Increased Risk of Psychopathological Abnormalities in Subjects With Unilateral Hearing Loss: A Cross-Sectional Study

Objectives@#. Although unilateral hearing loss (UHL) has been proven to be associated with educational and behavioral problems, few studies have investigated psychopathological abnormalities in this population. The aim of this study was to evaluate the psychopathological influence of UHL among Korean 19-year-old males. @*Methods@#. The authors retrospectively compared the objective personality test profiles of 602 subjects with UHL with those of 23,790 peers with normal hearing. All participants in the current study were 19-year-old males who underwent a physical examination and completed the Korean Military Multiphasic Personality Inventory for conscription at the Military Manpower Administration from February 2015 to December 2016. @*Results@#. Significantly higher scores were found on neurosis scales in the UHL group than in the normal-hearing group (50.9± 10.8 vs. 44.9±6.0 for anxiety; 51.0±10.5 vs. 44.9±5.2 for depression; 51.1±10.4 vs. 45.1±6.81 for somatization, all P<0.001). The psychopathy scales were also significantly higher in the UHL group than in the normal-hearing group (49.3±9.4 vs. 46.3±5.7 for schizophrenia; 51.1±11.2 vs. 44.3±5.8 for personality disorders; 51.1±10.5 vs. 45.7±3.7 for paranoia, all P<0.001). @*Conclusion@#. Nineteen-year-old males with UHL tended to have more abnormal results on personality tests than controls with normal hearing, suggesting that UHL may be related with a higher risk of psychopathology.

Vestibulocochlear Symptoms Caused by Vertebrobasilar Dolichoectasia

Objectives@#. Vertebrobasilar dolichoectasia (VBD), an elongation and distension of vertebrobasilar artery, may present with cranial nerve symptoms due to nerve root compression. The objectives of this study are to summarize vestibulocochlear manifestations in subjects with VBD through a case series and to discuss the needs of thorough oto-neurotologic evaluation in VBD subjects before selecting treatment modalities. @*Methods@#. Four VBD subjects with vestibulocochlear manifestations were reviewed retrospectively. VBD was confirmed by either brain or internal auditory canal magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). Patient information, medical history, MRI/MRA findings, and audiometry or vestibular function tests were reviewed according to patient’s specific symptom. @*Results@#. Of the four subjects, three presented with ipsilesional sensorineural hearing loss (SNHL), three with paroxysmal recurrent vertigo, and two with typewriter tinnitus. The MRI/MRA of the four subjects revealed unilateral VBD with neurovascular compression of cisternal segment or the brainstem causing displacement, angulation, or deformity of the cranial nerve VII or VIII that corresponded to the symptoms. @*Conclusion@#. Vestibulocochlear symptoms such as SNHL, recurrent paroxysmal vertigo, or typewriter tinnitus can be precipitated from a neurovascular compression of the vestibulocochlear nerve by VBD. Because proper medical or surgical treatments may stop the disease progression or improve audio-vestibular symptoms in subjects with VBD, a high index of suspicion and meticulous radiologic evaluation are needed when vestibulocochlear symptoms are not otherwise explainable, and if VBD is confirmed to cause audiovestibular manifestation, a thorough oto-neurotologic evaluation should be performed before initial treatment.

In vitro generation of mature midbrain-type dopamine neurons by adjusting exogenous Nurr1 and Foxa2 expressions to their physiologic patterns

Developmental information aids stem cell biologists in producing tissue-specific cells. Recapitulation of the developmental profile of a specific cell type in an in vitro stem cell system provides a strategy for manipulating cell-fate choice during the differentiation process. Nurr1 and Foxa2 are potential candidates for genetic engineering to generate midbrain-type dopamine (DA) neurons for experimental and therapeutic applications in Parkinson's disease (PD), as forced expression of these genes in neural stem/precursor cells (NPCs) yields cells with a complete battery of midbrain DA neuron-specific genes. However, simple overexpression without considering their expression pattern in the developing midbrain tends to generate DA cells without adequate neuronal maturation and long-term maintenance of their phenotype in vitro and in vivo after transplantation. We here show that the physiological levels and timing of Nurr1 and Foxa2 expression can be replicated in NPCs by choosing the right vectors and promoters. Controlled expression combined with a strategy for transgene expression maintenance induced generation of fully mature midbrain-type DA neurons. These findings demonstrate the feasibility of cellular engineering for artificial cell-fate specification.

Functional Neuroimaging in Neuro-Otology / 대한평형의학회지

Neuro-otologic symptoms such as dizziness, hearing loss, or tinnitus give rise to peripheral change-induced neuroplasticity or central pathology-induced structural or functional changes. In this regard, functional neuroimaging modalities such as positron emission tomography (PET), functional magnetic resonance imaging (fMRI), magnetoencephalography (MEG), quantitative electroencephalography (qEEG), or functional near infrared spectroscopy have provided researchers with possibility to observe neuro-otologic disease-induced central functional changes. Among these methods, PET and fMRI are advantageous over qEEG or MEG with regard to spatial resolution, while qEEG and MEG are advantageous over PET or fMRI with regard to temporal resolution. Also, fMRI or MEG is not suitable for patients with implanted devices, whereas PET is not ideal for repetitive measures due to radiation hazard. In other words, as these modalities are complementary to one another, researchers should choose optimum imaging modality on a case by case basis. Hereinafter, representative functional neuroimaging modalities and their application to neuro-otologic research will be summarized.

Immediate and Sustained Improvement in Behavior and Life Quality by Adenotonsillectomy in Children With Sleep-Disordered Breathing

OBJECTIVES: To investigate longitudinal changes in behavior and quality-of-life (QoL) in children with sleep disordered breathing (SDB) after adenotonsillectomy (AT). METHODS: This study prospectively enrolled 148 children who underwent AT for SDB. Caregivers filled out Korean attention deficit hyperactivity disorder (ADHD) rating scale (K-ARS) and Korean version of the obstructive sleep apnea-18 (KOSA-18) at preoperative 1 day, postoperative 1 month, and postoperative 6 months. Longitudinal changes in K-ARS and KOSA-18 were evaluated and compared among subgroups. RESULTS: Both K-ARS and KOSA-18 scores improved immediately at postoperative 1 month and were maintained at postoperative 6 months. The mean preoperative K-ARS scores of the non-ADHD, ADHD-trait, and overt-ADHD groups showed uniformly immediate and sustained improvements. Also, the mean preoperative KOSA-18 scores of the small, moderate, and large impact groups were significantly improved both at postoperative 1 month and 6 months. The changes in behavior and QoL showed no significant differences with regard to the aforementioned factors. CONCLUSION: After AT, we may expect immediate and sustained improvements in behavior and QoL in children with SDB regardless of preoperative symptom severity. Moreover, by performing AT, improvements in behavior and QoL are expected regardless of sex, age, allergic trait, or concurrent coblation-assisted turbinoplasty. These findings may help our colleague physicians counselling pediatric SDB patients and their caregivers.

Functional Neuroimaging of Tinnitus: State-of-the-Art / 대한이비인후과학회지

Non-pulsatile subjective tinnitus is a phantom sound percept without any objective physical sound source. Recent advances in tinnitus research have suggested central rather than peripheral changes as the culprit of tinnitus perception. Moreover, researchers have shown that these central functional changes can be observed not only in the auditory cortex but also in non-auditory regions such as the frontal, parietal, and limbic areas in patients with tinnitus. In this regard, functional neuroimaging modalities such as positron emission tomography (PET), functional magnetic resonance imaging (fMRI), magnetoencephalography (MEG), and quantitative electroencephalography (qEEG) provided researchers with a window into the cerebral cortical activity orchestrating tinnitus. Among these methods, qEEG and MEG are advantageous over PET or fMRI with regard to temporal resolution, while PET and fMRI are advantageous over qEEG or MEG with regard to spatial resolution. In other words, there is no gold standard functional neuroimaging modality in the field of tinnitus, but these four modalities are complementary to one another. In this review article, these four representative functional neuroimaging modalities and their application to tinnitus research will be introduced. Moreover, future direction of functional neuroimaging research on the pathophysiology of tinnitus will be discussed briefly.

Pulsatile Tinnitus due to Intracranial Dural Arteriovenous Fistula after Head Trauma by a Golf Ball / 대한이비인후과학회지

Diagnosis of traumatic arteriovenous fistula (AVF) is usually delayed because it takes time to be enlarged enough to emerge radiologically or because symptoms occur a few months after the trauma. A 56-year-old woman presented with a newly developed tinnitus immediately after a head trauma. Pulsatile high-frequency tinnitus was heard also by examiner and recorded using a transcanal microphone. Angiography revealed an intracranial dural AVF fed by the middle meningeal artery, draining the superior sagittal sinus on the affected side. After percutaneous transarterial embolization, tinnitus successfully disappeared. The clinical presentation, radiologic and angiographic features, and management are discussed. To our knowledge, this is the first reported case of pulsatile tinnitus caused by AVF developed immediately after a trauma. We emphasize that precise physical examination, laboratory tests, and appropriate radiographic imaging are essential for accurate diagnosis and treatment when a patient presents with pulsatile tinnitus, especially after a head trauma.

Long-term Outcome of Cochlear Implant in Patients with Chronic Otitis Media: One-stage Surgery Is Equivalent to Two-stage Surgery

This study compared long-term speech performance after cochlear implantation (CI) between surgical strategies in patients with chronic otitis media (COM). Thirty patients with available open-set sentence scores measured more than 2 yr postoperatively were included: 17 who received one-stage surgeries (One-stage group), and the other 13 underwent two-stage surgeries (Two-stage group). Preoperative inflammatory status, intraoperative procedures, postoperative outcomes were compared. Among 17 patients in One-stage group, 12 underwent CI accompanied with the eradication of inflammation; CI without eradicating inflammation was performed on 3 patients; 2 underwent CIs via the transcanal approach. Thirteen patients in Two-stage group received the complete eradication of inflammation as first-stage surgery, and CI was performed as second-stage surgery after a mean interval of 8.2 months. Additional control of inflammation was performed in 2 patients at second-stage surgery for cavity problem and cholesteatoma, respectively. There were 2 cases of electrode exposure as postoperative complication in the two-stage group; new electrode arrays were inserted and covered by local flaps. The open-set sentence scores of Two-stage group were not significantly higher than those of One-stage group at 1, 2, 3, and 5 yr postoperatively. Postoperative long-term speech performance is equivalent when either of two surgical strategies is used to treat appropriately selected candidates.

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.

Transglutaminase 2 Expression Predicts Progression Free Survival in Non-Small Cell Lung Cancer Patients Treated with Epidermal Growth Factor Receptor Tyrosine Kinase Inhibitor

Transglutaminase 2 (TG2), a cross-linking enzyme, is involved in drug resistance and in the constitutive activation of nuclear factor kappa B (NF-kappaB). We investigated the association of non-small cell lung cancer (NSCLC) treatment efficacy with TG2 and NF-kappaB expression in 120 patients: 102 with adenocarcinoma and 18 with other histologic types. All patients underwent surgery; 88 received adjuvant chemotherapy, with 28 receiving platinum-based doublet chemotherapy as first-line treatment and 29 receiving epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitor (TKI) therapy. Patients' TG2 and NF-kappaB expression values were calculated semiquantitatively. The median TG2 value was 50 (range, 0-300) and the median NF-kappaB value was 20 (range, 0-240). Disease-free survival did not differ between the low- and high-TG2 groups. Among patients who received palliative platinum-based doublet chemotherapy, progression free survival (PFS) was longer in the low-TG2 group than in the high-TG2 group (11.0 vs. 7.0 months; P=0.330). Among those who received EGFR-TKI therapy, PFS was also longer in the low-TG2 group than in the high-TG 2 group (11.0 vs. 2.0 months; P=0.013). Similarly, in EGFR wild-type patients treated with EGFR-TKI, PFS was longer in patients with low TG2 expression (9.0 vs. 2.0 months; P=0.013). TG2 expression levels can predict PFS in patients with NSCLC treated with EGFR-TKI.

Multiple Myeloma: Report of a Case Manifested as Facial Nerve Palsy / 대한이비인후과학회지

Multiple myeloma is a clonal B-cell tumor of slowly proliferating plasma cells within the bone marrow. It is a relatively well known disease, but temporal bone involvements have rarely been reported. It is not easy to diagnose multiple myeloma of the temporal bone in the early stage, since it presents with general, nonspecific otologic symptoms and pathologic confirmation in the temporal bone is difficult. Multiple myeloma involving the temporal bone area is considered to be in its terminal stage, but the treatment strategy is the same as that for conventional multiple myeloma involving other organs. We experienced a case of facial palsy had suddenly developed and was later diagnosed with multiple myeloma after a pathologic review and systemic evaluation. Herein we report this rare case with a review of literature.

A Case of Posterior Fossa Meningioma Presenting with Meniere's-Like Symptoms / 대한이비인후과학회지

Posterior fossa meningioma located on the endolymphatic sac may present Meniere's disease-like symptoms possibly due to endolymphatic hydrops by tumor compression. A 70-year-old woman presented with recurrent vertigo and combined hearing loss that had lasted for 6 years. She had been diagnosed as left-sided posterior fossa meningioma with a maximum diameter of 20.3x7.7x12 mm about 10 years ago and had been followed up regularly. Since then she was found with a slightly increased tumor and thus referred to our department for the treatment of intractable recurrent vertigo accompanying Tumarkin attacks with secondary head trauma. Since pharmaceutical treatment was not effective, intratympanic gentamicin was repeatedly injected. After successful ablation of vestibular function, no further recurrence of vertigo or Tumarkin attack was reported. This case illustrates that posterior fossa meningioma can be intimately involved with endolymphatic sac and present symptoms and signs suggestive of endolymphatic hydrops.

Acute Mastoiditis Cases Mandating Simple Mastoidectomy

Acute mastoiditis continues to be a potentially dangerous infection in the antibiotic era. Despite use of antibiotics to treat suppurative acute otitis media in children, the incidence of subsequent development of acute mastoiditis and its complications, including periauricular subperiosteal abscess, facial paralysis, meningitis, and brain abscess has not plunged down below a certain level. Mastoidectomy has been the routine procedure for treating acute mastoiditis with subperiosteal abscess but recently many authors suggest that subperiosteal abscess complicating acute coalescent mastoiditis can be successfully treated by broad-spectrum antibiotics, myringotomy, and minor surgical procedures such as incision and drainage, not necessarily requiring simple mastoidectomy. Contrary to the recent trend shift from a surgical disease to a medical disease, we report two consecutive acute mastoiditis cases definitely mandating simple mastoidectomy to emphasize the role of surgical intervention even in the recent trend shift from a surgical to a medical disease.

The Analysis of Prognostic Factor and Treatment Outcome of Malignancies of the External Auditory Canal / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The purpose of this study was to analyze the survival rate treatment modalities and certain prognostic factors in patients with external auditory canal (EAC) cancer using the University of Pittsburgh TNM Staging System. SUBJECTS AND METHOD: Medical records and radiological findings of 33 patients treated for EAC cancer between January 1995 and January 2009 were reviewed retrospectively. The median follow-up period was 65 months (range, 12-182 months). RESULTS: The most common histological type was squamous cell carcinoma (19 patients; 57.6%), followed by adenoid cystic carcinoma (9 patients; 27.3%), basal cell carcinoma (1 patient), adenocarcinoma (1 patient), rhabdomyosarcoma (1 patient), undifferentiated carcinoma (1 patient) and malignant small round cell tumor (1 patient). Seven of 33 patients died of EAC cancer and the overall survival rate was 70.6%. There was a significant difference in the survival rate with respect to TNM stages (p=.031). The 10 yr-survival rate of patients with stage I disease was 100.0%, whereas those of stages II, III and IV were 50.0, 80.0 and 58.3%, respectively. Five of 7 patients with recurrence had a history of initial positive resection margin. CONCLUSION: Early detection and proper surgical treatment with sufficient resection margin are essential for disease-specific survival and prevention of recurrence in patients with EAC cancer.

A Case of Cholesteatoma Extended Both into the Cochlea and IAC / 대한이비인후과학회지

Labyrinthine fistula caused by cholesteatoma can make various complications according to the site of the lesion. The lateral canal, superior canal ampulla, posterior canal and promontory of the cochlea are the common areas of fistulization. However, invasion into the cochlea or internal auditory canal (IAC) is very rare. We have experienced a case of cholesteatoma extending into the cochlea and IAC, which was successfully treated via the transotic approach. Herein we report this case with a review of literature

Clinical Manifestation and Treatment of Malleus Fixation / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Malleus fixation is one of the important causes of persistent conductive hearing loss. This study was designed to evaluate the clinical manifestation and the treatment outcome of malleus fixation. SUBJECTS AND METHOD: This study was a retrospective review of medical records between 1989 and 2008. Patients diagnosed as malleus fixation by intraoperative findings at the Department of Otorhinolaryngology, Seoul National University Hospital were included. Those cases with chronic otitis media and congenital ossicular anomaly involving the incus or stapes were excluded. A total of 13 patients were included (M:F=4:9, Mean age: 24.4 years old). RESULTS: All patients (100%) complained of hearing loss. Preoperative mean air-bone conduction gap was 34.4 dB and 5 (83.3%) out of 6 patients had A-type in impedence audiometry and 3 (75%) out of 4 patients had negative results in Gelle test. In the temporal bone computed tomography (TBCT) findings, we could identify bony spicules (2 cases), sclerosis of ligament (3 cases) and epitympanic contraction (2 cases). According to the different causes of conductive hearing loss, corrective surgery was performed: the release of bony ankylosis (2 cases), lysis of ligament (6 cases) and ossiculoplasty (5 cases) were performed. There was a statistically significant improvement in air-bone conduction gap (34.4 dB vs. 20.3 dB, p=0.009) after surgery. CONCLUSION: Considering the improvement of air-bone conduction gap after the corrective surgery, clinical suspicion of malleus fixation is important in cases of unknown conductive hearing loss.

Treatment of Meniere's Disease and Intratympanic Gentamicin Injection / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Several treatment options have been introduced to control Meniere's disease (MD). Among them, intratympanic injection of gentamicin (ITGM) is now accepted as a standard treatment method for intractable vertigo in patients with hearing impaired MD. The purpose of this study was to analyze the clinical course and treatment flow in patients with MD and to analyze the efficacy and problems after ITGM. SUBJECTS AND METHOD: A retrospective study was performed on 458 patients who were diagnosed as definite MD from May 2003 to October 2007 and were followed up at least 1 year. The authors investigated the course of disease and the results in our patients according to the treatment options. Several clinical parameters including frequency of vertigo, audiometry, caloric and rotation test were reviewed and analyzed according to the guideline of the AAO-HNS (1995), if necessary. Efficacy and problems after ITGM were reviewed. RESULTS: Among 458 patients, vertigo was resolved or improved in 399 patients (87.1%) with medical management. Fifty-nine patients needed further treatment (endolymphatic sac decompression: 4, Meniette: 2, ITGM: 53). Among 53 patients who got ITGM, 40 patients were followed up for more than 1 year after injection. Thirty-six patients (87.5%) showed successful control of vertigo. The average pure-tone threshold was changed from 54.1 dB to 56.9 dB after treatment. Three patients (7.5%) revealed more than 20 dB aggravation. CONCLUSION: Vertigo was controlled by supportive treatments or medication in 87% of definite MD patients. And ITGM could effectively and reasonably control vertigo for intractable MD patients.

Genetic Background of Congenital Hearing Loss

Understanding the genetic background of hearing loss is important since almost 50% of the cases of profound hearing loss are caused by genetic factors. Until now, more than 150 causative genes have been identified. In this review, classification of genetic hearing loss (syndromic versus non-syndromic, recessive versus dominant, X-linked and mitochondrial), pitfalls in elucidating causative genes, anatomy of the inner ear, introduction of the most common syndromic hearing loss, introduction of the most common non-syndromic hearing loss-causing genes, mitochondrial and multifactorial hearing losses were discussed. Moreover, clinical approaches to the patients with hereditary hearing loss and genetic counseling were also explained briefly. Finally, future directions of the hereditary hearing loss research in Korean population were presented.

A Novel Meatoplasty Method in Canal Wall Down Tympanomastoidectomy: A Perichondrial Posterior Fixation Technique

OBJECTIVES: Although it is well recognized that a small meatus after canal wall down (CWD) tympanomastoidectomy can cause a lifetime problem, unsatisfactory results are frequently encountered. We herein introduce a novel technique, perichondrial posterior fixation (PPF), to maintain a wide external auditory canal (EAC), to minimize postoperative wound infection due to the smaller dead space and to improve the posterior auricular cosmetic outcome. METHODS: A total of 73 patients who underwent CWD tympanomastoidectomy were included. Interventions are CWD tympanomastoidectomy with the PPF technique. Review of the medical records and evaluation of the postoperative size of the meatus and the extent of the cavum conchal cartilage buried within the mastoid cavity by taking digital photographs. RESULTS: Thirty males and 43 females were included and the mean age was 44.1 yr (range, 6 to 66 yr). The mean follow-up duration was 26 months (range, 12 to 56 months). All ears maintained a clean and large external meatus. The posterior auricular cavum conchal cartilage was successfully prevented from being buried into the mastoid cavity in all ears. The extent of cartilage buried into the mastoid cavity was much reduced compared to the conventional technique. CONCLUSION: The PPF technique, which is a novel meatoplasty technique in CWD tympanomastoidectomy, seems to be effective in maintaining a large external meatus and improving the cosmetic outcome with minimal risk of complications.

Clinical Manifestations and Surgical Results of Malleostapedotomy and Malleostapedectomy / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Incus stapedotomy is usually performed for the patients with otosclerosis and congenital ossicular fixation. However, for the patients whose incus is absent, anomalous or fixed, the piston wire cannot be placed around the incus. For these cases, malleostapedotomy or malleostapedectomy could be a proper treatment instead of incus stapedotomy. The aim of this study was to evaluate the clinical manifestation and treatment outcome of malleostapedotomy and malleostapedectomy. SUBJECTS AND METHOD: From January 1993 through March 2007, there were four malleostapedotomies and three malleostapedectomies. The medical records of these patients were retrospectively reviewed. The length of piston, ossicular condition, hearing improvement and postoperative complications were investigated. RESULTS: Conventional incus stapedotomy was not possible due to incus anomaly in three cases, incus fixation in two cases, incus removal during the previous surgery in one case and incus defect due to previous surgery in one case. The median length of prosthesis was 5.5 mm. The median preoperative air-bone gap was 42 dB and postoperative air-bone gap was 12 dB. The postoperative air-bone gap level was smaller than 10 dB in three patients, 11-20 dB in one patient, 21-30 dB in one patients, and 31-49 dB in one patient. Except for one case that had extrusion of the prosthesis after eight years, there was no intraoperative or postoperative complication. CONCLUSION: Malleostapedotomy or malleostapedectomy may be a good alternative surgical procedure to routine incus stapedotomy in cases of absence, anomaly or fixation of the incus.