Labyrinthitis ossificans is the formation of pathological new bone within the membranous labyrinth of the inner ear due to various local and systemic pathologies. Most commonly it occurs as a sequelae of meningitis spreading to the labyrinth, from the subarachnoid space via the cochlear aqueduct and the internal auditory canal. We are comparing three different etiological presentations of labyrinthitis ossificans; namely, tympanogenic, meningitic, and traumatic, together with their management in the light of recent advances.

Labyrinthitis most commonly results from an infectious and less commonly from an inflammatory process of the inner ear, but it can be associated with temporal bone trauma, hemorrhage, or tumor. This inflammation (regardless of the etiology) disrupts the transmission of sensory information from the ear to the brain. Labyrinthitis ossificans is the pathological ossification of the membranous labyrinthine spaces in response to an insult to the inner ear involving membranous labyrinth or the endosteum of the otic capsule. Herein, we present a case of a 67-year-old female with progressive bilateral sensorineural hearing loss and positional vertigo.

OBJECTIVE: The aim of this study was to report a case of cochlear implantation (CI) for a patient with an otic capsule-sparing traumatic brain injury (TBI) and to review the relevant literature.
METHODS: A patient with history of TBI received a CI for bilateral profound hearing loss. A systematic review of the literature was performed to identify and compare similar cases.
RESULTS: A 36-year-old male with a history of hearing loss from right acute labyrinthitis was referred for bilateral profound sensorineural hearing loss (SNHL) after a fall with associated injury to the central auditory nervous system (CANS) including the brainstem. On the right, behavioral acoustic threshold measurements were in the profound range with absent OAEs. On the left, testing revealed no measurable behavioral acoustic thresholds and variable physiologic measures. A right unilateral cochlear implant was performed with most recent follow-up demonstrating speech awareness thresholds of 25 dB HL with excellent detection of all 6 Ling sounds. However, the patient also continues to suffer from other neurologic sequelae related to his TBI, which challenge his ability to demonstrate objective and subjective benefit. A systematic review of the literature demonstrates variable outcomes for patients with TBI and SNHL.
CONCLUSIONS: Patients with profound SNHL and TBI present a distinct rehabilitative challenge for clinicians. CI may provide meaningful benefit in this population, though care should be taken in patient selection and counseling.

The underlying pathophysiology of idiopathic sudden sensorineural hearing loss (ISSNHL) is still unknown. However, an increasing number of observational studies report intralabyrinthine signal alterations in patients with ISSNHL using three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI). These findings warrant a meta-analysis.
To conduct a meta-analysis assessing the value of 3D-FLAIR MRI in identifying possible underlying labyrinthine pathophysiologic mechanisms and prognostication in patients with ISSNHL.
Two reviewers independently searched the Pubmed, Embase and Cochrane Library from inception until October 10, 2018 and evaluated eligibility based on titles and abstracts of all retrieved studies. All studies reporting on 3D-FLAIR imaging in ISSNHL were included. Subsequently, the full text of eligible studies were evaluated.
Adhering to the MOOSE guideline, two independent reviewers extracted data, assessed risk of bias and evaluated the relevance and quality of evidence. Data on the number of patients and events were extracted and hearing levels were converted to standardised mean differences (SMD) for conducting meta-analyses. Random effects models for meta-analyses were applied.
Eight observational studies met our inclusion criteria (n = 638 patients). In 29%, high signal intensity was found on 3D-FLAIR imaging, suggesting labyrinthine pathology (labyrinthitis [79%], intralabyrinthine haemorrhage [21%]). High signal intensity on 3D-FLAIR was associated with poorer hearing (SMD: 14 dB, 95% CI 5.67-22.94) and vertigo (RR: 1.92, 95% CI 1.16-3.17) at baseline. Multivariate analyses demonstrated that patients with high 3D-FLAIR signal intensity had 21 dB lower final hearing pure-tone averages (SMD: 21 dB, 95% CI 9.08-33.24).
Three-dimensional fluid-attenuated inversion recovery MR imaging can identify an underlying labyrinthine condition in up to 29% of patients with sudden hearing loss in whom previously no cause could be identified. Their final pure-tone averages are more than 20 dB worse than 3D-FLAIR-negative patients, suggesting more severe labyrinthine damage. Findings such as these may contribute to our understanding of pathophysiologic mechanisms of ISSNHL.

Conductive hearing loss (CHL) commonly results from middle ear fluid and inflammation (otitis media). Less commonly in patients with CHL, the middle ear cleft is well aerated or \'dry\' with absence of soft tissue or fluid clinically and on imaging. There are numerous causes for this but they can be clinically challenging to diagnose. This pictorial review aims to illustrate and discuss the CT features of both common and less common causes of CHL in patients with a \"dry middle ear cavity\".

To report the presentation, diagnosis, management, and convalescence of labyrinthine sequestrum (LS) and summarize all previously published cases.
Eleven-year-old female with LS.
Multidisciplinary diagnostic evaluation and treatment.
Imaging and laboratory findings, medical and surgical treatment.
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.
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.

BACKGROUND: Auditory implantation into the inner ear is increasingly performed for a variety of indications. Infective complications are rare, but when they occur they can have devastating consequences.
METHODS: This paper reports two cases where vestibular sequestration of the bony labyrinth developed following implantation into the middle ear.
CONCLUSIONS: To the authors\' knowledge, these are the first reported cases where vestibular sequestration has resulted from auditory implant surgery. This paper outlines the radiological changes characteristic of this pathology. It also describes the surgical and conservative treatment options for this condition, challenging the previously accepted belief that affected patients always require aggressive surgical intervention.

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.

Deafness is a common and often permanent neurological sequel of Streptococcus (S.) suis meningitis. Suppurative labyrinthitis, rather than direct auditory nerve infection, has been found to be the site responsible for deafness. Neuroimaging is important to localise the site involved in hearing loss and to assess the feasibility of a cochlear implantation. S. suis is very sensitive to penicillin. Although a relapse of S. suis meningitis is uncommon, it can occur despite an adequate duration of appropriate antibiotic therapy. We describe a patient with S. suis meningitis, who developed permanent deafness from haemorrhagic labyrinthitis, as shown on magnetic resonance imaging. She suffered a relapse despite a seven-week course of intravenous antibiotics. A review on six cases of relapse reported in the literature shows that relapses occurred despite two to four weeks of antibiotics being administered to the patients. The clinical implications and treatment of relapse are discussed.

METHODS: A case of a 54-year-old woman with a three month history of recurrent bilateral chondritis of the auricles, cochlear and vestibular inner ear damage and conjunctivitis is described. The diagnosis was based only on clinical criteria (auricular chondritis, conjunctivitis, inner ear damage). Antinuclear antibodies, ANCA and HLA-DR 4 antigen were negative. The only laboratory abnormality was an elevated erythrocyte sedimentation rate. The patient has been treated successfully with Methotrexate 20 mg 1 x /week and Prednisone 15 mg/die for 4 month now.
CONCLUSIONS: The relapsing polychondritis (RP) is a rare, multisystemic and potentially fatal disease. The pathogenesis and optimal therapeutic approach is poorly understood. The disease is characterized by episodic inflammation of cartilage such as auricular, nasal and laryngotracheal. Many other proteoglycan-rich structures like inner ear, eye, kidney and blood vessels, may be involved as well. RP has an equal sex prevalence. The majority of cases appear between 40 and 60 years. Auricular inflammation is the most common feature. Effectiveness of non-steroidal anti-inflammatory drugs, dapsone, immunosuppressive drugs and prednisone has been described. The overall survival rates were 74 % at 5 years and 55 % at 10 in one 1986 series.
CONCLUSIONS: The most common clinical presentation of RP regularly involves ENT-structures. Therefore ENT-specialists should be familiar with this disease. A close interdisciplinary cooperation is essential for therapy and follow-up, because pulmonary and cardiac involvement are limiting prognostic factors.