Symmetric Bilateral Congenital Middle Ear Cholesteatoma: A Case Report

Congenital middle ear cholesteatoma (CMEC) accounts for 2%-5% of all cases of middle ear cholesteatoma. CMEC is rare and diagnostically challenging; therefore, only a few cases are reported in the literature, and the pathophysiology of this condition remains largely unclear. Currently, epidermoid formation, amniotic fluid contamination, lack of the tympanic ring, and squamous metaplasia are among the mechanisms implicated in the pathogenesis of CMEC; however, no single theory satisfactorily explains the pathophysiology of this disorder. We report a case of CMEC in a young male patient, who showed a symmetric, binaural whitish mass posterior to the anterosuperior quadrant of the tympanic membrane. A few reports have described bilateral CMEC; however, no study has reported symmetrical CMEC as observed in this case. Bilateral tympanoplasty concomitant with cholesteatoma removal was performed, and histopathological evaluation of the resected specimen showed closed-type cholesteatoma. In this report, we describe a rare case of symmetric CMEC, which supports the epidermoid formation theory associated with CMEC, together with a literature review.

Treatment Efficacy of Various Maneuvers for Lateral Canal Benign Paroxysmal Positional Vertigo With Apogeotropic Nystagmus: A Randomized Controlled Trial

Objectives@#The aim of this study was to determine the most effective treatment approach by comparing the impacts of various otolith reduction techniques in patients with apogeotropic lateral semicircular canal benign paroxysmal positional vertigo (LC-BPPV). @*Methods@#We performed a multicenter randomized prospective study from January to December 2015, involving 72 consecutive patients with apogeotropic LC-BPPV. The patients were divided into three treatment groups: therapeutic head-shaking (group A), the Gufoni-Appiani maneuver (group B), and the cupulolith repositioning maneuver (CuRM; group C). Each group underwent evaluation and treatment up to the fourth week. Treatment success was defined as the disappearance of positional vertigo and nystagmus. @*Results@#This study included 72 patients (49 male and 23 female), with a mean (±standard deviation) age of 55.4±13.5 years. The mean duration of vertigo experienced prior to treatment was 3.9±4.4 days. The mean latency and duration of nystagmus were 2.7±3.0 seconds and 47.9±15.8 seconds, respectively. The overall treatment frequency was 2.0±0.9. The number of treatments differed significantly among the three groups (P0.05). However, CuRM was the only method with a 100% treatment success rate. @*Conclusion@#While no clear difference was observed among the three treatments for LC-BPPV, CuRM was found to be superior to the other approaches in the long term.

A Case of Autoimmune Sensorineural Hearing Loss Responding to Cytotoxic Agent / 대한이비인후과학회지

Autoimmune sensorineural hearing loss is a rare disease characterized by bilateral sensorineural hearing loss, some in a progressive pattern and others in idiopathic or fluctuating pattern, often accompanied by vestibular symptoms. This disease is also known as autoimmune inner ear disease (AIED), which primarily involves the vestibulo-cochlear system. However, in some cases, it occurs in the context of systemic autoimmune disorder such as wegner granulomatosis, or the Bechet disease. Response to steroids has been the requisite clinical criterion for diagnosis. Treatment usually includes corticosteroids and immunosuppressive drugs. However, after continuous steroid treatment development of corticosteroids, resistance is common in many AIED. To patients with AIED, response to steroids is not only a criterion for diagnosis but also a criterion to test steroid-sparing therapies such as methotrexate. A 10 year-old boy presenting a fluctuating pattern of bilateral sensorineural hearing loss was treated with a cytotoxic agent in suspicion of autoimmune sensorineural hearing loss. We reviewed this case with reference to relevant publications in the medical literature.

A Case of Middle Ear Neuroendocrine Adenoma in a Patient with Hearing Loss and Facial Palsy / 대한이비인후과학회지

Middle ear adenoma is a very rare disease which is benign and originates from the middle ear mucosa. Patients of middle ear adenoma usually come to the clinic for unilateral hearing loss or tinnitus, but rarely for accompanied facial palsy. It is non-gender specific and occurs over a wide range of ages. The recurrence rate is known to be very low, but few authors argue that neuroendocrine adenoma should be considered as a low grade carcinoma due to some cases of recurrence. A 18 years-old male who had a left side facial palsy about 3 years ago but has currently improved as compared with the initial onset, visited our clinic for the left side hearing loss. Pure tone audiogram showed about 30 dB of conductive hearing loss and a pinkish polypoid mass involving the left tympanic membrane. We removed a tumor via transmastoid approach. The final diagnosis was middle ear adenoma with neuroendocrine differentiation. Neither signs of complication nor recurrence were observed after six months of the surgery.

A Case of Middle Ear Neuroendocrine Adenoma in a Patient with Hearing Loss and Facial Palsy / 대한이비인후과학회지

Middle ear adenoma is a very rare disease which is benign and originates from the middle ear mucosa. Patients of middle ear adenoma usually come to the clinic for unilateral hearing loss or tinnitus, but rarely for accompanied facial palsy. It is non-gender specific and occurs over a wide range of ages. The recurrence rate is known to be very low, but few authors argue that neuroendocrine adenoma should be considered as a low grade carcinoma due to some cases of recurrence. A 18 years-old male who had a left side facial palsy about 3 years ago but has currently improved as compared with the initial onset, visited our clinic for the left side hearing loss. Pure tone audiogram showed about 30 dB of conductive hearing loss and a pinkish polypoid mass involving the left tympanic membrane. We removed a tumor via transmastoid approach. The final diagnosis was middle ear adenoma with neuroendocrine differentiation. Neither signs of complication nor recurrence were observed after six months of the surgery.

A Case of Autoimmune Sensorineural Hearing Loss Responding to Cytotoxic Agent / 대한이비인후과학회지

Autoimmune sensorineural hearing loss is a rare disease characterized by bilateral sensorineural hearing loss, some in a progressive pattern and others in idiopathic or fluctuating pattern, often accompanied by vestibular symptoms. This disease is also known as autoimmune inner ear disease (AIED), which primarily involves the vestibulo-cochlear system. However, in some cases, it occurs in the context of systemic autoimmune disorder such as wegner granulomatosis, or the Bechet disease. Response to steroids has been the requisite clinical criterion for diagnosis. Treatment usually includes corticosteroids and immunosuppressive drugs. However, after continuous steroid treatment development of corticosteroids, resistance is common in many AIED. To patients with AIED, response to steroids is not only a criterion for diagnosis but also a criterion to test steroid-sparing therapies such as methotrexate. A 10 year-old boy presenting a fluctuating pattern of bilateral sensorineural hearing loss was treated with a cytotoxic agent in suspicion of autoimmune sensorineural hearing loss. We reviewed this case with reference to relevant publications in the medical literature.

Clinical Application and Update of Video Head Impulse Test / 대한평형의학회지

Recently with the introduction of video head impulse test (vHIT), it can be easily performed quantitative and objective measurement of vestibulo-ocular reflex (VOR). vHIT has been used as a clinical vestibular function test that can individually evaluate the function of each semicircular canal. Loss of VOR gain and corrective catch-up saccades that occur during the vHIT usually indicate peripheral vestibular hypofunction, whereas in acute vestibular syndrome, normal vHIT should prompt a search for a central lesion. In this study, we will examine the principle of vHIT and its interpretation, and explain its clinical application in peripheral and central vestibulopathy. In addition, we will compare the caloric test and the differences, and review the most recently introduced suppression head impulse paradigm test.

Short-Term Clinical Outcome of Neuro One® System Cochlear Implantation in Korean Adults / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: This study analyzed short-term clinical outcome and safety of Neuro One® (Oticon Medical) cochlear implantation on postlingually deafened Korean adults. SUBJECTS AND METHOD: A prospective study was performed on five postlingually deafened Korean adults who underwent Neuro One® cochlear implantation between April and June, 2016. All cases were successfully implanted via round window approach. Preoperative pure tone audiometry and speech perception score [phonetically balanced (PB) words, bi-syllable words, Korean version-central institute for the deaf (K-CID)], intraoperative evoked compound action potential (ECAP), postoperative audiometry, aided warble-tone thresholds and speech perception scores after 6 months were evaluated. Residual hearing group was defined as patients with below 40 dB of average audiometric threshold in low frequency (250 and 500 Hz). Preservation of residual hearing and postoperative complication were also evaluated. RESULTS: The mean age of five patients (3 men, 2 women) was 37.6 years. All of the recipients were unsatisfied about the conventional hearing aid. Two patients met the criteria for belonging to a residual hearing group. The audiometric threshold on the implanted side was 102.8 dB, and the opposite side was 89.6 dB. The speech perception score with hearing aid resulted in the PB word score of 35.5%, bi-syllable word score of 50%, and the open set sentence test of 62.5% on the average. The mean operation time was 87 minutes. Three patients showed normal ECAP response throughout the entire electrode intraoperatively, but the other two patients could not detect ECAP response intraoperatively. However, we could eventually identify ECAP responses in all cases postoperatively. All patients of residual hearing group could preserve their residual hearing without deteriorating more than 10 dB on audiometry. At 6 months after implantation, there was significant improvement of speech perception scores (PB word score was 54.1%; bi-syllable word score was 68.1% and K-CID was 86.2%). CONCLUSION: Neuro One® cochlear implant system was able to preserve residual hearing with relative short operation time. Short-term clinical outcome showed no significant difference with other cochlear implant devices. However, intraoperative ECAP measurement was not consistent. Further investigation was necessary.

Clinical Usefulness of Speech Mapping for Fitting of Hearing Aids / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The aim of this study was to evaluate the usefulness of speech mapping based on real ear measurement for routine hearing aid fitting and to compare functional gains for evaluating subjective satisfaction of hearing aid users. SUBJECTS AND METHOD: Twenty two participants with bilateral symmetric (< 10 dB HL difference) sensorineural hearing loss were enrolled in this study. All participants were fitted unilateral hearing aids with speech mapping using the National Acoustic Laboratories-Nonlinear 2 formula. After the initial fitting, patients were followed with the 2nd and 3rd fitting at two weeks and six weeks, respectively, and measured for aided pure tone average (PTA), aided speech discrimination score (SDS), the difference between target gain and real ear insertion gain (REIG) using speech mapping and subjective satisfaction via Korean Adaptation of the International Outcome Inventory for Hearing Aids (K-IOI-HA) questionnaire before further fitting was performed. We analyzed correlation of each parameter at 2nd and 3rd fitting with the K-IOI-HA score. RESULTS: Every sequential aided PTA and SDS at 2nd fitting and 3rd fitting were significantly improved after repeated hearing aid fitting (all p < 0.01). In the correlation analysis between K-IOI-HA and each parameter, the aided PTA and aided SDS did not show significant correlations with subjective satisfaction from the 2nd and 3rd fittings. But the difference between the target gain and R EIG in speech mapping showed significant negative correlations with the satisfaction scores at various speech level (r=–0.609 to –0.709, all p < 0.01). CONCLUSION: Speech mapping using real ear measurement was useful to expect subjective satisfaction of hearing aid users and it would be a valuable tool for fine tuning to achieve individual preferences.

The Light Cupula: An Emerging New Concept for Positional Vertigo

Benign paroxysmal positional vertigo (BPPV) is the most common type of positional vertigo. A canalolithiasis-type of BPPV involving the lateral semicircular canal (LSCC) shows a characteristic direction-changing positional nystagmus (DCPN) which beats towards the lower ear (geotropic) on turning the head to either side in a supine position. Because geotropic DCPN in LSCC canalolithiasis is transient with a latency of a few seconds, the diagnosis can be challenging if geotropic DCPN is persistent without latency. The concept of “light cupula” has been introduced to explain persistent geotropic DCPN, although the mechanism behind it requires further elucidation. In this review, we describe the characteristics of the nystagmic pattern in light cupula and discuss the current evidence for possible mechanisms explaining the phenomenon.

Comparison of Video Head Impulse Test with Posterior Semicircular Canal Plane and Cervical Vestibular Evoked Myogenic Potential / 대한평형의학회지

OBJECTIVE: Cervical vestibular evoked myogenic potential (cVEMP) is thought to be assessing the function of the saccule and inferior vestibular nerve. Therefore, cVEMP indirectly reflects the function of the posterior semicircular canal. Recently, the video head impulse test (vHIT) is considered as useful clinical tool to detect each semicircular canal dysfunction. Goal of this study was to evaluate and compare the results of cVEMP with posterior canal plane of vHIT (p-vHIT). METHODS: Retrospectively, we compared the results of cVEMP with p-vHIT in 43 patients who visited with dizziness. We analyzed the inter-test agreement of cVEMP with p-vHIT. RESULTS: Positive asymmetry of cVEMP was present in 37.2% (16/43), and no responses of both ears were identified in 16.3% (7/43). In p-vHIT analysis, unilateral positive was 27.9% (12/43), bilateral positive was 11.6% (5/43) and negative in both sides was 60.5% (26/43). The inter-test agreement between cVEMP and p-vHIT was 75.8% (25/33) as we considered even in lesion side. And, Fleiss's kappa value showed a fair to good agreement (kappa value=0.559). In bilateral no response group (7 patients) in cVEMP, variable additional information could be obtained using p-vHIT. CONCLUSION: cVEMP and p-vHIT showed relatively lower inter-test agreement than expected. But, p-vHIT could be easily performed, and give additional information for differential diagnosis.

A Case of Perilymphatic Fistula Misdiagnosed as Benign Paroxysmal Positional Vertigo / 대한이비인후과학회지

Perilymphatic fistula (PLF) is an uncommon disease characterized by abnormal leakage of the perilymph from the inner ear into the middle ear. The symptoms of PLF include sudden-onset hearing loss, tinnitus, ear fullness, and vertigo resulting from inner-ear trauma, stapedial surgery or barotrauma such as valsalva, and nose blowing. As nystagmus can be induced by the affected-ear in down position, benign positional paroxysmal vertigo (BPPV) should be considered for differential diagnosis. About 40-50% of PLF patients have experienced spontaneous healing, but surgical repair should be considered when the hearing symptom and dizziness are not alleviated or are aggravated within a few days. Reported herein, with a review of the relevant literature, is a case of PLF initially misdiagnosed as BPPV but where successful fistula repair was finally achieved.

A Case of Foreign Bodies in Middle Ear and Eustachian Tube When Making Impression of Hearing Aid / 대한이비인후과학회지

Making impression is the first step of making hearing aid. Because it needs silicon injection on external auditory canal and lateral side of tympanic membrane, careful evaluation is required regarding the presence of any tympanic perforation or problems of external auditory canal prior to making ear impression. Recently, we experienced encountering one case of foreign body in ear while making impression of hearing aid by a local hearing aid seller. Silicone mold material, impacted into the middle ear, had perforated the tympanic membrane; a metallic foreign body was also found in the eustachian tube. Reporting this case, we suggest that special attention be paid to tympanic membrane and external auditory canal conditions when making an ear impression for hearing aids. Therefore, physical examination by otolaryngologist should precede any application of hearing aid.

A Case of Vestibular Schwannoma with Acute Spontaneous Vertigo / 대한이비인후과학회지

Vestibular schwannoma (VS) is a rare benign tumor arising from the vestibular portion of the 8th cranial nerve. Patients with VS complain of diverse symptoms, such as asymmetrical hearing loss, tinnitus, vertigo, and unsteadiness. The most common symptom of VS is unilateral and gradual loss of hearing, which may also be associated with tinnitus. We experienced an unusual case of 62-year-old woman, who presented with acute vertigo without any typical otologic symptoms of hearing loss and tinnitus. Because of the unusual symptom, the patient was initially diagnosed with vestibular neuronitis. However, atypical nystagmus was presented after head shaking test. Internal auditory canal MRI scanning confirmed the diagnosis of VS. We removed the patient's VS by middle cranial fossa approach. We present this case with a review of relevant peer-reviewed medical articles and literature.

Two Cases of Central Vertigo Presenting as Apogeotropic Direction Changing Positional Nystagmus / 대한평형의학회지

Positional vertigo and nystagmus without focal neurological symptoms and signs are characteristic features of benign paroxysmal positional vertigo (BPPV). And the apogeotropic positional nystagmus can be diagnosed as cupulolithiasis of the horizontal semicircular canal. However, cerebellar lesion involving especially nodulus could be initially presented as positional vertigo like a BPPV without other neurologic signs. In most of the patients with cerebellar involvement, initial presentation shows dysarthria, ataxia, headache, nausea, vomiting and unsteadiness. But in some central lesions, positional nystagmus might be observed in head roll test as if BPPV was presented. It is very important for clinicians of dizziness care unit to differentiate central positional vertigo (CPV) from BPPV. But it is difficult to diagnose CPV at initial visit by history and physical exam only. Therefore, we introduce two cases with cerebellar infarction and hemorrhage initially presenting isolated positional vertigo mimicking BPPV.

Two Cases of Cerebellar Hemangioblastoma with Isolated Vertigo / 대한평형의학회지

Hemangioblastoma is solid or cystic benign vascular tumor that may arise anywhere in the body. It is rarely presented tumor accounting for only 1% to 2.5% of all intracranial neoplasms. Usually, hemangioblastoma is located in the cerebellum and posterior cranial fossa and it occurs in a variety of symptoms depending on where the tumor is located. The initial symptoms in 80% to 90% of hemangioblastoma patients are headache and vomiting due to elevated intracranial pressure, and also dizziness and balance problems are initially presented in about half of the patients. We experienced 2 cases of hemangioblastoma who presented with isolated vertigo. All of them initially showed unidirectional spontaneous nystagmus during head impulse test. Finally, hemangioblastoma of the cerebellum has been diagnosed on magnetic resonance imaging scan. In one case, the tumor was successfully removed by retrosigmoid approach and the other case was treated conservatively due to pregnancy.

A Case of Multiple Sclerosis with Isolated Vertigo / 대한이비인후과학회지

Multiple sclerosis (MS) is a chronic disease characterized by multiple areas of demyelination, inflammation, and glial scarring in the central nervous system. MS is the most common chronic and usually progressive neurologic disease whose clinical course varies from a benign and symptom-free disease to a rapidly progressive and disabling disorder. MS can be presented with various symptoms, but isolated vertigo, represented in only 5%, is very rare. Nevertheless, in such a case, differential diagnosis of peripheral vestibular disease is very important for neuro-otologist. We recently experienced a 39 year-old female patient of multiple sclerosis with isolated vertigo mimicking vestibular neuritis. We report our case with a review of literature.

Acute Central Vertigo / 대한이비인후과학회지

Acute vertigo is a common symptom at the otolaryngology clinics or the Emergency Department. Also, many otolaryngologists see these patients first or they are referred from the Emergency Department. Although the stroke incidence in all dizzy patients is relatively low, the misdiagnosis of central vertigo may lead to serious morbidity and even mortality. Nevertheless, it is difficult to diagnose in vertigo patients whether the origin is central or not, especially at the initial visit. The purpose of this clinical review was to investigate clinical findings of acute central vertigo and to suggest the recent advance of differential diagnosis in these patients from peripheral vestibulopathy. We also suggest an appropriate practice for the radiologic investigation in these patients.

Multicenter Study on the Clinician's Diagnostic and Therapeutic Approaches for Benign Paroxysmal Positional Vertigo in Korea / 대한평형의학회지

BACKGROUND AND OBJECTIVES: It is necessary to establish the most efficient diagnostic and therapeutic method for benign paroxysmal positional vertigo (BPPV), which is appropriate for Korean healthcare system. We aimed to evaluate current state of Korean clinician's diagnostic and therapeutic approaches for BPPV. MATERIALS AND METHODS: A 16-item survey was emailed to the members of dizziness department of Otology Research Interest Group in the Korean Otologic Society (n=68). 43 were returned and analyzed. RESULTS: All respondents (100%) used Dix-Hallpike test as a diagnostic tool for vertical canal-BPPV. Supine roll test was used for diagnosing lateral canal BPPV in nearly all the respondents (97.7%). Epley maneuver was chosen as otolith repositioning maneuver (ORM) for posterior canal BPPV in all respondents and barbecue rotation (BBQ) was used for treating lateral canal BPPV with geotropic nystagmus in 95.3% of respondents. Extreme variation was noted for therapeutic approach of lateral canal BPPV with ageotropic nystagmus BBQ, with 4 kinds of ORM and adjunctive measures to liberate otolith from cupula, while BBQ was again the most commonly used ORM (76.7%). CONCLUSION: The development of practical and efficient ORM for lateral canal BPPV with ageotropic nystagmus is necessary.

Clinical Value of Vestibular Evoked Myogenic Potential in Assessing the Stage and Predicting the Hearing Results in Meniere's Disease

OBJECTIVES: Our goal was to find the clinical value of cervical vestibular evoked myogenic potential (VEMP) in Meniere's disease (MD) and to evaluate whether the VEMP results can be useful in assessing the stage of MD. Furthermore, we tried to evaluate the clinical effectiveness of VEMP in predicting hearing outcomes. METHODS: The amplitude, peak latency and interaural amplitude difference (IAD) ratio were obtained using cervical VEMP. The VEMP results of MD were compared with those of normal subjects, and the MD stages were compared with the IAD ratio. Finally, the hearing changes were analyzed according to their VEMP results. RESULTS: In clinically definite unilateral MD (n=41), the prevalence of cervical VEMP abnormality in the IAD ratio was 34.1%. When compared with normal subjects (n=33), the VEMP profile of MD patients showed a low amplitude and a similar latency. The mean IAD ratio in MD was 23%, which was significantly different from that of normal subjects (P=0.01). As the stage increased, the IAD ratio significantly increased (P=0.09). After stratification by initial hearing level, stage I and II subjects (hearing threshold, 0-40 dB) with an abnormal IAD ratio showed a decrease in hearing over time compared to those with a normal IAD ratio (P=0.08). CONCLUSION: VEMP parameters have an important clinical role in MD. Especially, the IAD ratio can be used to assess the stage of MD. An abnormal IAD ratio may be used as a predictor of poor hearing outcomes in subjects with early stage MD.