Speech Perception and Language Outcome in Congenitally Deaf Children Receiving Cochlear Implants in the First Year of Life / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The objective of this study was to examine the effect of early cochlear implantation (CI) performed in infants less than 12 months of age. SUBJECTS AND METHOD: Twenty-five children who received their first CI before 12 months of age were included in this study (infant group). The speech perception and language outcomes of these children were compared with those of 14 children who received their first CI between 13 and 24 months of age (older group). All children received sequential bilateral CI with the inter-stage interval of less than 2 years. Speech perception was measured using Categories of Auditory Performance, monosyllabic word test and sentence test, and language ability was measured using Sequenced Language Scale for Infants, Preschool Receptive-Expressive Language Scale, or Receptive & Expressive Vocavulary Test, depending on the age at the time of testing. RESULTS: There were no significant differences in speech perception abilities between the infant group and the older group. The mean expressive language score of infant group was higher than that of the older group, but the difference was not statistically significant. However, the receptive language score of infant group was significantly higher than that of the older group. CONCLUSION: Children who received CI before 12 months of age achieved better receptive language ability than those who received it after 12 months of age. Thus CI should be performed as early as before 12 months of age to achieve better language ability.

A Novel Frameshift Mutation of the USH2A Gene in a Korean Patient with Usher Syndrome Type II

Usher syndrome type II (USH2) is the most common form of Usher syndrome, characterized by moderate to severe hearing impairment and progressive visual loss due to retinitis pigmentosa. It has been shown that mutations in the USH2A gene are responsible for USH2. The authors herein describe a 34-year-old Korean woman with the typical clinical manifestation of USH2; she had bilateral hearing disturbance and progressive visual deterioration, without vestibular dysfunction. Molecular genetic study of the USH2A gene revealed a novel frameshift mutation (c.2310delA; Glu771LysfsX17). She was heterozygous for this mutation, and no other mutation was found in USH2A, suggesting the possibility of an intronic or large genomic rearrangement mutation. To the best of our knowledge, this is the first report of a genetically confirmed case of USH2 in Korea. More investigations are needed to delineate genotype-phenotype correlations and ethnicity-specific genetic background of Usher syndrome.

Repair of Spontaneous Cerebrospinal Fluid Otorrhea from Defect of Middle Cranial Fossa

Spontaneous cerebrospinal fluid (CSF) otorrhea is defined as CSF otorrhea where there are no identifiable causes including previous trauma, surgery, infection, neoplasm or congenital anomaly. The condition is rare. The origin of CSF leak is commonly a defect in the tegmen of the middle cranial fossa. The pathophysiology of spontaneous CSF otorrhea is unclear. Two theories of the etiology of bony defects of the temporal bone are the congenital bony defect theory and arachnoid granulation theory. The authors experienced a case of a 49-year-old female patient admitted with the complaint of persistent right ear fullness. Computed tomography revealed a large defect of the middle fossa and suspicious CSF otorrhea through the defect of tegmen tympani. Repair was successful with multiple bone chips using the transmastoid approach. The postoperative course was good and there has been no recurrence of the CSF leakage.

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 Analysis of Auricular Benign Masses

BACKGROUND AND OBJECTIVES: Auricular masses are growths on the outer ear that have not been well characterized clinically. We assessed the clinical nature and treatment of auricular benign masses in patients at our institution. SUBJECTS AND METHODS: We retrospectively identified 63 patients with auricular benign masses who underwent excision and biopsy from May 1970 to April 2011. We determined the site, cause, size, pathology and postoperative results of these auricular masses. RESULTS: Auricular benign masses occurred most commonly on the lobule (44.4%), followed by the tragus (20.6%), crus of helix (11.1%), triangular fossa (6.3%), crus of antihelix-antitragus (3.1%) and scapha (1.5%). Pathologically, the most common type of auricular mass was epidermal cysts (25.3%), followed by hypertrophic scar (12.6%), fibrous tissue-accessory ear (9.5%), chronic inflammation-nevus (7.9%), keloid (6.3%), hemangioma (4.7%), and skin tag-seborrheic keratosis (3.1%). CONCLUSIONS: The most common site of auricular benign masses is the lobule and most common pathology is an epidermal cyst.

Rate of Isolation and Trends of Antimicrobial Resistance of Multidrug Resistant Pseudomonas Aeruginosa from Otorrhea in Chronic Suppurative Otitis Media

OBJECTIVES: To assess the rate of isolation of Pseudomonas aeruginosa (PA) and multidrug-resistant PA (MDR-PA) from patients with chronic suppurative otitis media (CSOM) otorrhea and the annual trend of antibiotic-resistance. METHODS: Otorrhea samples were collected aseptically from 1,598 CSOM patients. The rate of bacterial isolation and the results of antibiotic susceptibility testing were evaluated retrospectively. RESULTS: The PA isolation rate from CSOM otorrhea was 24.4%. Of the 398 isolated strains tested for their susceptibilities to 10 antibiotics, 395 strains showed definitive results. Of these, 183 (46.3%) were susceptible to whole antibiotics and 212 (53.7%) was resistant to more than 1 antibiotics, with the frequency of antibiotics-resistance increasing significantly over time. Although strains susceptible to all antibiotics decreased over time, the rate of isolation of MDR-PA did not change significantly. Resistance to aminoglycosides and quinolones was higher than to other antibiotics and significantly increased over time, whereas resistance to other antibiotics showed no trend. CONCLUSION: MDR-PA, assessed using five individual antibiotics and six antibiotic-classes, showed no tendency to increase or decrease over time. This may have been due to increased concern about antibiotic-resistant bacterial strains, leading to improved infection control within hospitals and healthcare centers.

Clinical Characteristics and Suggested Diagnostic Criteria of Obliterative Otitis Media / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Obliterative otitis media is a condition in which the entire middle ear cavity is filled up with fibrous tissue and with intact tympanic membrane. The aim of this study was to define the clinical features and analyze the treatment outcomes of this disease. SUBJECTS AND METHOD: Between January 2000 and August 2011, 16 patients consistent with the criteria of obliterative otitis media were analyzed retrospectively. RESULTS: All patients had intact but thickened eardrum, with mixed hearing loss of mild to moderate degree. The video pneumatic otoscopic examination revealed very limited mobility of eardrum. Myringotomy did not reveal any air-filled spaces or fluid in the tympanic cavity. There was no significant improvement of hearing after myringotomy or ventilation tube insertion. CONCLUSION: Obliterative otitis media is a discrete clinical entity and should not be confused with otitis media with effusion or adhesive otitis media.

Three Cases of Dermoid Cysts of Auriculotemporal Area / 대한이비인후과학회지

Dermoid cysts, also referred as "dysontogenetic cyst", are ectodermal cysts that contain epithelial linings as well as adnexal structures. They are divided into 3 categories : epidermoids, dermoids and teratoids. Although dermoid cysts represent a separate entity, the term "dermoid" is generally used to indicate all 3 categories. Dermoid cysts may occur in numerous areas of the body. However, dermoid cysts on the head and neck region are relatively rare. A few cases of dermoid cysts have been reported especially in the auriculotemporal area but not in the external auditory canal or preauricular area. We experienced 3 unusual cases of dermoid cysts occurring in the auriculotemporal area that includes external auditory canal, preauricular and postauricular area. Because of its location and rarity, we report these cases of dermoid cyst in the auriculotemporal area with a brief review of literatures.

Open-Set Speech Perception Performance and Its Contributing Factors for Children Received Cochlear Implantation at Older Age / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: This study aims to investigate the development of open-set speech perception abilities in children who received cochlear implantation at older age and to examine the preoperative variables contributing to the postoperative speech perception outcomes. SUBJECTS AND METHOD: Open-set speech perception abilities were assessed with the Monosyllabic Word and Common Phrases Tests for 37 prelingually deafened children who received implantation between the ages of 7 and 17 years. Their performance was compared to that of 60 children who received implantation before 7 years of age. To identify the factors contributing to the postoperative open-set speech perception abilities, we analyzed age at hearing aid fitting, age at implantation, duration of implant use, preoperative language and speech perception abilities, and pure tone thresholds in the better ear. RESULTS: The children who received implantation after 7 years of age significantly improved during the first year of implant use, but reached a plateau by 1.5 to 2 years after implantation, reaching scores between 60% and 70%. Children who received implantation after age 7 showed higher speech perception scores than the younger children over the first year of implant use, but showed lower scores than the younger children after 2-3 years of implant use. The variables associated with the postoperative open-set speech perception abilities were speech perception ability, language ability, and hearing levels before implantation. CONCLUSION: Prelingually deafened children who received implantation at older age can obtain substantial open-set speech perception understanding, but tended to reach a plateau between 1.5-2 years after implantation. Speech perception, language abilities, and hearing level before implantation contribute to the outcome.

A Case of Neurilemmoma in the Nasal Vestibule

A schwannoma, also known as neurilemmoma, is a relatively rare benign tumor originating from the schwann cells of the nerve sheath. It occurs along any somatic, sympathetic and cranial nerves except the olfactory and optic nerves. It occurred most frequently in the head and neck but rarely in the nasal cavity and paranasal sinuses. Occurrence was especially rare in the nasal vestibule. Recently we experienced an unusual case of the schwannoma involving the left nasal vestibule. The mass was completely removed via a sublabial approach and histopathological examination and immunohistochemical staining study confirmed the schwannoma. We report this case with review of literatures.

A Case of Type 2 Usher Syndrome / 대한이비인후과학회지

The Usher syndrome is an autosomal recessive disorder that cause bilateral sensorineural hearing loss and progressive loss of vision. It is genetically heterogeneous and is the most frequent cause of hereditary deafness and blindness in human. There are three types of Usher syndrome that can be distinguished clinically and into different subtypes. Type 2 Usher syndrome is the most common form and less severe than Type 1. It is characterized by congenital, moderate to severe, high frequency sloping hearing loss, retinitis pigmentosa which is typically diagnosed in late adolescence, and normal vestibular function. Recently, we have experienced a case of clinically diagnosed Type 2 Usher syndrome in a 34 years old female. We report this case with a brief review of literature. This is the first Type 2 Usher Syndrome to be reported in the otolaryngologic field in Korea.

A Case of Herpes Zoster Oticus Involving Vestibular Nerve without Facial Nerve Palsy

Herpes zoster oticus (Ramsay Hunt syndrome) is characterized by facial nerve paralysis associated with vesticular eruptions and cochleovestibular symptoms. Many evidences have supported that it is caused by the reactivation of latent varicella-zoster virus in the geniculate ganglion. Recently we experienced a case 49-year-old man presented severe vertigo and a vesicular eruptions of auricle and external ear canal. It is an unusual variant of herpes zoster oticus that involves only vestibular nerve without facial nerve palsy and hearing loss. We believe this case results from reactivation of latent varicella zoster virus in the vestibular ganglion and report with a review of literatures.

Factors Affecting Treatment of Benign Paroxysmal Positional Vertigo

BACKGROUND AND OBJECTIVES: Benign paroxysmal positional vertigo (BPPV) is one of the most common clinical entities encountered in a dizziness clinic. Treatment of this disease, canalith repositioning procedures, have been reported to be successful in 44-90%. Treatment requires only one treatment visit in most patients. However, there are significant numbers of patients who require multiple treatment visits for relief. The goal of this study is to identify variables that may be associated with these difficult to treat cases. METHODS: Retrospective review was performed for the patients diagnosed as BPPV at Samsung medical center. Variables for statistical analysis included age, sex, involved canal, presence of bilateral disease, involvement of unilateral multiple canals, presence of recent head trauma, presence of chronic otitis media, history of middle ear surgery, history of otologic surgery, unilateral vestibular loss, accompanying sudden SNHL, underlying disease such as hypertension or diabetes, change of involved canal during treatment course and number of treatment visits. Statistical analysis using Pearson chi-square test was performed. RESULTS: Three hundred thirty-one patients with BPPV who received treatment were identified from 2001 to 2005. 85.2% required one treatment visit, 12.4% required a second treatment visit, and 98.2% were successfully treated after three treatment visits. Variables such as bilateral disease, anterior canal BPPV, post-traumatic BPPV, duration of symptom before treatment and change of involved canal during treatment were significantly related with number of treatments. CONCLUSION: Patients with anterior semicircular canal BPPV or bilateral BPPV or with recent head trauma or longer duration of symptom are more likely to require multiple visits for canalith repositioning.

Effect of Epitympanoplasty with Mastoid Obliteration Technique on Postoperative Rehabilitation of Hearing Impaired / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: This study was designed to identify whether canal type hearing aids can be used by the patients who have undergone epitympanoplasty with mastoid obliteration for postoperative rehabilitation of hearing impaired and also to investigate the advantages of the complete in the canal (CIC) hearing aid for the postoperative rehabilitation of hearing impaired. SUBJECTS AND METHOD: Fourty-three ears belonging to 40 adults, who were treated by epitympanoplasty with mastoid obliteration for cholesteatoma from April 1995 to May 1999 were involved in this study. The impressions were taken and measured postoperatively by Vernier calipers anteroposteriorly (AP) and superoinferiorly (SI). It is well known that the size of the external auditory canal (EAC) should be over 7 mm in diameter and over 2 cm in length to be able to wear the CIC type hearing aid. The shape of the EAC was also taken into consideration. RESULTS: Thirty-five out of 43 ears were large enough to wear CIC hearing aids and all 43 for the canal (ITC) hearing aids. No ears were excluded from being able to wear a CIC or ITC hearing aid with respect to the shape of the EAC. CONCLUSION: Canal type hearing aides can be used after epitympanoplasty with mastoid obliteration which is used as a treatment of cholesteatoma.

Results of Epitympanoplasty with Mastoid Obliteration Technique / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: There have been heated controversies over the choice of the canal wall down mastoidectomy (CWD) and canal wall up mastoidectomy (CWU), which are operational methods used to eliminate the lesion of chronic otitis media including cholesteatoma. The CWD method can secure a good operation field and remove the lesion easily, but it accompanies a cavity problem. The CWU method also has its shortcomings as it is difficult to eliminate cholesteatoma completely using this method. Combining the advantages of both methods, we invented a new operational method. The present study assesses the results of its use after a follow up of 55 months. MATERIALS AND METHODS: From December of 1994 to March of 1997, epitympanoplasty with mastoid obliteration was conducted on 44 adults (42 patients). Of these, 38 cases were cholesteatomas, 2 cases adhesive otitis media and 4 cases chronic otitis media with poor E-tube function. The postoperative observation period ranged from 41 to 68 months, with the average period of 55.2 months. RESULTS: There were 3 cases of residual cholesteatoma in the mastoid cavity and 3 cases in the middle ear cavity. All residual cholesteatomas in the mastoid cavity were treated with CWU, and there was no recurrent cholesteatoma or otorrhea, nor retraction pocket. CONCLUSION: Although the observation period of the postoperative process was not long enough, there was no recurrence. We suggest that it is advantageous to operate on cholesteatoma using the new method.

Changes of Ear Canal with Mandibular Movement / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Although the necessity of hearing aids as one of the rehabilitation methods is increasing, many hearing impaired persons are avoiding the use of behind-the-ear (BTE) hearing aids and prefer the complete-in-the-canal (CIC) hearing aids. CIC hearing aids have cosmetic and acoustic advantages but have problems of acoustic feedback and discomfort in wearing. Changes in the external auditory canal (EAC) by mandibular movements are a primary factor for the acoustic feedback and discomfort. The purpose of this research is to study the pattern of changes in the EAC by mandibular movement using the Korean population as subjects and to get basic data concerning the manufacture of impressions and shells for CIC hearing aids. MATERIALS AND METHODS: Subjects were 19 men with a fit external ear diameter to make CIC hearing aid. Impressions were made by taking 5 different conditions of jaw into consideration : fully opened state, 1/2 opened state, biting state, chewing state and resting state. Impressions were scanned at intervals of O.1mm using a 3D Laser digitizing system. We measured anterior to posterior (AP) width and superior to inferior (SI) height of each impression at the first bend, interbend and the 2nd bend of 5 conditions. RESULTS: Compared with the resting state, statistically significant changes in the AP width of the ear canal were observed at the interbend in the following order : fully opened jaw, 1/2 opened jaw (p <0.05), at 1st bend: 1/2 opened jaw (p<0.05). The 2nd bend biting state showed maximal AP width of the ear canal (p<0.05). The SI height of ear canal revealed statistically insignificant changes. CONCLUSION: We recommend making of ear impressions in the chewing state and shells to be supported at the interbend of the ear canal. The SI dimension of a shell tip should be more ground than AP dimension.