Comparison of Intratympanic, Systemic, and Combined Steroid Therapies for Sudden Sensorineural Hearing Loss in Patients with Diabetes Mellitus / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES@#We investigated whether the intratympanic steroid injections might be an effective initial treatment for sudden sensorineural hearing loss (SSNHL) in patients with diabetes mellitus (DM).SUBJECTS AND METHOD: We assessed a total of 167 patients with DM and SSNHL who visited a tertiary referral otolaryngology department between January 2010 and April 2018. Forty-two patients with DM and SSNHL received intratympanic steroid injections; 48 patients with DM and SSNHL received systemic steroid treatment; and 77 patients with DM and SSNHL received a combination of systemic and intratympanic treatment. Initial and post treatment hearing levels and fasting blood sugar (FBS) were assessed, and correlations between hearing gain and the duration of DM, HbA1c, FBS were investigated.@*RESULTS@#After steroid treatment, hearing levels were 38.87±25.35 dB in the intratympanic injection group, 41.09±28.49 dB in the systemic steroid treatment group, and 47.81±27.12 dB in the combined treatment group. Final hearing levels and hearing gain in the three groups did not differ significantly. FBS after treatment in the systemic steroid treatment and combined steroid treatment group worsened relative to the intratympanic injection group (202.00±9.40 mg/dL to 326.63±7.85 mg/dL). FBS, duration of DM, and HbA1c levels did not affect the hearing gain in patients with DM and SSNHL.@*CONCLUSION@#Intratympanic steroid injections are comparable to the systemic steroid treatment modality for SSNHL in patients with DM. Thus, intratympanic steroid injections may serve as an effective initial treatment modality for SSNHL in patients with DM.

Microscopic versus Endoscopic Inlay Butterfly Cartilage Tympanoplasty

BACKGROUND AND OBJECTIVES: Inlay butterfly cartilage tympanoplasty makes the graft easy, and reduces operating time. The present study aimed to investigate the outcomes of microscopic versus endoscopic inlay butterfly cartilage tympanoplasty. SUBJECTS AND METHODS: In this retrospective study, the outcomes of 63 patients who underwent inlay butterfly cartilage tympanoplasty with small to medium chronic tympanic membrane perforation were evaluated. Twenty-four patients underwent conventional microscopic tympanoplasty and 39 underwent endoscopic tympanoplasty. The outcomes were analyzed in terms of the hearing gain and graft success rate. RESULTS: The surgical success rate was 95.8% in the patients who underwent conventional microscopic tympanoplasty and 92.3% in those who underwent endoscopic tympanoplasty. In both groups of patients, the postoperative air-bone gap (ABG) was significantly lower than the preoperative ABG. There were no significant differences between the preoperative and postoperative ABG values in either group. CONCLUSIONS: Endoscopic inlay tympanoplasty using the butterfly cartilage technique appears to be an effective alternative to microscopic tympanoplasty and results in excellent hearing.

Outcomes of Modified Canal Wall Down Mastoidectomy and Mastoid Obliteration Using Autologous Materials

OBJECTIVES: The traditional canal wall down mastoidectomy (CWDM) procedure commonly has potential problems of altering the anatomy and physiology of the middle ear and mastoid. This study evaluated outcomes in patients who underwent modified canal wall down mastoidectomy (mCWDM) and mastoid obliteration using autologous materials. METHODS: Our study included 76 patients with chronic otitis media, cholesteatoma, and adhesive otitis who underwent mCWDM and mastoid obliteration using autologous materials between 2010 and 2015. Postoperative hearing air-bone gap and complications were evaluated. RESULTS: During the average follow-up of 64 months (range, 20 to 89 months), there was no recurrent or residual cholesteatoma or chronic otitis media. No patient had a cavity problem and anatomic integrity of the posterior canal wall was obtained. There was a significant improvement in hearing with respect to the postoperative air-bone gap (P<0.05). A retroauricular skin depression was a common complication of this technique. CONCLUSION: The present study suggests that our technique can prevent various complications of the classical CWDM technique using autologous tissues for mastoid cavity obliteration. It is also an appropriate method to obtain adequate volume for safe obliteration.

Comparison of Intratympanic, Systemic, and Combined Steroid Therapies for Sudden Sensorineural Hearing Loss in Patients with Diabetes Mellitus / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: We investigated whether the intratympanic steroid injections might be an effective initial treatment for sudden sensorineural hearing loss (SSNHL) in patients with diabetes mellitus (DM). SUBJECTS AND METHOD: We assessed a total of 167 patients with DM and SSNHL who visited a tertiary referral otolaryngology department between January 2010 and April 2018. Forty-two patients with DM and SSNHL received intratympanic steroid injections; 48 patients with DM and SSNHL received systemic steroid treatment; and 77 patients with DM and SSNHL received a combination of systemic and intratympanic treatment. Initial and post treatment hearing levels and fasting blood sugar (FBS) were assessed, and correlations between hearing gain and the duration of DM, HbA1c, FBS were investigated. RESULTS: After steroid treatment, hearing levels were 38.87±25.35 dB in the intratympanic injection group, 41.09±28.49 dB in the systemic steroid treatment group, and 47.81±27.12 dB in the combined treatment group. Final hearing levels and hearing gain in the three groups did not differ significantly. FBS after treatment in the systemic steroid treatment and combined steroid treatment group worsened relative to the intratympanic injection group (202.00±9.40 mg/dL to 326.63±7.85 mg/dL). FBS, duration of DM, and HbA1c levels did not affect the hearing gain in patients with DM and SSNHL. CONCLUSION: Intratympanic steroid injections are comparable to the systemic steroid treatment modality for SSNHL in patients with DM. Thus, intratympanic steroid injections may serve as an effective initial treatment modality for SSNHL in patients with DM.

Tumor Growth and Hearing Changes in the Natural Progress of Vestibular Schwannoma / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES@#Vestibular schwannoma is a benign neoplasm originating from the vestibular nerve, and the most common symptom caused by the tumor is unilateral hearing loss. This study is aimed to review the clinical outcomes of tumor growth and hearing changes in the natural progress of vestibular schwannoma.SUBJECTS AND METHOD: We retrospectively reviewed 28 patients who were diagnosed with vestibular schwannoma and treated with the ‘wait and scan’ modality for more than a year. We analyzed the patients' audiological changes and tumor growth by reviewing the temporal bone MR images. Patients were classified into an intrameatal group and extrameatal group according to the involvement of the cerebropontine angle.@*RESULTS@#The overall mean follow-up was 45.6±25.8 months. Among the 28 patients, 6 (21.4%) showed tumor growth after ‘wait and scan.’ Hearing thresholds and speech discrimination scores showed deterioration after ‘wait and scan’ in both groups. Among the 12 patients with serviceable hearing at initial diagnosis, 9 (75.0%) preserved serviceable hearing after ‘wait and scan.’ When the pure tone average and speech discrimination scores were applied to the scattergram, most values were positioned near the center of the scattergram, which implied slight changes after ‘wait and scan.’@*CONCLUSION@#The percentage of patients showing tumor growth after the ‘wait and scan’ policy was low. Patients with serviceable hearing at diagnosis would most likely preserve their hearing after ‘wait and scan.’ Considering the limited tumor growth and minimal hearing changes, rather than microsurgery or radiation surgery, the conservative ‘wait and scan’ policy can be an alternative treatment modality in patients with small-sized, non-growing vestibular schwannomas.

Evaluation of the Self-Testing Blood Glucose Monitoring System GlucoDr.S According to ISO 15197:2013 Guidelines

BACKGROUND: The performance of the self-monitoring of blood glucose in patients with diabetes should be properly evaluated to ensure strict glycemic control. This study evaluated the self-testing Blood Glucose Monitoring System GlucoDr.S™ (All Medicus Co., Ltd., Korea). METHODS: This study recruited 120 patients. Use of the glucometer was evaluated according to ISO 15197:2013 guidelines. The YSI 2300 STAT PLUS Glucose Analyzer (YSI Life Sciences, USA) was used as the reference device. RESULTS: The standard deviation and coefficients of variation ranges for measurement repeatability and intermediate measurement precision conducted with 10 meters and 3 reagent lots on the same day were 2.7–3.2 mg/dL (0.99. The influence effect of hematocrit and the 24 interference agents was not significant, except for xylose. A system accuracy test was conducted with 100 subjects taking duplicate measurements from each of the 3 reagent lots. When glucose levels were 95% of the samples were within ±15 mg/dL and within ±15% of the average measured values of the reference measurement, respectively. In Consensus Error grid analysis, all results were distributed in zone A and B. The results of the user performance evaluation using 115 lay persons were also included in the acceptance range. CONCLUSION: The GlucoDr.S™ showed acceptable performance according to the ISO 15197:2013 guidelines and could be a clinically useful self-testing glucometer.

Vestibular Function and Prognosis of Sudden Sensorineural Hearing Loss with Vertigo / 대한평형의학회지

OBJECTIVES: Vertigo combined with sudden sensorineural hearing loss (SSNHL) is known as a poor prognostic factor. We investigated clinical findings and vestibular function tests in patients of SSNHL with vertigo to find the prognostic factors. METHODS: We retrospectively evaluated data on the patients diagnosed with SSNHL with vertigo at Bucheon Soonchunhyang University Hospital from March 2009 to February 2018. We reviewed medical records and the results of vestibular function tests and audiometry. RESULTS: Of the 68 patients, 30 (44.1%) showed profound hearing loss and 53 (77.9%) showed poor recovery. Age and the degree of initial hearing loss showed negative prognostic factor in hearing recovery. Abnormal results of cervical vestibular evoked myogenic potentials (cVEMP) also showed significantly differences between good and poor recovery groups. CONCLUSIONS: In this study, most of the patients of SSNHL with vertigo showed poor recovery. Age, degree of initial hearing loss, and the abnormal result of the cVEMP have a negative effect on the prognosis of hearing recovery.

Computed Tomography and Magnetic Resonance Imaging Evaluation in Pediatric Unilateral Sensorineural Hearing Loss / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Children with unilateral sensorineural hearing loss (USNHL) are not actively evaluated by physicians. The diagnostic tool for evaluation of USNHL is also controversial, and no strategy for diagnosing USNHL through imaging studies has been established. We examined the results of temporal bone computed tomography (TBCT) imaging and magnetic resonance imaging (MRI) studies on children with USNHL. SUBJECTS AND METHOD: Eighty-nine patients with USNHL were reviewed. Of these patients, 21 underwent both TBCT and MRI, 51 underwent temporal MRI only, and 17 underwent TBCT only. RESULTS: The etiology of USNHL were determined through imaging studies in 20 patients. The most common abnormal finding (65%) was a narrow internal auditory canal identified on TBCT and cochlear nerve aplasia on temporal MRI. Incomplete partition (20%), common cavity (10%), and labyrinthitis ossificans (5%) were also observed in imaging studies. The hearing threshold was lower in USNHL patients with normal findings (76.1±28.7 dB) than in USNHL patients with abnormal findings on TBCT or temporal MRI (100.1±22.3 dB). CONCLUSION: Cochlear and cochlear nerve abnormalities can be detected through imaging studies in approximately 25% of patients with USNHL. Therefore, we suggest that children should undergo TBCT when USNHL is confirmed through audiologic evaluation.

Limitation of High Pitch Sound Perception in Nontumor Patients with Auditory Brainstem Implantation / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Auditory brainstem implantation (ABI) is another option for hearing rehabilitation in non-neurofibromatosis type 2 patients who cannot undergo cochlear implantation (CI). However, the average performance of ABI is worse than that of CI. We analyzed the psycho-electrical parameters of each electrode and psycho-acoustic response to different frequency sounds in nontumor patients with ABI. SUBJECTS AND METHOD: Sixteen patients with ABI from July 2008 to May 2013 were included in the study. They were followed up for 4 to 56 months. Among them, 12 were prelingual deaf with a narrow internal auditory canal or cochlear ossification. The remaining four were post-lingual deaf adults with severely ossified cochleae. We analyzed the electrical parameters [impedance, threshold level (T level), and dynamic range] of each of the 12 electrodes. We also evaluated the sound field pure-tone threshold, Ling 6 sound detection-identification test (Ling 6 test), and pitch ranking data of these patients. RESULTS: The impedance, T level, and dynamic range did not significantly differ among electrodes. However, the pure-tone threshold to sound field stimulation was elevated in the high tone area, where more variables were found than in the low frequency area. Patients could not identify /S/ and /Sh/ sounds in the Ling 6 test. The mean T level and the dynamic range of the three highest pitch-perceiving electrodes in each patient was higher and narrower, respectively, than those of the three lowest pitch-perceiving electrodes. CONCLUSION: The nontumor patients with ABI have difficulty perceiving high pitch sound. More sophisticated penetrating type electrodes and, if possible, bimodal stimulation with CI, could be considered.

The Comparison of Short-Term Therapeutic Effects of Acute Low-Tone Sensorineural Hearing Loss according to Steroid Capacity / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Acute low-tone sensorineural hearing loss refers to a condition wherein thresholds increase only at low frequencies in pure-tone audiometry. Systemic steroid treatment is commonly used for acute low-tone sensorineural hearing loss without vertigo, but there has been no established treatment in most clinical researches. The purpose of this study is to compare and analyze the short-term effects of high- and half-dose oral steroid therapy on patients suffering from acute low-tone sensorineural hearing loss without vertigo. SUBJECTS AND METHOD: Forty-six patients diagnosed with acute low-tone sensorineural hearing loss were divided into two groups and treated with high-dose (24 patients) and half-dose (22 patients) steroids, respectively. A retrospective analysis was conducted on their medical records. RESULTS: There were no significant differences between the two groups in any of the following: age, sex ratio, duration of symptoms before treatment, pure-tone hearing thresholds, low frequency hearing thresholds and speech intelligibility. However, both groups showed significant differences in hearing thresholds before and after treatment. In addition, no significant difference was noted in the recovery and recurrence rate between the two groups. CONCLUSION: According to the results of this study, there is no difference in treatment effect between the high- and half-dose groups. Therefore, half-dose steroids may be used as a treatment option for acute low tone sensorineural hearing loss.

Computed Tomography and Magnetic Resonance Imaging Evaluation in Pediatric Unilateral Sensorineural Hearing Loss / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES@#Children with unilateral sensorineural hearing loss (USNHL) are not actively evaluated by physicians. The diagnostic tool for evaluation of USNHL is also controversial, and no strategy for diagnosing USNHL through imaging studies has been established. We examined the results of temporal bone computed tomography (TBCT) imaging and magnetic resonance imaging (MRI) studies on children with USNHL.SUBJECTS AND METHOD: Eighty-nine patients with USNHL were reviewed. Of these patients, 21 underwent both TBCT and MRI, 51 underwent temporal MRI only, and 17 underwent TBCT only.@*RESULTS@#The etiology of USNHL were determined through imaging studies in 20 patients. The most common abnormal finding (65%) was a narrow internal auditory canal identified on TBCT and cochlear nerve aplasia on temporal MRI. Incomplete partition (20%), common cavity (10%), and labyrinthitis ossificans (5%) were also observed in imaging studies. The hearing threshold was lower in USNHL patients with normal findings (76.1±28.7 dB) than in USNHL patients with abnormal findings on TBCT or temporal MRI (100.1±22.3 dB).@*CONCLUSION@#Cochlear and cochlear nerve abnormalities can be detected through imaging studies in approximately 25% of patients with USNHL. Therefore, we suggest that children should undergo TBCT when USNHL is confirmed through audiologic evaluation.

Analysis of Prognostic Factors in Malignant External Otitis

OBJECTIVES: Malignant external otitis (MEO) is a potentially fatal infection of the external auditory canal, temporal bone, and skull base. Despite treatment with modern antibiotics, MEO can lead to skull base osteomyelitis. Until now, there have been few studies on the prognostic factors of MEO. METHODS: We performed a retrospective study to identify prognostic factors of MEO, and a meta-analysis of other articles investigating MEO. On the basis of disease progression the 28 patients in our study were divided into ‘controlled’ and ‘uncontrolled’ groups, consisting of 12 and 16 patients, respectively. We identified three categories of prognostic factors: those related to patient, disease, and treatment. We compared these prognostic factors between the controlled and uncontrolled groups. RESULTS: In our study, the duration of diabetes mellitus (DM), presence of inflammatory markers (C-reactive protein and erythrocyte sedimentation rate), and computed tomography or magnetic resonance imaging findings influenced the prognosis of MEO. In contrast, prognosis was unrelated to age, gender, mean glucose level, hemoglobin A1c level, pathogen, comorbidity, or cranial nerve involvement. No factor related to treatment modality was correlated with prognosis, such as surgery, steroid therapy, or interval to the first appropriate treatment. Cranial nerve involvement has been proven to be associated with disease progression, but the relationship between cranial nerve involvement and the prognosis of MEO remains controversial. As a part of this study, we conducted a meta-analysis of cranial nerve involvement as a prognostic factor of MEO. We found that cranial nerve involvement has a statistically significant influence on the prognosis of MEO. CONCLUSION: We found that glycemic control in diabetes mellitus, cranial nerve involvement, and the extent of disease determined from various imaging modalities influence the prognosis of MEO. We suggest that significant prognostic factors should be monitored to determine the prognosis of patients with MEO.

A Case of Recurrent Vertigo in Vestibular Schwannoma Treated with Chemical Labyrinthectomy / 대한평형의학회지

Vestibular schwannoma (VS) can have various symptoms and almost half of the patients have balance problems. Some patients with VS can suffer from recurrent vertigo mimicking Meniere's disease. We present the case of a 61-year-old male patient who was suffering from recurrent vertigo due to VS while symptoms improved by chemical labyrinthectomy with gentamicin. We consider that chemical labyrinthectomy can be an option in the treatment of patients with recurrent vertigo for VS.

The Difference of Clinical Symptoms and Audiovestibular Function between Intrameatal and Extrameatal Acoustic Neuroma / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Various hearing tests and vestibular function tests are used to diagnose acoustic neuroma. We analyzed the clinical characteristics and the results of audiovestibular function tests between patients of intrameatal and extrameatal acoustic neuroma. SUBJECTS AND METHOD: Reviewing the medical records for 64 patients with acoustic neuroma between March 2007 and February 2014, we divided the patients into two groups, intrameatal (31 patients) and extrameatal acoustic neuroma (33 patients) according to the involvement of cerebropontine angle. We compared the clinical characteristics, pure tone audiograms, speech audiometry, caloric test, and vestibular evoked myogenic potential (VEMP) between the two groups. RESULTS: While hearing loss was the most frequent presenting symptom in patients with intrameatal acoustic neuroma, dizziness was the most common symptom in patients with extrameatal acoustic neuroma. Hearing thresholds measured by pure tone audiometry and speech discrimination scores were significantly worse for patients with extrameatal acoustic neuroma. Abnormal unilateral canal paresis of caloric test was significantly higher for extrameatal acoustic neuroma than for intrameatal acoustic neuroma. Most patients with acoustic neuroma showed abnormal findings in the VEMP test, but the number of patients between intrameatal and extrameatal acoustic neuroma did not differ significantly. CONCLUSION: Clinical symptoms and the results of audiovestibular function tests differed according to the tumor size of acoustic neuroma. Physicians should counsel patients presenting with audiovestibular symptoms of the possibility of acoustic neuroma.

Multicenter Randomized Study on the Efficacy of Isosorbide in Patients with Mèniére's Disease / 대한평형의학회지

OBJECTIVES: Mèniére's disease is one of the most common causes of episodic vestibular syndrome that shows symptom complexes of recurrent vertigo, hearing loss, tinnitus and ear fullness. Isosorbide (Isobide) is a osmotic diuretics which has been used for Meniere's disease to reduce the endolymphatic pressure. The purpose of this study was to evaluate the efficacy of isosorbide in the treatment of Mèniére's disease by comparing the combined therapy of isosorbide and betahistine (Meniace) to betahistine only. METHODS: Among 220 patients enrolled with Mèniére's disease from 9 centers, 187 patients completed this clinical study. Patients were randomly subjected either to betahistine alone therapy at dose of 6 mg three times a day (n=97) or to combined therapy with isosorbide (dose of 30 mL three times a day) and betahistine (n=90) for 12 weeks. Two groups were compared at 4 and 12 weeks after treatment on frequency of vertigo, hearing level (pure tone audiometry, speech audiometry), electocochleography (ECoG), tinnitus (Tinnitus Handicap Inventory, THI) and quality of life (Korean functional level scale, Korean dizziness handicap inventory). RESULTS: During first 4 weeks after treatment, the frequency of vertigo was not reduced in either betahistine alone therapy group or combined therapy group. However, between 8 and 12 weeks, the frequency of vertigo was significantly reduced in either group, and in the combined group frequency of vertigo was more significantly reduced than in the betahistine alone therapy group (p=0.041). The hearing level, ECoG, tinnitus and quality of life was not significantly different between two groups. CONCLUSION: Isosorbide and betahistine combined therapy were more effective for vertigo control than betahistine alone therapy. Isosorbide is an effective diuretic in vertigo control in definite Mèniére's disease.

A Case of Middle Ear Implantation with Vibroplasty Coupler at the Stapes / 대한이비인후과학회지

Middle ear implantation is an effective treatment for sensorineural, conductive and mixed hearing loss. The active middle ear implant's floating mass transducer (FMT) is placed on the middle ear structure or coupled to the inner ear fluid. Since the device is not directly placed on the external auditory canal, patients are free from feedback phenomenon and occlusion effect resulting from hearing aids. A 66-year-old patient suffered from bilateral hearing loss. The patient had difficulties in adapting to a conventional hearing aid because of feedback phenomenon in the right ear. The patient underwent a middle ear implantation using a CliP-Coupler to connect stapes head and FMT in the left ear. To our knowledge, this is the first case reported in Korea on middle ear implantation with CliP-Coupler at the stapes, for which hearing results were successful at postoperative 7 months.

SLC26A4 Mutations in Korean Population / 대한이비인후과학회지

SLC26A4 mutations are common cause of congenital hearing loss in East Asia. The carrier frequency of SLC26A4 mutations is 1 in 75 in Korean populations. The SLC26A4 mutation spectrum varies according to the population. The most common mutation in Korean is replacement of histidine by arginine at codon 723 followed by exchange of guanine for adenine at the consensus acceptor splice site of intron 7, adenine to guanine change at position +3 transition donor splice site of intron 9, methionine to valine at position 147, and frameshift mutation by insertion T at N-terminal 2. Recent studies analyzed the genotype-phenotype correlation of SLC26A4 mutation and suggested that surface expression ratio of pendrin and residual anion exchange activity was related to the genotype of SLC26A4 mutations. The targeted drug to Korean SLC26A4 mutations would be helpful in preserving hearing in patients with SLC26A4 mutations.

A Case of Cerebellar Arteriovenous Malformation with Positional Down Beat Nystagmus / 대한평형의학회지

Brain arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system in the central nervous system. The first symptom of AVM is intracranial hemorrhage, followed by headache and seizure. However, dizziness as a symptom of AVM is very rare. We report a 16-year-old female patient who presented with disequilibrium. She showed down-beating nystamgus on Dix-Hallpike test and falling tendency on Romberg test with eyes open. Magnetic resonance image showed cerebellar AVM.

A Case of Inflammatory Pseudotumor in Temporal Bone Treated with Methotraxate / 대한이비인후과학회지

Inflammatory pseudotumor is a pathologically benign condition, but it demonstrates a wide range of clinical features ranging from silent small sized tumors to aggressive features mimicking malignancy. Pseudotumors most commonly occur in the orbital area, and the involvement of the middle ear cavity is extremely rare. Several modalities are known for the treatment of pseudotumors, including complete surgical excision, oral steroid therapy, and radiation therapy. We describe a 35-year-old woman with inflammatory pseudotumor involving the middle ear cavity. The patient was treated with canal wall up tympanomastoidectomy and additional treatments with steroid and radiation therapy. However, she showed side effects to high dose steroid treatment and no response to radiation therapy. Therefore, we decided to use methotrexate with low dose steroid. After treatment, symptoms were completely resolved and there was no evidence of recurrence 1 year after maintaining immunosuppressant treatment.

Two Cases of Malleostapedotomy in Congenital Oval Window Atresia

Congenital anomaly of the oval window with an abnormal facial nerve course is an uncommon embryological defect, which is related to the underdevelopment of second branchial arch derivatives. Some treatments for improving hearing levels are available; these include hearing aids, vestibulotomy, neo-oval window formation, and stapes surgeries, including incudostapedotomy and malleostapedotomy. However, surgery for congenital anomalies of the oval window has rarely been described, usually in very small series of patients. We describe two cases of congenital anomalies of the oval window with aberrant facial nerve courses. One was a 40-year-old male diagnosed with unilateral congenital oval window atresia; the other was a 10-year-old male diagnosed with bilateral congenital oval window atresia. We also describe the clinical manifestations and treatment outcomes of malleostapedotomy for congenital anomalies of the oval window with aberrant facial nerve courses.