Evaluation of cholesteatoma depends on clinical history and examination, with microscope and/or endoscope. A history of hearing loss with a chronic draining ear, refractory to ototopical medication, raises suspicion for cholesteatoma. Symptoms of Eustachian tube dysfunction or prior ear surgery including ear tubes should be elicited. Inflammation can be severe and should be suppressed if possible. Once cholesteatoma is diagnosed or strongly suspected, further workup includes audiometry prior to surgical excision. Imaging may supplement the workup and is especially helpful if there are concerning features including vertigo, third window symptoms, asymmetric bone line, facial nerve weakness, or for anticipatory guidance.

When surgically managing cholesteatoma, the Bondy modified radical mastoidectomy (MRM) and retrograde mastoidectomy can enhance visualization while minimizing the extent of surgery required for disease removal. The Bondy MRM can be used for disease limited to the atticoantral space, when the ossicles and middle ear space are otherwise healthy, and for canal cholesteatomas. The retrograde mastoidectomy offers a safe alternative for patients with unfavorable tegmen and sigmoid sinus positions and poorly pneumatized mastoids. Both approaches achieve favorable recidivism rates and postoperative hearing outcomes. This article serves as a reference regarding indications, techniques, and complications for these approaches.

Petrous bone cholesteatoma, or cholesteatoma that extends beyond the middle ear and mastoid, represents a rare but destructive pathology. Diagnosis can be difficult before substantial morbidity is incurred, and patients can present with life-threatening complications. Determination of disease extent and the functional status of the facial nerve and cochleovestibular system are critical in surgical planning. Typically, surgery involves ablative procedures with the goal of complete disease resection given the low likelihood of preserved inner ear function. In experienced hands, disease control and facial nerve outcomes are favorable; however, disease recidivism is not uncommon and, thus, these patients require lifelong surveillance.

Congenital cholesteatoma is a cyst of keratinizing squamous cell epithelium in the setting of an intact tympanic membrane, in a patient without a history of otorrhea, tympanic membrane perforation, or otologic surgery. The most common presentation of a congenital cholesteatoma is that of an asymptomatic pearly white mass in the anterosuperior quadrant of the tympanic cavity. The etiology of congenital cholesteatoma has been debated at length, with the leading theory being the epithelial rest theory. Treatment for congenital cholesteatoma is surgical, with advances in endoscopic ear surgery allowing for improved intraoperative visualization and postoperative lowered recidivism rates.

BACKGROUND: Middle ear active implants, such as the Vibrant Soundbridge (VSB), offer an alternative to reconstructive surgery and other implantable hearing aid systems for patients with conductive, mixed, or sensorineural hearing loss. The primary objective of this work is to describe the auditory results obtained with VSB in our patient cohort, measuring the auditory gain in terms of average tonal thresholds and spoken word discrimination at 65 dB. Secondly, auditory gain differences between different types of hearing loss, coupling to the ossicular chain compared to round and oval windows, and the impact of open versus more conservative surgical approaches, were analyzed.
METHODS: A cross-sectional observational study, with retrospective data collection, was conducted at a tertiary care center. Clinical and audiometric data pre- and post-implantation were included, from patients who underwent VSB device placement surgery between 2001 and 2024.
RESULTS: 55 patients with an average age of 62.58 ± 17.83 years and a slight preference in terms of the female gender (52.72%) were included in the study. The average gain in the PTA for all types of hearing loss was 41.56 ± 22.63 dB, while for sensorineural hearing loss (SNHL) the gain was 31.04 ± 8.80 dB. For mixed-conductive hearing loss (C-MHL) a gain of 42.96 ± 17.70 was achieved, notably, in terms of absolute values, at frequencies of 4000 and 6000 Hz, with gains reaching 49.25 ± 20.26 dB at 4 K and 51.16 ± 17.48 dB at 6 K. In terms of spoken word discrimination, for all types of hearing loss, an improvement of 75.20 ± 10.11% was achieved. However, patients with C-MHL exhibited an approximately 13% higher gain compared to those with SNHL (69.32 ± 24.58% vs. 57.79 ± 15.28%). No significant differences in auditory gain were found between open and closed surgical techniques, nor in the proportion of adverse effects, when comparing one technique with the other.
CONCLUSIONS: The VSB is effective in improving hearing in patients with mixed, conductive, and sensorineural hearing loss, with significant gains at high frequencies, especially through the round window membrane approach. The choice of surgical technique should consider the patient\'s anatomical characteristics and specific needs in order to optimize auditory outcomes and minimize postoperative complications.

OBJECTIVE: The purpose of this study was to explore the characteristics of external auditory canal cholesteatoma (EACC) among children and to describe its radiological findings on high-resolution computed tomography (CT) of the temporal bone in order to improve the diagnostic accuracy of primary EACC.
METHODS: The clinical records and CT imaging features of 44 patients who were diagnosed with EACC between January 2017 and May 2022 at Shenzhen Children\'s Hospital were retrospectively reviewed. Clinical features, including external auditory canal wall findings, hearing damage, symptoms and physical examination findings, were analysed against the level of lesion involvement. The correlation between different types of EACC and the incidence of different clinical symptoms was analysed, and the degree of hearing impairment and the rate of bone wall destruction were examined using CT.
RESULTS: The mean age at EACC onset was 9.02 ± 3.15 years, and the mean age at onset for EACC involving the right ear was older than that of EACC involving the left ear (P < 0.05). There were 44 patients (46 ears), including 10 ears with type I EACC, 23 ears with type II EACC, and 13 ears with type III EACC. Conductive hearing loss was the main type of hearing impairment observed among EACC patients. There were differences in types I, II and III EACC in terms of hearing impairment; specifically, there was a significant difference in moderate hearing impairment between type II and type III EACC patients (P < 0.05). The four most common symptoms were otorrhea, otalgia, itching and bleeding. The incidence of itching symptoms was greater in type I EACC than the incidence of otorrhea, and the incidence of otorrhea symptoms in type II and type III EACC was significantly greater than that in type I EACC(P < 0.05). There were no significant differences in the fracture rates of the anterior, posterior, superior or inferior walls of the external auditory canal within or between type II and type III EACC patients (P > 0.05). The failure rate of scute damage was significantly higher in type III EACC patients than in type II EACC patients (P < 0.05).
CONCLUSIONS: The presence of otorrhea and hearing loss as well as the identification of granulation tissue during otoscopy suggest the need for a temporal bone CT scan. This imaging modality can aid in the early detection and accurate classification of EACC, thereby guiding the selection of appropriate surgical interventions and greatly assisting in preventing further progression of hearing impairment.

Pediatric cholesteatomas (PC) have multifactorial aetiology, spread aggressively and there are high chances of residual/ recurrent disease after treatment. The surgical technique to manage this entity has been debatable. This study was done to (i) enumerate the presentation of PC and the surgical techniques adopted (ii) analyse the outcomes viz., residual/ recurrence rates and hearing results. A cross sectional record based study was done on 618 cases of PC operated between 1983 and 2015, at a centre dedicated to otology and lateral skull base surgery. The data which was maintained on the basis of clinical and peri- operative findings was analysed. Otorrhea (59.2%) and hearing loss (54.2%) were the common symptoms. The surgeries done were: canal wall up (CWU) (44.3%), canal wall down (CWD) (41.1%), modified bondy\'s mastoidectomy (5.7%), radical mastoidectomy (4.9%), trans canal excision (1.8%) and subtotal petrosectomy (2.3%). The residual and recurrence rates were 12.6% and 7.9% respectively. A significant difference between the pre and post operative hearing was observed. The mean improvement in air bone gap was 7.7db. Residual/ recurrent disease were higher in CWU as compared to CWD group. The surgery should be individualised so that the patient remains disease free.

UNASSIGNED: Cholesteatoma usually harbors a poly-microbial infection. As the diversity of bacterial pathogens in the Indian COM is unknown, we set out to identify the bacteria associated with cholesteatoma disease in different patients of North India using targeted metagenomic analysis of the 16 S rRNA gene.
UNASSIGNED: We recruited 15 patients of cholesteatomatous chronic otitis media (COM), who underwent surgical disease clearance. We divided these patients into four groups based on the four clinic-radiological stages categorized as per the EAONO/JOS joint consensus statement classification. Representative samples were extracted during the surgery and sent for bacterial culture and sensitivity and 16 S rRNA gene metagenomic analysis.
UNASSIGNED: While 12 (80%) of the patients belonged to clinical Stage I/II; one patient had an extracranial complication (stage III) and two patients had an intracranial complication (stage IV). Our detailed bacterial metagenomics analyses showed that while phylum Proteobacteria was most abundant (reads up to ∼ 95%) in specimens from nine patients, phylum Firmicutes was most abundant (up to ∼ 80%) in specimens from four patients. Gamma (γ) Proteobacteria and Epsilon (ε) Proteobacteria were the most abundant class amongst Proteobacteria. Class Tissierellia stood out as the most abundant Firmicutes (40-60%), followed by Clostridia (20%) and Bacilli (10%). There was negligible difference in the bacterial profiles across all four clinical stages.
UNASSIGNED: Cholesteatoma is primarily associated with Proteobacteria and Firmicutes phyla, even in complicated disease. Further studies with a larger sample size are required to validate our findings.
UNASSIGNED: The online version contains supplementary material available at 10.1007/s12070-024-04678-9.

Sinus tympani is one of the hidden space in the retrotympanic area of middle ear which is commonly invaded by cholesteatoma and known for recidivism. Conventional surgical approaches sometimes fail to access deep sinuses and eradicate the disease. The aim of our study was to measure endoscopic depth of Sinus tympani in Indian Population. Total of 30 patients underwent mastoid exploration and intraoperatively depth of Sinus Tympani was measured using a 0.5 mm calibrated probe with 30 degree rigid Hopkins endoscope. Endoscopic depth in the present study ranges between 2.5 and 5.5 mm. It was higher in right ear in case of females however no significant difference were seen in males and females on left side. It is difficult to clear disease from Sinus Tympani because of its inaccessible location. Sinus Tympani can also show variations in terms of its depth further making difficult to clear disease.

BACKGROUND: Temporal bone computed tomography (CT) helps diagnose chronic otitis media (COM). However, its interpretation requires training and expertise. Artificial intelligence (AI) can help clinicians evaluate COM through CT scans, but existing models lack transparency and may not fully leverage multidimensional diagnostic information.
OBJECTIVE: We aimed to develop an explainable AI system based on 3D convolutional neural networks (CNNs) for automatic CT-based evaluation of COM.
METHODS: Temporal bone CT scans were retrospectively obtained from patients operated for COM between December 2015 and July 2021 at 2 independent institutes. A region of interest encompassing the middle ear was automatically segmented, and 3D CNNs were subsequently trained to identify pathological ears and cholesteatoma. An ablation study was performed to refine model architecture. Benchmark tests were conducted against a baseline 2D model and 7 clinical experts. Model performance was measured through cross-validation and external validation. Heat maps, generated using Gradient-Weighted Class Activation Mapping, were used to highlight critical decision-making regions. Finally, the AI system was assessed with a prospective cohort to aid clinicians in preoperative COM assessment.
RESULTS: Internal and external data sets contained 1661 and 108 patients (3153 and 211 eligible ears), respectively. The 3D model exhibited decent performance with mean areas under the receiver operating characteristic curves of 0.96 (SD 0.01) and 0.93 (SD 0.01), and mean accuracies of 0.878 (SD 0.017) and 0.843 (SD 0.015), respectively, for detecting pathological ears on the 2 data sets. Similar outcomes were observed for cholesteatoma identification (mean area under the receiver operating characteristic curve 0.85, SD 0.03 and 0.83, SD 0.05; mean accuracies 0.783, SD 0.04 and 0.813, SD 0.033, respectively). The proposed 3D model achieved a commendable balance between performance and network size relative to alternative models. It significantly outperformed the 2D approach in detecting COM (P≤.05) and exhibited a substantial gain in identifying cholesteatoma (P<.001). The model also demonstrated superior diagnostic capabilities over resident fellows and the attending otologist (P<.05), rivaling all senior clinicians in both tasks. The generated heat maps properly highlighted the middle ear and mastoid regions, aligning with human knowledge in interpreting temporal bone CT. The resulting AI system achieved an accuracy of 81.8% in generating preoperative diagnoses for 121 patients and contributed to clinical decision-making in 90.1% cases.
CONCLUSIONS: We present a 3D CNN model trained to detect pathological changes and identify cholesteatoma via temporal bone CT scans. In both tasks, this model significantly outperforms the baseline 2D approach, achieving levels comparable with or surpassing those of human experts. The model also exhibits decent generalizability and enhanced comprehensibility. This AI system facilitates automatic COM assessment and shows promising viability in real-world clinical settings. These findings underscore AI\'s potential as a valuable aid for clinicians in COM evaluation.
BACKGROUND: Chinese Clinical Trial Registry ChiCTR2000036300; https://www.chictr.org.cn/showprojEN.html?proj=58685.