Evaluation of the Impact of Cochlear Implantation on Patients' Working Life: A Cross-Sectional Study.

Hearing loss that arises from various causes at different stages of life has a direct impact on individuals' physical and mental well-being. This paper aimed to evaluate the employment, workplace adaptation, productivity, and professional success of individuals who have hearing loss and whose hearing loss is corrected with a cochlear implant. In this cross-sectional study, data were collected between November 2022 and March 2023 with the participation of individuals with cochlear implants living in several settlements in all regions of Türkiye. A total of 142 participants with severe hearing loss who were corrected with a cochlear implant were included in this study. The survey method was used to collect data for the study. The questionnaire consisted of 32 questions and was distributed to the participants online. In the first part of the questionnaire (questions 1-10), the general characteristics of implant patients were investigated. In the second part (questions 11-32), the positive or negative effects of implantation on the work lives of the participants were evaluated. Almost half of the research group (49.3%, n = 70) consisted of women, and the mean age of the participants was determined to be 35.8 ± 14.8 years. There was no significant difference between gender, educational status, implanted side, working time, working style (physical, desk), and factors affecting work life (p > 0.05). Professional satisfaction and success at work increased significantly more in those with acquired hearing loss (p = 0.010). Post-implantation workplace compliance, success, and productivity were found to be higher in those with acquired hearing loss (p = 0.013). Hearing loss had a significantly less negative impact on work performance in those implanted in childhood than in those implanted in adulthood (p = 0.043). It was observed that hearing loss had a greater negative impact on the work life of married people (p = 0.006). Cochlear implantation greatly enhances workplace satisfaction, increases self-confidence, and has a positive impact on the future of profoundly deaf individuals.

Effects of Unilateral Audio-Vestibular Insufficiency on Spatial Hearing.

This study aimed to compare spatial hearing performance between adult individuals with the unilateral sensorineural hearing loss and unilateral loss of horizontal semicircular canal function (termed canal paresis/weakness) in the same ear and adults with normal hearing thresholds and normal vestibular function and to examine associated factors (duration of hearing loss and rate of canal paresis).The study participants consisted of 20 adults (aged 48±11 years) with unilateral sensorineural hearing loss and unilateral canal paresis (unilateral weakness≥25%) in the same ear. The control group comprised 25 adults (aged 45±13 years) with normal hearing and a unilateral weakness rate below 25%. Pure tone audiometry, bithermal binaural air caloric test, Turkish Spatial Hearing Questionnaire (T-SHQ), and Standardized Mini-Mental State Exam were applied to all the individuals. When the performance of the participants in T-SHQ was examined both in terms of the subscales and the total scale, there was a statistically significant difference between the two groups in relation to the scores. A statistically significant, high, negative correlation was detected between the duration of hearing loss, the rate of canal paresis and all the subscale scores and total score of T-SHQ. According to these results, as the duration of hearing loss increased, the scores obtained from the questionnaire decreased. As the rate of canal paresis increased, vestibular involvement increased, and the T-SHQ score decreased. This study showed that adults with unilateral hearing loss and unilateral canal paresis in the same ear had lower spatial hearing performance than those with normal hearing and balance. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-022-03442-1.

Anesthesia for a Child with Congenital Long QT Syndrome, a Case Report and Literature Review.

Long QT syndrome is an inherited disorder of the heart's electrical activity that may also be associated with malignant arrhythmia and cause sudden death. In addition to this inherited condition, several commonly used anesthetic drugs can prolong the QT interval. We present here a 17-month-old male patient who underwent general anesthesia for a cochlear implant. No cardiac arrhythmia was observed in the patient, whose muscle relaxant effect was reversed using sugammadex. The application of intravenous anesthetics was preferred to maintain anesthesia for this patient and was safely applied.

Evaluation of the Vestibulocochlear System in Patients with Pseudoexfoliation Syndrome

Objectives: Patients with pseudoexfoliation syndrome (PES) can also have sensorineural hearing loss as well as balance problems. Our aim was to evaluate vestibulocochlear system involvement in PES patients. Materials and Methods: The study included 16 subjects with PES (study group) with a mean age of 66.12±5.64 years and 17 healthy subjects (control group) with a mean age of 61.70±8.46 years. Both groups underwent ophthalmological, neuro-otological, audiological, and vestibular evaluation. Pure-tone audiometry and tympanometry were performed as audiological tests and bithermal caloric test and vestibular-evoked myogenic potential (VEMP) testing were used as vestibular tests. The Romberg, tandem Romberg, and Unterberger tests were also performed. Results: In the PES group, bithermal caloric tests revealed right canal paresis in 6 patients, left canal paresis in 3 patients, and bilateral stimulation loss in 2 patients, despite no clinical evidence of balance loss. Paresis was not detected in any of the control subjects. Unilateral VEMP responses could not be obtained in 3 patients in the PES group. The ocular PES patients whose VEMP waves were obtained differed significantly from the control group (p<0.05). In office tests for vestibular evaluation, pathologic findings were found in 7 of 16 patients in the study group and only 4 subjects in the control group. Audiological evaluation with pure-tone thresholds revealed sensorineural decline at 4000 and 8000 Hertz in the PES patients. A statistically significant difference was found between the study group and the control group (p<0.05). Conclusion: Patients with PES showed elevation in pure-tone thresholds and a decrease in superior and inferior vestibular nerve function, demonstrating that the vestibular system as well as the auditory system are affected in PES.

Effects of Oxygen Therapies in Experimental Acute Acoustic Trauma.

BACKGROUND: Acute acoustic trauma is defined as a sudden sensorineural hearing loss that occurs after an exposure to acoustic overstimulation. Increasing the oxygen in perilymph can be a treatment modality. Our study aims to investigate the influence of normobaric oxygen therapy on the recovery of acute acoustic trauma and to compare it with the hyperbaric oxygen therapy. METHODS: Three groups of rats (5 rats each) were exposed to white noise for 1 hour. Sensorineural hearing loss was identified using distortion product otoacoustic emission. Subsequently, the first group was treated with hyperbaric oxygen therapy, the second group was treated with normobaric oxygen therapy, and the third group did not receive any treatment and was used as a control group. RESULTS: There was a statistically significant difference within time for frequencies of 1, 1.5, and 2 kHz, but there was no statistically significant difference between groups. For frequencies of 3, 4, 5, and 6 kHz, there was a statistically significant difference within time and between groups. Between groups, recovery of distortion product otoacoustic emission values in all frequencies was better in the control group by the third, fifth, and seventh days. Comparing the values of hyperbaric oxygen therapy and normobaric oxygen therapy groups, it was observed that by the third day, the hyperbaric oxygen therapy values were better than those of the normobaric oxygen therapy values. However, by the fifth and seventh days, the normobaric oxygen therapy values were better (except at a frequency of 1 kHz). CONCLUSION: Because there is a high rate of spontaneous recovery, physicians should be more selective to treat patients with oxygen therapies.

Analysis of cochlear implant revision surgeries.

PURPOSE: This study aimed to evaluate the reasons for cochlear implant (CI) revision surgeries in pediatric and adult groups. METHODS: A total of 490 CIs were used for 423 patients between August 2005 and August 2019. Among these, patients who underwent a CI revision surgery were identified retrospectively. The reasons for CI revision surgeries were classified mainly as medical problems and device failure. These were also determined according to implant brands. RESULTS: Of 423 patients with a CI surgery, 27 (6.3%) underwent a CI revision surgery. The revision implant rate was 4.9% for the pediatric group and 1.4% for the adult group. Five patients underwent a CI revision surgery due to medical problems (23.8%) and 16 due to device failure (76.2%) in the pediatric group, while 3 patients (50.0%) underwent a CI revision surgery due to medical problems and 3 (50.0%) due to device failure in adults. CONCLUSIONS: A CI surgery is safe for patients with severe-to-profound sensorineural hearing loss, although a CI revision surgery has some potential. Device failure is the most common cause in children. It may be due to early implantation, frequent fall when children learn walking, or impaired vestibular function.

Perception of male otolaryngologists on gender discrimination: a comparative study.

PURPOSE: To gather information on perception of male otolaryngologists (MORLs) about gender discrimination towards female otolaryngologists (FORLs). METHODS: MORLs were invited to participate to an online survey. Minimum participation requirement was completion of at least their first year of residency. The responses were analyzed and compared vis-a-vis with the previously conducted similar survey among FORLs. RESULTS: Statistically significant responses on the Likert scale are classified in four main groups. MORLs and FORLs share the same views about financial factors, benefits and opportunities, housework as burden, establishing work-life balance and physical strength requirements. They have opposing views about FORLs being meticulous, exposed to more negative attitude of the opposite gender and men's dominance in decision-making. FORLs don't have consensus, but MORLs disagree about MORLs being favored in pursuing academic careers. On the other hand, MORLs don't have consensus, but FORLs agree about patients having more confidence in MORLs. CONCLUSION: MORLs don't usually have any confrontation with FORLs in regards to the roles of women in the society such as their motherhood role. On the other hand, MORLs show rather a contradiction on their perception towards the gender discrimination mainly in achieving career goals by FORLs such as growing in the profession and holding managing roles. When the views of the both gender group are compared, MORLs don't seem to fully acknowledge FORLs' gender discrimination experience.

Association Between Incomplete Partition Type III and Abnormal Hypothalamic Morphology: Further Imaging Evidence.

PURPOSE: Incomplete partition III (IP-III), characterized by congenital mixed or sensorineural hearing loss, is a rare genetic disease transmitted through X-linked mode of inheritance. Inner ear findings of IP-III have been well described and allow an immediate diagnosis to be made. Recently, an association between IP-III and distinct hypothalamic malformations has been reported in some of the patients with IP-III. The purpose of this study was to investigate the morphologic abnormalities of the hypothalamus in IP-III. MATERIALS AND METHODS: Magnetic resonance imaging studies of 8 subjects, including 1 set of brothers, who were diagnosed with IP-III based on their clinical and inner ear imaging findings, were analyzed. RESULTS: Of the 8 subjects, 7 demonstrated some degree of morphologic abnormality of the hypothalamus. Of these, 2 showed asymmetrical thickening, 1 showed symmetrical thickening, and 4 showed mass-like enlargement of the hypothalamus. Six of 7 subjects with hypothalamic abnormalities showed asymmetry in caudal extension of the abnormalities, which was more discernible on coronal oblique T2-weighted images. Clinically, none of the subjects had endocrinologic or neurologic symptoms. CONCLUSIONS: This retrospective analysis presents further magnetic resonance imaging evidence on the association between the rare IP-III malformations and the presence of hypothalamic morphologic abnormalities.

Long-range cis-regulatory elements controlling GDF6 expression are essential for ear development.

Molecular mechanisms governing the development of the mammalian cochlea, the hearing organ, remain largely unknown. Through genome sequencing in 3 subjects from 2 families with nonsyndromic cochlear aplasia, we identified homozygous 221-kb and 338-kb deletions in a noncoding region on chromosome 8 with an approximately 200-kb overlapping section. Genomic location of the overlapping deleted region started from approximately 350 kb downstream of GDF6, which codes for growth and differentiation factor 6. Otic lineage cells differentiated from induced pluripotent stem cells derived from an affected individual showed reduced expression of GDF6 compared with control cells. Knockout of Gdf6 in a mouse model resulted in cochlear aplasia, closely resembling the human phenotype. We conclude that GDF6 plays a necessary role in early cochlear development controlled by cis-regulatory elements located within an approximately 500-kb region of the genome in humans and that its disruption leads to deafness due to cochlear aplasia.

Irregular Contour of Inner Ear Structures and Hypomineralized Areas at Otic Capsule: Are They Other Additional Imaging Findings of Incomplete Partition-III?

PURPOSE: Incomplete partition III (IP-III) characterized by congenital mixed or sensorineural hearing loss is a rare genetic disease transmitted through X-linked inheritance. Incomplete partition III can be easily achieved based on pathognomonic computed tomography findings. The aims of this study were to investigate the otic capsule abnormalities in IP-III and to report irregular contour of membranous labyrinth and hypomineralized areas at otic capsule, which have not previously been described. MATERIALS AND METHODS: The otic capsule features of 10 subjects (8 affected patients, 1 of whom is a female; 2 carrier mothers), who were diagnosed on clinical and typical radiologic findings, were analyzed. RESULTS: All patients had typical IP-III as described in the literature. Seven of 10 patients had irregular contour. Seven of 10 patients demonstrated hypomineralized areas, which were very hypodense to normally develop otic capsule areas. One affected patients and 2 carrier mothers had a normal-looking membranous labyrinth contour and normal mineralization at otic capsule. CONCLUSIONS: We report for the first time the irregular contour of inner ear structures and hypodense otic capsule areas in patients with IP-III. We think that though speculative, abnormal development of the inner endosteal layer results in irregular contour of inner ear structures. Hypomineralized areas at otic capsule could be explained by abnormal development of middle enchondral layer due to reduced or absent vascular supply from middle ear mucosa during fetal life. These findings may be accepted as additional criteria of IP-III.

The Effectiveness of Medical Prophylactic Treatment on Vestibular Migraine and Its Effect on the Quality Of Life.

OBJECTIVES: The aim of the present study was to determine the efficacy of propranolol treatment in patients with vestibular migraine by the Visual Analog Scale, Dizziness Handicap Inventory (DHI), Vertigo Symptom Scale, and Vestibular Disorders Activities of Daily Living Scale (VADL) and its effect on the quality of life. MATERIALS AND METHODS: The study population consisted of 38 patients with vertigo/dizziness who underwent routine evaluation and vestibular examinations, were diagnosed with definitive vestibular migraine, and received the same medical treatment protocol (propranolol). The questionnaires and scales that were applied to the patients before and after treatment were evaluated. The results were evaluated with 95% confidence interval, and p<0.05 was accepted as statistically significant. RESULTS: The mean age of the patients was 47.55 (18-75) years, and 27 (71%) patients were female, and 11 (29%) were male. The mean total scores of the DHI before and after treatment were 50.21±22.39 (range: 8-92) and 9.31±9.86 (range: 0-58), respectively (p<0.001). The degree of disability after treatment was low in all patients (p<0.001). The total scores of the VADL before and after treatment were 186.63±79.65 (range: 32-280) and 55.52±51.89 (range: 28-273), respectively (p<0.001). There was no correlation between these two scales (p=0.235). CONCLUSION: To our knowledge, this is the first study to evaluate both the efficacy of propranolol treatment and its effects on the quality of life in vestibular migraine. The severity, frequency, and number of attacks and disability scores were reduced, and the quality of life was improved in patients with vestibular migraine with propranolol treatment.

Women in otolaryngology in Turkey: Insight of gender equality, career development and work-life balance.

OBJECTIVE: The aim of this study is to collect information about the demographics, academic ambitions, job satisfactions, career development and work-life balance of female otorhinolaryngologists (FORL) in Turkey and to determine their experiences with, and attitudes towards gender discrimination throughout their academic careers. STUDY DESIGN AND SETTING: A prospective survey study. SUBJECTS AND METHODS: The study was aimed to include all FORL who completed their residency program. An online survey was used to collect their responses. RESULTS: Out of 208 invitations, 156 FORL participated to the survey. The mean age of the participants was 39.7 ±â€¯6.1 (29-75) years. 16.6% of the FORL used to occupy or still occupying administrative positions in their respective medical centers; 15.4% of them used to be assigned or still being assigned to a duty in otorhinolaryngology associations. Gender discrimination was 2.5 fold higher (p = 0.006 OR: 2.55 (95% CI 1.31, 4.99) in departments where there were no female faculty members. 53.2% of the female surgeons were both physically and psychologically harassed for their gender during their residency programs to deter from completing their program in otolaryngology. This finding is 4.1 fold higher than those who stated that they had not been exposed to any such gender discrimination (p = 0.001 OR: 4.094 (95% CI 2.22, 7.57). CONCLUSION: Healthcare policy-makers and institutions should consider taking all the necessary actions to prevent gender discrimination in order to increase job satisfaction and achievements of female specialists in the field of otorhinolaryngology.

FOXF2 is required for cochlear development in humans and mice.

Molecular mechanisms governing the development of the human cochlea remain largely unknown. Through genome sequencing, we identified a homozygous FOXF2 variant c.325A>T (p.I109F) in a child with profound sensorineural hearing loss (SNHL) associated with incomplete partition type I anomaly of the cochlea. This variant is not found in public databases or in over 1000 ethnicity-matched control individuals. I109 is a highly conserved residue in the forkhead box (Fox) domain of FOXF2, a member of the Fox protein family of transcription factors that regulate the expression of genes involved in embryogenic development as well as adult life. Our in vitro studies show that the half-life of mutant FOXF2 is reduced compared to that of wild type. Foxf2 is expressed in the cochlea of developing and adult mice. The mouse knockout of Foxf2 shows shortened and malformed cochleae, in addition to altered shape of hair cells with innervation and planar cell polarity defects. Expressions of Eya1 and Pax3, genes essential for cochlear development, are reduced in the cochleae of Foxf2 knockout mice. We conclude that FOXF2 plays a major role in cochlear development and its dysfunction leads to SNHL and developmental anomalies of the cochlea in humans and mice.

International Otology Outcome Group and the International Consensus on the Categorization of Tympanomastoid Surgery.

The International Otology Outcome Group (IOOG) was founded in 2017 to encourage and facilitate international collaboration with regard to the surgical outcome of ear surgery. This report outlines the methodology and recommendations of the consensus-based categorization of tympanomastoid surgery produced by the IOOG. The IOOG Steering Committee used the acronym SAMEO-ATO to categorize tympanomastoid operations, representing the stage of surgery, approach, mastoid bone extirpation, external bony wall repair, obliteration of the mastoid cavity, access to the middle ear, tympanic membrane reconstruction, and ossicular reconstruction. A modified Delphi technique was used to obtain international consensus. The expert panels included the chairpersons from 21 otology societies. The approval rate of the SAMEO-ATO system from the otology societies was 95%. The SAMEO-ATO scheme was presented at the 31st Politizer Meeting for field testing. There were no objections or serious concerns raised. Some international otologists wished to see more surgical categories included to reflect the varieties of surgical techniques, but they accepted that it would make the whole system cumbersome. In addition to providing an international categorization of tympanomastoid surgery, the IOOG Steering Committee plans to introduce a common otology dataset that the international otology community could use to record their surgical outcome. The high level of international consensus on the IOOG categorization of tympanomastoid surgery supports this tool for surgeons to pool their surgical data into a large database for research and comparative audit.

MPZL2 is a novel gene associated with autosomal recessive nonsyndromic moderate hearing loss.

While recent studies have revealed a substantial portion of the genes underlying human hearing loss, the extensive genetic landscape has not been completely explored. Here, we report a loss-of-function variant (c.72delA) in MPZL2 in three unrelated multiplex families from Turkey and Iran with autosomal recessive nonsyndromic hearing loss. The variant co-segregates with moderate sensorineural hearing loss in all three families. We show a shared haplotype flanking the variant in our families implicating a single founder. While rare in other populations, the allele frequency of the variant is ~ 0.004 in Ashkenazi Jews, suggesting that it may be an important cause of moderate hearing loss in that population. We show that Mpzl2 is expressed in mouse inner ear, and the protein localizes in the auditory inner and outer hair cells, with an asymmetric subcellular localization. We thus present MPZL2 as a novel gene associated with sensorineural hearing loss.

Efficacy of Platelet-Rich Plasma on Fat Grafts in the Repair of Tympanic Membrane Perforations: An Experimental Study.

OBJECTIVE: We investigated the use of autologous platelet-rich plasma (PRP) to improve the success rate of fat graft myringoplasty in perforated tympanic membranes of rats. MATERIALS AND METHODS: A total of 20 healthy Wistar albino female rats were divided into two groups. In Group 1, the left tympanic membranes were perforated and grafted with a fat graft that was harvested from the inguinal region. In Group 2, the left tympanic membranes were perforated, and a fat graft was also harvested from the inguinal region. Then, the fat was soaked in 0.5 mL PRP and grafted at the perforation. After the procedure, the rats were examined, and the graft situation was assessed at 3, 5, 7, 10, and 14 days. All of the rats were sacrificed 21 days after perforation, and a histopathological examination was made. RESULTS: We compared fat graft histopathological and otomicroscopic findings between the groups. While we did not observe graft rejections in Group 2, we saw 1 case of graft rejection in Group 1. In the histopathological examination, PRP prevents fat graft resorption by the terms of the adipocyte area, granulation tissue area, and vacuolization area. CONCLUSION: This study demonstrated the efficacy of fat grafts prepared with PRP on rat tympanic membranes. The fat graft with PRP did not statistically improve the success rate compared to the graft without PRP. Histopatologic findings of the study showed that PRP prevents fat graft resorption. Further studies are needed to further examine the advantages of the graft with PRP.

Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases.

BACKGROUND: Keratitis-ichthyosis-deafness (KID) syndrome is a syndrome which presents with hearing loss and visual and keratinization disorders. In such patients, hearing aids cannot be effectively used in the rehabilitation of hearing loss because of the frequent blockage of the external ear canal with epithelial debris and due to dry and tense skin of the external ear canal. Moreover, severe or profound hearing loss also limits the benefits gained from the conventional hearing aids. On the other hand, cochlear implantation is a method that has been used in limited cases in the literature. CASE REPORT: This study presents the results of cochlear implantation applied in our clinic to two children who had been diagnosed with KID. Audiological assessments before and after the cochlear implant operation were performed using pure-tone audiometry, immittance audiometry, and auditory brainstem response (ABR), and the postoperative follow-up was conducted using pure-tone audiometry. CONCLUSION: Skin problems, visual disturbances, and other additional problems complicate the short-term and long-term rehabilitation after implantation in individuals with KID syndrome. Close monitoring should be exercised due to possible skin complications that may develop during the postoperative period. The families and rehabilitation teams should be warned about the possible visual disturbances and skin complications.

Novel pathogenic variants underlie SLC26A4-related hearing loss in a multiethnic cohort.

OBJECTIVES: The genetics of sensorineural hearing loss is characterized by a high degree of heterogeneity. Despite this heterogeneity, DNA variants found within SLC26A4 have been reported to be the second most common contributor after those of GJB2 in many populations. METHODS: Whole exome sequencing and/or Sanger sequencing of SLC26A4 in 117 individuals with sensorineural hearing loss with or without inner ear anomalies but not with goiter from Turkey, Iran, and Mexico were performed. RESULTS: We identified 27 unique SLC26A4 variants in 31 probands. The variants c.1673A > G (p.N558S), c.1708-1G > A, c.1952C > T (p.P651L), and c.2090-1G > A have not been previously reported. The p.N558S variant was detected in two unrelated Mexican families. CONCLUSION: A range of SLC26A4 variants without a common recurrent mutation underlies SLC26A4-related hearing loss in Turkey, Iran, and Mexico.

EAONO/JOS Joint Consensus Statements on the Definitions, Classification and Staging of Middle Ear Cholesteatoma.

The European Academy of Otology and Neurotology (EAONO) has previously published a consensus document on the definitions and classification of cholesteatoma. It was based on the Delphi consensus methodology involving the broad EAONO membership. At the same time, the Japanese Otological Society (JOS) had been working independently on the "Classification and Staging of Cholesteatoma." EAONO and JOS then decided to collaborate and produce a joint consensus document. The EAONO/JOS joint consensus on "Definitions, Classification and Staging of Middle Ear Cholesteatoma" was formally presented at the 10th International Conference on Cholesteatoma and Ear Surgery in Edinburgh, June 5-8, 2016. The international otology community who attended the consensus session was given the chance to debate and give their support or disapproval. The statements on the "Definitions of Cholesteatoma" received 89% approval. The "Classification of Cholesteatoma" received almost universal approval (98%). The "EAONO/JOS Staging System on Middle Ear Cholesteatoma" had a majority of approval (75%). Some international otologists wanted to see more prognostic factors being incorporated in the staging system. In response to this, the EAONO/JOS steering group plans to set up an "International Otology Outcome Working Group" to work on a minimum common otology data set that the international otology community can use to evaluate their surgical outcome. This will generate a large database and help identify relevant prognostic factors that can be incorporated into the staging system in future revisions.