Cochlear Implantation via the Transmeatal Approach in an Adolescent with Hunter Syndrome—Type II Mucopolysaccharidosis

Type II mucopolysaccharidosis (MPS II) commonly known as Hunter syndrome, is a rare X-linked lysosomal storage disorder caused by iduronate-2-sulfatase deficiency, which in turn causes otorhinolaryngological manifestations, including sensorineural hearing loss (SNHL). Previously, the median survival age of patients with MPS was approximately 13.4 years. However, in the era of enzyme replacement therapy and other multidisciplinary care modalities, the life expectancy has increased. Herein, we report a rare case of an adolescent with MPS II who underwent SNHL treatment with cochlear implantation (CI). Based on unexpected findings of mastoid emissary veins and overgrowth of the vessels around the temporal bone, CI was performed using the transmeatal approach instead of the conventional transmastoid method, to avoid damage to the vessels. The average hearing threshold after CI was 35 dB and no surgical complications were encountered. Adolescent MPS II may present vessel abnormalities, which can reduce the success rate of surgery. In patients with MPS II with SNHL, CI should be performed under careful monitoring of vessel overgrowth. Moreover, with regard to feasibility of CI in adolescent patients with MPS II with SNHL, surgical techniques such as the transmeatal approach should be selected based on adequate assessment of the case.

Changes in Vestibular Symptoms and Function After Cochlear Implantation: Relevant Factors and Correlations With Residual Hearing

Objectives@#. The aim of this study was to evaluate vestibular function loss after cochlear implantation (CI) and the relationship between vestibular function and hearing changes. @*Methods@#. Seventy-five patients with CI were enrolled and divided into those with normal preoperative caloric function (group I) and those with a normal preoperative waveform in cervical vestibular evoked myogenic potential (c-VEMP) testing (group II). The relationship between hearing and changes in the vestibular system was analyzed preoperatively and at 3 and 6 months postoperatively. @*Results@#. In group I, unilateral weakness on the implanted side was detected in five (7.7%) and eight (12.3%) patients at 3 and 6 months post-CI, respectively. By 3 months post-CI, the total slow-phase velocity (SPV; warm and cold stimulations) was significantly different between the implanted and non-implanted sides (P=0.011), and the shift in total SPV from pre- to post-CI was significantly correlated with the average hearing threshold at 6 months post-CI. In group II, an abnormal c-VEMP was detected on the implanted side in six patients (16.2%) at 3 months post-CI, and in six patients (16.2%) at 6 months post-CI. Significant changes were noticed in the P1 and N1 amplitude at 3 months postCI (P=0.027 and P=0.019, respectively). @*Conclusion@#. Vestibular function and residual hearing function should be afforded equal and simultaneous consideration in terms of preservation.

Efficacy of Tympanoplasty Without Mastoidectomy for Treating Chronic Otitis Media in Patients With Mastoid Cavity Opacification in Temporal Bone Computed Tomography Findings

OBJECTIVES: Combined mastoidectomy is generally preferred to tympanoplasty alone when treating patients with chronic otitis media (COM), particularly when temporal bone computed tomography (TBCT) shows that the mastoid cavity contains opacification of soft tissue density. However, in cases with Eustachian tube dysfunction, a mastoid cavity volume may be a burden to its function. We hypothesized that tympanoplasty alone might be better than tympanoplasty combined with mastoidectomy because soft tissue in the mastoid cavity is a sequel to a protective physiological response. Thus, we explored the efficacy of tympanoplasty without mastoidectomy in COM patients exhibiting mastoid air cell opacification on TBCT. METHODS: Between 2010 and 2014, a total of 33 patients, diagnosed with COM and with evidence of mastoid cavity opacification on TBCT, underwent tympanoplasty without mastoidectomy. All ears had been dry for ≥3 months before surgery. All procedures were performed by the same surgeon. We retrospectively analyzed the preoperative otoscopic findings, pre- and postoperative pure tone averages (PTAs; the mean of the values at 0.5, 1, 2, and 4 kHz), surgical procedures, and complications or recurrence. RESULTS: Of the 33 patients, 28 (84.8%) exhibited hearing improvement after surgery. The mean pre- and postoperative PTAs were 46.9±21.2 dB and 29.4±17.0 dB, respectively (P < 0.001). The air-bone gap decreased from 25.7±10.7 dB to 10.3±8.7 dB (P < 0.001). Thirty-two patients (97.0%) did not develop any COM recurrence or cholesteatoma; one patient developed attic retraction of the tympanic membrane. Other minor complications were transient otorrhea caused by myringitis (two cases) and a pinpoint perforation (one case). CONCLUSION: Tympanoplasty alone, i.e., without mastoidectomy, may adequately control COM, if it shows dry-up status for at least 3 months even though mastoid cavity opacification is detected in TBCT.

Development of Simplified Ajou Version of MarkeTrak Survey to Evaluate Hearing Aid Use / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: MarkeTrak Survey is designed to evaluate the sociodemographic and HA factors of hearing aid (HA) users and their satisfaction of use. The questionnaire is inconvenient to use because it asked too many questions and requires long time to answer. The purpose of this study was to develop a simplified ("Ajou") version of MarkeTrak Survey and to evaluate its reliability. SUBJECTS AND METHOD: Forty-five HA users who visited the Ajou Hearing Center from 2014 to 2015 were included in the present study. To examine the correlation of the Korean version and the Ajou version of MarkeTrak Survey, 42 patients completed each survey simultaneously. To examine the reliability of Ajou version, test-retest and internal consistency methods were used. For the test-retest method, 22 HA users repeated the survey 2-4 weeks later. RESULTS: The newly developed "Ajou" version of MarkeTrak Survey consisted of 12 questions and was more simplified. Most of the questions of the two surveys showed significant correlation coefficient values (Spearman correlation, p<0.05). For the test-retest method, most questions showed significant correlation coefficient values (Spearman correlation, p<0.05), but they showed lower values than 0.6 in many cases. It showed high internal consistency of overall HA satisfaction. CONCLUSION: The Ajou version may be used as a simplified and alternative questionnaire for the Korean version of MarkeTrak Survey.

A Case of Lagophthalmos after a Corrective Rhinoplasty / 대한이비인후과학회지

Corrective rhinoplasty, a commonly performed plastic surgery, is occasionally followed by numerous risks and complications. In this report, we present, with discussion of the causes and proper management, a case of lagophthalmos that occurred after a rhinoplasty. A 35-year-old female visited our outpatient clinic due to continuous nasal obstruction even after septoplasty. After thorough evaluation, corrective rhinoplasty was performed to release the patient's nasal symptoms and manage the deviated external nose and nasal septum. During the surgery, we encountered excessive nasal bleeding after percutaneous osteotomy. In addition, immediate postoperative findings presented left periorbital edema without limited eye movement or reduced eye vision associated with the paralysis of the eyelid localized to the medial side of the left upper palpebra. Surgeons should be aware of rare but possible complications of corrective rhinoplasty such as lagophthalmos, and a rapid and intensive care is recommended for early management and better prognosis of postoperative complications.

Two Cases of Acute Mastoiditis with Subperiosteal Abscess

The incidence of mastoiditis in pediatric age has consistently increased over the last two decades due to increase of antibiotic-resistant bacteria. Compared to young patients, occurrence of acute otitis media complications such as acute mastoiditis and subperiosteal abscess is relatively low in adults. Various treatments for acute mastoiditis with subperiosteal abscess such as tympanostomy tube insertion, intravenous antibiotics, and postauricular incision and drainage have avoided the morbidity and necessity of mastoid surgery. Recently, many studies have indicated mastoidectomy only in cases of severe complications or failure of disease improvement after antibiotic treatment and myringotomy. In this report, we present two cases of successful treatment of subperiosteal abscess and discuss the management of acute mastoiditis with subperiosteal abscess in both child and adult.