Outcomes of Endoscopic Tympanoplasty for Large Perforations: A Multicenter Retrospective Study in South Korea.

OBJECTIVES: Endoscopic tympanoplasty (ET) provides minimally invasive transcanal access to the middle ear and improves middle ear visibility for the treatment of tympanic membrane (TM) perforations. However, the literature on surgical outcomes for large TM perforations is lacking and limited to small series. This study aimed to evaluate the clinical benefits of ET for large TM perforations. METHODS: This retrospective cohort study was conducted at nine tertiary referral hospitals in South Korea, where 252 patients who underwent ET as primary surgery from September 2019 to August 2021 were included. The outcome measures included the graft success rate and pre- and postoperative audiometric data. RESULTS: In 239 patients, the graft success rate of ET for large or subtotal perforations was 86.2% (206 patients), while the graft failure rate was 13.8% (33 patients). The graft failure rate was directly correlated with surgical techniques, including overlay and medial or lateral underlay tympanoplasty (P=0.027). Lateral underlay tympanoplasty showed the most favorable. RESULTS: Sex, laterality, etiology, site and size of perforation, operation time, and graft materials did not vary significantly between the graft success and failure groups (P>0.05). The mean air-bone gap (ABG) improved significantly in both groups (graft success group: 10.0±0.6 dB and graft failure group: 7.7±0.3 dB; P<0.001). However, the ABG improvement did not significantly differ between the groups. Analysis of covariance revealed that the postoperative 500-Hz bone conduction threshold improved after successful ET (adjusted coefficient, -11.351; 95% confidence interval, -21.491 to -1.212; P=0.028). CONCLUSION: This study involved the largest population to date of large TM perforations treated by ET. The study findings suggest that ET is feasible and effective in treating large TM perforations.

Outcomes of Endoscopic Congenital Cholesteatoma Removal in South Korea.

Importance: Transcanal endoscopic ear surgery (TEES) provides minimally invasive transcanal access to the middle ear and improves middle ear visibility during cholesteatoma resection. However, the literature on outcomes following TEES alone for the removal of congenital cholesteatoma (CC) is lacking and limited to small series. Objective: To assess outcomes of TEES for CC limited to the middle ear and/or mastoid antrum and to explore the risk factors associated with recidivism (ie, recurrent and/or residual cholesteatoma). Design, Setting, and Participants: This cohort study evaluated retrospective, multicenter data for 271 children with CC who underwent TEES at 9 tertiary referral hospitals in South Korea between January 1, 2013, and December 31, 2021, and had a follow-up of at least 6 months after surgery. Main Outcomes and Measures: Outcomes included the incidence of residual cholesteatoma and audiometric data after TEES. A multivariable analysis using Cox proportional hazards regression models was used to assess associations between cholesteatoma characteristics and recidivism, with hazard ratios (HRs) and 95% CIs reported. Results: Of the 271 patients (mean [SD] age, 3.5 [2.9] years; 194 [71.6%] boys, 77 [28.4%] girls), 190 had Potsic stage I CC (70.1%), 21 (7.7%) had stage II, 57 (21.0%) had stage III, and 3 (1.1%) had stage IV. Thirty-six patients (13.3%) with residual cholesteatoma were found, including 15 (7.9%) with Potsic stage I, 3 (14.3%) with stage II, and 18 (31.6%) with stage III. In the multivariable analysis, invasion of the malleus (HR, 2.257; 95% CI, 1.074-4.743) and posterosuperior quadrant location (HR, 3.078; 95% CI, 1.540-6.151) were associated with the incidence of recidivism. Overall, hearing loss (>25 dB on auditory behavioral test or >30 dB of auditory evoked responses) decreased from 24.4% to 17.7% after TEES. Conclusions and Relevance: This cohort study involved the largest known population to date of CC removed by TEES. The findings suggest that TEES may be feasible and effective for the removal of CC limited to the middle ear and/or mastoid antrum in children.

The ECRG4 cleavage product augurin binds the endotoxin receptor and influences the innate immune response during otitis media.

Otitis media (OM), the most common disease of childhood, is typically characterized by bacterial infection of the middle ear (ME). Prominent features of OM include hyperplasia of the ME mucosa, which transforms from a monolayer of simple squamous epithelium with minimal stroma into a full-thickness respiratory epithelium in 2-3 days after infection. Analysis of the murine ME transcriptome during OM showed down-regulation of the tumor suppressor gene Ecrg4 that was temporally related to mucosal hyperplasia and identified stromal cells as the primary ECRG4 source. The reduction in Ecrg4 gene expression coincided with the cleavage of ECRG4 protein to release an extracellular fragment, augurin. The duration of mucosal hyperplasia during OM was greater in Ecrg4 -/- mice, the number of infiltrating macrophages was enhanced, and ME infection cleared more rapidly. ECRG4-null macrophages showed increased bacterial phagocytosis. Co-immunoprecipitation identified an association of augurin with TLR4, CD14 and MD2, the components of the lipopolysaccharide (LPS) receptor. The results suggest that full-length ECRG4 is a sentinel molecule that potentially inhibits growth of the ME stroma. Processing of ECRG4 protein during inflammation, coupled with a decline in Ecrg4 gene expression, also influences the behavior of cells that do not express the gene, limiting the production of growth factors by epithelial and endothelial cells, as well as the activity of macrophages.

Prolonged Positional Downbeat Nystagmus in Benign Paroxysmal Positional Vertigo: A Case Report and Literature Review.

Purpose This study aimed to report an unusual case of benign paroxysmal positional vertigo (BPPV), who showed prolonged positional downbeat nystagmus without latency and was diagnosed with cupulolithiasis of the anterior canal (AC). We compared this case with one of typical AC-BPPV, and possible mechanisms underlying the atypical characteristics were discussed. Method Two patients diagnosed with AC-BPPV were reported. Positional testing using video-oculography goggles was performed, and outcomes were measured via medical records and analysis of videos of the nystagmus. Results Downbeat nystagmus was observed in the contralateral Dix-Hallpike test in both cases. The torsional component was subtle or absent, but motion was induced toward the affected ear. The two cases differed in latency and duration of vertigo, as well as habituation. The patient with atypical nystagmus showed little or no latency and longer duration. Moreover, there was no habituation on repeated tests. The nystagmus showed several differences from that of typical AC-BPPV. Conclusions Based on our case, AC-BPPV may induce various unusual clinical manifestations of nystagmus. Accurate diagnosis requires careful consideration of the patient's symptoms and the characteristics of the nystagmus. Supplemental Material https://doi.org/10.23641/asha.14265356.

IgG4-related disease presenting as otogenic skull base osteomyelitis.

IgG4-related disease (IgG4-RD) is an emerging clinical disease entity characterized by tumefactive lesions at multiple sites with a dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells. Although almost any organ can be affected, IgG4-RD is most likely to involve the submandibular, lacrimal, or parotid glands in the head and neck region. However, skull base involvement presenting as otogenic skull base osteomyelitis (SBO) is rare. We encountered a 70-year-old male with IgG4-RD presenting primarily with severe otalgia and otorrhea. He had uncontrolled diabetes mellitus and showed clinical manifestations of otogenic SBO. Tissue immunostaining revealed typical features of increased IgG4-positive plasma cells, and hematological examination showed elevated serum IgG4 concentrations. Treatment with corticosteroids significantly improved well-being and partially resolved the lesion based on computed tomography (CT) scan.

Macrophage Depletion in CCR2-/- Mice Delays Bacterial Clearance and Enhances Neutrophil Infiltration in an Acute Otitis Media Model.

BACKGROUND: Otitis media (OM) is a common and potentially serious disease of childhood. Although OM is multifactorial on origin, bacterial infection is a unifying component. Many studies have established a critical role for innate immunity in bacterial clearance and OM resolution. A key component of innate immunity is the recruitment of immune and inflammatory cells, including macrophages. METHODS: To explore the role of macrophages in OM, we evaluated the expression of genes related to macrophage function during a complete episode of acute OM in the mouse caused by middle ear (ME) inoculation with Haemophilus influenzae. We also combined CCR2 deficiency with chlodronate liposome toxicity to deplete macrophages during OM. RESULTS: Macrophage genes were robustly regulated during OM. Moreover, macrophage depletion enhanced and prolonged the infiltration of neutrophils into the infected ME and increased the persistence of bacterial infection. CONCLUSIONS: The results illustrate the critical role played by macrophages in OM resolution.

Pseudo-spontaneous nystagmus in lateral semicircular canal benign paroxysmal positional vertigo: Correlation with bow and lean test in a pitch plane.

OBJECTIVES: We investigated the incidence and characteristics of pseudo-spontaneous nystagmus (PSN) in benign paroxysmal positional vertigo involving the lateral semicircular canal (LC-BPPV) and evaluated the correlation between PSN and the bow and lean test. METHODS: We examined nystagmus in the sitting position using video-oculography goggles in 131 LC-BPPV patients. The positioning test and bow and lean test were also performed. Patients were divided into canalolithiasis and cupulolithiasis groups according to the character of nystagmus. In each group, the incidence and direction of PSN, correlation with the bow and lean test, and treatment outcome were analyzed. RESULTS: PSN was observed in 25 cases (19.1%) in LC-BPPV patients, 7 of which were canalolithiasis and 18 of which were cupulolithiasis (p = 0.098). Of the 25 patients with PSN, 21 (84%) exhibited nystagmus consistent with the lean test whereas 4 (16%) exhibited nystagmus consistent with the bow test. In patients with PSN, nystagmus was observed in the bow and lean test in all cases (23/23), but in patients without PSN, no nystagmus was observed in 13 cases (13/87) in the bow and lean test (p = 0.048). The number of barbecue maneuvers performed until the end of treatment was 1.4 ± 0.7 in patients with PSN and 1.4 ± 0.9 in those without PSN (p = 0.976). CONCLUSION: We identified PSN in patients with LC-BPPV irrelevant of subtype. Moreover, all patients with PSN showed nystagmus in the bow and lean test. The direction of PSN was mostly consistent with that of the lean test (21/25, 84%). The presence of PSN was not related to the treatment outcome in this study.

Periostin induces kidney fibrosis after acute kidney injury via the p38 MAPK pathway.

Periostin plays a crucial role in fibrosis, and acute kidney injury results in a high risk of progression to chronic kidney disease. Therefore, we hypothesized that periostin was involved in the progression of acute kidney injury to kidney fibrosis. Unilateral ischemia-reperfusion injury (UIRI) was induced in 7- to 8-wk-old male wild-type and periostin-null mice, and the animals were observed for 6 wk. In vitro, human kidney-2 cells and primary-cultured human tubular epithelial cells were incubated under hypoxic conditions (5% O2, 5% CO2, and 90% N2) for 5 days. The cells were also cultured with recombinant periostin (rPeriostin) and a p38 mitogen-activated protein kinase (MAPK) inhibitor in a hypoxic incubator. At 6 wk after UIRI, interstitial fibrosis/tubular atrophy was significantly alleviated in periostin-null mice compared with wild-type controls. In addition, periostin-null mice had attenuated expression of fibrosis/apoptosis markers and phosphorylated-p38 MAPK compared with wild-type controls. In vitro, hypoxic injury increased the expression of fibrosis markers, periostin, and phosphorylated-p38 MAPK, which was comparable to or substantially greater than their expression levels following treatment with recombinant transforming growth factor-ß1 under normoxic conditions. Furthermore, rPeriostin treatment under hypoxic conditions enhanced fibrosis/apoptosis markers and phosphorylated-p38 MAPK. In contrast, p38 MAPK inhibition ameliorated hypoxia-induced fibrosis, and the addition of the p38 MAPK inhibitor to rPeriostin significantly ameliorated the changes induced by rPeriostin. In conclusion, periostin promotes kidney fibrosis via the p38 MAPK pathway following acute kidney injury triggered by a hypoxic or ischemic insult. Periostin ablation may protect against chronic kidney disease progression.

Idiopathic benign paroxysmal positional vertigo in the elderly: a long-term follow-up study.

BACKGROUND: Benign paroxysmal positional vertigo (BPPV) is the most common cause of peripheral-type vertigo in the elderly. While some studies have investigated BPPV among the elderly, no study has focused on the isolated idiopathic BPPV (iBPPV) in the elderly. AIMS: To investigate the clinical features and recurrence rate in elderly patients diagnosed with iBPPV. METHODS: The authors prospectively reviewed the medical records of 627 patients diagnosed with BPPV, and a total of 370 patients fulfilled the inclusion criteria. The subjects were divided into two groups by age (experimental group ≥65 years and control group 17-64 years), and the gender, numbers of canalith reposition maneuver (CRM) for successful treatment, time elapsed from onset of symptoms to clinic visit, the affected semicircular canal, and the 1- and 5-year recurrence proportion were analyzed. RESULTS: The gender, numbers of CRM for successful treatment, affected semicircular canal, and the 1- and 5-year recurrence proportion showed no statistically significant difference between two groups (p > 0.05). The only exception was the experimental group which took a longer duration from manifestation of symptoms to hospital visit than control group (student's t test, p = 0.021). DISCUSSION: Several previous studies report that the elderly with iBPPV shows more protracted clinical course and much higher recurrence rate than younger adult. Unlike those reports, our study showed no significant differences about the clinical features and recurrence rate between age groups. CONCLUSION: The elderly with iBPPV could be treated as effective as general population.

Spontaneous temporomandibular joint herniation into the external auditory canal through a patent foramen of Huschke: A case report.

Temporomandibular joint herniation (TMJ) can be caused by inflammation, trauma, tumor, or otologic procedures. However, spontaneous TMJ herniation can also occur as a result of a congenital bony defect in the external auditory canal (EAC), known as the patent foramen of Huschke (PFH), and occurs in 0.4% of the population. Herein, the authors present a case of spontaneous TMJ herniation through the PFH with clicking tinnitus. The patient underwent the surgical repair of bony defect in the EAC with placement of titanium mesh, and the symptom disappeared after surgery. They also review the relevant literature regarding this disease and discuss its embryologic development and clinical significance.

Ethmoidal mucocele presenting as oculomotor nerve paralysis.

A 56-year-old male was admitted with an acute headache and sudden ptosis on the right side. No ophthalmological or neurological etiologies were apparent. A mucocele of the right posterior ethmoid sinus was observed with radiology. After the marsupialization of the mucocele via a transnasal endoscopic approach, the patient's symptoms (oculomotor nerve paralysis and headache) resolved in 4 weeks. Oculomotor paralysis is a rare symptom of an ethmoidal mucocele. In this article, we report this rare case along with a literature review.

Effect of high dose intravenous vitamin C on idiopathic sudden sensorineural hearing loss: a prospective single-blind randomized controlled trial.

The aim of this prospective single-blind randomized controlled study was to evaluate the therapeutic efficacy of high dose intravenous vitamin C (HDVC) added to systemic steroid in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). Between August 2010 and August 2011, 72 ISSNHL patients who participated in this study were randomly allocated to two groups: 36 to a control group, members of which were given systemic steroid treatment for 15 days, and 36 to a HDVC group, members of which were given HDVC (200 mg/kg/day) for 10 days in addition to steroid therapy followed by oral vitamin C (2,000 mg) for 30 days after discharge. Finally, we analyzed each group: 35 as a control group and 32 as a HDVC group. Auditory evaluations were performed by pure tone audiometry (PTA) before and ~1 month after treatment using Siegel's criteria. HDVC group showed significantly greater complete and partial recovery improvement (p = 0.035). In addition, the complete recovery rate in the HDVC group was more than twice that of the control group (p = 0.031). In the HDVC group, PTA improved from 67.6 ± 19.8 dB HL before treatment to 37.1 ± 28.8 dB HL at 1 month after treatment, whereas in the control group, PTA improved from 70.3 ± 12.4 to 47.6 ± 25.2 dB HL, which represented a significant intergroup difference (p = 0.030). In conclusion, HDVC may enhance hearing recovery in ISSNHL patients, which suggests that HDVC reduces levels of reactive oxygen metabolites produced by inner ear ischemia or inflammation, and that HDVC could be considered for the treatment of ISSNHL.

Periostin may play a protective role in the development of eosinophilic chronic rhinosinusitis with nasal polyps in a mouse model.

OBJECTIVES/HYPOTHESIS: Several genes have been reported to be upregulated in human nasal polyps in previous genetic analyses. Among these genes, periostin is known to be overexpressed in nasal polyps obtained from aspirin-sensitive patients. Using periostin-null mice, we investigated the role of periostin in a murine model of eosinophilic rhinosinusitis with nasal polyps. STUDY DESIGN: Animal study. METHODS: Eosinophilic rhinosinusitis was induced in both periostin-null and wild-type mice according to previously established protocols. In brief, ovalbumin (OVA) was used for sensitization and prolonged intranasal stimulation. Staphylococcus aureus enterotoxin B was applied intranasally to develop polyplike lesions. To examine the inflammation and mucosal lesions, hematoxylin and eosin, Sirius red, and Giemsa staining were performed. RESULTS: There was no definite difference in the maximal mucosal thickness between periostin-null and wild-type mice. In contrast, some parameters of inflammation, including the number of polyplike lesions and mast cells, were aggravated in the periostin-null mice compared to wild type. Eosinophilic infiltration was aggravated in the OVA-stimulated periostin-null mice, compared to OVA-stimulated wild-type mice, whereas there was no apparent difference between wild-type and periostin-null mice challenged with additional S aureus enterotoxin B. CONCLUSIONS: The loss of periostin appears to enhance polyplike lesion formation and mast cell infiltration in a mouse model of eosinophilic rhinosinusitis with nasal polyps.

Molecular and clinical characterization of the variable phenotype in Korean families with hearing loss associated with the mitochondrial A1555G mutation.

Hearing loss, which is genetically heterogeneous, can be caused by mutations in the mitochondrial DNA (mtDNA). The A1555G mutation of the 12S ribosomal RNA (rRNA) gene in the mtDNA has been associated with both aminoglycoside-induced and non-syndromic hearing loss in many ethnic populations. Here, we report for the first time the clinical and genetic characterization of nine Korean pedigrees with aminoglycoside-induced and non-syndromic hearing loss. These Korean families carry in the A1555G mutation of 12S rRNA gene and exhibit variable penetrance and expressivity of hearing loss. Specifically, the penetrance of hearing loss in these families ranged between 28.6% and 75%, with an average of 60.8%. These results were higher than the 29.8% penetrance that was previously reported in a Chinese population but similar to the 65.4% and 54.1% penetrance observed in a large Arab-Israeli population and nineteen Spanish pedigrees, respectively. The mutational analysis of the complete mtDNA genome in these families showed that the haplogroups of the Korean population, which belongs to the eastern Asian population, were similar to those of the Chinese population but different from the Spanish population, which belongs to the European-Caucasian population. The mtDNA variants that may act as modifier factors were also found to be similar to the Chinese population. Although the mtDNA haplogroups and variants were similar to the eastern Asian population, we did find some differing phenotypes, although some subjects had the same variants. This result suggests that both the ethnic background and environmental factors lead to a variable phenotype of the A1555G mutation.

Roles of periostin in symptom manifestation and airway remodeling in a murine model of allergic rhinitis.

PURPOSE: Periostin was originally identified as a secreted factor during screening of a mouse osteoblastic library. In a recent study, periostin was found to directly regulate eosinophil accumulation in allergic mucosal inflammation. Chronic eosinophilic inflammation is related to the development of remodeling. The present study examined the expression of periostin and evaluated its role in the inflammatory process and remodeling associated with allergic rhinitis. METHODS: A murine model of allergic rhinitis was established in periostin knockout mice. We analyzed the expression of periostin, manifestation of nasal symptoms, eosinophilic inflammation, and subepithelial fibrosis as well as the expression of MMP-2, TIMP-1, and type 1 collagen in nasal tissue. RESULTS: Periostin was mainly distributed in the subepithelial tissue of the nasal mucosa. The subepithelial tissue was thinner in the knockout group than in the control group. No differences in the expression of MMP-2 or TIMP-1 were found in the knockout group. However, after a month of allergen challenge, type I collagen in the nasal tissue was lower in the knockout group than in the control group. The number of eosinophils and the symptom score were also lower in the knockout group. CONCLUSIONS: Periostin is expressed in nasal tissues of murine models of allergic rhinitis. Periostin deficiency may affect the remodeling of nasal tissue with reduced subepithelial fibrosis, and lead to less eosinophilic inflammation.

Clinical characteristics and natural course of recurrent vestibulopathy: a long-term follow-up study.

OBJECTIVES/HYPOTHESIS: To investigate the clinical characteristics and the natural course of recurrent vestibulopathy (RV). STUDY DESIGN: Retrospective study. METHODS: During the period April 2002 to February 2008, we reviewed the clinical records of 98 patients diagnosed with RV. All patients were approached by telephone and using a questionnaire. The analysis included age, sex distribution, natural history, pure-tone audiometry, caloric response, age at onset, and the characteristics of vertigo. RESULTS: Median follow-up was 63.1 months (range, 24-103 months). Patients had a mean age at onset of 39 years and a mean duration of 4.2 years. An obvious female predilection was found, and unilateral caloric paresis (≥ 25%) was seen in 35%. Of the 98 patients, symptoms resolved in 82% but were unchanged in 12%. RV developed to Ménière's disease in four patients and to migraine in two. No patient with RV developed a central nervous system disease or benign paroxysmal positional vertigo during follow-up. CONCLUSIONS: The study suggests that in the majority of cases, vertigo spontaneously resolves and that the risks of development to Ménière's disease or migraine are low.

Endoscope-assisted intraoral resection of external dermoid cyst.

BACKGROUND: Surgical removal of a dermoid cyst is usually accomplished through an external neck incision. However, this procedure inevitably results in a neck scar. METHODS: We report the case of a 17-year-old woman with a submental mass. We implemented a modified approach to dermoid cyst removal through the floor of the mouth using an endoscope system. RESULTS: The patient received a modified approach to dermoid cyst removal and remains free of disease 6 months after excision. CONCLUSION: Resection of the submental type dermoid cyst can be performed by an intraoral endoscope-assisted approach through the floor of the mouth. We describe the procedure of the endoscope-assisted intraoral resection.

Sensation recovery of auricle following chronic ear surgery by retroauricular incision.

The aim of this prospective analysis was to objectify and quantify the sensory loss in the auricular area that occurs following surgery for chronic otitis media or cholesteatoma, and to assess the exact recovery time of the auricular sensation. Till now, no study has yet been conducted on the sensory loss that is developed after retroauricular incision is performed for chronic ear surgery. Forty-eight patients underwent surgery via retroauricular incision for chronic otitis media or for chronic otitis media or cholesteatoma between March 2009 and January 2010. The skin around the auricle was divided into six areas. The sensation of each area was assessed before the surgery and 3 days, 7 days, 1 month, 3 months, and 6 months after the surgery, using the Semmes-Weinstein monofilaments. The patients were also asked to record the sensation that they felt using the visual analog scale (VAS). In the objective sensory assessment, only area 5, which corresponds to the retroauricular area, showed significant sensory loss. Three months after the surgery, the sensation was recovered to a level comparable to that before the surgery in most of the patients. The mean subjective VAS score was 10 prior to the surgery, 8.56 (± 1.08) 3 months after the surgery, and 9.32 (± 0.74) 6 months after the surgery, respectively. In conclusion, following chronic ear surgery, the sensation of the auricle was recovered to the previous level within 3 months. Therefore, patients who are to undergo retroauricular incision should be informed that they will experience temporary sensory loss for approximately 3 months after the surgery.

Immunohistochemical and biomolecular identification of melatonin 1a and 1b receptors in rat vestibular nuclei.

OBJECTIVE: The aim of this study was to examine the localizations and expressions of melatonin 1a (MT1a) and 1b (MT1b) receptors in rat vestibular nuclei by immunohistochemical staining and reverse transcriptase-polymerase chain reaction. MATERIALS AND METHODS: Twenty male Sprague-Dawley rats were used in this study. Antibodies for the MT1a and MT1b receptors were used in 10 rats, respectively. A further 10 animals were sacrificed for RT-PCR. Tissues containing medial vestibular nuclei were selectively isolated from brain stem slices for RT-PCR. RESULTS: MT1a and MT1b receptor immunopositive neurons were found to be distributed throughout the four major vestibular nuclei. Both receptors were primarily detected in neuronal somata and their proximal dendrites. The presences of the mRNAs of the MT1a and MT1b receptors were confirmed by RT-PCR in medial vestibular nuclei and trigeminal ganglia. CONCLUSIONS: The present study demonstrates, for the first time, that MT1a and MT1b receptors are localized and expressed in rat vestibular nuclei. This study provides additional insight into the role of melatonin receptors during vestibular signal processing.