Cochlear Implantation in Patients With Osteolytic Labyrinthitis: A Case Series.

OBJECTIVE: To describe the rare process of osteolytic labyrinthitis, previously referred to as labyrinthine sequestrum, which involves progressive obliteration of the bony and membranous labyrinth with eventual supplantation with soft tissue and, in some cases, bony sequestrum. PATIENTS: Three patients with diverse presentations of osteolytic labyrinthitis from two tertiary care academic medical centers. INTERVENTIONS: Case series report analyzing the relevant clinical, radiologic, pathologic, and surgical data on our patients with osteolytic labyrinthitis and comparing these index cases to the existing literature. MAIN OUTCOME MEASURES: We describe the varying image findings seen in osteolytic labyrinthitis on computed tomography and magnetic resonance imaging. Also, we report successful surgical intervention and hearing rehabilitation with cochlear implantation in patients with osteolytic labyrinthitis. RESULTS: Our three patients presented with profound sudden sensorineural hearing loss and vertigo consistent with labyrinthitis. None of the three patients had a history of chronic otitis media. Imaging workup revealed varying degrees of erosion to the otic capsule bone demonstrating the spectrum of disease seen in osteolytic labyrinthitis. Although two cases showed osteolytic changes to the semicircular canals and vestibule, the first case revealed frank bony sequestrum within the obliterated labyrinth. The three cases were taken for surgical debridement and cochlear implantation. CONCLUSIONS: We propose the new term, osteolytic labyrinthitis-previously referred to as labyrinthine sequestrum-to describe the rare spectrum of disease characterized by destruction of the osseous and membranous labyrinth and potential supplantation with bony sequestrum. Cochlear implantation is a viable option in selected patients with osteolytic labyrinthitis.

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.

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.

Reversible Canalith Jam of the Horizontal Semicircular Canal Mimicking Cupulolithiasis.

OBJECTIVE: To describe a case of benign paroxysmal positional vertigo (BPPV) resulting in reversible horizontal semicircular canalith jam successfully treated with horizontal canal occlusion. A brief literature review of similar cases was performed. METHODS: Case report and literature review. RESULTS: A 68-year-old female presented with apogeotropic positional nystagmus, attributed to reversible horizontal canalith jam mimicking cupulolithiasis that was refractory to tailored repositioning maneuvers across months. She was unable to work due to the severity of her symptoms. She underwent surgical occlusion of the affected canal with immediate resolution of her symptoms. A literature review revealed similar cases of canalith jam mimicking cupulolithiasis. CONCLUSIONS: Reversible canalith jam, in which particles moving with horizontal head position alternate between obstructing the semicircular canal and resting on the cupula, can mimic signs of cupulolithiasis. This variant of BPPV can be effectively managed with surgical canal occlusion should symptoms fail to resolve after tailored repositioning maneuvers.

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.

"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.

Cochlear implant in Thalassemia patient - Case report.

INTRODUCTION: Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin. Iron overload occurs in thalassemia, with blood transfusion therapy being the major cause. Deferoxamine continues to be the mainstay of therapy to remove excess iron in patients requiring long-term transfusions. One of the most important complications of deferoxamine therapy is neurosensory toxicity, including sensorineural hearing loss (SNHL). Labyrinthine hemmorhage (LH) is thought to result from altered capillary hemodynamics or reperfusion injury. It is theorized that LH incites a reparative response that cascades from fibrosis to sclerosis and ultimately ossification of the inner ear structures. CASE PRESENTATION: We present a case of 3-year-old thalassemic child with bilateral profound sensorineural hearing loss. Patient was on regular blood transfusions with chelation therapy. HRCT temporal bone and MRI brain and temporal bone had features of labyrinthitis ossificans (LO). Child underwent uniateral cochlear implantation and postimplantation speech perception and production outcomes were normal. DISCUSSION: This case illustrates the unique feature of labyrinthitis ossificans in a thalassemia patient which has not yet reported in the English literature. Hearing screening of all thalassemia patients and therefore early diagnosis of SNHL prompts early intervention and improved quality of life.

Facial Nerve Stimulation Patterns Associated With Cochlear Implantation in Labyrinthitis Ossificans.

OBJECTIVE: To describe and characterize facial nerve stimulation (FNS) patterns in patients with labyrinthitis ossificans who underwent cochlear implantation (CI) for sensorineural hearing loss. PATIENTS: Five ears in four patients with labyrinthitis ossificans who underwent CI and subsequently developed FNS. INTERVENTIONS: CI, electrode mapping, and/or explantation to resolve FNS. MAIN OUTCOME MEASURES: FNS, postoperative computed tomography imaging, and resolution of FNS. RESULTS: Fourteen ears with labyrinthitis ossificans underwent CI over an 11-year period at a single institution; 5 of these ears exhibited postoperative FNS (35.7% incidence). Four cases had involvement of basal electrodes, while all five cases had middle and/or apical electrode stimulation. All cases had resolution of FNS with CI reprogramming, however, the resultant map in two cases provided minimal audiologic benefit and patients became nonusers in that ear. CONCLUSIONS: FNS arising from all cochlear regions is possible in patients with labyrinthitis ossificans who undergo CI. Mapping and electrode deactivation can resolve symptoms, but resultant audiologic benefit is variable and may lead to explantation.

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.

Acoustic brainstem implant in a post-meningitis deafened child-Lessons learned.

We report a case of a deafened child with advanced labyrinthitis ossificans resulting from meningo-encephalitis. She received a cochlear implant in the right ear, following a drill-out procedure. Post-implant hearing outcomes were satisfactory initially, but deteriorated over time as a result of partial electrode migration. The child subsequently received a left auditory brainstem implantation with improvement of hearing outcomes. Post-operatively, a sub-dural hematoma developed not on the side of the operation but on the opposite side. Simultaneous use of the cochlear implant on one side and the acoustic brainstem on the other, aggravated the non-auditory side effects of the ABI and compromised its potential for optimal hearing results.

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.

Anatomy of the middle-turn cochleostomy.

OBJECTIVE: Middle-turn cochleostomies are occasionally used for cochlear implant electrode placement in patients with labyrinthitis ossificans. This study evaluates the anatomic characteristics of the middle-turn cochleostomy and its suitability for placement of implant electrodes. METHODS: Ten cadaveric human temporal bones were dissected using a facial recess approach. A middle-turn cochleostomy was drilled 2 mm anterior to the oval window and just inferior to the cochleariform process. The preparations were then stained with osmium tetroxide and microdissections were performed. The location of the cochleostomy on the cochlear spiral and its path through the various cochlear compartments were evaluated in all 10 specimens. A Cochlear Corporation depth gauge was inserted in five of the specimens and insertion trauma, number of contact rings, and depth of insertion were recorded. RESULTS: Eight of the 10 cochleostomies were placed at approximately 360 degrees on the cochlear spiral, near the transition between the basal and middle turns. In one case, the cochleostomy was found to enter the cochlear apex and in another it entered scala vestibuli of the proximal basal turn. The cochleostomy entered scala media in six bones and scala vestibuli in four specimens. A depth gauge was inserted in five specimens. The number of contacts placed within the cochlear lumen ranged from four to nine. There was evidence of insertional trauma to the lateral wall of the cochlear duct, basilar membrane, and Reissner's membrane, but no evidence of fractures to the osseous spiral lamina or modiolus. CONCLUSION: This study demonstrates that electrodes inserted via a middle-turn cochleostomy are likely to enter scala vestibuli and have access to the middle- and apical-cochlear turns. It is also possible that the electrode could be directed into the descending portion of the basal turn depending on cochleostomy orientation. Middle-turn cochleostomy seems to be a viable alternative for electrode placement when preservation of residual hearing is not a concern.

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.

Nontypeable Haemophilus influenzae meningitis complicated by hearing loss in a 9-year-old HIV-infected boy.

A 9-year old boy with perinatal HIV infection developed meningitis due to nontypeable Haemophilus influenzae. His course was complicated by progressive hearing loss due to labyrinthitis ossificans. Placement of cochlear implant improved hearing thresholds. Nontypeable H. influenzae meningitis and use of cochlear implants have not previously been in HIV-infected children.