The Merits of Bilateral Application of Middle Ear Implants in Patients With Bilateral Conductive and/or Mixed Hearing Loss.

This study investigated sound localization abilities in patients with bilateral conductive and/or mixed hearing loss (BCHL) when listening with either one or two middle ear implants (MEIs). Sound localization was measured by asking patients to point as quickly and accurately as possible with a head-mounted LED in the perceived sound direction. Loudspeakers, positioned around the listener within a range of +73°/-73° in the horizontal plane, were not visible to the patients. Broadband (500 Hz-20 kHz) noise bursts (150 ms), roved over a 20-dB range in 10 dB steps was presented. MEIs stimulate the ipsilateral cochlea only and therefore the localization response was not affected by crosstalk. Sound localization was better with bilateral MEIs compared with the unilateral left and unilateral right conditions. Good sound localization performance was found in the bilaterally aided hearing condition in four patients. In two patients, localization abilities equaled normal hearing performance. Interestingly, in the unaided condition, when both devices were turned off, subjects could still localize the stimuli presented at the highest sound level. Comparison with data of patients implanted bilaterally with bone-conduction devices, demonstrated that localization abilities with MEIs were superior. The measurements demonstrate that patients with BCHL, using remnant binaural cues in the unaided condition, are able to process binaural cues when listening with bilateral MEIs. We conclude that implantation with two MEIs, each stimulating only the ipsilateral cochlea, without crosstalk to the contralateral cochlea, can result in good sound localization abilities, and that this topic needs further investigation.

Taste Disorders After Middle Ear Surgery: Chorda Tympani Nerve Injury and Quality of Life.

OBJECTIVE: To evaluate taste disorders after middle ear surgery, their modifying factors especially chorda tympani nerve injury or underlying otologic disease. We investigated consequences of taste disorders on quality of life. STUDY DESIGN: Prospective cohort study. SETTING: Monocentric study in tertiary care center. METHODS: A total of 214 patients who underwent middle ear surgery were included. Data regarding taste disorders were collected by questionnaires over a 1-year follow-up period. RESULTS: Taste disorders were reported in 42.7% at 10 days, in 23.3% at 4 months, and in 9.2% 1 year postoperatively. When the chorda tympani nerve was initially healthy, taste disorders were more frequent after its transection throughout the follow-up period. When it was involved in a cholesteatoma or inflammatory process, postoperative taste disorders were more frequent after nerve stretching. Postoperative discomfort in daily life was rated on the Likert scale at 3.5 out of 10. Dietary modifications were reported by 25.8% of patients, and mood alterations by 15% of patients. CONCLUSION: Taste disorders are frequent after middle ear surgery although they mostly improve in the first months. When the CTN is healthy, cutting it leads to more taste disorders than stretching it, thus advocating its preservation to prevent these symptoms. However, in cases of pathological CTN, cutting this nerve, which is sometimes necessary to control the disease, is less likely to cause taste disorders than stretching it. These taste disorders are a source of discomfort and may present risks of dietary modifications and emotional impact.

Applicability of ChOLE staging to preoperative HRCT temporal bone in chronic otitis media with cholesteatoma.

PURPOSE: Chronic otitis media with cholesteatoma is a frequent disease entity in otology, requiring surgery in overwhelming majority of cases. Despite the huge burden there is no established grading system available to assess the severity and extent of disease preoperatively until date. Aim of our study is to assess the applicability of ChOLE staging to preoperative HRCT temporal bone in Chronic otitis media with cholesteatoma. METHODOLOGY: Patients clinically diagnosed as COM with cholesteatoma, who underwent preoperative HRCT temporal bone imaging and mastoid exploration at our tertiary care centre were included. Preoperative radiology was assessed and a radiological ChOLE (r-ChOLE) was given by radiologist. This was then compared with the postop ChOLE. RESULTS: 21 patients were included in the study. Data was linear and normally distributed (Shapiro wilk test). Pearson's product-moment correlation used to see relationship between radiological and postop Total score showed strong statistically significant positive correlation with correlation coefficient (r) of 0.977. Paired t test showed p value was 0.329 (p > 0.05) suggesting no significant difference between radiological and postop Total scores. Cohen kappa test of agreement was applied. It revealed an overall strong agreement (p < 0.001). CONCLUSION: ChOLE staging may be extended to preoperative HRCT of temporal bone in COM with cholesteatoma (rCHOLE). A preoperative radiological staging will help in better prioritizing, planning and execution of tympanomastoid surgeries.

A Rare Case of Abducens Nerve Palsy Caused by Primary Squamous Cell Carcinoma of the Middle Ear.

Abducens nerve palsy is the most common ocular motor nerve palsy, and its possible aetiologies are numerous and diverse. Primary malignancy rarely occurs in the middle ear, with most cases associated with long-standing ear discharge and peak age of presentation in the sixties. We report a rare case of a 64-year-old male who presented with right abducens nerve palsy, which led to the diagnosis of primary squamous cell carcinoma of the right middle ear, and to our knowledge, this has not been reported previously in English literature.

Endoscopic ear surgery in the treatment of chronic otitis media with atelectasis.

PURPOSE: Atelectasis otitis media (AtOM) is a chronic condition where the tympanic membrane (TM) becomes retracted towards the middle ear and the ossicular chain. Surgical treatment for this condition could be indicated based on stage of atelectasis, patient's clinical condition and hearing loss. Over the years, AtOM has been treated with various types of tympanoplasty under microscopic view. The aim of this study is to present the results of endoscopic ear surgery in AtOM. METHODS: Forty-five patients who underwent endoscopic trans-canal tympanoplasty were included in the study. Preoperative features, intraoperative findings and postoperative outcomes were collected. RESULTS: Preoperatively, none of the study's patients were classified with a Sadè Grade I, whereas grades II, III and IV were 3 (6.6%), 23 (32.1%) and 19 (67.8%) respectively. The 3 patients with Sadè grade II showed a conductive hearing loss higher than 20 dB and a continuous ear fullness, therefore they were surgically treated. The postoperative graft success rate was estimated at 95.5%. During follow-up, 2 patients showed a TM perforation (at 6 and 12 months after surgery) whereas 1 patient experienced a recurrence of atelectasis in the TM (16 months after surgery). The overall success rate at the final follow-up was calculated at 88.8%. The average preoperative air-conduction threshold was 51.1 ± 21.5, which reduced to 34.6 ± 22.1 (p = 0.04) at follow-up. The preoperative air-bone gap decreased from 28 ± 7.2 to 11.8 ± 10 (p = 0.002) after surgery. CONCLUSION: Atelectasis otitis media might be suitable for exclusive endoscopic surgical treatment, as it appears to exhibit a low recurrence rate and promising audiological outcomes.

To Study the Pre Operative Indicators of Incudal Necrosis in Tubo-Tympanic Chronic Otitis Media.

To assess the pre-operative indicators of incudal necrosis in tubo-tympanic CSOM. A total of 80 patients with tubo-tympanic type of CSOM attending the SMGS Hospital ENT OPD/IPD of both genders were enrolled for the study. Pre-operative findings on oto-microscopic examination, pure-tone audiometry, x-ray mastoid and intra-operative microscopy was recorded. It was found that patients with age > 30 years and more than 10 years of CSOM had had higher incidence of ossicular necrosis as compared to patients age < 30 years and less than 10 years of CSOM (P < 0.05). Patients who had granulation tissue and moderate to moderately severe hearing loss had higher incidence of ossicular necrosis as compared to other patients. The difference was seen significant (P < 0.05). In this study, it was observed that the presence of granulations over the perforation margins, disease persisting for more than 10 years, moderate to moderately severe hearing loss appear to be significant reliable indicators of incudal necrosis in tubo-tympanic type of CSOM. All patients of tubo-tympanic type of CSOM should be assessed in detail so as to reach early diagnosis of ossicular erosion that helps in surgical decision making and preparedness regarding ossiculoplasty and patient consent.

[The effect of different degrees of Eustachian tube dysfunction on hearing threshold in patients with acquired primary middle ear cholesteatoma after balloon eustachian tuboplasty].

Objective:To investigate the changes in hearing threshold of the acquired primary cholesteatoma of the middle ear with different degrees of eustachian tube dysfunction after balloon eustachian tuboplasty. Methods:This retrospective study included forty cases with middle ear cholesteatoma and eustachian tube dysfunction who underwent open mastoidectomy + tympanoplasty + balloon eustachian tuboplasty were enrolled. All patients were admitted from November 2020 to April 2022. The preoperative eustachian tube score of 0-2 were defined as the lower group, and the scores of 3-5 were defined as the higher group. Pure tone audiometry was measured preoperatively and 1, 3, 6 and 12 months postoperatively. The average value of bone conduction threshold and air conduction threshold of 250-4 000 Hz were calculated, and the air-bone gap was calculated simultaneously. SPSS 25.0 was used for statistical analysis. P<0.05 was considered statistically significant. Results:In the lower group, the air conduction threshold and air-bone gap at 3 months postoperatively were significantly decreased in comparison with those preoperatively（P<0.05），as was the air-bone gap at 6 months postoperatively（P<0.05）. In the higher group, the air conduction threshold and air-bone gap were significantly decreased at 3, 6 and 12 months postoperatively（P<0.05）. Conclusion:The air conduction threshold and air-bone gap of patients with the acquired primary cholesteatoma of the middle ear and eustachian tube dysfunction were significantly decreased after eustachian tube balloon dilatation. Hearing improvement lasted longer in patients with slight eustachian tube dysfunction.

[Therapeutic effect of ß-tricalcium phosphate in mastoid cavity obliterationunder endoscope].

Objective:To investigate the therapeutic effect of ß-tricalcium phosphate in mastoid cavity obliteration for middle ear cholesteatoma under endoscope. Methods:Sixty patients with middle ear cholesteatoma admitted to our department from September 2021 to March 2022 were included in this study. The observation group（n=30） received ß-tricalcium phosphate during mastoid cavity obliteration. The control group（n=30） received autologous tissue during mastoid cavity obliteration. Pure tone audiometry was performed before surgery and after surgery in both groups, and the air conduction thresholds of 500, 1 000, 2 000 and 4 000 Hz were recorded. The external acoustic meatus cross-sectional area within 1 cm of the external acoustic meatus opening was measured during the operation and after the operation. The differences of postoperative ear drying time, hearing change and mastoid cavity healing were compared between the two groups. Results:The duration of postoperative dry ear in the observation group was 2-14 weeks, with an average of （9.4±2.7） weeks, while that in the control group was 4-26 weeks, with an average of（16.0±5.7） weeks. The difference in dry ear time between the two groups was statistically significant（P<0.05）. In the observation group, the threshold change was -19-27 dB, with an average of（6.4±10.7） dB, and in the control group, the threshold change was -9-17 dB, with an average of （4.7±7.1） dB. There was no significant difference in hearing change between the two groups（P>0.05）. In the observation group, the cross-sectional area of 1 cm inside the ear canal opening was -5.9-8.2 mm², with an average of （-0.6±2.6） mm², and in the control group, the cross-sectional area of 1 cm inside the ear canal opening was -5.5-5.2 mm², with an average of （-0.4±2.3） mm². There was no significant difference in intraoperative cavity changes between the two groups（P>0.05）. Conclusion:The application of ß-tricalcium phosphate to fill the mastoid cavity during the operation of endoscopic middle ear cholesteatoma has no adverse effect on the hearing of patients, can shorten the postoperative dry ear time, and results in good postoperative healing, which is worth promoting.

Goodhill syndrome: a case report.

Conductive hearing loss with a normal tympanic membrane is a common reason for otolaryngology consultation, with otospongiosis being the most frequent cause and House syndrome being extremely rare, requiring systematic investigation. We report the case of a 31-year-old woman who presented with conductive hearing loss with a normal tympanic membrane. A temporal bone computed tomography (CT) scan confirmed a House-Goodhill syndrome due to fixation of the malleus head. Surgical intervention was considered to remove the attic bone synostosis with the malleus head, resulting in a significant clinical improvement. The Goodhill syndrome is a rare condition that causes hearing loss with a normal eardrum. The surgery can highly improve the hearing function.

Surgical management of chondrosarcomas of the skull-base and temporal bone.

OBJECTIVES: To analyze the overall long-term outcome of surgically treated skull base and temporal bone chondrosarcomas. METHODS: The medical records of patients with surgically treated skull base and temporal bone chondrosarcomas between 1983 and 2024 were thoroughly evaluated. RESULTS: Out of a total of over 5000 skull base surgeries performed at our center, only 29 patients had histopathologically confirmed chondrosarcomas of the skull base and temporal bone. The mean of patients age was 45.6, and the male-to-female ratio was 1.9:1. The most common symptoms included hearing loss (58.6%), tinnitus (41.4%), diplopia (31%), dysphonia (24.1%), dysphagia (20.7%), vertigo (10.3%), and dizziness (10.3%). The most frequent locations of lesions among the 29 patients are as follows: petroclival region (34.5%), jugular foramen (27.6%), petrous apex (17.2%), middle ear (13.8%), others (3.4%). TO, IFTA, IFTB, IFTC, POTS, and combined surgical approaches were commonly used. The rate of gross total removal and recurrence was 82.6% and 13.8% respectively. The follow-up duration of 6 patients was more than five years and less than ten years whereas ten patients had more than ten years of follow-up. CONCLUSIONS: Chondrosarcoma of the skull base and temporal bone is a very rare pathology. Due to its multiple potential sites of origin and histological specificity, it presents us with significant challenges. Gross total removal is the primary treatment for chondrosarcoma of the skull base and temporal bone. Personalized decision-making should be considered based on the following aspects: tumor, patient, and surgeon's factors. Postoperative radiation therapy is complementary to surgical treatment in grades II and III lesions to achieve long-term survival.

Stochastic modeling of the human middle ear dynamics under pathological conditions.

BACKGROUND: Although the dynamics of the middle ear (ME) have been modeled since the mid-twentieth century, only recently stochastic approaches started to be applied. In this study, a stochastic model of the ME was utilized to predict the ME dynamics under both healthy and pathological conditions. METHODS: The deterministic ME model is based on a lumped-parameter representation, while the stochastic model was developed using a probabilistic non-parametric approach that randomizes the deterministic model. Subsequently, the ME model was modified to represent the ME under pathological conditions. Furthermore, the simulated data was used to develop a classifier model of the ME condition based on a machine learning algorithm. RESULTS: The ME model under healthy conditions exhibited good agreement with statistical experimental results. The ranges of probabilities from models under pathological conditions were qualitatively compared to individual experimental data, revealing similarities. Moreover, the classifier model presented promising results. DISCUSSION: The results aimed to elucidate how the ME dynamics, under different conditions, can overlap across various frequency ranges. Despite the promising results, improvements in the stochastic and classifier models are necessary. Nevertheless, this study serves as a starting point that can yield valuable tools for researchers and clinicians.

N6-methyladenosine methylation analysis of circRNAs in acquired middle ear cholesteatoma.

Introduction: Middle ear cholesteatoma is a chronic middle ear disease characterized by severe hearing loss and adjacent bone erosion, resulting in numerous complications. This study sought to identify pathways involved in N6-methyladenosine (m6A) modification of circRNA in middle ear cholesteatoma. Methods: A m6A circRNA epitranscriptomic microarray analysis was performed in middle ear cholesteatoma tissues (n = 5) and normal post-auricular skin samples (n = 5). Bioinformatics analyses subsequently explored the biological functions (Gene Ontology, GO) and signaling pathways (Kyoto Encyclopedia of Genes and Genomes, KEGG) underlying middle ear cholesteatoma pathogenesis. Methylated RNA immunoprecipitation qPCR (MeRIP-qPCR) was performed to verify the presence of circRNAs with m6A modifications in middle ear cholesteatoma and normal skin samples. Results: Microarray analysis identified 3,755 circRNAs as significantly differentially modified by m6A methylation in middle ear cholesteatoma compared with the normal post-auricular skin. Among these, 3,742 were hypermethylated (FC ≥ 2, FDR < 0.05) and 13 were hypomethylated (FC ≤ 1/2, FDR < 0.05). GO analysis terms with the highest enrichment score were localization, cytoplasm, and ATP-dependent activity for biological processes, cellular components, and molecular functions respectively. Of the eight hypermethylated circRNA pathways, RNA degradation pathway has the highest enrichment score. Peroxisome Proliferator-Activated Receptor (PPAR) signaling pathway was hypomethylated. To validate the microarray analysis, we conducted MeRIP-qPCR to assess the methylation levels of five specific m6A-modified circRNAs: hsa_circRNA_061554, hsa_circRNA_001454, hsa_circRNA_031526, hsa_circRNA_100833, and hsa_circRNA_022382. The validation was highly consistent with the findings from the microarray analysis. Conclusion: Our study firstly presents m6A modification patterns of circRNAs in middle ear cholesteatoma. This finding suggests a direction for circRNA m6A modification research in the etiology of cholesteatoma and provides potential therapeutic targets for the treatment of middle ear cholesteatoma.

Safety and efficacy of the mAXIS stapes prosthesis.

PURPOSE: Otosclerosis leads to a fixed stapes footplate and thus to hearing loss. The predominant treatment method is surgery, with various types of stapes prostheses available. The aim of this study was to investigate the safety and efficacy of the new mAXIS Stapes Prosthesis. METHODS: 34 cases of otosclerosis were implanted with the new mAXIS Stapes Prosthesis. Comprehensive clinical assessments, including pre- and postoperative pure tone audiometry was performed at short-term (ST) follow-up at 25 (± 15) days and mid-term (MT) follow-up at 181 (± 107) days. The pure tone average of 0.5, 1, 2 and 3 kHz (PTA4) was calculated. RESULTS: In all cases, the application of the prosthesis was successful and straightforward. The postoperative PTA4 air-bone gap was 10.7 ± 5.2 dB at ST follow-up (n = 34) and 8.3 ± 4.1 dB at MT follow-up (n = 18). In 61% of cases, the ABG-closure was within 10 dB and in 100% of cases within 20 dB at MT follow-up. CONCLUSION: Findings of this study support that the mAXIS Stapes Prosthesis is safe for implantation and shows promising audiological outcome. Future investigations will contribute its long-term efficacy and safety profile.

Assessing the Efficacy of Tragal Pumping in a Novel Tympanostomy Tube-Rat Model.

Objective: Tragal pumping (TP) is a practice of pushing on the tragus to raise pressure within the external auditory canal and is a commonly recommended adjunctive maneuver believed to facilitate the introduction of ototopical medications into the middle ear cavity via a tympanostomy tube. To investigate the efficacy of TP in the penetration of eardrops into the middle ear cavity via tympanostomy tube, we established the novel tympanostomy tube-rat model. We investigated the histology of the middle ear to determine the efficacy in moving fluid into the middle ear. Study Design: Prospective controlled animal study. Setting: Animal laboratory in a university hospital. Methods: Ten rats were recruited, and a tympanostomy tube insertion and green dye eardrops into outer ears were performed on bilateral ears. TP was performed only on 1 ear and was not applied on the other ear in each rat. Green dye in a middle ear cavity in hematoxylin and eosin-stained temporal bone sections was evaluated by blinded experts in microscopic anatomy (staining grade) and by using Image J software (staining level). The results of these 2 methods were statistically validated. Results: The staining grade (P < .001) and the staining level (P < .001) were significantly higher in the ears which we applied TP than in the control ears. The results of 2 methods were significantly and positively correlated (r = .898, P < .001). Conclusion: Our results showed that the TP accelerate the penetration of eardrops into the middle ear cavity in the tympanostomy tube-rat model.

Ergonomics of Various Modalities for Ear Surgery.

Objective: Evaluate ergonomic differences of various modalities for performing middle ear surgery. Study Design: Observational study. Setting: Two academic tertiary care centers. Methods: Attending physicians and residents performing middle ear surgery were photographed intraoperatively. Intraoperative photographs were analyzed using the validated Rapid Upper Limb Assessment (RULA) tool to measure musculoskeletal disease (MSD) risk. Descriptive statistics and significance testing were used to characterize and compare ergonomic differences between surgical modalities. Multivariable ordinal regression was performed to assess factors associated with increased MSD risk, as determined by the final RULA score. Results: Most of our 110 intraoperative photos featured attendings (82.7%) performing combined middle ear surgery and mastoidectomy (60.0%). Body angles and the final RULA score varied significantly among modalities. On subset analysis, microscopic surgery exhibited significantly worse wrist, trunk, and neck angles compared to endoscopic and exoscopic surgery. Exoscopic surgery had significantly lower final RULA scores than both endoscopic and microscopic surgery, indicating significantly lower MSD risk. Microscopic and endoscopic surgery final scores did not vary significantly. In a multivariable ordinal regression of factors associated with increased RULA score, exoscopic surgery had statistically significantly less ergonomic risk relative to microscopic surgery (odds ratio = 0.12, 95% confidence interval = [0.03-0.43]). Conclusion: Exoscopic, endoscopic, and microscopic surgery all featured low ergonomic risk, although exoscopic middle ear surgery demonstrated the lowest risk profile among studied surgical modalities. This demonstrates the importance of using each modality in combination with other ergonomic interventions to provide meaningful musculoskeletal benefits.

A Clinicopathological Study of Aural Polyps: A Retrospective Analysis in a Tertiary Care Hospital.

Introduction Aural polyps are a misnomer. Any lesion can present as a mass in the external auditory canal. Aural polyps are proliferation of the granulation tissue due to long standing inflammatory process with associated otalgia and otorrhea. Objectives To document the clinicoradiological presentations, intraoperative findings, and histopathological diagnosis of aural polyp, correlating them. Methods In our study 81 patients underwent treatment for aural polyps in the department of Ear, Nose, and Throat (ENT) from April 1997 to April 2022. Results were tabulated, a simple descriptive analysis was done using the Statistical Package Social Sciences software, and the results obtained were represented as percentages and presented in tables. Results The majority (38) of the patients presenting with aural polyps were diagnosed with mucosal and squamous type of CSOM, and 22 with simple granulation polyps. There were also 5 patients with malignant otitis externa, 3 patients had glomus tumors, 2 patients with retained foreign bodies, and 3 patients with brain herniation. We also identified aberrant internal carotid artery, high jugular bulb, one patient had facial nerve neuroma, one patient had polyp from the tragus diagnosed with tuberculosis, one patient with keratosis obturans, and one with exostosis. Conclusion A thorough detailed examination and mastoid exploration with radiological and histopathological evaluation is mandatory for better defining the definitive treatment. Utmost care and meticulousness are advised for the surgeons while dealing with aural polyps to avoid any complications.

Pediatric Myringoplasty using the Periosteum: An Institutional Overview.

Introduction Myringoplasty is a common otologic procedure to restore the integrity of the tympanic membrane in cases of traumatic or pathologic perforations. Many grafting materials have been used with different techniques. Objective In the present work, we evaluate the surgical and audiological outcomes of periosteal graft overlying the mastoid cortex through a retroauricular incision in a pediatric cohort. Methods A retrospective study was carried out involving all children aged ≤ 16 years who underwent periosteal graft myringoplasty for the treatment of chronic suppurative otitis media with dry central perforation in our hospital from April 2019 to April 2021. All patients were followed up for one year to assess the anatomical success and functional outcomes by comparing the preoperative and postoperative (after six months) results of pure tone audiometry (PTA). Results The sample was composed of 36 patients; 20 of them were female (55.6%) and 16 were male (44.4%) subjects, with ages ranging from 7 to 16 (mean: 12.7) years. Four patients underwent surgery in both ears (with an interval of 6 to 9 months). Out of 40 surgeries performed, 38 ears have shown anatomical success (95%). A highly significant improvement in hearing was obtained (the mean difference between the pre- and postoperative results of the PTA was of 14.6 ± 3.45 dB ( p < 0.001). Conclusion We advocate the use of periosteal graft in the pediatric population as a good alternative for other types of grafts, with comparable and even better functional and anatomical outcomes.

Preliminary results of classifying otosclerosis and disarticulation using a convolutional neural network trained with simulated wideband acoustic immittance data.

Current noninvasive methods of clinical practice often do not identify the causes of conductive hearing loss due to pathologic changes in the middle ear with sufficient certainty. Wideband acoustic immittance (WAI) measurement is noninvasive, inexpensive and objective. It is very sensitive to pathologic changes in the middle ear and therefore promising for diagnosis. However, evaluation of the data is difficult because of large interindividual variations. Machine learning methods like Convolutional neural networks (CNN) which might be able to deal with this overlaying pattern require a large amount of labeled measurement data for training and validation. This is difficult to provide given the low prevalence of many middle-ear pathologies. Therefore, this study proposes an approach in which the WAI training data of the CNN are simulated with a finite-element ear model and the Monte-Carlo method. With this approach, virtual populations of normal, otosclerotic, and disarticulated ears were generated, consistent with the averaged data of measured populations and well representing the qualitative characteristics of individuals. The CNN trained with the virtual data achieved for otosclerosis an AUC of 91.1 %, a sensitivity of 85.7 %, and a specificity of 85.2 %. For disarticulation, an AUC of 99.5 %, sensitivity of 100 %, and specificity of 93.1 % was achieved. Furthermore, it was estimated that specificity could potentially be increased to about 99 % in both pathological cases if stapes reflex threshold measurements were used to confirm the diagnosis. Thus, the procedures' performance is comparable to classifiers from other studies trained with real measurement data, and therefore the procedure offers great potential for the diagnosis of rare pathologies or early-stages pathologies. The clinical potential of these preliminary results remains to be evaluated on more measurement data and additional pathologies.

Middle Ear Fibrosis Contributes to Hearing Loss in Patients With Myhre Syndrome.

Myhre syndrome (MS) is a rare genetic condition that presents with multiple genetic anomalies including cleft lip and palate and Eustachian tube dysfunction. These patients are at a high risk for airway scarring from intubation and mucosal inflammation. Hearing loss (conductive or mixed, of varying severity) is a common comorbidity in these patients, the exact etiology of which is still unclear. We present the cases of 2 unrelated children with MS who suffered progressive mixed hearing loss from fibrosis and obliteration of the middle ear spaces. Both patients had multiple sets of ear tubes that demonstrated early extrusion. The older patient underwent bone conduction implantation at age 11 which resulted in dramatic improvement of speech recognition and interactive skills. The other younger patient demonstrates a similar trajectory but has not yet undergone implantation. Otolaryngologists should take a cautious approach to surgery of the eardrum and middle ear to avoid unnecessary induction of fibrosis in this susceptible patient population. These cases highlight a newly described etiology for hearing loss and suggest a benefit to bone conduction implantation.