Feasibility of Speech Testing Using Wireless Connection in Single-Sided Cochlear Implant Users

Background and Objectives@#The speech tests used to evaluate language performance in patients with bilateral deafness (BiD) and cochlear implant (CI) are problematic if applied to patients with single-sided deafness (SSD) because normal ear hearing should be excluded. Thus, we investigated the feasibility of using wireless connection to evaluate speech intelligibility of the CI ear in patients with SSD. @*Subjects and Methods@#Patients with BiD and SSD were administered the word recognition scores (WRS) and speech intelligibility tests using an iPadbased wireless connection and conventional methods. To exclude normal side hearing in patients with SSD, masking noise and “plugged and muffed” method were used in the WRS and speech intelligibility tests, respectively. @*Results@#In patients with BiD, the WRS and speech intelligibility tests results using wireless connection and conventional methods were similar. In patients with SSD, the WRS using masking noise in the normal hearing ear was similar to that of using wireless connection. However, 3 of 11 patients with SSD showed under-masked results if using the “plugged and muffed” method. @*Conclusions@#Speech intelligibility testing using wireless connection is a convenient and reliable method for evaluating CI performance in patients with SSD. The “plugged and muffed” method is not recommended for evaluating CI performance in patients with SSD.

Patients Requiring Gastrostomy Tube Insertion After Total Laryngectomy Have a Higher Incidence of Otitis Media

Background and Objectives@#Some reports propose an increased risk of otitis media and hearing impairment after total laryngectomy. However, the incidence of otitis media following laryngectomy and the mechanism remain unclear. This study aimed to identify the incidence and risk factors of otitis media after total laryngectomy. @*Subjects and Methods@#This retrospective cohort study assessed 77 patients who underwent total laryngectomy from 2010 to 2020 in a tertiary referral center. Serial imaging studies (computed tomography [CT], magnetic resonance imaging, and positron emission tomography-CT) were used to assess otitis media. @*Results@#The study enrolled 58 patients (mean age, 67.0±7.7 years; male, 56 [96.6%]); nine (15.5%) underwent a gastrostomy tube (four preoperatively and five postoperatively). Otitis media was confirmed in seven (12.1%) patients. Gastrostomy tube insertion was the only significant risk factor for otitis media (p=0.012). Of the nine patients who underwent gastrostomy tube insertion, four developed otitis media; all four had the procedure after laryngectomy. @*Conclusions@#This study found an increased incidence of otitis media after total laryngectomy. Swallowing difficulties likely contribute to otitis media as it occurred more frequently in patients requiring postoperative gastrostomy tube insertion.

Effect of Dexamethasone Combination With Gentamicin in Chemical Labyrinthectomy on Hearing Preservation in Patients With Meniere’s Disease / 대한이비인후과학회지

Chemical labyrinthectomy may be performed in patients with Meniere’s disease who have intractable vertigo that does not respond to drug. By using aminoglycosides, the surgical procedure ablates vestibular type 1 hair cells. However, the risk of hearing loss remains a main concern for clinicians because gentamicin ablates cochlear hair cells as well as vestibular hair cells. To deal with the concern for hearing loss, dexamethasone can be combined with gentamicin during chemical labyrinthectomy. Herein, we show that chemical labyrinthectomy using gentamicin combined with dexamethasone preserve hearing at high-frequency compared to the conventional method.

Association Between Eustachian Tube Dysfunction Questionnaire-7 Scores and Eustachian Tube Function Test Results in Symptomatic Patients With a Normal Drum

Background and Objectives@#We investigated the clinical validity of and correlation between the Eustachian Tube Dysfunction Questionnaire-7 (ETDQ-7) scores and the eustachian tube function test (ETFT) results in patients with a normal drum. @*Subjects and Methods@#The study included 49 patients (93 ears) with unilateral or bilateral ear fullness over >3 months. All patients were administered the ETDQ-7 survey and underwent the ETFT on the same day. The receiver operating characteristic (ROC) curve and the association between the results were statistically analyzed. @*Results@#ETDQ-7 scores were not significantly correlated with the ETFT results or with middle ear pressure. ETDQ-7 scores in patients with eustachian tube dysfunction (ETD) were significantly higher than those in patients with normal ETFT results (p=0.039) when ETD was defined as a pressure change <10 daPa on the ETFT. The area under the ROC curve was 0.631, with a sensitivity of 37.0% and specificity of 89.4%. @*Conclusions@#The ETDQ-7 has limited clinical significance in patients with ETD but a normal drum. Therefore, concomitant objective tests should be performed to diagnose patients with ETD.

3D FLAIR Cochlear Abnormality Does Not Predict on Sudden Sensorineural Hearing Loss Prognosis: A Retrospective Cohort Study / 대한이비인후과학회지

Background and Objectives@#Previous studies reported abnormalities in MRI as a poor prognostic indicator of sudden sensorineural hearing loss (SSNHL). Since abnormalities in three-dimensional (3D) fluid-attenuated inversion recovery (FLAIR) are strongly correlated with the initial hearing function, the prognostic value of the 3D FLAIR images should be carefully evaluated to avoid collinearity. We aimed to evaluate abnormalities on the 3D FLAIR images as an independent prognostic factor in the matched SSNHL groups.Subjects and Method We retrospectively reviewed medical records of 179 patients with SSNHL who underwent temporal MRI, including the 3D FLAIR sequence, between January 2015 and December 2019. Patients were divided based on the presence of cochlear abnormalities on the 3D FLAIR images. Hearing prognosis was evaluated with and without matching for initial hearing and treatment interval. @*Results@#The groups were similar in sex (p=0.091), age (p=0.925), treatment interval (p= 0.216), and MRI interval (p=0.828). Notably, patients with cochlear abnormalities on the 3D FLAIR images showed distinctly more severe hearing loss (p<0.001) at the initial pure tone average (PTA) assessment and poorer outcomes (p<0.001) compared to those without abnormality. After matching for initial hearing and treatment interval, the hearing outcome, measured by PTA, was similar between the groups (p=0.681). @*Conclusion@#Cochlear signal abnormality in 3D FLAIR MRI was associated with poor initial hearing. However, it did not affect hearing recovery outcomes when the groups were matched.

Inflammatory Monocytes Infiltrate the Spiral Ligament and Migrate to the Basilar Membrane After Noise Exposure

Objectives@#. Noise exposure leads to an increase in the macrophage population. This increment is thought to be caused by the transformation of infiltrated monocytes into macrophages rather than by proliferation of the cochlear resident macrophages. However, studies on infiltrated monocytes in the cochlea are scarce. Thus, we aimed to investigate the infiltration of monocytes and their transformation into macrophages after noise exposure. @*Methods@#. In wild-type and CX3CR1+/GFP C57/B6 mice, inflammatory monocytes were identified by immunofluorescence of mouse cochlear cells. The findings were confirmed and quantitated by flow cytometry. @*Results@#. One day after noise exposure, monocytes were identified in the spiral ligament. Flow cytometric analysis confirmed that the monocyte population peaked on post-noise exposure day 1 and decreased thereafter. On day 3 after noise exposure, amoeboid-type macrophages increased in the crista basilaris, and on day 5, they spread to the basilar membrane. @*Conclusion@#. Infiltrated monocytes were successfully observed 1 day after noise exposure, preceding the increase in the macrophage population. This finding supports the proposal that infiltrated monocytes transform into macrophages.

Cochlear Implants for Patients With Common Cavity Deformities and the Impact of Electrode Positioning

Objectives@#. Common cavity deformity is a rare congenital bony labyrinth malformation associated with profound hearing loss. Cochlear implants are widely used for hearing rehabilitation for common cavity deformities; however, the reported prognosis is poor. Due to the deformed anatomical structure, it is important to consider the position of the electrodes to maximize the performance of the cochlear implant. The present study discusses the impact of electrode placement on hearing outcomes. @*Methods@#. A retrospective medical chart review of eight common cavity deformity patients (10 cochlear implants) who received cochlear implants was performed at a single university hospital. In all eight patients, implant surgery was performed using single-slit labyrinthotomy. Electrodes wer e manually bent before insertion to prevent misplacement and to reduce physical damage to the neuroepithelium. @*Results@#. Four of the 10 electrodes were misplaced, with their tips placed in the anterior semicircular canal or internal auditory canal. However, after implant surgery, all patients—including those with misplaced electrodes—gained auditory perception and improved hearing function. One patient who had electrodes that did not contact the inner wall of the cavity showed limited activity of the electrodes (27%) compared to others (64%–100%). @*Conclusion@#. Proper contact of the electrode with the inner wall was more likely to be important for cochlear implant success in cases of common cavity deformity than appropriate placement of the electrode tip.

Relationship between Hearing Loss and Dementia Differs According to the Underlying Mechanism

Background@#and Purpose The associations between hearing loss (HL) and the mechanisms underlying cognitive impairment (CI) remain unclear. We evaluated the effects of clinical factors, vascular magnetic resonance imaging (MRI) markers, and CI mechanisms on HL. @*Methods@#In total, 112 patients with CI (59% demented) and subjective HL prospectively underwent MRI, amyloid positron-emission tomography (PET), hearing evaluations, and neuropsychological tests including a language comprehension test. Patients were categorized into pure-Alzheimer’s disease-related CI (ADCI), pure-Lewy-body disease-related CI (LBCI), mixed-ADCI/LBCI, and non-ADCI/LBCI groups based on clinical features and PET biomarkers. @*Results@#The risk of peripheral HL [defined as a pure-tone average (PTA) threshold >40 dB] was higher in the pure-LBCI group than in the pure-ADCI and mixed-ADCI/LBCI groups, and lower in the presence of ADCI. The non-ADCI/LBCI group had the most-severe vascular MRI markers and showed a higher risk of peripheral HL than did the pure-ADCI and mixed-ADCI/LBCI groups. While the pure-LBCI group had a higher risk of comprehension dysfunction than the pure-ADCI group regardless of the PTA and the score on the Korean version of the Mini Mental State Examination (K-MMSE), those in the pure-LBCI group even with a better K-MMSE score had a risk of comprehension dysfunction comparable to that in the mixed-ADCI/LBCI group due to a worse PTA. @*Conclusions@#Peripheral HL could be associated with the absence of significant β-amyloid deposition in patients with CI and characteristic of the pure-LBCI and non-ADCI/LBCI groups.

Three-Dimensional Distribution of Cochlear Macrophages in the Lateral Wall of Cleared Cochlea

Objectives@#. Resident macrophages are well known to be present in the cochlea, but the exact patterns thereof in spiral ligaments have not been discussed in previous studies. We sought to document the distribution of macrophages in intact cochleae using three-dimensional imaging. @*Methods@#. Cochleae were obtained from C-X3-C motif chemokine receptor 1+/GFP mice, and organ clearing was performed. Three-dimensional images of cleared intact cochleae were reconstructed using two-photon microscopy. The locations of individual macrophages were investigated using 100-μm stacked images to reduce bias. Cochlear inflammation was then induced by lipopolysaccharide (LPS) inoculation into the middle ear through the tympanic membrane. Four days after inoculation, three-dimensional images were obtained. @*Results@#. Macrophages were scarce in areas adjacent to the stria vascularis, particularly the area just beneath it even though many have suspected macrophages to be abundant in this area. This finding remained consistent upon LPS-induced cochlear inflammation, despite a significant increase in the number of macrophages, compared to non-treated cochlea. @*Conclusion@#. Resident macrophages in spiral ligaments are scarce in areas adjacent to the stria vascularis.

Importance of High-Frequency Vestibular Function in the Prognosis of Bilateral Vestibulopathy

Objectives@#. The aim of this study was to investigate whether preserved vestibular function in the high-frequency range influences the prognosis of patients with bilateral vestibulopathy (BVP) after vestibular rehabilitation. @*Methods@#. Twenty-four patients followed up with vestibular rehabilitation were recruited. The enrolled patients were divided into two groups according to the preservation of the high-frequency vestibulo-ocular reflex (VOR) based on the video head impulse test (vHIT). The results of computerized dynamic posturography and the Dizziness Handicap Inventory (DHI) survey collected at baseline and at the 6-month follow-up after vestibular rehabilitation therapy were analyzed. @*Results@#. Both groups showed significantly increased composite and DHI scores after follow-up with vestibular rehabilitation. The group with preserved high-frequency VOR showed a better composite score (p=0.064) and vestibular score (p= 0.008) than the group with lost high-frequency VOR at the 6-month follow up. The DHI score significantly decreased only in the group with lost high-frequency VOR (p=0.047). Among the three vestibular function tests (caloric test, rotary chair test, and vHIT) used to diagnose BVP, only vHIT showed a significant correlation (p=0.015) with a favorable prognosis (composite score ≥70). @*Conclusion@#. Better treatment outcomes are likely in patients with BVP with preserved vestibular function in response to high-frequency stimulation, as measured by the vHIT.

Relationship between Hearing Loss and Dementia Differs According to the Underlying Mechanism

Background@#and Purpose The associations between hearing loss (HL) and the mechanisms underlying cognitive impairment (CI) remain unclear. We evaluated the effects of clinical factors, vascular magnetic resonance imaging (MRI) markers, and CI mechanisms on HL. @*Methods@#In total, 112 patients with CI (59% demented) and subjective HL prospectively underwent MRI, amyloid positron-emission tomography (PET), hearing evaluations, and neuropsychological tests including a language comprehension test. Patients were categorized into pure-Alzheimer’s disease-related CI (ADCI), pure-Lewy-body disease-related CI (LBCI), mixed-ADCI/LBCI, and non-ADCI/LBCI groups based on clinical features and PET biomarkers. @*Results@#The risk of peripheral HL [defined as a pure-tone average (PTA) threshold >40 dB] was higher in the pure-LBCI group than in the pure-ADCI and mixed-ADCI/LBCI groups, and lower in the presence of ADCI. The non-ADCI/LBCI group had the most-severe vascular MRI markers and showed a higher risk of peripheral HL than did the pure-ADCI and mixed-ADCI/LBCI groups. While the pure-LBCI group had a higher risk of comprehension dysfunction than the pure-ADCI group regardless of the PTA and the score on the Korean version of the Mini Mental State Examination (K-MMSE), those in the pure-LBCI group even with a better K-MMSE score had a risk of comprehension dysfunction comparable to that in the mixed-ADCI/LBCI group due to a worse PTA. @*Conclusions@#Peripheral HL could be associated with the absence of significant β-amyloid deposition in patients with CI and characteristic of the pure-LBCI and non-ADCI/LBCI groups.

Three-Dimensional Distribution of Cochlear Macrophages in the Lateral Wall of Cleared Cochlea

Objectives@#. Resident macrophages are well known to be present in the cochlea, but the exact patterns thereof in spiral ligaments have not been discussed in previous studies. We sought to document the distribution of macrophages in intact cochleae using three-dimensional imaging. @*Methods@#. Cochleae were obtained from C-X3-C motif chemokine receptor 1+/GFP mice, and organ clearing was performed. Three-dimensional images of cleared intact cochleae were reconstructed using two-photon microscopy. The locations of individual macrophages were investigated using 100-μm stacked images to reduce bias. Cochlear inflammation was then induced by lipopolysaccharide (LPS) inoculation into the middle ear through the tympanic membrane. Four days after inoculation, three-dimensional images were obtained. @*Results@#. Macrophages were scarce in areas adjacent to the stria vascularis, particularly the area just beneath it even though many have suspected macrophages to be abundant in this area. This finding remained consistent upon LPS-induced cochlear inflammation, despite a significant increase in the number of macrophages, compared to non-treated cochlea. @*Conclusion@#. Resident macrophages in spiral ligaments are scarce in areas adjacent to the stria vascularis.

Importance of High-Frequency Vestibular Function in the Prognosis of Bilateral Vestibulopathy

Objectives@#. The aim of this study was to investigate whether preserved vestibular function in the high-frequency range influences the prognosis of patients with bilateral vestibulopathy (BVP) after vestibular rehabilitation. @*Methods@#. Twenty-four patients followed up with vestibular rehabilitation were recruited. The enrolled patients were divided into two groups according to the preservation of the high-frequency vestibulo-ocular reflex (VOR) based on the video head impulse test (vHIT). The results of computerized dynamic posturography and the Dizziness Handicap Inventory (DHI) survey collected at baseline and at the 6-month follow-up after vestibular rehabilitation therapy were analyzed. @*Results@#. Both groups showed significantly increased composite and DHI scores after follow-up with vestibular rehabilitation. The group with preserved high-frequency VOR showed a better composite score (p=0.064) and vestibular score (p= 0.008) than the group with lost high-frequency VOR at the 6-month follow up. The DHI score significantly decreased only in the group with lost high-frequency VOR (p=0.047). Among the three vestibular function tests (caloric test, rotary chair test, and vHIT) used to diagnose BVP, only vHIT showed a significant correlation (p=0.015) with a favorable prognosis (composite score ≥70). @*Conclusion@#. Better treatment outcomes are likely in patients with BVP with preserved vestibular function in response to high-frequency stimulation, as measured by the vHIT.

Two Cases of Seronegative Paraneoplastic Neurologic Syndrome with Opsoclonus Nystagmus / 대한이비인후과학회지

The dizziness associated with paraneoplastic neurologic syndrome is hard to diagnose clinically because the prevalence of disease is rare, and radiologic and serologic examination result may come out normal. Opsoclonus-myoclonus symdrome is a representative of classical paraneoplastic neurologic syndromes. In this paper, we report 2 cases of paraneoplastic neurologic syndromes with negative serologic auto-antibody test and no brain lesion on MRI. Both cases were eventually diagnosed through PET. Patients with opsoclonus-myoclonus type nystagmus should be evaluated for paraneoplastic neurologic syndrome even if their radiologic and serologic findings are normal.

Educational Status in Bilateral Prelingual Deaf Children with Cochlear Implantation

BACKGROUND AND OBJECTIVES: This study was undertaken to investigate the educational status in bilateral prelingual deaf children with a cochlear implant (CI), also known as early cochlear implantees (CIs). Type of schooling and enrollment rate of tertiary education were analyzed as primary results. SUBJECTS AND METHODS: Participants in this study comprised a highly homogeneous group of deaf patients who underwent cochlear implantation at a similar age. Sixty-four Korean patients were enrolled. Statistical data for disabled populations and the general population were obtained from the National Statistics Korea. RESULTS: Among 64 patients, 46, 8, and 10 attended mainstream, integrated, and special schools, respectively. Notably, there was a significant difference in the type of school between hearing-impaired and CI groups (p=0.007). Ten of 13 patients enrolled in tertiary education. CONCLUSIONS: CI users were more likely than hearing impaired students to attend mainstream school. The enrollment rate of CI users in tertiary education was the same as that of the general population.

Hyperbilirubinemia and Follow-up Auditory Brainstem Responses in Preterm Infants

OBJECTIVES.: Neonatal hyperbilirubinemia is considered one of the most common causative factors of hearing loss. Preterm infants are more vulnerable to neuronal damage caused by hyperbilirubinemia. This study aimed to evaluate the effect of hyperbilirubinemia on hearing threshold and auditory pathway in preterm infants by serial auditory brainstem response (ABR). In addition, we evaluate the usefulness of the unconjugated bilirubin (UCB) level compared with total serum bilirubin (TSB) on bilirubin-induced hearing loss. METHODS.: This study was conducted on 70 preterm infants with hyperbilirubinemia who failed universal newborn hearing screening by automated ABR. The diagnostic ABR was performed within 3 months after birth. Follow-up ABR was conducted in patients with abnormal results (30 cases). TSB and UCB concentration were compared according to hearing threshold by ABR. RESULTS.: The initial and maximal measured UCB concentration for the preterm infants of diagnostic ABR ≥40 dB nHL group (n=30) were statistically higher compared with ABR ≤35 dB nHL group (n=40) (P=0.031 and P=0.003, respectively). In follow-up ABR examination, 13 of the ABR ≥40 dB nHL group showed complete recovery, but 17 had no change or worsened. There was no difference in bilirubin level between the recovery group and non-recovery group. CONCLUSION.: UCB is a better predictor of bilirubin-induced hearing loss than TSB in preterm infants as evaluated by serial ABR. Serial ABR testing can be a useful, noninvasive methods to evaluate early reversible bilirubin-induced hearing loss in preterm infants.

Two Cases of Seronegative Paraneoplastic Neurologic Syndrome with Opsoclonus Nystagmus / 대한이비인후과학회지

The dizziness associated with paraneoplastic neurologic syndrome is hard to diagnose clinically because the prevalence of disease is rare, and radiologic and serologic examination result may come out normal. Opsoclonus-myoclonus symdrome is a representative of classical paraneoplastic neurologic syndromes. In this paper, we report 2 cases of paraneoplastic neurologic syndromes with negative serologic auto-antibody test and no brain lesion on MRI. Both cases were eventually diagnosed through PET. Patients with opsoclonus-myoclonus type nystagmus should be evaluated for paraneoplastic neurologic syndrome even if their radiologic and serologic findings are normal.

Prognosis of Sudden Low Frequency Hearing Loss during Long-term Follow-up / 대한평형의학회지

OBJECTIVES: The prognosis of sudden low frequency hearing loss (SLFHL) is relatively good, but recurrences of hearing loss and possible progression to Meniere's disease is still a clinically important concern. This study was conducted to confirm the rate at which SLFHL proceeds to Meniere's disease. METHODS: We retrospectively analyzed the medical records of 160 SLFHL patients who were followed up for more than 6 months from September 2005 to August 2013. Progression, initial hearing level, recovery and recurrence of hearing loss were reviewed. RESULTS: 106 patients (66.25%) had complete hearing recovery, 32 (20%) had recurrent hearing loss. Of the 32 recurrent group, 15 (9.38%) had progressed to Meniere's disease after average of 1.7±1.4 years. The mean age of nonrecurrent group was higher than recurrent group (55.3±14.6 and 48.0±13.4, respectively, p=0.011). The threshold of 250Hz was significantly higher in the nonrecurrent group compared with recurrent group (p=0.047). CONCLUSIONS: In patients with SLFHL, recurrence at relatively young age should be considered with the possibility of progression to Meniere's disease.

A Case of Central Origin Vertigo in Breast Cancer with Brain Metastasis / 대한이비인후과학회지

The incidence of symptomatic brain metastasis among women with breast cancer ranges from 10 to 16%. A metastatic spread of breast cancer to either the brain parenchyma or the leptomeninges is generally a late feature of metastatic progression. The present case of a 55-year-old patient had been considered cured from breast cancer. However, she recently complained of severe dizziness, hearing impairment, and headache. Initially, it was considered to be Meniere's disease or vestibular migraine, although later dizziness, hearing impairment, and headache were aggravated in spite of medication. Pure tone audiometry showed left-sided sensorineural hearing loss, but her vestibular function was within normal range. She underwent a Gadolinium-enhanced magnetic resonance imaging, and a metastatic brain lesion was noted. With a review of the literature, we present this case of breast cancer with brain metastasis in a 55-year-old woman with vertigo and hearing loss.