Diagnosis of Primary Ciliary Dyskinesia via Whole Exome Sequencing and Histologic Findings

Purpose@#To assess the diagnostic potential of whole-exome sequencing (WES) and elucidate the clinical and genetic characteristics of primary ciliary dyskinesia (PCD) in the Korean population. @*Materials and Methods@#Forty-seven patients clinically suspected of having PCD were enrolled at a tertiary medical center. WES was performed in all patients, and seven patients received biopsy of cilia and transmission electron microscopy (TEM). @*Results@#Overall, PCD was diagnosed in 10 (21.3%) patients: eight by WES (8/47, 17%), four by TEM. Among patients diagnosed as PCD based on TEM results, two patients showed consistent results with WES and TEM of PCD (2/4, 50%). In addition, five patients, who were not included in the final PCD diagnosis group, had variants of unknown significance in PCD-related genes (5/47, 10.6%).The most frequent pathogenic (P)/likely pathogenic (LP) variants were detected in DNAH11 (n=4, 21.1%), DRC1 (n=4, 21.1%), and DNAH5 (n=4, 21.1%). Among the detected 17 P/LP variants in PCD-related genes in this study, 8 (47.1%) were identified as novel variants. Regarding the genotype–phenotype correlation in this study, the authors experienced severe PCD cases caused by the LP/P variants in MCIDAS, DRC1, and CCDC39. @*Conclusion@#Through this study, we were able to confirm the value of WES as one of the diagnostic tools for PCD, which increases with TEM, rather than single gene tests. These results will prove useful to hospitals with limited access to PCD diagnostic testing but with relatively efficient in-house or outsourced access to genetic testing at a pre-symptomatic or early disease stage.

Prevalence and Clinical Characteristics of Mitochondrial DNA Mutations in Korean Patients With Sensorineural Hearing Loss

Background@#Mutations in mitochondrial DNA (mtDNA) are associated with several genetic disorders, including sensorineural hearing loss. However, the prevalence of mtDNA mutations in a large cohort of Korean patients with hearing loss has not yet been investigated. Thus, this study aimed to investigate the frequency of mtDNA mutations in a cohort of with pre- or post-lingual hearing loss of varying severity. @*Methods@#A total of 711 Korean families involving 1,099 individuals were evaluated. Six mitochondrial variants associated with deafness (MTRNR1 m.1555A>G, MTTL1 m.3243A>G, MTCO1 m.7444G>A and m.7445A>G, and MTTS1 m.7471dupC and m.7511T>C) were screened using restriction fragment length polymorphism. The prevalence of the six variants was also analyzed in a large control dataset using whole-genome sequencing data from 4,534 Korean individuals with unknown hearing phenotype. @*Results@#Overall, 12 of the 711 (1.7%) patients with hearing loss had mtDNA variants, with 10 patients from independent families positive for the MTRNR1 m.1555A>G mutation and 2 patients positive for the MTCO1 m.7444G>A mutation. The clinical characteristics of patients with the mtDNA variants were characterized by post-lingual progressive hearing loss due to the m.1555A>G variant (9 of 472; 1.9%). In addition, 18/4,534 (0.4%) of the Korean population have mitochondrial variants associated with hearing loss, predominantly the m.1555A>G variant. @*Conclusion@#A significant proportion of Korean patients with hearing loss is affected by the mtDNA variants, with the m.1555A>G variant being the most prevalent. These results clarify the genetic basis of hearing loss in the Korean population and emphasize the need for genetic testing for mtDNA variants.

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.

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.

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.

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.

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.

Audiological and Vestibular Functions in Patients With Lateral Semicircular Canal Dysplasia and Aplasia

Objectives@#. The aim of the present study was to evaluate audiologic and vestibular functions in patients with lateral semicircular canal (LSCC) dysplasia/aplasia. @*Methods@#. We conducted a retrospective study of a patients with LSCC dysplasia and aplasia at tertiary referral center. The subjects included 15 patients with LSCC dysplasia or aplasia, with or without combined inner ear anomalies. Medical history, temporal bone computed tomography scans, pure-tone audiograms, and vestibular function test results were analyzed. @*Results@#. LSCC anomaly was identified in 15 patients (20 ears). Nine patients had unilateral LSCC dysplasia only and showed a mean pure-tone average of 45.5±28.7 dB, while three patients (33.3%) among them had normal hearing. Six patients had bilateral LSCC dysplasia/aplasia combined with other inner ear anomalies and profound bilateral hearing loss. Notably, only four out of 15 patients (26.7%) had dizziness symptoms. On caloric test, patients with isolated LSCC dysplasia showed a 51.8%±29.3% level of canal paresis (eight out of nine patients showed anomalies), whereas patients with bilateral LSCC dysplasia/aplasia presented bilateral vestibular loss. One patient with isolated LSCC underwent video-head impulse test; horizontal canal gain decreased to 0.62 (17% asymmetry) and anterior canal gain was 0.45 (52.6% asymmetry), whereas posterior canal gain was normal. @*Conclusion@#. Bilateral LSCC dysplasia/aplasia is comorbid with other inner ear anomalies and presents as profound bilateral hearing loss and vestibulopathy. In contrast, isolated unilateral LSCC dysplasia presents as ipsilateral horizontal canal paresis. Hearing function in isolated LSCC dysplasia is usually, but not always, impaired with varying severity.

Which Patients With a Unilateral Hearing Aid for Symmetric Sensorineural Hearing Loss Have Auditory Deprivation?

Objectives@#. The aim of study is to find conditions that aggravate auditory deprivation in patients with symmetric hearing loss after unilateral digital, non-linear hearing aid (HA). @*Methods@#. In the retrospective case-comparison study, we assessed 47 patients with symmetric sensorineural hearing loss (SNHL), wearing unilateral conventional HAs. Audiological outcomes were assessed >1 year after HA fitting (mean duration, 31.0 months). Pure-tone audiometry in HA-aided and HA-unaided conditions was performed over time. Word recognition score (WRS) was evaluated at the most comfortable listening level. @*Results@#. The initial pure tone average of four frequency thresholds at 500, 1,000, 2,000, and 4,000 Hz (PTA4) did not show a difference of >5 dB HL between HA-aided and HA-unaided ears. WRS progressively decreased for both HA-aided and HA-unaided ears although the extent of decrease was significantly greater for HA-unaided (7.6%) than for HA-aided ears (5.1%, P<0.05). Notably, auditory deprivation in HA-unaided ears was significantly greater in patients with an initial PTA4 ≥53 dB HL (P<0.001). @*Conclusion@#. Bilateral HAs are strongly recommended, particularly for patients with moderate to severe SNHL to prevent auditory deprivation in the contralateral ear.

Long-Term Changes in Video Head Impulse and Caloric Tests in Patients with Unilateral Vestibular Neuritis / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Video head impulse tests (vHITs) and caloric tests are widely used to assess the loss of vestibular function in acute vestibular neuritis. Although previous studies have reported on the results of each test, longitudinal comparison of these tests is rare. In the present study, vHITs and caloric tests were performed in patients with unilateral vestibular neuritis during the acute phase and after a long follow-up period (>6 months). The goal of this study was to evaluate the changes in vHIT and caloric test results and to analyze the relationships between them. SUBJECTS AND METHOD: Between September 2013 and December 2015, charts from 13 patients with unilateral vestibular neuritis were retrospectively reviewed. Among the 13 patients, caloric tests and vHITs were performed in 9 and 10 patients, respectively. Results of the vHITs and caloric tests were analyzed and the changes were compared. RESULTS: During the acute phase of vestibular neuritis, the results of the caloric test showed an increase in canal paresis (CP), and the results of the vHIT showed a decrease in horizontal gain. Although subjective symptoms improved in all patients after a long follow-up period (mean: 13.9 months), the occurrence of CP determined from the caloric test was not significantly changed (p=0.889). On the other hand, the mean horizontal gain of the vHIT had improved significantly (p < 0.05). CONCLUSION: While CP determined from the caloric test did not change after a long follow-up period, the decreased horizontal gain in the vHIT was significantly recovered in patients with unilateral vestibular neuritis.

Feasibility of Revision Cochlear Implant Surgery for Better Speech Comprehension

BACKGROUND AND OBJECTIVES: The purpose of this study was to evaluate the efficacy of revision cochlear implant (CI) surgery for better speech comprehension targeting patients with low satisfaction after first CI surgery. SUBJECTS AND METHODS: Eight patients who could not upgrade speech processors because of an too early CI model and who wanted to change the whole system were included. After revision CI surgery, we compared speech comprehension before and after revision CI surgery. Categoies of Auditory Performance (CAP) score, vowel and consonant confusion test, Ling 6 sounds, word and sentence identification test were done. RESULTS: The interval between surgeries ranged from eight years to 19 years. Same manufacturer’s latest product was used for revision surgery in six cases of eight cases. Full insertion of electrode was possible in most of cases (seven of eight). CAP score (p-value=0.01), vowel confusion test (p-value=0.041), one syllable word identification test (p-value=0.026), two syllable identification test (p-value=0.028), sentence identification test (pvalue=0.028) had significant improvement. Consonant confusion test (p-value=0.063), Ling 6 sound test (p-value=0.066) had improvement but it is not significant. CONCLUSIONS: Although there are some limitations of our study design, we could identify the effect of revision (upgrade) CI surgery indirectly. So we concluded that if patient complain low functional gain or low satisfaction after first CI surgery, revision (device upgrade) CI surgery is meaningful even if there is no device failure.

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.

Non-syndromic hearing loss caused by the dominant cis mutation R75Q with the recessive mutation V37I of the GJB2 (Connexin 26) gene

GJB2 alleles containing two cis mutations have been rarely found in non-syndromic hearing loss. Herein, we present a Korean patient with non-syndromic hearing loss caused by the R75Q cis mutation with V37I, which arose de novo in the father and was inherited by the patient. Biochemical coupling and hemichannel permeability assays were performed after molecular cloning and transfection of HEK293T cells. Student's t-tests or analysis of variance followed by Tukey's multiple comparison test was used as statistical analysis. Biochemical coupling was significantly reduced in connexin 26 (Cx26)-R75Q- and Cx26-V37I-transfected cells, with greater extent in Cx26-R75Q and Cx26-R75Q+V37I cells. Interestingly, our patient and his father with the mutations had more residual hearing compared with patients with the dominant mutation alone. Although the difference in hemichannel activity between R75Q alone and R75Q in combination with V37I failed to reach significance, it is of note that there is a possibility that V37I located upstream of R75Q might have the ability to ameliorate R75Q expression. Our study emphasizes the importance of cis mutations with R75Q, as the gene effect of R75Q can be modulated depending on the type of additional mutation.

Two Cases of Diffuse Large B-cell Lymphoma of Sinonasal Tract

Nasal lymphoma is an uncommon neoplasm in the sinonasal tract. The NK/T-cell type of lymphoma is more commonly found in Asian populations. However, B-cell lymphoma is more common in the western countries whereas it is rare in Asia. Recently, we experienced two cases of sinonasal B-cell lymphoma, which are rare cases in Korea, one in an advanced stage and one in an early stage. Both patients had no specific nasal symptoms or systemic B symptoms (fever, night sweat, weight loss). As our cases demonstrate, diagnosis of B cell lymphoma is often delayed in many cases because B-cell lymphoma does not show any specific symptoms. Thus, careful physical examination and biopsy under suspicion of malignancy are important for early diagnosis of nasal lymphoma.

Clinical Presentation and Management of Jugular Foramen Paraganglioma

OBJECTIVES: Jugular foramen paraganglioma is a locally invasive, benign tumor, which grow slowly and causes various symptoms such as pulsatile tinnitus and low cranial nerve palsy. Complete surgical resection is regarded as the ideal management of these tumors. The goal of this study is to identify the clinical characteristics and most effective surgical approach for jugular foramen paraganglioma. METHODS: Retrospective analysis of 9 jugular foramen paraganglioma patients who underwent surgical resection between 1986 and 2005 was performed. Clinical records were reviewed for analysis of initial clinical symptoms and signs, audiological examinations, neurological deficits, radiological features, surgical approaches, extent of resection, treatment outcomes and complications. RESULTS: Most common initial symptom was hoarseness, followed by pulsatile tinnitus. Seven out of 9 patients had at least one low cranial nerve palsy. Seven patients were classified as Fisch Type C tumor and remaining 2 as Fisch Type D tumor on radiologic examination. Total of 11 operations took place in 9 patients. Total resection was achieved in 6 cases, when partial resection was done in 3 cases. Two patients with partial resection received gamma knife radiosurgery (GKS), when remaining 1 case received both GKS and two times of revision operation. No mortality was encountered and there were few postoperative complications. CONCLUSION: Neurologic examination of low cranial nerve palsy is crucial since most patients had at least one low cranial nerve palsy. All tumors were detected in advanced stage due to slow growing nature and lack of symptom. Angiography with embolization is crucial for successful tumor removal without massive bleeding. Infratemporal fossa approach can be considered as a safe, satisfactory approach for removal of jugular foramen paragangliomas. In tumors with intracranial extension, combined approach is recommended in that it provides better surgical view and can maintain the compliance of the patients.

A Case of Inverted Papilloma Originating from the Middle Ear / 대한이비인후과학회지

Inverted papilloma (Schneiderian-type papilloma) involving the middle ear is extremely rare. Most of cases originate from the mucosa of the lateral nasal wall, extending into the paranasal sinuses and orbits. Inverted papilloma involving the middle ear has a high recurrence rate and a possibility of malignant change. We experienced a case of inverted papilloma of the middle ear secondary to congenital cholesteatoma surgery. A nine-year old male who was diagnosed with congenital cholesteatoma was treated with intact canal wall tympanomastoidectomy. Nine months later, he underwent a second look operation. In the second surgery, a 3 mm round pinkish mass which was located at the tympanic orifice of Eustachian tube was identified as inverted papilloma. There was no recurrence of cholesteatoma or inverted papilloma four months after the 2nd operation.

Statistical Analysis of Pure Tone Audiometry and Caloric Test in Herpes Zoster Oticus

OBJECTIVES: Pure tone audiometry and caloric test in patients with herpes zoster oticus were performed to determine the biologic features of the varicella zoster virus (VZV) and the pathogenesis of vestibulocochlear nerve disease in herpes zoster oticus. Study Design: A retrospective chart review of 160 patients with herpes zoster oticus was designed in order to determine the classic characteristics of vestibulocochlear nerve disease associated with the syndrome. Speech frequency and isolated high frequency acoustic thresholds were analyzed based on severity of facial paralysis and patient age. Patients without cochlear symptoms were selected randomly, and audiological function was evaluated. Patients with symptoms of vestibular dysfunction underwent the caloric test, and canal paresis was analyzed according to the severity of facial paralysis and the age of each patient. RESULTS: Among the 160 patients, 111 exhibited pure tone audiometry; 26 (79%) of the patients with cochlear symptoms and 44 (56%) of the patients without cochlear symptoms had abnormal audiological data. Among the patients without cochlear symptoms, 15 (19%) had hearing loss at speech frequency, and 42 (54%) had hearing loss isolated to high frequency. The incidence of cochlear symptoms in herpes zoster oticus was not related to the severity of facial paralysis. The incidence of patients with isolated high frequency hearing loss statistically increased with age, however the incidence of patients with speech frequency hearing loss did not increase. Thirteen patients complained vertigo, and the incidence of vestibular disturbances and the value of canal paresis in the caloric test increased to statistical significance in parallel with increasing severity of facial paralysis. CONCLUSION: Mild or moderate cochlear symptoms with high frequency hearing loss were related to age, and severe vestibular symptoms were related to the severity of facial paralysis after onset of herpetic symptoms. This study might suggest us a clue to the pathogenesis of vestibulocochlear nerve disease in herpes zoster oticus.

Statistical Analysis of Pure Tone Audiometry and Caloric Test in Herpes Zoster Oticus

OBJECTIVES: Pure tone audiometry and caloric test in patients with herpes zoster oticus were performed to determine the biologic features of the varicella zoster virus (VZV) and the pathogenesis of vestibulocochlear nerve disease in herpes zoster oticus. Study Design: A retrospective chart review of 160 patients with herpes zoster oticus was designed in order to determine the classic characteristics of vestibulocochlear nerve disease associated with the syndrome. Speech frequency and isolated high frequency acoustic thresholds were analyzed based on severity of facial paralysis and patient age. Patients without cochlear symptoms were selected randomly, and audiological function was evaluated. Patients with symptoms of vestibular dysfunction underwent the caloric test, and canal paresis was analyzed according to the severity of facial paralysis and the age of each patient. RESULTS: Among the 160 patients, 111 exhibited pure tone audiometry; 26 (79%) of the patients with cochlear symptoms and 44 (56%) of the patients without cochlear symptoms had abnormal audiological data. Among the patients without cochlear symptoms, 15 (19%) had hearing loss at speech frequency, and 42 (54%) had hearing loss isolated to high frequency. The incidence of cochlear symptoms in herpes zoster oticus was not related to the severity of facial paralysis. The incidence of patients with isolated high frequency hearing loss statistically increased with age, however the incidence of patients with speech frequency hearing loss did not increase. Thirteen patients complained vertigo, and the incidence of vestibular disturbances and the value of canal paresis in the caloric test increased to statistical significance in parallel with increasing severity of facial paralysis. CONCLUSION: Mild or moderate cochlear symptoms with high frequency hearing loss were related to age, and severe vestibular symptoms were related to the severity of facial paralysis after onset of herpetic symptoms. This study might suggest us a clue to the pathogenesis of vestibulocochlear nerve disease in herpes zoster oticus.