Rapidly progressive labyrinthitis ossificans in an immunocompromised pediatric patient

In this report, we present a case of rapid otic capsule obliteration within an exceedingly short timeframe in the setting of Chronic Suppurative Otitis Media (CSOM) in an immunocompromised pediatric patient with Down Syndrome. Following maximal therapy for a right sided cholesteatoma, the patient developed a multi-drug resistant infection that cause CSOM, which within 6 weeks progressed to complete obliteration of the right cochlea and otic capsule. The possibility of congenital temporal bone microscopic dehiscence allowing infection propagation cannot be excluded. Nonetheless, this case highlights the importance of appreciating how quickly chronic middle ear disease can progress to involve the labyrinth and cause intracranial complications, even with adequate concurrent medical therapy in the form of antibiotics and surgical therapy. A greater awareness as physicians should be made on management of refractory chronic middle ear disease to better treat their potential complications, which is made apparent in this case report. Keywords: Otic capsule; Chronic suppurative otitis media; Otic capsule; Tympanomastoidectomy; Multi-drug resistance. Abbreviations: CSOM: Chronic Suppurative Otitis Media; MDR: Multi-Drug Resistant; CT: Computed Tomography; MRI: Magnetic Resonance Imaging; ID: Infectious Disease.

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.

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.