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Objective: Recommendations for air travel after stapes surgery, specifically stapes surgery, vary, with no standard recommendation to guide patients and surgeons. According to our search, no previous article has explored the physics of middle ear changes during flight and its effects on poststapedectomy patients in a systematic way. The aim of this study is to bring together 2 arms of expertise, otology, and aviation, to produce an evidence-based recommendation for flight after stapes surgery. Data Sources: The database MEDLINE was searched during August 2022. The search strategy had the goal of identifying studies that discovered the effects of flying on stapes surgery patients and the effects of atmospheric pressure on middle ear structures. Review Methods: The articles yielded from the search strategy were transferred to the online citation manager Rayyan. Included in the review were those studies reporting patient outcomes after flying following ear surgery; additional studies included those reporting pressure changes in the middle ear and ossicular chain displacement whether in experimental or animal conditions. Conclusion: Modern-day commercial air travel is safe for patients who have undergone stapedotomy surgery, even very shortly after hospital discharge if they have to. Implications for Practice: If stapedotomy patients wish to fly after hospital discharge, otologists are to reassure them that it is safe to do so. Patients are to be reminded to perform a gentle Valsava maneuver about every 4 minutes during airplane descent.

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OBJECTIVE: The effect of hearing aid use on the evolution of presbycusis has not been well described in the literature, with only a handful of publications addressing this topic. This paper aims to evaluate the long-term use of amplification and its effect on pure-tone thresholds and word recognition scores. METHOD: Monaurally fitted patients were followed with serial audiograms. Data was collected from hearing aid centers. Seventy-seven patients with presbycusis met the inclusion criteria and participated in the present study. The progression of hearing loss in both pure tone thresholds and word recognition scores were compared between the hearing aid ears (HA), and the non-hearing aid ears (NHA). Pure tone thresholds were analyzed by comparing the pure tone average at the initial and last audiograms. Word Recognition Scores (WRS) were analyzed using the model of Thornton and Raffin (1978), and by comparing the change in the absolute values of WRS from the initial to the last audiogram between the HA ear and the NHA ear. RESULTS: No significant difference in pure-tone thresholds between the HA ear and NHA ear was found at the last audiogram (P = .696), even after dividing the patients into groups based on the duration of amplification. Both methods of analysis of patients' WRS showed a statistically significant worsening in NHA (P < .05). CONCLUSION: The present study supports the previously defined auditory deprivation effect on non-fitted ears, which showed worsening of word recognition over time and no effect on pure tone average. It provides an additional argument for the counseling of patients with presbycusis considering amplification, and highlights the importance of bilateral amplification in preserving the residual hearing of hearing impaired patients.

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BACKGROUND: Hearing loss (HL) represents the most common congenital sensory impairment with an incidence of 1-5 per 1000 live births. Non-syndromic hearing loss (NSHL) is an isolated finding that is not part of any other disorder accounting for 70% of all genetic hearing loss cases. METHODS: In the current study, we reported a polygenic mode of inheritance in an NSHL consanguineous family using exome sequencing technology and we evaluated the possible effect of the detected single nucleotide variants (SNVs) using in silico methods. RESULTS: Two bi-allelic SNVs were detected in the affected patients; a MYO15A (. p.V485A) variant, and a novel MITF (p.P338L) variant. Along with these homozygous mutations, we detected two heterozygous variants in well described hearing loss genes (MYO7A and MYH14). The novel MITF p. Pro338Leu missense mutation was predicted to change the protein structure and function. CONCLUSION: A novel MITF mutation along with a previously described MYO15A mutation segregate with an autosomal recessive non-syndromic HL case with a post-lingual onset. The findings highlight the importance of carrying whole exome sequencing for a comprehensive assessment of HL genetic heterogeneity.

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OBJECTIVES: Review of the literature for studies involving the use of hyaluronic acid (HA) in otology. METHODS: Pubmed and OvidMedline were searched using a combination of the following words in different variations: hyaluronic acid, hyaluronate, otolaryngology, otology, ear, tympanic membrane, perforation, tympanostomy, tympanoplasty, myringoplasty, packing, middle ear, cochlea, gene delivery, gene therapy, cochlear implant, hearing loss, meniere, vertigo, otitis and cholesteatoma. RESULTS: The papers relevant for this review were triaged based on abstracts and titles and were then categorized based on topic/disease entity/procedure. The papers were read and summarized in order to use their findings in this review. CONCLUSIONS: HA is being recently used as adjuvant therapy for multiple inflammatory conditions and in tissue repair. These immunomodulatory properties and biocompatibility have interested researchers specially in the field of otology for repair, gene delivery, immunomodulation etc. Recent data in the field show optimistic results for the use of HA in several conditions especially tympanic membrane perforations and gene delivery. It also establishes the role of HA as ancillary treatment in many other otologic pathologies. This review presents the most recent findings on the use of HA in otology. The results could be used to guide clinical practice and incite further research based on the presented results of the literature.

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BACKGROUND: The study aims at reporting our experience with loop underlay tympanoplasty, a modification of the underlay technique previously reported, for the reconstruction of anterior, subtotal or total tympanic membrane perforations. METHODS: A retrospective review of charts of patients who have undergone loop underlay tympanoplasty from January 2002 to January 2012 was performed. One thousand one hundred patients were included. Hearing test results preoperatively and postoperatively were reported. On follow up visits, the closure of the tympanic membrane perforation and the improvement of hearing compared to preoperative measurements with absence of complications were considered as successful outcomes of the surgery. RESULTS: At the three-month follow-up visit, the perforation closure rate was found to be 99.3% and Air-Bone Gap closure rate to less than 10 dB was 99.5%. The complication rate, including post-operative infection, was 0.72%. CONCLUSION: The loop underlay technique combines advantages of both underlay and overlay techniques with excellent postoperative outcomes.

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AIM: To screen for the genetic basis of congenital hearing loss in a Syrian family. METHODS: A Syrian patient living in Lebanon presented with moderate congenital hearing loss. The patient's large nonconsanguineous family was recruited. DNA was extracted from blood samples and sent for whole-exome sequencing. A detailed clinical examination along with audiograms was obtained for all subjects. RESULTS: Hearing loss was noted to be mild to moderate in the low and mid frequencies, sloping to moderate to severe in the high frequencies for all affected members. Results of DNA analysis showed the presence of a previously described p.Arg925* mutation in the OTOGL gene on both alleles in affected family members, whereas nonaffected members either had the wild type or one copy of the mutated allele. DISCUSSION: Mutations affecting the OTOGL gene have been recently connected with nonsyndromic sensorineural hearing loss. Seven such mutations have already been described. The p.Arg925* reported in this study has been found once in a French family. The current report is the first to describe this mutation in a Middle Eastern family.

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OBJECTIVE: To translate the Tinnitus Handicap Inventory (THI) into literary Arabic to come up with a unified Arabic version and to determine its validity and reliability in assessing the quality of life of Arabic-speaking patients with tinnitus. STUDY DESIGN: Clinical measurement study. SETTING: Tertiary care center. SUBJECTS AND METHODS: The original English THI was translated into literary Arabic by a forward- and back-translation process according to the published guidelines for cross-cultural adaptation of health-related quality-of-life measures and applied to 100 patients with chronic tinnitus. Internal consistency reliability was then assessed by calculating Cronbach's alpha coefficient. Pearson correlation coefficients were also calculated for the different scales and the different baseline characteristics. RESULTS: Results showed high internal consistency and reliability coefficients (total THI: 0.93, functional subscale: 0.86, emotional subscale: 0.86, catastrophic subscale: 0.66) comparable to those of the original English THI. CONCLUSION: The Arabic version of the THI is a valid and reliable tool for the assessment of the impact of tinnitus on the quality of life of Arabic-speaking patients with the complaint of chronic tinnitus.

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Although proteomics has been exploited in a wide range of diseases for identification of biomarkers and pathophysiological mechanisms, there are still biomedical disciplines such as otology where proteomics platforms are underused due to technical challenges and/or complex features of the disease. Thus, in the past few years, healthcare and scientific agencies have advocated the development and adoption of proteomic technologies in otological research. However, few studies have been conducted and limited literature is available in this area. Here, we present the state of the art of proteomics in otology, discussing the substantial evidence from recent experimental models and clinical studies in inner-ear conditions. We also delineate a series of critical issues including minute size of the inner ear, delicacy and poor accessibility of tissue that researchers face while undertaking otology proteomics research. Furthermore, we provide perspective to enhance the impact and lead to the clinical implementation of these proteomics-based strategies.

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OBJECTIVE: To identify the relationship between the vertical portion of the facial nerve and the tympanic annulus, using computed tomographic (CT) scans of healthy adult and pediatric patients. STUDY DESIGN: A retrospective review of CT scans. SETTING: The study was conducted in a tertiary referral medical center. PATIENTS: After excluding ears with noted pathology, a total of 241 ears were included in the final review (121 right and 120 left ears). The mean age of the patients was 33.3 years (2 mo to 87 yr). INTERVENTION(S): Both structures were identified at three distinct locations: the superior and the inferior most margins of the tympanic ring and at the umbo. Measurements were made in both anteroposterior and a mediolateral planes. Comparisons between different age groups were made to analyze the relative change in position that happens with age. RESULTS: The vertical portion of the facial nerve, as it proceeds distally, takes a more anterior and lateral course, and crosses the plane of the annulus almost consistently in the inferior third. The average distance at each location is reported. Statistically significant differences were found between age groups, in the areas around the second genu and the distal most segment of the facial nerve. CONCLUSIONS: This is the largest anatomic study to date analyzing the relationship of the facial nerve to the tympanic annulus. It also allows comparison between age groups, demonstrating a significant difference between adult and pediatric groups, especially in the distal portion of the fallopian canal.

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Aspergillus mastoiditis usually occurs in immunocompromised patients. There are a few isolated reports in the literature involving immunocompetent patients. We hereby describe the case of an immunocompetent patient diagnosed with invasive Aspergillus mastoiditis, which was treated successfully, and review the literature pertaining to this condition. The common clinical presentations, putative pathophysiology, and recommended therapy are discussed.

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OBJECTIVE: Determine whether more conservative management for surgical salvage after failed radiation leads to better facial nerve outcomes. STUDY DESIGN: "Retrospective" review using prospectively planned database. SETTING: Private practice, tertiary neurotology/neurosurgery referral center. PATIENTS: A series of 73 patients with vestibular schwannoma, who underwent primary radiosurgery with no other intervention and then surgical salvage tumor removal using the translabyrinthine approach. INTERVENTION(S): Translabyrinthine craniotomy for vestibular schwannoma salvage surgery after failed radiation, with either gross total or partial tumor removal. MAIN OUTCOME MEASURES: Long-term (1 yr) House-Brackmann (H-B) facial nerve grade and change in facial nerve grade from before to after surgery. RESULTS: Of the 73 patients, 79.5% had gross total removal, 5.5% had planned partial resection (subtotal or near total), and 15.1% had intraoperatively elected partial removal with most of these being near total removal. At 1 year of follow-up, good facial nerve function (H-B I/II) was found in 50% of patients with gross total removal and 85.7% of those with partial removal (p ≤ 0.03). The H-B grade was maintained postoperatively in 45.8% and 78.6% of the 2 groups, respectively (p ≤ 0.037), with 21.7% of the total removal group having unsatisfactory outcomes (H-B V or VI) compared with 7.1% of patients with partial removal. To date, no patient has required additional treatment. CONCLUSION: Failed radiosurgery is an increasing indication for salvage surgery in patients with posterior fossa tumors. A conservative approach with a willingness to perform partial and near-total tumor removals leads to better facial nerve outcomes with no current evidence of treatment compromise.

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DFNA9 is an autosomal dominant cause of non-syndromic adult-onset sensorineural hearing loss with associated variable vestibular dysfunction caused by mutations in the COCH gene. DFNA9 has previously been characterized by the presence of unique histopathologic features limited to the cochlear and vestibular labyrinth. This report describes newly discovered extralabyrinthine findings within the middle ear in DFNA9 and discusses their implications. The histopathologic anatomy of extralabyrinthine structures was reviewed in 12 temporal bones from seven individuals with DFNA9 and compared with age-matched controls. All temporal bones with DFNA9 had abnormal deposits within the tympanic membrane, incudomalleal joint, and incudostapedial joint. Hematoxylin and eosin stain and Movat's pentachrome stain both revealed different staining patterns of the extralabyrinthine deposits compared with the intralabyrinthine deposits suggesting that the composition of the deposits varies with location. The deposits within the tympanic membrane resembled cartilage morphologically and stained positively for aggrecan, an extracellular matrix protein found in cartilage. However, the cellular component of the tympanic membrane deposits did not stain with immunomarkers for chondrocytes (s100 and connective tissue growth factor). These novel findings in DFNA9 have implications for the phenotypic expression of the disorder and the clinical workup of adult-onset sensorineural hearing loss.

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OBJECTIVE: To identify and define the imaging characteristics of children with auditory neuropathy spectrum disorder (ANSD). DESIGN: Retrospective medical records review and analysis of both temporal bone computed tomographic (CT) and magnetic resonance images (MRI) in children with a diagnosis of ANSD. SETTING: Tertiary referral center. PATIENTS: One hundred eighteen children with the electrophysiologic characteristics of ANSD with available imaging studies for review. INTERVENTIONS: Two neuroradiologists and a neurotologist reviewed each study, and consensus descriptions were established. MAIN OUTCOME MEASURES: The type and number of imaging findings were tabulated. RESULTS: Sixty-eight (64%) MRIs revealed at least 1 imaging abnormality, whereas selective use of CT identified 23 (55%) with anomalies. The most prevalent MRI findings included cochlear nerve deficiency (n = 51; 28% of 183 nerves), brain abnormalities (n = 42; 40% of 106 brains), and prominent temporal horns (n = 33, 16% of 212 temporal lobes). The most prevalent CT finding from selective use of CT was cochlear dysplasia (n = 13; 31%). CONCLUSION: Magnetic resonance imaging will identify many abnormalities in children with ANSD that are not readily discernable on CT. Specifically, both developmental and acquired abnormalities of the brain, posterior cranial fossa, and cochlear nerves are not uncommonly seen in this patient population. Inner ear anomalies are well delineated using either imaging modality. Because many of the central nervous system findings identified in this study using MRI can alter the treatment and prognosis for these children, we think that MRI should be the initial imaging study of choice for children with ANSD.

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OBJECTIVE: Review publications reporting radiation treatment of vestibular schwannomas (VS) and describe how data, patient characteristics, and study endpoints were reported. DATA SOURCES: PubMed search for English language articles on radiation treatment of VS published from January 2002 to July 2007. STUDY SELECTION: Studies presenting outcomes were selected, yielding 56 articles (58 studies) in journals of neurosurgery (30), oncology (18), otolaryngology (6), and other (2). DATA EXTRACTION/SYNTHESIS: Data included type of study, number of subjects, demographics, follow-up times, type of radiation, tumor size, tumor control definition, control rates, facial nerve function measure and outcome, type of hearing and vestibular testing and outcomes, and complications. Descriptive statistics were performed. RESULTS: Studies (72.9%) were retrospective reviews with stated sample sizes ranging from 5 to 829. Gamma-knife (49.2%), linear accelerator (35.6%), and proton beam (6.8%) were used with various doses. Average follow-up was less than 5 years in 79.6% of studies, and 67.4% included patients at less than or equal to 1 year. Tumor size was reported as diameter (23.7%), volume (49.2%), both (11.9%), other (3.4%), or not reported (11.9%). Definition of tumor control varied: less than or equal to 2 mm growth (22.0%), no visible/measurable change (16.9%), required surgery (10.2%), other (17.0%), and not clearly specified (33.9%). Facial nerve outcome was reported as House-Brackmann (64.4%), normal/abnormal (11.9%), other (1.7%), or was not reported (22%). CONCLUSION: The lack of uniform reporting criteria for tumor control, facial function and hearing preservation, and variability in follow-up times make it difficult to compare studies of radiation treatment for VS. We recommend consideration of reporting guidelines such as those used in otology for reporting VS resection results.

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OBJECTIVES/HYPOTHESIS: Surgical approaches to the pituitary have undergone numerous refinements over the last 100 years. The introduction of the endoscope and the advent of minimally invasive pituitary surgery (MIPS) have revolutionized pituitary surgery. This study aims to compile and evaluate outcomes of all of the MIPS performed at our institution. STUDY DESIGN: Retrospective, cases series of 176 consecutive patients undergoing MIPS. METHODS: Patient demographics, tumor characteristics, and intraoperative or postoperative complications for of 176 consecutive patients undergoing MIPS were complied. Statistical analysis for categorical variables and incidence across series were conducted using Pearson's chi test and Fisher's exact tests. Odd ratios were calculated to relate the discrete variables to outcomes and designing clinical prediction of risk. RESULTS: One hundred seventy-six patients who underwent 193 procedures. Pathologic evaluation revealed 147 of the tumors to be pituitary adenomas. Only one death occurred (mortality rate of 0.5%). The rate of diabetes insipidus occurred in 20.2% of the procedures. Vascular complications occurred in 5.2% of the procedures. Intraoperative cerebrospinal fluid (CSF) leaks were identified in 19.7% whereas postoperative CSF leak was noted in 10.3%. Resection of Rathke's cleft cyst correlated higher risk of both intraoperative and postoperative CSF leak (OR = 2.6, P <.001). Resection of tumors other than adenomata correlated with significantly higher risk of CSF leak (OR = 9.0, P = <.001). Sinusitis occurred after eleven resections (5.7%). Meningitis occurred in 2 of 193 resections (1.0%) in our series. Two neurologic complications occurred in our series, pneumocephalus and cranial neuropathy (1.0%). No other complications occurred. CONCLUSIONS: MIPS is a safe and efficacious marriage of the endoscope to the transsphenoidal approach. Thus, a brightness and clarity of vision is combined with the unique ability to explore the tumor bed with angled views and hydroscopy. Outcomes and complication rates comparable to traditional transsphenoidal approaches have resulted but with less dissection and tissue manipulation, reduced need for packing, and greater patient comfort and acceptance.

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OBJECTIVE: To describe the case of a true false-positive finding on magnetic resonance imaging, mimicking a vestibular schwannoma, and review the pertinent literature. PATIENTS: The case of a single patient with a suspicion of a vestibular schwannoma. INTERVENTION: A middle fossa craniotomy was performed for resection of the presumed schwannoma. MAIN OUTCOME MEASURES: Intraoperative findings and postoperative magnetic resonance imaging (MRI). RESULTS: : Intraoperative findings and a postoperative MRI confirm the absence of a neoplasm. CONCLUSION: False-positive findings on MRI, though rare, do occur. The clinician should have a high index of suspicion and consider reimaging in some cases.

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