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1.
Development ; 148(16)2021 08 15.
Article in English | MEDLINE | ID: mdl-34351428

ABSTRACT

Neocortical progenitor cells generate subtypes of excitatory projection neurons in sequential order followed by the generation of astrocytes. The transcription factor zinc finger and BTB domain-containing protein 20 (ZBTB20) has been implicated in regulation of cell specification during neocortical development. Here, we show that ZBTB20 instructs the generation of a subset of callosal projections neurons in cortical layers II/III in mouse. Conditional deletion of Zbtb20 in cortical progenitors, and to a lesser degree in differentiating neurons, leads to an increase in the number of layer IV neurons at the expense of layer II/III neurons. Astrogliogenesis is also affected in the mutants with an increase in the number of a specific subset of astrocytes expressing GFAP. Astrogliogenesis is more severely disrupted by a ZBTB20 protein containing dominant mutations linked to Primrose syndrome, suggesting that ZBTB20 acts in concert with other ZBTB proteins that were also affected by the dominant-negative protein to instruct astrogliogenesis. Overall, our data suggest that ZBTB20 acts both in progenitors and in postmitotic cells to regulate cell fate specification in the mammalian neocortex.


Subject(s)
Astrocytes/metabolism , Neocortex/growth & development , Neurogenesis/genetics , Neurons/metabolism , Transcription Factors/metabolism , Abnormalities, Multiple/genetics , Animals , Calcinosis/genetics , Ear Diseases/genetics , Female , Gene Knockout Techniques , Intellectual Disability/genetics , Male , Mice , Mice, Inbred C57BL , Mice, Knockout , Muscular Atrophy/genetics , Mutation, Missense , Neocortex/metabolism , Signal Transduction/genetics , Stem Cells/metabolism , Transcription Factors/genetics
2.
Am J Med Genet A ; 194(8): e63610, 2024 08.
Article in English | MEDLINE | ID: mdl-38517161

ABSTRACT

Primrose syndrome (PS) is a rare genetic disease characterized by developmental delay, intellectual disability, sensorineural hearing loss, and dysmorphic features. PS is caused by de novo pathogenic variants in the ZBTB20 gene, which encodes a transcription factor modulating neurogenesis. We describe resolution with sertraline of neurobehavioral difficulties in a 17-year-old Hispanic male with PS with de novo heterozygous c.1916G > A (p.C639Y) variant of ZBTB20. Neurobehavioral difficulties included aggression towards self and others, irritability, tearfulness, and mood liability that did not respond to behavioral interventions or aripiprazole. Treatment with sertraline, a medication indicated for psychiatric disorders including anxiety and depression, led to the resolution of neurobehavioral difficulties after 2 weeks of initiation of medication. The treatment course suggests that selective serotonin reuptake inhibitors, such as sertraline, may be a useful tool for neurobehavioral difficulties in PS over antipsychotics that are accompanied by complex side effect profiles, and suggest that anxiety is the primary cause of the neurobehavioral difficulties in this patient.


Subject(s)
Intellectual Disability , Sertraline , Transcription Factors , Humans , Sertraline/therapeutic use , Male , Adolescent , Intellectual Disability/genetics , Intellectual Disability/drug therapy , Transcription Factors/genetics , Hearing Loss, Sensorineural/genetics , Hearing Loss, Sensorineural/drug therapy , Selective Serotonin Reuptake Inhibitors/therapeutic use , Abnormalities, Multiple/genetics , Abnormalities, Multiple/drug therapy , Abnormalities, Multiple/psychology , Calcinosis , Ear Diseases , Muscular Atrophy , Nerve Tissue Proteins
3.
Ear Hear ; 45(5): 1241-1251, 2024.
Article in English | MEDLINE | ID: mdl-38797886

ABSTRACT

OBJECTIVES: During an initial diagnostic assessment of an ear with normal otoscopic exam, it can be difficult to determine the specific pathology if there is a mechanical lesion. The audiogram can inform of a conductive hearing loss but not the underlying cause. For example, audiograms can be similar between the inner-ear condition superior canal dehiscence (SCD) and the middle-ear lesion stapes fixation (SF), despite differences in pathologies and sites of lesion. To gain mechanical information, wideband tympanometry (WBT) can be easily performed noninvasively. Absorbance , the most common WBT metric, is related to the absorbed sound energy and can provide information about specific mechanical pathologies. However, absorbance measurements are challenging to analyze and interpret. This study develops a prototype classification method to automate diagnostic estimates. Three predictive models are considered: one to identify ears with SCD versus SF, another to identify SCD versus normal, and finally, a three-way classification model to differentiate among SCD, SF, and normal ears. DESIGN: Absorbance was measured in ears with SCD and SF as well as normal ears at both tympanometric peak pressure (TPP) and 0 daPa. Characteristic impedance was estimated by two methods: the conventional method (based on a constant ear-canal area) and the surge method, which estimates ear-canal area acoustically.Classification models using multivariate logistic regression predicted the probability of each condition. To quantify expected performance, the condition with the highest probability was selected as the likely diagnosis. Model features included: absorbance-only, air-bone gap (ABG)-only, and absorbance+ABG. Absorbance was transformed into principal components of absorbance to reduce the dimensionality of the data and avoid collinearity. To minimize overfitting, regularization, controlled by a parameter lambda, was introduced into the regression. Average ABG across multiple frequencies was a single feature.Model performance was optimized by adjusting the number of principal components, the magnitude of lambda, and the frequencies included in the ABG average. Finally, model performances using absorbance at TPP versus 0 daPa, and using the surge method versus constant ear-canal area were compared. To estimate model performance on a population unknown by the model, the regression model was repeatedly trained on 70% of the data and validated on the remaining 30%. Cross-validation with randomized training/validation splits was repeated 1000 times. RESULTS: The model differentiating between SCD and SF based on absorbance-only feature resulted in sensitivities of 77% for SCD and 82% for SF. Combining absorbance+ABG improved sensitivities to 96% and 97%. Differentiating between SCD and normal using absorbance-only provided SCD sensitivity of 40%, which improved to 89% by absorbance+ABG. A three-way model using absorbance-only correctly classified 31% of SCD, 20% of SF and 81% of normal ears. Absorbance+ABG improved sensitivities to 82% for SCD, 97% for SF and 98% for normal. In general, classification performance was better using absorbance at TPP than at 0 daPa. CONCLUSION: The combination of wideband absorbance and ABG as features for a multivariate logistic regression model can provide good diagnostic estimates for mechanical ear pathologies at initial assessment. Such diagnostic automation can enable faster workup and increase efficiency of resources.


Subject(s)
Acoustic Impedance Tests , Hearing Loss, Conductive , Humans , Acoustic Impedance Tests/methods , Hearing Loss, Conductive/diagnosis , Adult , Female , Male , Stapes , Middle Aged , Young Adult , Ear, Middle , Regression Analysis , Semicircular Canals/physiopathology , Ear Diseases/diagnosis
4.
BMC Med Imaging ; 24(1): 102, 2024 May 09.
Article in English | MEDLINE | ID: mdl-38724896

ABSTRACT

Precision and intelligence in evaluating the complexities of middle ear structures are required to diagnose auriculotemporal and ossicle-related diseases within otolaryngology. Due to the complexity of the anatomical details and the varied etiologies of illnesses such as trauma, chronic otitis media, and congenital anomalies, traditional diagnostic procedures may not yield accurate diagnoses. This research intends to enhance the diagnosis of diseases of the auriculotemporal region and ossicles by combining High-Resolution Spiral Computed Tomography (HRSCT) scanning with Deep Learning Techniques (DLT). This study employs a deep learning method, Convolutional Neural Network-UNet (CNN-UNet), to extract sub-pixel information from medical photos. This method equips doctors and researchers with cutting-edge resources, leading to groundbreaking discoveries and better patient healthcare. The research effort is the interaction between the CNN-UNet model and high-resolution Computed Tomography (CT) scans, automating activities including ossicle segmentation, fracture detection, and disruption cause classification, accelerating the diagnostic process and increasing clinical decision-making. The suggested HRSCT-DLT model represents the integration of high-resolution spiral CT scans with the CNN-UNet model, which has been fine-tuned to address the nuances of auriculotemporal and ossicular diseases. This novel combination improves diagnostic efficiency and our overall understanding of these intricate diseases. The results of this study highlight the promise of combining high-resolution CT scanning with the CNN-UNet model in otolaryngology, paving the way for more accurate diagnosis and more individualized treatment plans for patients experiencing auriculotemporal and ossicle-related disruptions.


Subject(s)
Ear Ossicles , Tomography, Spiral Computed , Humans , Tomography, Spiral Computed/methods , Ear Ossicles/diagnostic imaging , Deep Learning , Ear Diseases/diagnostic imaging , Temporal Bone/diagnostic imaging , Adult , Neural Networks, Computer
5.
J Ultrasound Med ; 43(2): 411-414, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37929614

ABSTRACT

Primrose syndrome is a very rare congenital malformation. Symptoms of this disorder may appear during childhood, but the diagnosis is identified in adulthood in the majority of cases. The prenatal diagnosis of Primrose syndrome is not developed in the literature. We present herein a case series of 3 cases with characteristic sonographic features. A dysmorphic metopic suture, downslanting palpebral fissures, a wide forehead, and agenesis of corpus callosum are the main signs. A missense mutation in ZBTB20 identified in whole exome sequencing can confirm the prenatal diagnosis of Primrose syndrome.


Subject(s)
Abnormalities, Multiple , Calcinosis , Ear Diseases , Intellectual Disability , Muscular Atrophy , Pregnancy , Female , Humans , Abnormalities, Multiple/diagnostic imaging , Intellectual Disability/diagnosis , Intellectual Disability/genetics , Prenatal Diagnosis , Agenesis of Corpus Callosum/diagnostic imaging , Agenesis of Corpus Callosum/genetics
6.
Am J Otolaryngol ; 45(5): 104397, 2024.
Article in English | MEDLINE | ID: mdl-39059160

ABSTRACT

PURPOSE: To investigate the treatment time and efficiency of constricted ears of different severity after correction. MATERIALS AND METHODS: We included the patients with constricted ear presented to our hospital for treatment between December 2021 and December 2023 in this retrospective analysis. The patients were divided into class I, II and III groups based on the severity of the constriction. Then we collected the data on classification of severity from each patient, together with sex, family history, age at initial correction, being informed upon diagnosis after birth, as well as utilization of auricle correction system. Logistic regression analysis was performed to identify the factors associated with the treatment time and efficiency. RESULTS: The correction system yielded a high effective rate in the constricted ears. The treatment time in class II was significantly longer compared with those of class I after adjusting these parameters. Compared with the cases of class I, those with a class III showed significant attenuation in the symptoms and conditions (95 % CI: 0.034, 0.365; P < 0.001), after adjusting the age at initial correction, being informed upon diagnosis after birth, and utilization of auricle correction system. There were no statistical differences between class II and III in the treatment efficiency after correction. CONCLUSIONS: The Amazing Ear Correction System was effective in treating constricted ear, yielding satisfactory treatment efficiency. Patients with class II constriction required longer treatment time compared with those of class I. The treatment outcome in the class I constriction was better than that of class III.


Subject(s)
Severity of Illness Index , Humans , Female , Male , Retrospective Studies , Treatment Outcome , Constriction, Pathologic/therapy , Ear Auricle/abnormalities , Ear Auricle/surgery , Child , Child, Preschool , Time Factors , Ear, External/abnormalities , Infant , Ear Diseases/therapy , Ear Diseases/diagnosis
7.
Eur Arch Otorhinolaryngol ; 281(10): 5363-5373, 2024 Oct.
Article in English | MEDLINE | ID: mdl-38977475

ABSTRACT

PURPOSE: This study aimed to assess the efficacy and safety of ballon dilation of the eustachian tube (BDET) in adult population suffering from Eustachian tube dysfunction (ETD). METHODS: Following PRISMA criteria, a systematic review was conducted by searching PubMed, Cochrane, and Embase databases from January 2015 to March 2024. The primary outcomes included Eustachian Tube Score (ETS), tympanometry, and Valsalva maneuver. The quality of studies was assessed using the Quality in Prognostic Studies (QUIPS) instrument. RESULTS: Overall, 11 studies were incorporated into the systematic review: two RCTs, three prospective investigations, and six retrospective studies. The balloon dilation in all investigations was performed using either Spiggle & Theis or Acclarent catheters for balloon dilation. There was heterogeneity across studies examining the effect of BDET on persistent ETD in terms of patient selection, period of follow-up, administration of conservative or surgical therapies, and use of assessment methods. Overall, the treatment yielded alleviation of symptoms, which either exhibited stability over time or demonstrated further improvement after an average duration of follow-up. Moreover, the incidence of complications was categorized as low and resolving spontaneously. The majority of the studies exhibited a high risk of bias related to confounding variables, and consequently, the overall risk of bias across most studies was considered high. CONCLUSION: The findings suggest BDET holds promise for ETD treatment, reducing symptom severity with minimal complications. Nonetheless, there is a need for improved studies that adhere to established indications, methodologies, and outcomes to establish a more robust body of evidence.


Subject(s)
Dilatation , Ear Diseases , Eustachian Tube , Humans , Eustachian Tube/physiopathology , Dilatation/methods , Ear Diseases/therapy , Adult , Treatment Outcome
8.
Eur Arch Otorhinolaryngol ; 281(4): 1761-1771, 2024 Apr.
Article in English | MEDLINE | ID: mdl-37917164

ABSTRACT

PURPOSE: A variety of lesions could arise from the GG area, or extend into this region from adjacent sites. The management of perigeniculate lesions includes observation, surgery, and radiation, according to the nature, the size of the lesion, and the accompanying symptoms. Preliminary experiences on the exclusive transcanal endoscopic approach to the GG area have shown safety and feasibility avoiding of any postauricular incision, or brain manipulation. The experience from two referral centers on patients treated for a GG lesion with a totally endoscopic approach is herein reported. METHODS: Data about patients who underwent exclusive endoscopic approach to the GG area at the Otolaryngology Departments of the University Hospitals of Modena and Bologna between May 2017 and February 2022 were retrospectively collected. RESULTS: The total number of patients included in our study was 11. 10 patients (91%) had progressive unilateral facial paralysis and 1 patient (11%) presented with chronic otorrhea. The mean largest diameter of the treated lesions was of 8 mm. The resection was extended to the fundus of the IAC in 2 patients (expanded approach). The remaining 9 patients (82%) underwent partial ossicular replacement prosthesis (PORP). No major complications occurred. Facial nerve outcomes were good in all patients and the mean ABG worsened from 12 dB pre-operatively to 22 dB post-operatively. CONCLUSIONS: The exclusively endoscopic approach to GG lesions represents a viable alternative to traditional microscopic approaches and may be included in the armamentarium of ear surgeons.


Subject(s)
Ear Diseases , Geniculate Ganglion , Humans , Geniculate Ganglion/surgery , Retrospective Studies , Endoscopy , Facial Nerve , Ear Diseases/surgery , Treatment Outcome
9.
Eur Arch Otorhinolaryngol ; 281(10): 5135-5143, 2024 Oct.
Article in English | MEDLINE | ID: mdl-38809266

ABSTRACT

PURPOSE: This study aims to evaluate the validity and reliability of tubomanometry (TMM) in diagnosing obstructive Eustachian tube dysfunction (OETD) before balloon Eustachian tuboplasty (BET). METHODS: A prospective cohort study was conducted, involving 25 patients with sinonasal pathology, 75 patients with middle ear disease, and 25 healthy subjects, totaling 250 ears. All participants underwent comprehensive physical examinations, including TMM, nasal videoendoscopy, otoendoscopy, Valsalva maneuver (VM), tympanometry, and audiometry. Additionally, various scales such as ETDQ-7 and ETS-7 were employed. Tympanometry served as the gold standard for assessing OETD, and comparisons were made among the measures across the three groups. RESULTS: Among the 125 participants, 44.8% (n = 56) were female, and 55.2% (n = 69) were male, with ages ranging from 19 to 93 years (M = 48.5; SD = 15.6). In our study, VM demonstrated high sensitivity (86.3%) when tympanometry was considered the gold standard. Conversely, TMM, ETDQ-7, and ETS-7 exhibited high specificity, with ETDQ-7 showing the highest specificity (87.4%). Regarding TMM, all R values in patients within the middle ear disease group were pathological (R > 1). CONCLUSION: TMM has exhibited notable specificity as a diagnostic tool compared to tympanogram and VM. Nonetheless, the combination of TMM and ETDQ-7 has enabled us to conduct a diagnostic assessment with high sensitivity and specificity for chronic OETD diagnosis.


Subject(s)
Acoustic Impedance Tests , Ear Diseases , Eustachian Tube , Humans , Eustachian Tube/physiopathology , Female , Male , Middle Aged , Adult , Acoustic Impedance Tests/methods , Aged , Prospective Studies , Ear Diseases/diagnosis , Ear Diseases/physiopathology , Reproducibility of Results , Aged, 80 and over , Young Adult , Case-Control Studies , Sensitivity and Specificity
10.
Eur Arch Otorhinolaryngol ; 281(4): 1693-1700, 2024 Apr.
Article in English | MEDLINE | ID: mdl-37847285

ABSTRACT

PURPOSE: This study retrospectively evaluated the efficacy and versatility of reopening procedures for the permanent occlusion of the cartilaginous Eustachian tube (POET) by analyzing four consecutive cases. METHODS: The study included all patients diagnosed with POET who suffered from Eustachian tube occlusion and glue ear. A combined approach of endoscopic transnasal/transoral laser surgery was utilized to reopen the POET. This was subsequently followed by balloon dilation (BET) and stenting for a duration of six weeks. In one distinct case, the Eustachian tube orifice was approached via a transtympanic method, where a balloon catheter was placed. The primary outcome measures targeted the success rate of reopening, which was quantified using audiological outcomes and Eustachian tube patency verified by a positive Valsalva maneuver. RESULTS: Four patients, with an age range of 14-62 years (mean age of 29.3 years), were subject to Eustachian tube reopening. The duration of follow-up varied between 10 and 24 months, averaging at 16.2 months. Notably, 75% of the surgically treated ears displayed no evidence of glue ear upon their last follow-up and showed restoration of Eustachian tube patency. The procedures were executed without any surgical complications. The causes for POET in these patients were heterogeneous: two were attributed to scarring post adenoidectomy, one to occlusion following orthognathic surgery and the remaining one due to prior radiotherapy treatment for squamous cell carcinoma located at the soft palate. DISCUSSION: Total occlusion of the cartilaginous Eustachian tube may be linked to persistent middle ear diseases. It is imperative to conduct nasopharyngeal endoscopy in these cases. The findings from this study suggest that the Eustachian tube reopening procedure is predominantly effective and safe for patients with POET stemming from a variety of pathologies. Future research should focus on exploring advanced stenting devices and necessitate longer follow-up periods for comprehensive understanding.


Subject(s)
Ear Diseases , Eustachian Tube , Laser Therapy , Otitis Media , Humans , Adult , Adolescent , Young Adult , Middle Aged , Eustachian Tube/surgery , Eustachian Tube/pathology , Retrospective Studies , Ear Diseases/surgery , Otitis Media/surgery , Laser Therapy/methods , Dilatation/methods , Treatment Outcome
11.
Eur Arch Otorhinolaryngol ; 281(6): 2883-2891, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38151540

ABSTRACT

INTRODUCTION: Patulous Eustachian tube (PET) is a condition affecting approximately 0.3% to 6.6% of the population, with autophony being the predominant complain. The management of PET lacks a well-defined standard in the literature as no effective medical treatments have been documented but various surgical options are available. This study aims to report mid-term outcomes following surgical management of PET. MATERIALS AND METHODS: All patients who underwent surgical intervention for PET between September 2017 and June 2022 were enrolled. Data encompassing general demographics, quality of life (GBI), and procedure-specific data were collected. RESULTS: A total of 30 PET cases (in 19 patients) underwent surgical intervention including 9 injections of hyaluronic acid, 13 fat injections, 6 endoscopic shim insertions, 1 cartilage graft, and 1 injection of hydroxy apatite. After an average follow-up of 22 ± 14 months, 16 cases (53%) achieved complete symptom relief, while 8 cases (26.6%) reported partial relief. Additionally, 11(36%) cases required multiple surgeries. No specific surgical technique demonstrated superiority. Quality of life improved in 77% of cases based on 10 out of 13 GBI collected. Recurrence of PET symptoms occurred on average 10.6 ± 9.7 months after initial surgery, with an estimated global risk of 75% at 3 years. Transient serous otitis media was observed in only 4 cases (13.3%). CONCLUSION: Surgical intervention for PET was found to be effective, achieving complete symptom relief in 53% of cases and significantly improving quality of life 2 years post-surgery. However, a substantial portion of cases necessitated one or more re-interventions. The durability of effectiveness appears to diminish over time.


Subject(s)
Ear Diseases , Eustachian Tube , Quality of Life , Humans , Eustachian Tube/surgery , Eustachian Tube/physiopathology , Female , Male , Ear Diseases/surgery , Middle Aged , Adult , Aged , Treatment Outcome , Hyaluronic Acid/administration & dosage , Hyaluronic Acid/therapeutic use , Endoscopy/methods , Otologic Surgical Procedures/methods , Cartilage/transplantation , Durapatite
12.
Eur Arch Otorhinolaryngol ; 281(3): 1259-1265, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37725135

ABSTRACT

PURPOSE: Chronic obstructive Eustachian tube dysfunction (OETD) can lead to tympanic membrane (TM) retraction and middle ear effusion (MEE) which can cause conductive hearing impairment, which among other ear symptoms can lower the quality of life (QoL). In this prospective study we assess hearing results and subjective changes in QoL following balloon Eustachian tuboplasty (BET) in treatment of OETD. METHODS: Totally 25 ears with TM retraction and 18 ears with MEE due to chronic OETD underwent BET as the sole intervention. Outcomes including otoscopy, ability to perform the Valsalva maneuver, tympanometry, audiometry, Eustachian tube inflammation scale and the Glasgow Benefit Inventory questionnaire (GBI) were obtained on all patients preoperatively and 6 months postoperatively. RESULTS: Hearing thresholds improved statistically significantly (p < 0.05) with means of 3 dB in the TM retraction group and 9 dB in the MEE group. Total GBI results indicated a positive influence on patients' QoL. Valsalva success rate was 80% in patients with TM retraction and 67% in patients with MEE. Tympanometry results improved in 50% of TM retraction patients and in 33% of MEE patients. CONCLUSIONS: Here we demonstrated that the BET has a positive impact on patients' conductive hearing loss and QoL in patients with TM retraction or MEE. Results were better in TM retraction group than in MEE group.


Subject(s)
Ear Diseases , Eustachian Tube , Otitis Media with Effusion , Humans , Quality of Life , Eustachian Tube/surgery , Prospective Studies , Dilatation/methods , Ear Diseases/surgery , Otitis Media with Effusion/complications , Otitis Media with Effusion/surgery , Otitis Media with Effusion/diagnosis , Acoustic Impedance Tests , Hearing , Hearing Loss, Conductive/etiology , Hearing Loss, Conductive/surgery , Treatment Outcome
13.
Eur Arch Otorhinolaryngol ; 281(4): 2031-2035, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38367073

ABSTRACT

PURPOSE: Achromobacter xylosoxidans is an emerging pathogen mainly associated with resistant nosocomial infections. This bacteria had been isolated in the ear together with other pathogens in cultures from patients with chronic otitis media, but it had never been reported as a cause of osteomyelitis of the external auditory canal. CASE PRESENTATION: We present a unique case of a healthy 81-year-old woman who presented with left chronic otorrhea refractory to topical and oral antibiotic treatment. Otomicroscopy revealed an erythematous and exudative external auditory canal (EAC) with scant otorrhea. The tympanic membrane was intact, but an area of bone remodeling with a small cavity anterior and inferior to the bony tympanic frame was observed. Otic culture isolated multi-drug-resistant A. xylosoxidans, only sensitive to meropenem and cotrimoxazole. Temporal bone computed tomography showed an excavation of the floor of the EAC compatible with osteomyelitis. Targeted antibiotherapy for 12 weeks was conducted, with subsequent resolution of symptoms and no progression of the bone erosion. CONCLUSIONS: Atypical pathogens such as A. xylosoxidans can be the cause of chronic otitis externa. Early diagnosis and specific antibiotherapy can prevent the development of further complications, such as osteomyelitis. In these cases, otic cultures play an essential role to identify the causal germ. This is the first case of EAC osteomyelitis due to A. xylosoxidans reported to date.


Subject(s)
Achromobacter denitrificans , Ear Diseases , Osteomyelitis , Otitis Externa , Female , Humans , Aged, 80 and over , Ear Canal/diagnostic imaging , Otitis Externa/diagnosis , Otitis Externa/drug therapy , Osteomyelitis/diagnosis , Osteomyelitis/drug therapy , Osteomyelitis/complications
14.
Eur Arch Otorhinolaryngol ; 281(6): 2921-2930, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38200355

ABSTRACT

PURPOSE: Patient-to-image registration is a preliminary step required in surgical navigation based on preoperative images. Human intervention and fiducial markers hamper this task as they are time-consuming and introduce potential errors. We aimed to develop a fully automatic 2D registration system for augmented reality in ear surgery. METHODS: CT-scans and corresponding oto-endoscopic videos were collected from 41 patients (58 ears) undergoing ear examination (vestibular schwannoma before surgery, profound hearing loss requiring cochlear implant, suspicion of perilymphatic fistula, contralateral ears in cases of unilateral chronic otitis media). Two to four images were selected from each case. For the training phase, data from patients (75% of the dataset) and 11 cadaveric specimens were used. Tympanic membranes and malleus handles were contoured on both video images and CT-scans by expert surgeons. The algorithm used a U-Net network for detecting the contours of the tympanic membrane and the malleus on both preoperative CT-scans and endoscopic video frames. Then, contours were processed and registered through an iterative closest point algorithm. Validation was performed on 4 cases and testing on 6 cases. Registration error was measured by overlaying both images and measuring the average and Hausdorff distances. RESULTS: The proposed registration method yielded a precision compatible with ear surgery with a 2D mean overlay error of 0.65 ± 0.60 mm for the incus and 0.48 ± 0.32 mm for the round window. The average Hausdorff distance for these 2 targets was 0.98 ± 0.60 mm and 0.78 ± 0.34 mm respectively. An outlier case with higher errors (2.3 mm and 1.5 mm average Hausdorff distance for incus and round window respectively) was observed in relation to a high discrepancy between the projection angle of the reconstructed CT-scan and the video image. The maximum duration for the overall process was 18 s. CONCLUSIONS: A fully automatic 2D registration method based on a convolutional neural network and applied to ear surgery was developed. The method did not rely on any external fiducial markers nor human intervention for landmark recognition. The method was fast and its precision was compatible with ear surgery.


Subject(s)
Neural Networks, Computer , Tomography, X-Ray Computed , Humans , Tomography, X-Ray Computed/methods , Temporal Bone/diagnostic imaging , Temporal Bone/surgery , Augmented Reality , Otoscopy/methods , Female , Video Recording , Male , Ear Diseases/surgery , Ear Diseases/diagnostic imaging , Otologic Surgical Procedures/methods , Middle Aged , Algorithms , Surgery, Computer-Assisted/methods , Adult , Tympanic Membrane/diagnostic imaging , Tympanic Membrane/surgery , Malleus/diagnostic imaging , Malleus/surgery , Endoscopy/methods
15.
Ann Plast Surg ; 92(6): 635-641, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38747571

ABSTRACT

BACKGROUND: Keloid is a dermal fibroproliferative disease unique to humans. Due to the ambiguity in its pathophysiology and the frequent recurrence of keloid, there is no clear consensus on the treatment of keloid and there are many treatment methods defined. In order to benefit from the positive effects of fat grafting on pathological scars, we applied fat grafting to patients who underwent keloid enucleation. METHODS: Fifteen ear keloid patients included in the study. All patients underwent the same surgical procedure by the same surgeon. Routine follow-ups and examinations were performed to evaluate the results and in addition, the Patient and Observer Scar Assessment Scale (POSAS) survey was used. RESULTS: In the study, 15 patients were followed for a median (IQR) period of 21 (13-28) months. No recurrence was observed in any patient during follow-up, which occurred for a median of 21 (13-28) months. In the questionnaire filled out by the patients, the preoperative median value was found to be 48 (IQR: 12), whereas the postoperative median value was found to be 14 (IQR: 8). According to the patients, there was a statistically significant ( P < 0.05) positive improvement after surgery. CONCLUSIONS: Historically, surgical procedures were avoided because the surgical recurrence rate was very high, but today, recurrence rates are decreasing with combined treatments. These treatment combinations may require more than one intervention and require frequent clinical follow-ups. With our technique of fat grafting after enucleation, the treatment was completed with a single operation and no additional intervention was required.


Subject(s)
Adipose Tissue , Keloid , Humans , Keloid/surgery , Male , Female , Adipose Tissue/transplantation , Adult , Follow-Up Studies , Treatment Outcome , Young Adult , Middle Aged , Adolescent , Ear Diseases/surgery
16.
J Craniofac Surg ; 35(1): e88-e90, 2024.
Article in English | MEDLINE | ID: mdl-37948616

ABSTRACT

The question mark ear is a rare abnormality characterized by a cleft between the helix and the earlobe, resulting in a protrusion of the upper part of the ear. The severity of this ear malformation can range from a minor notch in the helix to a complete separation of the helix and the earlobe. In this study, we present a case of a patient with a moderately severe right-sided unilateral question mark deformity. To address this issue, we utilized a novel technique that involves a combination of a Y-V flap with double opposing Z-plasty. Our clinical study demonstrates that using this technique for reconstructing the deformity yields excellent results in terms of the helical rim and fold contour, utilizing solely the local tissues.


Subject(s)
Ear Auricle , Ear Diseases , Ear/abnormalities , Plastic Surgery Procedures , Humans , Surgical Flaps/surgery , Ear Auricle/surgery
17.
Clin Otolaryngol ; 49(2): 235-242, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38178371

ABSTRACT

INTRODUCTION: There is an increased demand for well-validated PROMs in otology. This study will systematically assess the methodological quality of all published patient-reported outcome measures (PROMS) for patients with multiple ear complaints and to identify the best suitable PROM for use by clinicians treating patients with multiple ear complaints. METHODS: An extensive systematic mapping review of all otology questionnaires was performed to identify questionnaires measuring multiple ear complaints. The 'Consensus-based standards for the selection of health measurement Instruments' (COSMIN) checklists were used to evaluate the quality of the questionnaire by two researchers. The worst item score per aspect of the methodological assessment counted. RESULTS: Twelve multiple-complaint questionnaires were included in the study for quality assessment. Ten questionnaires were disease-specific (COMQ-12, CES, ZCMEI-21, MD-POSI, PAN-QOL, ETDQ-7, MDOQ, GYSSCDQ, COMOT-15 and DEU-MDDS). Two questionnaires were ear domain-specific (OQUA and COQOL). The majority of multiple complaint questionnaires lacked good design with concept elicitation and patient involvement. CONCLUSION: For the majority of questionnaires, the quality assessment was inadequate as only a few authors consulted with patients affected by the complaints in the development. Modifications of earlier versions of PROMS or combinations of multiple questionnaires lead to ongoing (cross-cultural) validation of these questionnaires albeit mediocre design and validation. The two domain-specific questionnaires are the COQOL and OQUA, both with adequate quality but different focus. COQOL to quantify the quality of life and OQUA to measure and evaluate the severity and impact of ear complaints. [Correction added on 26 January 2024, after first online publication: In the preceding sentence, the spelling of the abbreviation COQOL has been corrected in this version.].


Subject(s)
Ear Diseases , Patient Reported Outcome Measures , Humans , Ear Diseases/diagnosis , Ear Diseases/therapy , Surveys and Questionnaires , Quality of Life
18.
Clin Otolaryngol ; 49(5): 652-659, 2024 Sep.
Article in English | MEDLINE | ID: mdl-38899484

ABSTRACT

INTRODUCTION: Pathway innovation using smartphone otoscopy and tablet-based audiometry technologies to deliver ear and hearing services via trained audiologists may improve efficiency of the service. An ENT-integrated-community-ear service (ENTICES-combining community audiology management, remote ENT review and novel technologies) was piloted. We aimed to assess the efficiency and safety of ENTICES. METHOD: ENTICES was a community-based and audiologist-led pathway. Patients with otological symptoms were self-referred to this service. Smartphone otoscopy and tablet-based audiograms were performed. Two otologists reviewed all decisions made in the community by audiologists based on video-otoscopy, hearing tests and chart reviews. Data on the first 50 consecutive new patients attending either consultant-led hospital otology clinics (HOC), audiologist-led hospital advanced audiology diagnostics (AAD) or ENTICES clinics were collected between 1 August 2021 and 31 December 2021. Data were collected through chart reviews and questionnaires to compare the three pathways with respect to efficiency, patient satisfaction, technology utility and safety. RESULTS: No audiology-led ENTICES decisions were amended by hospital otologists following remote review. Remote review of video-otoscopy with history was sufficient for a diagnosis in 80% of cases. Adding hearing tests and standardised history increased the diagnostic yield to 98%. Patient satisfaction scores showed 100% service recommendation. The cost per patient, per visit, was £83.36, £99.07 and £69.72 for AAD, HOC or ENTICES, respectively. CONCLUSION: ENTICES provides a safe ear and hearing service that patients rated highly. Thirty-two per cent of hospital otology patients were eligible for this service. For those patients, ENTICES is 20% more cost-effective and can reduce the number of clinic visits by up to 60% compared with HOC.


Subject(s)
Otolaryngology , Telemedicine , Humans , Pilot Projects , Male , Female , Middle Aged , Adult , Otoscopy/methods , Aged , Ear Diseases/diagnosis , Ear Diseases/therapy , Patient Satisfaction , Audiometry , Adolescent , Referral and Consultation
19.
HNO ; 72(1): 57-68, 2024 Jan.
Article in German | MEDLINE | ID: mdl-38047932

ABSTRACT

Congenital malformations of the pinna and aural atresia can result in major aesthetic and functional deficits. Knowledge about embryologic developments and established classification systems is an essential requirement when dealing with affected patients. Early detection of deficiencies and introduction of appropriate diagnostic measures is vital to initiate adequate therapies and prevent long-term disabilities. Treatment for malformations of the pinna-if requested-is mostly surgical, infrequently an epithesis is applied. As in other surgical fields, tissue engineering will likely play a crucial role in the future. Treatment of aural stenosis and atresia aims at improvement of hearing levels and prevention of secondary complications like cholesteatoma and chronic otorrhea. Auditory rehabilitation comprises a spectrum from conventional hearing aids to invasive hearing implants, the latter being favored in recent years.


Subject(s)
Congenital Abnormalities , Congenital Microtia , Ear Diseases , Humans , Congenital Abnormalities/therapy , Congenital Abnormalities/surgery , Congenital Microtia/diagnosis , Congenital Microtia/therapy , Congenital Microtia/complications , Ear Diseases/diagnosis , Ear Diseases/therapy , Ear, External , Hearing , Hearing Tests
20.
HNO ; 72(3): 192-198, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38289500

ABSTRACT

BACKGROUND: In 1873, Hermann Schwartze and Adolf Eysell described a new surgical technique for treating mastoid disease using a mallet, chisels, and gouges of various sizes instead of trephines or drill instruments also called "modern mastoidectomy." On the 150th jubilee of this landmark article, we pay tribute by studying the reception and implementation of mastoidectomy in the 2 years following its publication. METHODS: The commentaries published in the otological and medical literature between the second part of 1873 to the end of 1875 were studied with an emphasis on the three specialized otological journals and the otological textbooks that existed during this period. RESULTS AND CONCLUSION: The princeps paper Ueber die künstliche Eröffnung des Warzenfortsatzes ("On the artificial opening of the mastoid process") by Hermann Schwartze and Adolf Eysell published in 1873 was rapidly disseminated in the medical literature for nearly 1 year, and then entered a phase of evaluation followed by a phase of extension and implementation, before finding its definitive place in the history of mastoid process surgery.


Subject(s)
Ear Diseases , Mastoidectomy , Humans , Mastoid/surgery , Surgical Instruments
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