A Case of Intralabyrinthine Schwannoma Removal and Sequential BonebridgeTM Implantation

Intralabyrinthine schwannoma (ILS) is a rare benign tumor that arises within the membranous labyrinthine. Since hearing loss is inevitable after tumor removal in most cases, an appropriate rehabilitation method should be considered. A 41-year-old male, who was diagnosed with ILS underwent tumor removal via translabyrinthine approach, has subsequently experienced right-side deafness as a result. Seventeen months after the tumor removal, a surgery for hearing rehabilitation was performed. Since cochlear implantation was not doable due to cochlear ossification, an active transcutaneous bone conduction implant (BonebridgeTM , MEDEL) was placed at the sinodural angle. To the best of our knowledge, this is the first report describing hearing rehabilitation with BonebridgeTM implantation after ILS removal.

Otological IgG4-Related Disease With Inner Ear Involvement: A Case Report and Review of Literature

Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory disease involving multiple organs. Some studies have reported otological manifestations of IgG4-RD, although most studies describe involvement of the middle ear, and reports on inner ear manifestations are limited. Here, we describe a case of a 30-year-old man with IgG4-RD involving the inner ear. This case demonstrated that IgG4-RD affected the inner ear and caused cochlear ossification. Cochlear implants may be considered for milder cases, and hormone and immunosuppressive therapy may control disease progression.

Bilateral Hearing Loss and Unilateral Cochlear Ossification in a Patient With Chronic Myelogenous Leukemia

Bilateral sensorineural deafness and unilateral cochlear ossification have rarely been described in patients with chronic myeloid leukemia (CML). A 21-year-old man presented to a hospital with right-sided sudden hearing loss and tinnitus. He was diagnosed with CML. Five days later, sudden hearing loss appeared in the other ear. Abnormality of the right-sided inner ear structure was revealed by preoperative magnetic resonance imaging; honeycomb-like cochlear ossification was observed during cochlear implant surgery in the right ear. The patient’s auditory performance exhibited significant improvement after bilateral cochlear implantation in our hospital. Hematological disorders must be considered in patients with sensorineural hearing loss. Cochlear implantation is feasible in patients with CML who exhibit sensorineural deafness, but cochlear ossification should be carefully evaluated by means of preoperative imaging examinations.

The role of preoperative imaging for auditory implants in children

<b>Introduction:</b> Preoperative imaging, besides audiological evaluation, plays a major role in evaluation of candidacy for auditory implants, and in particular cochlear implants. It is essential to assess whether the basic criteria necessary for implantation are met. Diagnostic imaging is crucial not only in determining candidacy, but also determining the feasibility of cochlear implantation as it allow to anticipate surgical difficulties which could preclude or complicate the implantation of the device. The aim of the study is to present the protocol for the evaluation of preoperative imaging studies with particular focus on the factors potentially affecting clinical decisions in children qualified for cochlear implantation. <br><b>Material and method:</b> Preoperative imaging studies of 111 children performed prior to cochlear implantation were analyzed: high-resolution computed tomography (HRCT) of temporal bones and MRI. The assessment was made according to the presented protocol. <br><b>Results:</b> Pathologies and anomalies identified during the assessment of preoperative imaging studies significantly altered clinical decisions in 30% of patients. In the study group, in 17% of patients inner ear malformations were identified. 2.7% of children were disqualified from a cochlear implantation due to severe congenital inner ear malformations. 9% of the patients have had bacterial meningitis. In 50% of them difficulties related to complete or progressive cochlear ossification occurred. In 4.5% of patients less common surgical approaches other than mastoidectomy with a posterior tympanotomy were applied. <br><b>Discussion:</b> Preoperative imaging allow for the identification of significant pathologies and anomalies affecting qualification decisions and further treatment. HRCT and MRI are complementary to each other for preoperative imaging. The two modalities in combination allow accurate and optimal evaluation of the anatomical structures prior to implantation. Inner ear malformations and cochlear ossification following meningitis are relatively frequently encountered in children qualified for a cochlear implant.

Evaluation of Subtotal Petrosectomy Technique in Difficult Cases of Cochlear Implantation

<b><i>Objectives:</i></b> To assess the validity of the subtotal petrosectomy (STP) technique in problematic cases of cochlear implant (CI) surgery, and review indications, outcomes, and related controversies. <b><i>Study Design:</i></b> This is a retrospective review of data from a private quaternary referral center of otology and skull base surgery. <b><i>Patients and Methods:</i></b> A review of patients who underwent CI with STP (STP-CI) as the leading approach was performed. Demographics, indications, surgical details, and main outcomes were evaluated. The surgeries performed were usually single-stage procedures encompassing a comprehensive mastoidectomy, blind sac closure of the external auditory canal (EAC), and mastoid obliteration with autologous fat. <b><i>Results:</i></b> A total of 107 cases were included. Mean follow-up was 7.1 years (range 1–13 years). The most frequent indication for STP-CI was chronic otitis media with/without cholesteatoma (32.7%), followed by open mastoid cavity (26.1%), and cochlear ossification (17.7%). Other difficult conditions where STP facilitates successful implantation include inner-ear malformations, temporal-bone trauma, unfavorable anatomic conditions, and revision surgery. A planned staged procedure was performed in 3 cases. The rate of major complications was 5.6% (<i>n</i> = 6). Three patients developed postauricular wound dehiscence which eventually resulted in device extrusion. No cases of recurrent/entrapped cholesteatoma, EAC breakdown, or meningitis were encountered. This is the largest single-center series of STP-CI reported in the literature. <b><i>Conclusions:</i></b> When CI is intended in technically challenging cases or associated with a high risk of complications, STP is effective and reliable. Safe implantation and excellent long-term outcomes can be achieved provided surgical steps are properly followed. Single-stage procedures can be performed in most cases, even when there is active middle-ear disease.

Cochlear Ossification in a Patient with Cogan’s Syndrome Undergoing Bilateral Cochlear Implantation

We present the case of a young female patient diagnosed with Cogan’s syndrome after the rapid onset of profond hearing and vestibular loss with concomitant eye symptoms. After appropriate medical treatment, her hearing did not respond and she underwent bilateral simultaneous cochlear implantation with findings of extensive cochlear ossification in both ears. The case and outcome are described in the body of the paper.