlear implantation. Standard post-implantation speech outcomes include consonant-nucleus-consonant (CNC) word and phoneme scores and Az-Bio in quiet and noise. The patient noted subjective improvement in her hearing. Performance measures markedly improved when compared to her pre-operative evaluation, which showed no aided sound detection. This case report highlights the possibility of meningitis many years after splenectomy that can result in profound deafness with labyrinthitis ossificans and the potential for hearing rehabilitation for cochlear implantation.

A cadaveric temporal bone study of the anatomical landmarks for middle turn cochleostomy.

OBJECTIVE: This study aimed to determine anatomical landmarks for accurate and safe middle turn cochleostomy on cadaveric temporal bones. METHODS: In 17 cadaveric wet adult temporal bones, cortical mastoidectomy was performed, followed by extended posterior tympanotomy through which a middle turn opening was created anterior to the stapes footplate. Micro-measurements of various lengths were taken from the cochleostomy to normal middle-ear anatomical landmarks using a digital microscope. RESULTS: The mean length from the middle turn cochleostomy to the processus cochleariformis was 1.8 ± 0.3 mm and to the tympanic segment of the facial nerve was 2.2 ± 0.3 mm. The mean shortest length from the oval window to the osseous spiral lamina was 2.4 ± 0.3 mm and to the internal carotid artery was 5.0 ± 0.6 mm. The mean shortest length from the round window to the internal carotid artery was 4.3 ± 0.6 mm. CONCLUSION: A middle turn cochleostomy can be safely drilled by using the measured lengths in difficult cases.

Balance testing: does it make a difference?

OBJECTIVE: This study aimed to analyse whether referral for specialist balance testing influences diagnosis and management of patients with dizziness. METHOD: This was a retrospective study examining patients referred for vestibular function testing between 1 January 2018 and 30 June 2018. RESULTS: A total of 101 patients were referred, with 69 patients (68.3 per cent) receiving a preliminary 'pre-vestibular function testing balance diagnosis', which included benign paroxysmal positional vertigo (32.7 per cent), Ménière's disease (13.8 per cent) and migraine (14.9 per cent). Following vestibular function testing, revised diagnoses were achieved for 54 patients (53.5 per cent), including benign paroxysmal positional vertigo (14.9 per cent), Ménière's disease (3.0 per cent) and migraine (10.9 per cent). Pre-vestibular function testing balance diagnoses were confirmed for 32.4 per cent of patients. If no pre-vestibular function testing suspected diagnosis was provided, vestibular function testing was significantly more likely to be inconclusive. Following vestibular function testing, 38.6 per cent were discharged, 21.7 per cent were referred to another specialty and treatment was commenced for 17.8 per cent of patients. CONCLUSION: Referral for vestibular function testing has a role when attempting to answer a clear clinical question. Diagnosing the underlying aetiology of complex imbalance is challenging, but diagnosis can be assisted by judicious use of vestibular function testing.

Cochlear implantation through intracochlear fibrosis: A comparison of surgical techniques.

OBJECTIVE: While the implications of ossification on cochlear implantation (CI) have been extensively described, there is a paucity of data regarding the fibrotic stage. We examined the outcomes of different insertion techniques for managing intracochlear fibrosis. STUDY DESIGN: Retrospective review of case series with case-control comparison. SETTING: University-based tertiary-referral otology-neurotology practice. PATIENTS: Between 2009 to 2020, 384 patients underwent CI. Of those, 7 patients (8 ears) demonstrated intracochlear fibrosis. INTERVENTIONS: CI performed 1-4 months following meningitis/labyrinthitis and 12-24 months after idiopathic sudden SNHL. Fibrosis removal (38%) or dilation (63%) permitted implantation. A styleted-electrode was used in 63% due to dense fibrosis. MAIN OUTCOME MEASURES: Postoperative audiometry with CI in place, additional comparisons with audiometric outcomes in age-matched controls. RESULTS: Full insertion achieved in all except one ear with partial ossification. Mean ipsilateral pure tone average (PTA) improved to 29 ± 15 dB and speech discrimination to 72 ± 28%. Fibrosis removal vs. dilation resulted in no PTA differences (p = 0.76). Poorest outcomes occurred with the longest time to surgery. CONCLUSIONS: Good CI audiologic outcomes in the setting of cochlear fibrosis can be achieved and are independent of technique. Instead, they vary with time to implantation. Every attempt should be made to intervene as early as possible.

Magnetic Resonance Imaging in Otosyphilis: A Rare Manifestation of Neurosyphilis.

We report magnetic resonance imaging (MRI) in a case with otosyphilis, which is a rare manifestation of neurosyphilis. A 50-year-old male presented with progressive hearing loss on the left side and recent-onset palsy of the left 7th and 12th cranial nerves. Computed tomography imaging showed destructive bone lesions involving the petrous temporal bone, middle ear, and mastoid region with a pathognomonic pattern of bone destruction depicted in the volume rendering technique images. MRI showed features of destructive bone lesions (gummatous lesion), meningoneuritis, and labyrinthitis. Pathological examination and treponemal antibody absorption test favored the diagnosis. Otosyphilis should be considered as a differential diagnosis in an adult patient with destructive lesions of the petrous temporal bone. To the best of our knowledge, MRI in otosyphilis has not been reported.

SARS-CoV-2 infection with bilateral intralabyrinthine hemorrhage.

A 47-year-old woman presented with the complaint of sudden hearing loss associated with vertigo. Serological testing was positive for IgM and negative for IgG COVID-19 antibodies, with no other associated factors. Magnetic resonance imaging of the brain showed bilateral intralabyrinthine hemorrhage.

Unilateral sudden sensorineural hearing loss in post-COVID-19 patients: Case report.

Sudden sensorineural hearing loss (SSNHL) is an otologic emergency. Sensorineural hearing loss needs to be distinguished from conductive when patients present with sudden hearing loss at the primary care level. Prompt diagnosis of sensorineural hearing loss with pure tone audiometry and immediate treatment by an otolaryngologist can improve the hearing outcome. To date, few case reports exist about SSNHL among post-COVID-19 patients, and none were reported in Malaysia. Here, we present two cases of SSNHL in patients after COVID-19 infection. We wish to highlight the association of SSNHL following COVID-19 infection for timely referral towards better audiology outcomes. Permanent hearing loss will lead to another negative impact on the long-term quality of life of COVID-19 patients.

Differences in vestibulo-ocular reflex time constants in patients with vestibular migraine versus vestibular neuritis/labyrinthitis.

OBJECTIVE: Recurrent dizziness, vertigo, and imbalance, as well as nausea or vomiting, can be induced by central or peripheral vestibular pathology. Vestibular migraine (VM) is a central pathology in which migraines reciprocally interact with vestibular nuclei. Vestibular neuritis and labyrinthitis (VN/L) are peripheral diseases involving inflammation of structures in the vestibular nerve or labyrinth. Because VM and VN/L in early stages can produce similar symptoms, diagnosis may require prolonged clinical evaluation. It has been suggested that differences in a patient's Vestibulo-Ocular Reflex (VOR) measured during the rotary chair step rotation test (RCS Test) can differentiate VM from peripheral pathologies, allowing for faster diagnosis. We sought to compare VOR time constants (VORTcs) in patients with VM versus VN/L, with the goal of exploring potential diagnostic value. STUDY DESIGN: Retrospective chart review of patients seen at a hospital balance center between January 2010 and June 2019. SETTING: Tertiary referral center. PATIENTS: 68 patients (mean age = 58.15, 65 % female, 35 % male) were placed into two groups based on clinical diagnosis codes: 1) VM or 2) VN/L. MAIN OUTCOME MEASURE(S): Primary variable of VORTc, evoked by rotational step testing. RESULTS: Patients in the VM group demonstrated statistically significant elevated VOR Tcs outside the normed range of 10-19.5 compared to those in the VN/L group (p = 0.0003) while patients in the VN/L group demonstrated statistically significant shortened VOR Tcs (p = 0.0443) with the two populations having distinctly different VOR Tc profiles. CONCLUSIONS: Our results support the conclusion that elevated VOR Tcs are a sign of central vestibular pathology and highlight the benefit of utilizing rotary chair testing early in the diagnostic process as a powerful diagnostic tool when evaluating dizzy patients for central versus peripheral vestibular dysfunction. Our results suggest more particularly that elevated VOR Tcs can be utilized to identify patients with vestibular migraine, thus offering differentiation from patients with peripheral vestibular dysfunction earlier in the process of diagnosis, with an opportunity for earlier intervention.

Intratympanic injection of dexamethasone for management of labyrinthitis associated with COVID-19 disease resistant cases.

Objective: To evaluate the efficacy of mixed oral prednisolone and intratympanic dexamethasone (ITID) injection in labyrinthitis, due to COVID 19. Methods: Seventy-five post-COVID-19 labyrinthitis patients were included. Those patients were treated with systemic oral prednisolone for two weeks and ITID. Patients who refuse ITID were ordered to continue oral prednisolone treatment. Assessment of outcome and audiometry for hearing evaluation was done 1, 2 and 4 weeks as well as 3 months post-treatment. Results: Patients in oral steroid only group were 26 patients, while patients in oral steroid and ITID group were 49 patients. In oral steroid group; 11/26 patients showed complete recovery, 3/26 had partial recovery and 12/26 not recovered. In other group; 38/46 patients had complete recovery, 6 had partial recovery and 5/49 patients not recovered. Conclusion: Combined systemic corticosteroid with ITID showed a marked improvement of post-COVID vestibular disorder and hearing loss than only using oral corticosteroid therapy.

Inner ear modifications in Dar-es-Soltane II H5 (Morocco): A case of labyrinthitis ossificans.

OBJECTIVE: This paper presents the inner ear modifications in Dar-es-Soltane II H5, an Aterian fossil possibly dated to 100 ka. MATERIAL: The remains consist of a large portion of the cranium including the face, the left frontal and temporal bones, part of the left parietal bone and greater wing of the sphenoid. METHODS: The bony labyrinth anatomy was investigated on existing micro-CT data acquired by the MPI-EVA. RESULTS: The observation of micro-CT sections revealed a partial filling of the semi-circular canals that raises question about its origin. A careful examination of the micro-CT sections shows that the elements present in the semicircular canals were denser than the sediments observed in other regions and cavities of the temporal bone. CONCLUSIONS: The current evidence suggests a pathological origin of this condition with partial ossification of the membranous labyrinth. The differential diagnosis indicates a case of labyrinthitis ossificans in its early stages. SIGNIFICANCE: This pathological condition can be responsible for permanent hearing loss and is associated with dizziness and vertigo. Along with the Singa skull, Dar-es-Soltane II H5 represents one of the oldest known cases of labyrinthitis ossificans. LIMITATIONS: The early stage of disease and the absence of the right temporal bone limit conclusions about the degree of disability of the individual and their dependence on the rest of the group. SUGGESTIONS FOR FURTHER RESEARCH: To carry out a paleopathological study of all the fossils from Dar-es-Soltane II.

Case Report: Suppurative Labyrinthitis Induced by Chronic Suppurative Otitis Media.

A discussion on suppurative labyrinthitis associated with chronic suppurative otitis media (CSOM) may seem to be an outdated issue due to the advent of antibiotics in the last century. In previous literature, limited cases of suppurative labyrinthitis have been reported. This case, therefore, is an excellent and rare opportunity to study its clinical symptoms, diagnoses, and treatments. This report described the case of a 39-year-old female with a history of CSOM for 20 years, and she often presented with otorrhea, vestibular disorder, and hearing impairment. CT of the temporal bone revealed fistulae in both the basal turn of the cochlea and the horizontal semicircular canal. Combined with the otolaryngology examination results, suppurative labyrinthitis was considered. During a three-month follow-up, her symptoms were improved significantly after surgery. In conclusion, suppurative labyrinthitis must not be overlooked and neglected; early diagnosis and treatments are of vital importance.

Labyrinthitis ossificans following severe acute otitis media.

Labyrinthitis occurs because of the inflammation of the inner ear. We present a rare case of labyrinthitis ossificans following an acute otitis media. The T2-weighted magnetic resonance imaging showed decreased signal intensity in the right inner ear due to labyrinthitis ossificans, consistent with the clinical presentation.