Beyond the otoscope: an imaging review of congenital cholesteatoma.

Congenital cholesteatoma (CC) is a non-neoplastic lesion of keratin debris lined by epithelium found in the temporal bone. It is the lesser-known sibling of the acquired cholesteatoma and may be classified as congenital middle ear cholesteatoma and congenital petrous bone cholesteatoma. The incidence is rising, probably owing to increased recognition and advances in imaging modalities. Cone beam CT provides detailed anatomical information, highlighting quadrant location, ossicular involvement, and mastoid extension. MRI aids in lesion characterization and detection of complications. The classification systems for congenital middle ear and petrous bone cholesteatoma are helpful in the preoperative workup and have a role in predicting postoperative recurrence rates. Management almost invariably involves surgical intervention aimed at preserving middle and inner ear function. Follow-up of CC is mainly based on MRI together with otoscopic examination. Non-echo planar diffusion-weighted imaging, especially, has proven essential for detecting residual disease. This review article emphasizes the significance of imaging in the timely diagnosis and management of CCs. CLINICAL RELEVANCE STATEMENT: This article underscores the crucial role of imaging for prompt detection, preoperative assessment, and postoperative follow-up of CCs, a condition with rising incidence associated with potentially severe complications. KEY POINTS: Timely diagnosis of CCs is imperative for avoiding complications. Imaging is key in detection, preoperative evaluation, and postoperative management. Cone Beam CT and non-echo planar DWI represent state-of-the-art imaging techniques.

Duration of cochlear implant use in children with prelingual single-sided deafness is a predictor of word perception in the CI ear.

As part of a longitudinal study regarding the benefit of early cochlear implantation for children with single-sided deafness, the current work explored the children's daily device use, potential barriers to full-time device use, and the children's ability to understand speech with the cochlear implant (CI). Data were collected from 20 children with prelingual SSD who received a CI before the age of 2.5 years, from the initial activation of the sound processor until the children were 4.8 to 11.0 years old. Daily device use was extracted from the CI's data logging, while word perception in quiet was assessed using direct audio input to the children's sound processor. The children's caregivers completed a questionnaire about habits, motivations, and barriers to device use. The children with SSD and a CI used their device on average 8.3 h per day, corresponding to 63 % of their time spent awake. All children except one could understand speech through the CI, with an average score of 59 % on a closed-set test and 73 % on an open-set test. More device use was associated with higher speech perception scores. Parents were happy with their decision to pursue a CI for their child. Certain habits, like taking off the sound processor during illness, were associated with lower device use. Providing timely counselling to the children's parents, focused on SSD-specific challenges, may be helpful to improve daily device use in these children.

Virtual reality application matches the most established treatment for Mal de Debarquement Syndrome: A non-inferiority, randomized, open clinical trial.

Mal de Debarquement Syndrome (MdDS) is a debilitating neuro-otological disorder where individuals consistently feel self-motion, often triggered by motion like being on a boat (MT-MdDS). Due to the unknown pathophysiological mechanism, available treatment options for managing symptoms are limited. Our objective was to develop a virtual reality application (VRA) to simulate the full field optokinetic stimulation (OKS) booth and evaluate its efficacy compared to the standard treatment. In our randomized, open, non-inferiority clinical trial with 30 â€‹MT-MdDS patients, 15 received the OKS booth and 15 the new VRA over four consecutive days. Two 4-min treatment blocks were scheduled in the morning and afternoon, with a total of four blocks. Treatment effectiveness was evaluated through questionnaires and posturography. Our findings suggest that the choice of modality does not significantly differ in achieving an overall improvement in symptoms. We advocate that the VRA can be used as an accessible alternative to the booth method worldwide, effectively mitigating MdDS symptoms and enhancing the QoL of numerous MdDS patients.

Guideline for standardized approach in the treatment of the Mal de Debarquement syndrome.

Introduction: Mal de Debarquement Syndrome (MdDS) is a debilitating neuro-otological disorder. Patients experience almost continuously a perception of self-motion. This syndrome can be motion-triggered (MT-MdDS), such as on a boat, or occur spontaneously or have other triggers (SO-MdDS) in the absence of such motion. Because the pathophysiological mechanism is unknown, treatment options and symptom management strategies are limited. One available treatment protocol involves a readaptation of the vestibular ocular reflex (VOR). This study assesses the effectiveness of vestibulo-ocular reflex (VOR) readaptation in 131 consecutive patients with a fixed protocol. Methods: We administered 131 treatments involving optokinetic stimulation (OKS) paired with a fixed head roll at 0.167 Hz over two to five consecutive days. Each day, four-minute treatment blocks were scheduled twice in the morning and afternoon. Treatment effectiveness was evaluated through questionnaires and posturography. Results: We observed significant improvements in the visual analog scale (VAS), MdDS symptom questionnaire, and posturography measures from pre- to post-treatment. No significant differences were found in outcome variables between MT- and SO-MdDS onsets. Conclusion: Symptoms improved subjectively and objectively in patients' post-treatment. The overall success rate was 64.1%, with no significant difference between MT (64.2%) and SO (63.3%). This study supports the conclusion that VOR readaptation treatment provides relief for two-thirds of MdDS patients, irrespective of the onset type. Based on consistency in the findings, we propose a standardized method for treatment of MdDS based on the OKS with head roll paradigm.

Surgical Treatment of Acquired Atresia of the External Auditory Ear Canal

Abstract Introduction Acquired atresia of the external auditory canal is characterized by the formation of fibrous tissue in themedial part. The causes include chronic otitis externa, perforated chronic otitis media, postoperative or idiopathic healing problems. Acquired atresia presents with hearing loss and can be associated with otorrhea. Objective We analyzed the results of surgery after six months and two years by checking (1) pre- and postoperative hearing thresholds; (2) presence of otorrhea; and (3) whether a dry and patent ear canal is achieved. Methods We conducted this retrospective study at a tertiary referral center. In total, 27 ears underwent treatment with resection of the fibrotic plug followed by transplantation of a split-thickness skin graft covering the bare bone and tympanic membrane. When necessary, we combined this with a myringoplasty and a (meato-) canalplasty. Results Otorrhea was present in 59.3% of the patients initially and in 14.8% at six months and 11% at two years postoperative. A dry and patent ear canal was obtained in 55.6% after six months and in 89% of the patients after two years (n = 27). The pure tone average before surgery was 39.1 dBHL (SD = 20 dBHL), at six months 31.4 dBHL (SD = 16.4 dBHL), and at 24 months postop 30.9 dBHL (SD = 17.1 dBHL). We observed a statistically significant improvement of hearing in 63% of the patients at six months (p = 0.005) and in 65% after two years (p = 0.022). Conclusions Treatment of acquired atresia remains a challenge. Using the appropriate surgical technique, including skin-grafting and regular postoperative check-up, rendered excellent results regarding otorrhea and a moderate improvement of hearing was achieved in 65% of the patients after two years.