Histopathology of Labyrinthitis Ossificans From an Intralabyrinthine Surgical Specimen.

OBJECTIVE: Histologic characterization of labyrinthitis ossificans (LO) has mostly been limited to postmortem samples. In this report, we describe the histology of LO from a surgical specimen obtained from a patient undergoing labyrinthectomy with simultaneous cochlear implantation. PATIENT: A 38-year-old woman initially presenting to the emergency room with acute vertigo, left-sided hearing loss, tinnitus, and aural fullness. INTERVENTIONS: Contrast-enhanced magnetic resonance imaging (MRI) and computerized tomography (CT) were performed for the patient before labyrinthectomy and cochlear implantation. Audiometric testing was performed before and after surgical intervention. Histologic analysis was performed on a specimen obtained from the left lateral semicircular canal (SCC) during surgery. MAIN OUTCOME MEASURES: Preoperative CT and MRI findings, preoperative and postoperative hearing thresholds via air conduction and bone conduction, hematoxylin and eosin (H&E) stain of the surgical sample, and CD45 immunostain of the surgical sample. RESULTS: Preoperative CT and MRI confirmed the diagnosis of isolated LO of the left lateral SCC. Audiometric testing revealed significant improvement in hearing after labyrinthectomy and cochlear implantation. H&E stain demonstrated fibrosis and ossification in the left lateral SCC, and CD45 immunostain was negative. CONCLUSIONS: Histopathology of LO based on a surgical sample is comparable to heterotopic ossification (HO) seen in other human tissue. Given the similarities between LO and HO, agents effective in preventing HO may have utility in preventing LO.

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.

Human Otopathology of Cochlear Implant Drill-out Procedures.

OBJECTIVE: Human otopathology following drill-out procedures for cochlear implantation (CI) in cases with labyrinthitis ossificans (LO) has not been previously described. This study uses the high sensitivity of histopathology to (1) evaluate surgical drill-out technique with associated intracochlear findings and (2) quantify spiral ganglion neuron populations in a series of patients with LO who underwent CI. STUDY DESIGN: Retrospective otopathology study. SETTING: Otopathology laboratory. SUBJECTS AND METHODS: Temporal bone (TB) specimens from cases with evidence of preoperative intracochlear fibroossification that required a drill-out procedure for CI electrode array insertion were included. All cases were histopathologically evaluated and 3-dimensional reconstructions of the cochleae were performed to interpret drilling paths and electrode trajectories. RESULTS: Five TB specimens were identified, of which 4 underwent drill-out of the basal turn of the cochlea and 1 underwent a radical cochlear drill-out. In multiple TBs, drilling was imprecise with resultant damage to essential structures. Two TBs showed injury to the modiolus, which was associated with substantially decreased or even absent neuronal populations within these areas. In addition, 2 cases with inadequate drill-out or extensive LO of the basal turn resulted in extracochlear placement of electrode arrays into the vestibule due to persistent obstruction within the basal turn. CONCLUSION: Otopathology highlights the challenges of drill-out procedures in cases of LO. Imprecise drilling paths, due to distortion of normal cochlear anatomy, risk injury to the modiolus and adjacent neurons as well as extracochlear placement of electrode arrays, both of which may contribute to poorer hearing outcomes.

Refractory Positional Vertigo With Apogeotropic Horizontal Nystagmus After Labyrinthitis: Surgical Treatment and Identification of Dysmorphic Ampullae.

OBJECTIVES: To describe the rationale, intraoperative details, and histopathologic findings discovered when treating an unusual case of apogeotropic horizontal canal positional vertigo with a transmastoid labyrinthectomy. PATIENT: A single case report. INTERVENTION: Therapeutic. MAIN OUTCOME MEASURES: Resolution of apogeotropic nystagmus and improvement of positional vertigo. RESULTS: The apogeotropic variant of horizontal canal positional vertigo can be a difficult entity to treat. This report describes a patient who developed profound sensorineural hearing loss and vertigo after an acute left labyrinthitis. Ten months later, she developed vertigo with apogeotropic positional nystagmus involving the left horizontal semicircular canal. Particle repositioning maneuvers and vestibular physical therapy were unsuccessful. In addition, she developed intermittent positional vertigo affecting the ipsilateral vertical semicircular canals. Given the persistence of her vertigo, multiple canal involvement, and patient preference for definitive treatment, a transmastoid labyrinthectomy was performed. Intraoperatively, the ampulla of the horizontal canal as well as that of the other canals was grossly abnormal as later confirmed on histology. After surgery, her apogeotropic nystagmus and vertigo resolved, and her balance ability gradually improved to a highly functional level. CONCLUSION: This case illustrates a unique form of positional vertigo that developed and persisted after acute labyrinthitis. Conservative measures were unsuccessful and a transmastoid labyrinthectomy documented dense inflammatory tissue involving all three ampullae. We postulate that the post-labyrinthitic inflammatory changes resulted in mass loading of the membranous ampullae, causing abnormal nystagmus patterns and positional vertigo, which resolved after the labyrinthectomy.

The incidence of tympanogenic labyrinthitis ossificans.

OBJECTIVE: To estimate the incidence of tympanogenic labyrinthitis ossificans. METHODS: The records of patients treated with mastoidectomy for various tympanogenic aetiologies from January 2007 to December 2011 were retrospectively reviewed. Patients whose high-resolution computed tomography scans showed evidence of labyrinthine calcification of the temporal bone were enrolled. Patients with a history of head and neck cancer, meningitis, and otosclerosis, and patients with cochlear implants, were excluded from this study. RESULTS: A total of 195 patients were enrolled in this study; 4 of the patients presented with calcification in the inner ear. Therefore, the incidence of tympanogenic labyrinthitis ossification was 2 per cent. The computed tomography findings revealed: (1) cochlear calcifications of the basal and middle turn in two patients; and (2) vestibular, superior semicircular canal, posterior semicircular canal and lateral semicircular canal calcification in one, four, three and two patients, respectively. CONCLUSION: The incidence of tympanogenic labyrinthitis ossification in patients who had undergone a mastoidectomy was 2 per cent.

Clinical outcome after cochlear implantation in patients with unilateral hearing loss due to labyrinthitis ossificans.

OBJECTIVES: Cochlear implantation (CI) is the treatment of choice in bilateral labyrinthitis ossificans (LO). The aim of this clinical case study was to evaluate audiologic and subjective outcomes after CI treatment for unilateral hearing loss (UHL) because of LO and to identify optimal timing for treatment. PATIENTS: Three subjects (age 40, 54, and 68 yr) with UHL because of LO were enrolled. Duration of deafness was 1.5, 12, and 120 months. INTERVENTION: After extensive consultation, testing with conventional contralateral routing of signal hearing aid and bone-anchored hearing instrument, CI candidacy was confirmed and CI surgery performed. MAIN OUTCOME MEASURES: Test of open-set speech recognition in background noise and sound localization were performed preoperatively, in unaided and aided conditions, and in the CI-aided condition, at 6 and 12 months postoperatively. Subjective assessment via the Speech, Spatial and Qualities scale (SSQ) and the Tinnitus Visual Analogue Scale was performed at preimplant and 12 months postimplant. CONCLUSION: The data show moderate-to-high hearing benefit after CI in 2 cases and no benefit for the third. SSQ and tinnitus scales show benefit from CI use in both cases. CI treatment should be performed as early as possible, ideally before signs of obliteration are evident. Counseling on all rehabilitation options is important.

Labyrinthine sequestrum: four case studies.

Labyrinthine sequestrum, a rare form of labyrinthitis, is highly distinct from the more commonly encountered labyrinthitis ossificans based on its unique clinical, radiologic, and histologic characteristics. The study included 4 such patients who had undergone clinical and laboratory investigations, computed tomography (CT), and magnetic resonance imaging (MRI) assessments followed by surgical procedures and pathological evaluation. Their major symptoms were otorrhea, otalgia, tinnitus, and profound hearing loss. Imaging studies showed an osteolytic soft mass with calcified debris in the inner ear, and the bony labyrinth was eroded partly or completely by granulation mass, with loss of bony morphology. Further pathological examination was coincident with inflammatory granulation tissue with some calcification or osseous tissue. The disease process is attributed to chronic osteomyelitis due to the presence of osteonecrosis. Prompt CT and MRI examinations and optimal therapeutic management facilitate definitive diagnosis and protect against fatal complications.

Medical and surgical management of otitis in captive bongo (Tragelaphus eurycerus).

Six cases of severe otitis media-interna, an uncommon problem in nondomestic ruminants, were diagnosed in five captive bongo (Tragelaphus eurycerus). The cases were geographically clustered at zoological facilities in Florida. A visible ear droop, head shaking, and otic discharge were common at clinical presentation. Medical management with prolonged systemic and topical antimicrobial therapy, combined with repeated manual removal of debris from the otic canal, was successful in resolving two cases and effectively controlled a third case. Two bongo with severe otitis did not respond to medical management and required surgical intervention. A bulla osteotomy and total ear canal ablation were performed on these animals (bilaterally in one bongo). Surgery was successful in providing complete clinical resolution of the otitis and is recommended for severe cases that fail to respond to less invasive management.

Management of chronic otitis by middle ear obliteration with blind sac closure of the external auditory canal.

OBJECTIVE: Description of a technique of middle ear obliteration (MEO) with blind sac closure of the external auditory canal with discussion of the indications for its use in cases of recalcitrant chronic otitis and in far advanced disease. PATIENTS: All patients underwent otologic examination and audiologic and radiologic assessments in a quaternary center. RESULTS: Fifty-three cases of MEO were analyzed. For 9 patients, primary surgery was performed. One case of residual disease was identified. The minimum follow-up was 2 years. CONCLUSION: The decision to perform a MEO is one that is made only rarely. However, this is a technique that should be part of every otologist's armamentarium. Whereas the indications for its use are more straightforward in an ear with unserviceable hearing, a MEO is occasionally required in an ear with good cochlear reserve due the severity of disease. All of our patients managed by MEO have had an improvement in their quality of life and a high rate of successful eradication of disease.

[Labyrinthitis, or inflammatory pseudotumor after stapedectomy]. / Labyrinthite ou pseudotumeur inflammatoire après stapédectomie.

OBJECTIVES: To describe an extensive pseudotumor as a complication of stapes surgery. METHODS: Radiological workup and surgical exploration in a 38-year-old man suffering from postoperative hearing loss. The patient presented with tinnitus, inferior facial palsy, vertigo, and rapidly progressive hearing loss after his operation. RESULTS: The initial postoperative CT scan was normal. However, seven months after surgery, the CT scan showed an enlargement of the inner ear canal and complete vestibular destruction. The CISS sequence of the magnetic resonance imaging (MRI) enhanced after gadolinium injection revealed the presence of a mass filling the entire inner ear canal, the cochlear, the posterior labyrinth, and the middle ear. The aspect suggested an inflammatory pseudotumor. Surgical exploration confirmed the invasive aspect of the mass and pathological analysis revealed inflammatory tissue associated with microcalcifications. DISCUSSION: Hearing loss, vertigo, and tinnitus after stapes surgery require a radiologic workup. The CT scan is done first. It could be normal or eliminate other diagnoses. MRI may lead to a more precise diagnosis. It can reveal an inflammatory process of the inner ear after gadolinium injection. Surgical exploration is indicated in case of aggressive and extensive lesions. CONCLUSION: In the context of hearing loss complicating otosclerosis surgery, an imaging workup should include a CT scan. In case of a suspected expansive and inflammatory mass, it should be completed by an MRI (CISS sequence and gadolinium injection). An inflammatory lesion of the inner ear could indicate extensive pseudotumor.

[A case of labyrinthitis due to congenital cholesteatoma in a child]. / Przypadek zapalenia blednika w przebiegu perlaka wrodzonego u dziecka.

A rare case of congenital cholesteatoma as a cause of acute labyrinthitis is presented in the report. 14-years old boy was operated due to labyrinthitis caused by a huge cholesteatoma discovered behind intact tympanic membrane. On the first examination the symptoms indicated lack of right inner ear function, so unfortunately the referral was to late and the patient resulted in the dead ear. The diagnosis of congenital cholesteatoma as well as treatment of the entity and its subsequent complications is discussed in the report.

Labyrinthine fistula as a late complication of middle ear surgery using the canal wall down technique.

OBJECTIVES: To evaluate the clinical features of labyrinthine fistulae occurring as a late complication of middle ear surgery using the canal wall down technique. STUDY DESIGN: This was a retrospective study of the past 23 years, conducted at a single tertiary care center. The authors evaluated the backgrounds, clinical features, and surgical findings in 25 patients with labyrinthine fistulae, who had a history of ear surgery using the canal down technique and who underwent a second operation at their hospital. INTERVENTIONS: All the patients underwent revision surgery because of persistent or recurrent vertigo caused by labyrinthine fistulae, circumscribed labyrinthitis, or suppurative labyrinthitis. MAIN OUTCOME MEASURES: The clinical features of this disease entity were assessed by history, surgical findings, and the results of audiovestibular testing. RESULTS: The patients had a long history of repetitive postoperative aural discharge before experiencing vertigo, which initially occurred 4 to 64 years (average, 20.2 years) after the previous operation. At the first visit to the authors' clinic, the results of a fistula test conducted with a Politzer's bulb were positive in 14 patients and negative in 5 patients. In the remaining 6 ears, pressure loading of the ear canal induced the sensation of vertigo without accompanying nystagmus. Surgical intervention showed that the fistulae were located at the lateral semicircular canal in 19 ears, at the footplate of the stapes in 4 ears, and at the promontory in 2 ears. Labyrinthine fistulae were closed with conchal cartilage, bone paste (bone dust mixed with fibrin glue), and/or temporalis fascia. In some patients, the fistulae were further covered with pedicled temporalis muscle. In 2 cases complicated by acute suppurative labyrinthitis, the mastoid cavity was obliterated after completion of the labyrinthectomy. The postoperative courses in all patients were uneventful. CONCLUSIONS: A labyrinthine fistula may be created by repeated and insidious infection of a mastoid cavity that has been exposed to the outside during canal wall down surgery. Intensive care of the opened mastoid cavity is essential to avoid this late complication.

Topographic analysis of inner ear lesions in profoundly deafened patients with tympanogenic and meningogenic labyrinthitis using three-dimensional magnetic resonance imaging.

OBJECTIVE: High-resolution magnetic resonance imaging (MRI) provided clear images of three-dimensional (3D) reconstruction of the inner ear in candidates for cochlear implantation. In this study. semiquantitative analysis of the 3D MRI findings was performed to investigate topographic lesions of the inner ear caused by tympanogenic and meningogenic labyrinthitis. STUDY DESIGN: This was an observational study. SETTING: The study was performed in an academic, comprehensive, multispecialty group practice. PATIENTS: Postlingual deafened patients with cochlear implantation. The cause of deafness was tympanogenic or meningogenic labyrinthitis. INTERVENTION: High-resolution 3D MRI and postoperative speech recognition tests were used. RESULTS: Abnormal findings in the inner ear detected with MRI were found before surgery in 58.3% of the patients with meningogenic labyrinthitis, although the incidence was lower in patients with tympanogenic labyrinthitis. Abnormal MRI findings were frequently observed in the cochlear basal turn and semicircular canals more than in the middle and apical turn in cases with meningogenic etiology. Patients with tympanogenic labyrinthitis suffered less with a vestibular apparatus than did those patients with meningogenic labyrinthitis. Regarding the analysis of the inner ear lesions at the implanted side, the postoperative speech recognition ability did not correlate to the extent of abnormal MRI findings of the implanted ear. CONCLUSION: Improvement in 3D MRI technology provided an accurate preoperative picture of the inner ear apparatus. In cochlear implant patients with infectious labyrinthitis, the extent of the inner ear lesion detected with 3D MRI was different among etiologies of deafness.

Cholesteatoma extending into the internal auditory meatus.

We report our experiences in managing a patient with cholesteatoma complicated by meningitis, labyrinthitis and facial nerve palsy. The antero-inferior half of the tympanum was aerated but the postero-superior portion of the tympanic membrane was tightly adherent to the promontry mucosa. An attic perforation was present at the back of the malleolar head. High-resolution computed tomography also uncovered a fistula in the lateral semicircular canal. Surgical exploration of the middle ear cavity demonstrated that both the vestibule and cochlea were filled with cholesteatoma, and the cholesteatoma extended into the internal auditory meatus through the lateral semi-circular canal fistula. The cholesteatoma was removed by opening the vestibule and cochlea with a preservation of the facial nerve. Post-operatively, an incomplete facial palsy remained, but has improved slowly. There is no sign of recurrence to date after a 3-year period of observation.

Reconstruçäo tridimensional de acessos cirúrgicos para neurimoma do acústico, utilizando imagens de tomografia computadorizada / Threedimensional reconstruction of acustic neuroma approaches, using computerized tomography imaging

Os autores apresentam estudo preliminar sobre reconstruçäo tridimensional de superfície a partir de imagens de tomografia computadorizada. Para tal, foi realizada tomografia em osso temporal conservado em formol, antes e depois da confecçäo de acesso retrolabiríntico e translabiríntico. Os dados foram utilizados para reconstruçäo de imagens tridimensionais, com o intuito de visualizaçäo do conduto auditivo interno nos diferentes acessos e para o cálculo da quantidade de osso removida em cada um

[Sensorineural hearing loss caused by middle ear surgery].

It is well known that sensorineural hearing loss rarely occurs after middle ear surgery. Five cases of postoperative sensorineural hearing loss were reviewed. These operations amounted to 0.9% of all middle ear operations in which the first author was involved. The causes of hearing loss were believed to be labyrinthitis through the fistula in cholesteatoma case 1, and acoustic trauma from the bone-cutting burr in cases 2 and 3. In cases 4 and 5 postoperative sensorineural hearing loss occurred several months after stapes surgery and the causes of hearing loss were unknown. On the basis of the available literature discussion focused on management of labyrinthine fistula, the acoustic effect of the bone-cutting burr and the possibility of sensorineural hearing loss caused by stapes surgery.