Acute labyrinthitis: a manifestation of COVID-19 in a teenager.

Audiovestibular symptoms following COVID-19 have been long acknowledged, especially in adults. However, acute labyrinthitis presenting as an early manifestation of COVID-19 has not been reported in children. We report COVID-19-induced acute labyrinthitis in a teenager. We report on a boy in his early adolescence with a sudden onset of spinning sensation, imbalance and unilateral hearing loss with a positive SARS-CoV-2 test. Vestibular investigations point towards right labyrinthine hypofunction, and an audiometry test revealed right-sided severe hearing loss. Symptoms improved gradually with steroids and vestibular rehabilitation therapy. However, the long-term repercussions of post-COVID-19 acute labyrinthitis are unknown and must be followed up closely. To our knowledge, this is the first reported case of acute labyrinthitis secondary to COVID-19 in paediatrics. Additionally, we conducted a literature search to elucidate the outcome of COVID-19-induced acute vestibular syndrome in children.

Standard Programming of Retrograde Electrode Insertion via Middle-Turn Cochleostomy in Labyrinthitis Ossificans.

Successful cochlear implantation in the setting of labyrinthitis ossificans is challenging. Various surgical techniques are described to circumvent the region of ossification and retrograde insertion of the electrode array is one such option. While reverse programming is often recommended in the case of retrograde electrode insertion, we present our experience of retrograde electrode insertion for labyrinthitis ossificans, where standard programming was adopted due to patient preference and provided satisfactory outcomes.

Otopathologic Analysis of Patterns of Postmeningitis Labyrinthitis Ossificans.

OBJECTIVE: Labyrinthitis ossificans (LO) may occur following meningitis and, in cases where cochlear implantation is indicated, complicate electrode insertion. LO is critical to identify for successful cochlear implantation, and histopathology is more sensitive than imaging for identification of LO. Herein we utilize otopathologic techniques to study the timing and location of intracochlear tissue formation following meningitic labyrinthitis (ML). STUDY DESIGN: Retrospective review. SETTING: Academic institution. METHODS: Temporal bone specimens with a history of bacterial ML were histologically evaluated. The location and extent of intracochlear tissue formation within the scala tympani (ST) and scala vestibuli (SV) were graded, and spiral ganglion neurons were counted. RESULTS: Fifty-one temporal bones were identified: 32 with no intracochlear tissue formation, 9 with fibrosis alone, and 10 with LO. Fibrosis was identified as early as 1.5 weeks after ML, while ossification was found only in specimens that survived multiple years after ML. All LO cases showed ossification of the ST at the round window membrane (RWM) with continuous extension throughout the basal turn. Extent of SV ossification correlated with that in the ST but showed frequent isolated distal involvement of the cochlea. Spiral ganglion neuron counts were lower than those in age-matched controls. CONCLUSION: In this human temporal bone study, we found that postmeningitic LO results in ossification at the RWM with continuous extension into the ST of the basal turn and variable involvement of the SV. Identification of a patent basal turn beyond RWM ossification of the ST should permit full electrode insertion. LEVEL OF EVIDENCE: Retrospective review.

HHV 6-7 reactivation causing Pityriasis Rosea and labyrinthitis: A case report.

Pityriasis Rosea (PR) and labyrinthitis are most commonly caused by viral infections. PR presents with a characteristic rash while labyrinthitis presents with vertigo, tinnitus and hearing loss. However, the coexistence of PR and Labyrinthitis remains an uncommon event. Human Herpes Virus (HHV) 6 and 7, are common infections in childhood, and their reactivation causes Pityriasis Rosea. But these viruses are not known to have any involvement with the inner ear or the 8th cranial nerve (CN).

Doença de Creutzfeldt-Jakob: relato de caso / Creutzfeldt-Jakob disease: case report

As doenças priônicas fazem parte do grupo das síndromes de demência rapidamente progressiva com neurodegeneração. Em humanos, a doença de Creutzfeldt-Jakob é a mais prevalente. Atualmente, seu diagnóstico pode ser baseado em uma combinação do quadro clínico, ressonância magnética e eletroencefalograma com alterações típicas, juntamente da detecção de proteína 14- 3-3 no líquido cefalorraquidiano. Este relato descreve o caso de uma paciente de 74 anos, natural de Ubá (MG), admitida em um hospital da mesma cidade com quadro de demência de rápida progressão, com declínio cognitivo, ataxia cerebelar e mioclonias. No contexto clínico, aventou-se a possibilidade de doença de Creutzfeldt-Jakob e, então, foi iniciada investigação para tal, com base nos critérios diagnósticos. Também foram realizados exames para descartar a possibilidade de doenças com sintomas semelhantes. O caso foi diagnosticado como forma esporádica de doença de Creutzfeldt-Jakob. (AU)

Prion diseases are part of the rapidly progressive dementia syndromes with neurodegeneration. In humans, Creutzfeldt-Jakob disease is the most prevalent. Currently, its diagnosis may be based on a combination of clinical picture, magnetic resonance imaging, and electroencephalogram with typical changes, along with the detection of 14-3-3 protein in cerebrospinal fluid. This report describes the case of a 74-year-old woman from the city of Ubá, in the state of Minas Gerais, who was admitted to a hospital in the same city with a rapidly progressive dementia, cognitive decline, cerebellar ataxia and myoclonus. In the clinical context, the possibility of Creutzfeldt-Jakob disease was raised, and then investigation was started for this disease, based on the its diagnostic criteria. Tests have also been conducted to rule out the possibility of diseases with similar symptoms. The case was diagnosed as a sporadic form of Creutzfeldt-Jakob disease. (AU)

"Emergency" Cochlear Implantation in Labyrinthitis Ossificans Secondary to Polyarteritis Nodosa: How to Face a Rare Entity.

Polyarteritis nodosa (PAN) is a systemic vasculitis affecting the small- and medium-sized arteries that may present with hearing impairment. In rare cases, PAN may be associated with progressive labyrinthitis ossificans (LO), an otologic emergency requiring early cochlear implantation (CI) to restore hearing before the complete, irreversible cochlear ossification. We report the first case in the literature of a patient affected by PAN with bilateral sudden sensorineural hearing loss and rapid LO who underwent "emergency" bilateral simultaneous CI. This case report emphasizes the importance of an early audiological evaluation in patients with PAN when LO is suspected. Multidisciplinary approach is mandatory when facing organ-specific manifestations in patients with PAN. Detailed discussion is provided with particular regard to clinical and radiological presentation as well as CI outcomes in such a rare and challenging case.

Acute Otitis Media and Associated Complications in United States Emergency Departments.

OBJECTIVE: Complications due to acute otitis media (AOM), while rare, are associated with significant morbidity and are not well characterized from an epidemiological perspective. We analyze the pattern of presentation and emergency department (ED) utilization in patients with AOM and associated complications. STUDY DESIGN: Retrospective analysis of the Nationwide Emergency Department Sample (NEDS) from 2009 to 2011. SETTING: Emergency Department. PATIENTS: Patients who presented with a primary diagnosis of AOM or acute mastoiditis. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURES: NEDS was queried for patient encounters with a diagnosis of AOM or acute mastoiditis based on ICD-9 codes. Complications of severe infection, including petrositis, Gradenigo's syndrome, facial paresis, labyrinthitis, meningitis, intracranial abscess, venous sinus thrombosis, and cerebrospinal fluid leak, were assessed. Weighted estimates for demographics, types of complications, socioeconomic status, and trends over time were extracted. RESULTS: A weighted total of 5,811,127 ED visits were identified. The majority of patients were less than 18 years old (79.9%) with an average age of 10.1 years. Most were discharged (99.4%). There were 15,243 (0.26%) patients who presented with a complication. The most common complications were acute mastoiditis (0.16%), labyrinthitis (0.06%), and facial paresis (0.03%). Compared with patients with uncomplicated AOM, patients with complicated AOM were older (37 vs. 10-yr old), insured by Medicare (18% vs. 2.1%), and more likely to be admitted (43.6% vs. 0.4%) (p < 0.0001, for all comparisons). CONCLUSIONS: ED visits related to AOM or mastoiditis are common and complications are rare. An in-depth analysis on a national level is useful for assessing healthcare utilization trends.

Labyrinthine Sequestrum: A Case Report and Review of the Literature.

OBJECTIVE: To report the presentation, diagnosis, management, and convalescence of labyrinthine sequestrum (LS) and summarize all previously published cases. PATIENT(S): Eleven-year-old female with LS. INTERVENTION(S): Multidisciplinary diagnostic evaluation and treatment. MAIN OUTCOME MEASURES: Imaging and laboratory findings, medical and surgical treatment. RESULTS: We describe a case of LS secondary to medically recalcitrant suppurative otitis media in an 11-year-old female and review all eight previously reported cases. The index patient presented after 6 months of otitis media, profound unilateral hearing loss, with symptoms suggesting meningitis. Temporal bone CT demonstrated marked bony destruction of the left otic capsule. Gadolinium-enhanced MRI showed an enhancing process with evidence of meningitis and subdural empyema. The patient was treated with surgical debridement and culture directed antibiotic therapy. Posttreatment imaging showed resolution of intracranial infection with fibrous bony healing of the otic capsule resembling fibrous dysplasia. CONCLUSION: LS is a rare form of labyrinthitis characterized by centrifugal destruction of the otic capsule. The current index case highlights the importance of combined medical and surgical treatment and describes for the first time in the literature the fibrous ossification of the otic capsule following disease resolution.

Cochlear Implant Associated Labyrinthitis: A Previously Unrecognized Phenomenon With a Distinct Clinical and Electrophysiological Impedance Pattern.

OBJECTIVES: To report a unique clinical entity "cochlear implant associated labyrinthitis," characterized by a distinct constellation of clinical symptoms and pattern of electrode impedance fluctuations. STUDY DESIGN: Retrospective chart review. METHODS: All patients that underwent cochlear implantation between January 2014 and December 2016 were retrospectively reviewed. All subjects with acute onset dizziness, device performance decline, and characteristic erratic pattern of electrode impedances occurring after an asymptotic postoperative interval were identified and reported. RESULTS: Five patients with the above criteria were identified, representing 1.4% of all implant surgeries performed during this time. The median age at time of implantation was 71 years, and the median time interval between implantation and onset of symptoms was 126 days. All patients exhibited acute onset dizziness, subjective performance deterioration, erratic impedance pattern, and two experienced worsening tinnitus. Two of five patients underwent subsequent CT imaging, where good electrode placement was confirmed without cochlear ossification. Two of five patients received oral prednisone therapy. All patients reported a subjective improvement in symptoms and stabilization of electrode impedances. Three patients subsequently received vestibular testing, where significantly reduced peripheral vestibular function was identified. CONCLUSIONS: We describe a unique clinical entity, "cochlear implant associated labyrinthitis," characterized by a distinct constellation of clinical symptoms and corresponding electrode impedance anomalies. The exact cause for this event remains unknown, but may be related to viral illness, delayed foreign body reaction to the electrode, or a reaction to electrical stimulation. Future studies characterizing this unique clinical entity are needed to further elucidate cause and optimal management.

Indications and radiological findings of acute otitis media and its complications. / Indicaciones y hallazgos radiológicos de la otitis media aguda y sus complicaciones.

Most cases of acute otitis media resolve with antibiotics and imaging is not required. When treatment fails or a complication is suspected, imaging plays a crucial role. Since the introduction of antibiotic treatment, the complication rate has decreased dramatically. Nevertheless, given the critical clinical relevance of complications, the importance of early diagnosis is vital. Our objective was to review the clinical and radiological features of acute otitis media and its complications. They were classified based on their location, as intratemporal or intracranial. Imaging makes it possible to diagnose the complications of acute otitis media and to institute appropriate treatment. Computed tomography is the initial technique of choice and, in most cases, the ultimate. Magnetic resonance is useful for evaluating the inner ear and when accurate evaluation of disease extent or better characterization of intracranial complications is required.

Hearing Preservation After Penetrating Cochlear Injury.

OBJECTIVES: To share results and recommendations for management of penetrating cochlear injury. METHODS: A patient underwent repair of a penetrating cochlear injury after a projectile led to a traumatic cochleostomy with a narrow miss of the facial nerve and intracranial carotid artery. RESULTS: Postoperatively, the patient's audiogram demonstrated a pure tone average of 47.5 dB for air conduction and 35 dB for bone conduction, worse in the high frequencies, with a Word Recognition Score of 76%. CONCLUSIONS: Hearing loss from a penetrating cochlear injury can be mitigated with early repair, minimizing inner ear trauma, and steroid use to treat posttraumatic labyrinthitis.

Suppurative labyrinthitis associated with otitis media: 26 years' experience / Labirintite associada à otite média: experiência de 26 anos

ABSTRACT INTRODUCTION: Suppurative labyrinthitis continues to result in significant hearing impairment, despite scientific efforts to improve not only its diagnosis but also its treatment. The definitive diagnosis depends on imaging of the inner ear, but it is usually clinically presumed. OBJECTIVE: To analyze the clinical factors and hearing outcomes in patients with labyrinthitis secondary to middle ear infections and to discuss findings based on imaging test results. METHODS: Retrospective cohort study, based on the charts of patients admitted with middle ear infection-associated labyrinthitis. RESULTS: We identified 14 patients, eight (57%) of whom were females and six (43%) males. Mean age was 40 years. Cholesteatomatous chronic otitis media was diagnosed in six patients (43%), acute suppurative otitis media in six (43%), and chronic otitis media without cholesteatoma was diagnosed in two patients (14%). Besides labyrinthitis, 24 concomitant complications were identified: six cases (25%) of labyrinthine fistula, five cases (21%) of meningitis, five cases (21%) of facial paralysis, five cases (21%) of mastoiditis, two cases (8%) of cerebellar abscess, and one case (4%) of temporal abscess. There was one death. Eight (57%) individuals became deaf, while six (43%) acquired mixed hearing loss. CONCLUSION: Suppurative labyrinthitis was often associated with other complications; MRI played a role in the definitive diagnosis in the acute phase; the hearing sequel of labyrinthitis was significant.

RESUMO INTRODUÇÃO: Labirintite permanece resultando em deficiência auditiva significativa, apesar dos esforços científicos para melhorar não só o diagnóstico, como também o tratamento. O diagnóstico definitivo é dependente de imagens da orelha interna, mas geralmente é presumido clinicamente. OBJETIVO: Analisar os fatores clínicos e os resultados auditivos em pacientes com labirintite secundária à otite média e discutir os achados dos exames de imagem. MÉTODO: Estudo de coorte retrospectivo, com base nos prontuários de pacientes diagnosticados com labirintite associada à infecção da orelha média. RESULTADOS: Foram identificados 14 pacientes, oito (57%) do sexo feminino e seis (43%) masculino. Média etária de 40 anos. Otite média crônica colesteatomatosa foi diagnosticada em seis pacientes (43%), otite média aguda em seis pacientes (43%) e otite média crônica sem colesteatoma em dois pacientes (14%). Foram identificadas 24 complicações concomitantes: seis casos (25%) de fístula labiríntica, cinco casos (21%) de meningite, cinco (21%) de paralisia facial, cinco (21%) de mastoidite, dois casos (8%) de abscesso cerebelar e um caso (4%) de abcesso temporal. Houve uma morte. Oito (57%) indivíduos tornaram-se anacústicos, enquanto seis (43%) evoluíram para perda auditiva mista. CONCLUSÃO: Labirintite foi frequentemente associada a outras complicações; RNM auxiliou no diagnóstico definitivo da labirintite na sua fase aguda; a sequela auditiva da labirintite foi significativa.

Nystagmus in patients with unilateral acute otitis media complicated by serous labyrinthitis.

Conclusion The patients with serous labyrinthitis caused by acute otitis media (AOM) exhibited various patterns of nystagmus in which direction-fixed irritative-type nystagmus was the most common pattern. Differential effects on inner ear function by toxic or inflammatory mediators may be responsible for the various manifestation of nystagmus. Objective This study aimed to investigate nystagmus patterns in patients with serous labyrinthitis, and discuss possible mechanisms. Methods From October 2011 to March 2014, 13 consecutive patients with serous labyrinthitis were included. Eye movements of the patients were serially examined using video-nystagmography, and patterns of nystagmus were investigated. Results The most commonly observed pattern was direction-fixed nystagmus (nine of 13 patients). Of these, eight showed irritative-type, and one showed paretic-type. Direction of nystagmus, although the intensity gradually decreased, was not changed during the course of treatment. One patient showed direction-changing spontaneous nystagmus, which changed into paretic-type direction-fixed nystagmus 1 day after myringotomy. Three patients exhibited persistent direction-changing positional nystagmus in a supine head-roll test. Of them, two showed apogeotropic and one showed geotropic type. In all 13 patients, vertigo and hearing loss were improved after the treatment.

Suppurative labyrinthitis associated with otitis media: 26 years' experience.

INTRODUCTION: Suppurative labyrinthitis continues to result in significant hearing impairment, despite scientific efforts to improve not only its diagnosis but also its treatment. The definitive diagnosis depends on imaging of the inner ear, but it is usually clinically presumed. OBJECTIVE: To analyze the clinical factors and hearing outcomes in patients with labyrinthitis secondary to middle ear infections and to discuss findings based on imaging test results. METHODS: Retrospective cohort study, based on the charts of patients admitted with middle ear infection-associated labyrinthitis. RESULTS: We identified 14 patients, eight (57%) of whom were females and six (43%) males. Mean age was 40 years. Cholesteatomatous chronic otitis media was diagnosed in six patients (43%), acute suppurative otitis media in six (43%), and chronic otitis media without cholesteatoma was diagnosed in two patients (14%). Besides labyrinthitis, 24 concomitant complications were identified: six cases (25%) of labyrinthine fistula, five cases (21%) of meningitis, five cases (21%) of facial paralysis, five cases (21%) of mastoiditis, two cases (8%) of cerebellar abscess, and one case (4%) of temporal abscess. There was one death. Eight (57%) individuals became deaf, while six (43%) acquired mixed hearing loss. CONCLUSION: Suppurative labyrinthitis was often associated with other complications; MRI played a role in the definitive diagnosis in the acute phase; the hearing sequel of labyrinthitis was significant.

Silicone impression material foreign body in the middle ear: Two case reports and literature review.

We report two cases of impression material foreign body in the middle ear. The first case had been affected with chronic otitis media. The silicone flowed into the middle ear through a tympanic membrane perforation during the process of making an ear mold. About 4 years and 8 months after, the patient had severe vertigo and deafness. We found bone erosion of the prominence of the lateral semicircular canal and diagnosed labyrinthitis caused by silicone impression material. In the second case silicone flowed into the canal wall down mastoid cavity. Both cases required surgery to remove the foreign body. The clinical courses in such cases are variable and timing of surgery is sometimes difficult. In addition to reporting these two cases, we present here a review of the literature regarding impression material foreign bodies.

Progression of autoimmune inner ear disease to labyrinthitis ossificans: clinical and radiologic correlation.

We report the case of a 42-year-old man who presented with fluctuating bilateral sensorineural hearing loss that subsequently progressed to a complete hearing loss, and we describe the correlation between the clinical and radiologic features of this case. To the best of our knowledge, this is the first report to demonstrate imaging evidence of progression from autoimmune inner ear disease to labyrinthitis ossificans. This is also the first reported case of a reversal of a loss of labyrinthine CISS (constructive interference in a steady state) signal, suggesting that T2-weighted hyposignal may be attributable to an alteration in labyrinthine fluid content and not to fibrosis only.

Labyrinthitis Ossificans: On the Mechanism of Perilabyrinthine Bone Remodeling.

INTRODUCTION: It has been suggested that remodeling of the otic capsule is highly suppressed by the action of anti-resorptive signals emanating from structures of the inner ear space. Labyrinthitis ossificans (LO) is a severe complication to bacterial meningitis and is characterized by destruction of inner ear structures by the formation of new bone. The aim of this study was to explore the impact of LO on bone remodeling of the otic capsule. MATERIAL AND METHODS: In 11 human temporal bones with extensive LO and 10 control specimens, the degree of bone remodeling was explored indirectly by estimating the viability of osteocytes in perilabyrinthine bone and the mastoid. RESULTS: The viability of osteocytes was significantly lower in the perilabyrinthine bone compared to the mastoid in both groups. However, the loss of perilabyrinthine osteocytes was the same in the 2 groups, and the presence of cartilage remnants appeared to be the same. CONCLUSION: This study indicates that the factors affecting bone remodeling of the otic capsule and the degeneration of osteocytes are not altered by wholesale destruction of inner ear soft tissue and its replacement by bone. Therefore, alternative mechanisms may be implicated in the suppression of capsular bone remodeling.

Bilateral sensorineural hearing loss and labyrinthitis ossificans secondary to neurosarcoidosis.

OBJECTIVE: To report a case of cochlear ossification as a result of neurosarcoidosis in a patient with bilateral profound sensorineural hearing loss. STUDY DESIGN: Case report: Setting University teaching hospital, tertiary referral center. PATIENT: Forty-year-old man with neurosarcoidosis and bilateral profound sensorineural hearing loss. Intervention Unilateral cochlear implantation. OUTCOME MEASURE: Aided thresholds speech perception tests. RESULTS: Marked improvement in hearing following implant. CONCLUSION: Patients with neurosarcoidosis are at risk of labyrinthitis ossificans. Early imaging of these patients is recommended and if early cochlear ossification is identified they should be offered rapid access to rehabilitation with a cochlear implant.

Mastoiditis in adults: a 19-year retrospective study.

The objective of our study was to review retrospectively the clinical, radiological and therapeutic findings in 62 adults with acute mastoiditis treated at the ENT Department of Ferrara from 1992 to 2010. 62 adult cases fulfilled the following inclusion criteria: otoscopical evidence of co-existent or recent otitis media; postauricular swelling, erythema or tenderness; protrusion of the auricle; fever and/or significant radiological findings of mastoiditis. Conservative treatment comprehended antibiotic ± ventilation tube. Surgical procedures comprehended mastoidectomy or mastoido-tympanoplasty. The incidence of adult's mastoiditis in our district (0.99 cases/year/100.000 inhabitants) has maintained quite stable during the considered 19-year period. The typical clinical presentation was observed in 48% of cases. Complications were meningitis (15 cases), meningo-encephalitis (1), meningitis associated with lateral sinus thrombosis (1), facial nerve paralysis (11), and labyrinthitis (8). In all cases except one, the facial palsy recovered completely and no mortality was observed due to these complications. Complete cure was obtained with conservative treatment in 69% of uncomplicated cases and in 24% of patients with intracranial complications. Mastoiditis in adults may present as the acute classical form, as well as latent forms which often have prolonged and insidious development followed by a rapid clinical deterioration. Clinical features are frequently atypical, while incidence of meningitis and other complications is still high particularly in the most elderly. Thus, great care is required from clinicians to make an early diagnosis in order to promote adequate treatment.