Delineating the hearing loss in children with enlarged vestibular aqueduct

Objective/Hypothesis: To explore the clinical characteristics and audiologic outcomes in children with enlarged vestibular aqueduct (EVA). Study Design: Retrospective study in a pediatric tertiary care facility. Methods: A total of 54 cases (82 ears) of children with EVA were identified with complete records, including otologic evaluation, imaging studies, and audiologic assessments. The diagnosis of EVA was confirmed by computerized tomography scan/magnetic resonance imaging of the temporal bone. Hearing status was assessed using behavioral testing or auditory brainstem response (ABR). Tympanometry, acoustic reflex, and vestibular evoked myogenic potential (VEMP) testing were also performed when appropriate. Results: Fifty‐two percent of our EVA cases showed bilateral involvement, and 43% of all ears with EVA also had cochlear malformations, such as Mondini dysplasia. Sensorineural HL was initially diagnosed in 16 ears (20% of the total) with EVA whereas conductive or mixed HL was found in 66 ears (80% of the total). Further review of all EVA cases with sensorineural HL showed lack of proper bone conduction testing, so air‐bone gaps were missed. Despite air‐bone gaps in EVA ears, middle ear pressure and mobility were usually normal, along with present acoustic reflexes. VEMP responses were present with abnormally low thresholds. Conclusions: Air‐bone gap(s) can be found in most ears with EVA if both air and bone conduction thresholds are properly tested. Normal tympanometry, presence of acoustic reflex and low threshold VEMP responses suggest that the air‐bone gap in EVA is due to an inner ear anomaly, similar to the “third” labyrinthine window syndrome.     

Acute rhinosinusitis – are we forgetting the possibility of a dental origin? A retrospective study of 385 patients

Abstract

Background: Odontogenic sinusitis (OS) is a common but underdiagnosed form of acute rhinosinusitis (ARS). OS carries no specific characteristics, but unilateral symptoms and certain microbiological as well as radiological findings indicate odontogenic origin.

Aims/objectives: We studied the proportion of OS in ARS patients, the presence and associations of unilateral symptoms, and possible OS microbial and radiological findings. In addition, we investigated how this condition is recognised among ear, nose and throat specialists and radiologists.

Materials and methods: All 676 ARS patients treated in the Department of Otorhinolaryngology at Helsinki University Hospital in 2013 were retrospectively enrolled. The data were collected from patients’ hospital medical records, the laboratory database and radiological reports.

Results: Odontogenic origin of ARS was suspected in 59 (15.3%) patients. Altogether (29.9%) 115 patients complained of unilateral symptoms and these were found to associate with probable oral microbial findings (p?<?.001). These findings covered 20.2% of isolates. Teeth were mentioned in 89.6% of the radiological reports.

Conclusions and significance: OS is common among patients with ARS, and good diagnostic tools already exist in routine practice. Microbial and radiological findings should be carefully evaluated, especially in cases of unilateral symptoms.     

Long-term follow-up of hearing loss in children and young adults with enlarged vestibular aqueducts: relationship to radiologic findings and Pendred syndrome diagnosis

OBJECTIVE: To describe the long-term audiologic findings in pediatric patients with enlarged vestibular aqueducts (EVAs). The relationship between the hearing loss (HL) and the dimensions of the EVA, enlarged endolymphatic duct (EED), or enlarged endolymphatic sac (EES) was also investigated. The influence of a Pendred syndrome (PS) diagnosis on the audiologic phenotype was also examined. STUDY DESIGN: Retrospective analysis of case notes and imaging records, including measurement of the dimensions of the EVA, EED, and EES. SETTING: Tertiary referral center. PATIENTS: Twenty-seven patients (21 female, 6 male) had an EVA in at least one ear. Eighty-five percent had bilateral enlargements. Median age at onset of follow-up was 5.0 years, and median follow-up was 9.7 years. MAIN OUTCOME MEASURES: Hearing thresholds at the start and end of follow-up, rate of progression of HL, history of sudden drops in hearing. RESULTS:: All ears with an EVA had HL. Average HL at the start and end of follow-up was severe. Thirty-seven percent of patients had progressive HL, and 33% reported sudden drops in hearing. Progression was significantly associated with a history of sudden drops. PS patients had worse hearing at the end of follow-up as compared with nonsyndromic patients. There was no evidence of a relationship between the dimensions of the EVA, EED, or EES and the severity or progression of HL. CONCLUSIONS: Patients with EVAs should be advised to avoid known trigger factors for sudden drops in hearing (e.g., minor head trauma). A diagnosis of PS may be associated with a worse audiologic prognosis.     

An analysis of correlation between the unusual location of the jugular bulb and audiovestibular symptoms

OBJECTIVE: Case reports indicate that abnormal location of the jugular bulb (JB) may result with some vestibular and audiological symptoms. The aim of this study is to analyze the correlation between the radiological view of the JB and audiovestibular findings and to clarify its possible role for Meniere-like problems. METHOD: Fifty-six patients, who had abnormal view of the JB on computerized tomography (CT) were selected to the study in this prospective case review. They were classified in three groups (Group-I; Lateral, Group-II; Intermediate and Group-III; Medial), according to the location of the JB. Patients were subjected to audiogram and vestibular tests (Dix-Hallpike maneuver, fistula test, vestibulospinal tests, electronystagmography (ENG)). RESULTS: JB was compressing on the cochlear aqueduct (CA) in two patients in group-II. Seventeen out of 25 patients in group-III had radiological evidence of obstruction of vestibular aqueduct (VA). 19 patients had neurosensorial and four patients had conductive hearing loss. All of the patients in group-I and nine of the patients in group-II gave normal caloric response to stimulation. Twelve of 19 patients in group-III demonstrated canal paresis ranging from 22 to 80%. CONCLUSION: Present study points out that there is a high correlation between HJB and vestibular problems in symptomatic cases. In patients who have HJB without classic vertigo attacks subclinical involvement of the vestibular system has been observed on ENG recordings. These findings imply that radiological existence of HJB may be a predisposing factor for Meniere-like complaints.     

Electrocochleography and intranasal allergen challenge as investigational tools in patients with inhalant allergy and Ménière's disease

OBJECTIVES: To expand on a prior study investigating the relation between inhalant allergy and Ménière's disease using electrocochleography and to present data from five patients heretofore unmentioned in previous reports. STUDY DESIGN: Prospective study of five patients identified with Ménière's disease and inhalant allergy in the practices of two faculty otolaryngologists. METHODS: Patients were tested twice using electrocochleography: once as a baseline and again 20 minutes following intranasal challenge with the allergen to which they were most sensitive. RESULTS: Three patients had no prior history of immunotherapy, and all were found to have a >15% increase in summating potential (SP)/action potential (AP) ratio after antigen challenge. However, only one of these patients developed audiovestibular symptoms. Two patients had a history of immunotherapy. One of these patients was tested using three different antigens to which she was highly sensitive on skin testing, one of which provoked audiovestibular symptoms on environmental exposure. Postchallenge electrocochleography, however, demonstrated normal SP/AP ratios with only one antigen causing a >15% increase. The other patient had elevated SP/AP ratios both before and after challenge and developed no audiovestibular symptoms despite a >15% increase. CONCLUSIONS: Previous work using this investigational tool has identified that all patients with a normal electrocochleography were asymptomatic from an audiovestibular standpoint at the time of postchallenge testing. An elevated SP/AP was not reliably correlated with audiovestibular symptoms in this group of patients. Further investigation in this area will examine the utility of using the variability of the SP and AP to predict audiovestibular symptoms.     

Phenotype of DFNA11: a nonsyndromic hearing loss caused by a myosin VIIA mutation

OBJECTIVES/HYPOTHESIS: To characterize the audiovestibular phenotype of DFNA11, an autosomal dominant nonsyndromic hearing impairment caused by a mutation in the myosin VIIA gene (MYO7A), including whether DFNA11-affected subjects have retinal degeneration as is characteristic of Usher syndrome type 1B, caused by different MYO7A mutations. STUDY DESIGN: Retrospective study of audiovestibular and ophthalmological data in a Japanese family linked to DFNA11. METHODS: Otoscopic examination and pure-tone audiometry were performed in all participants in the family. Selected subjects underwent additional examinations including speech discrimination scoring, acoustic reflex measurements, Békésy audiometry, evoked and distortion-product otoacoustic emissions, auditory brainstem responses, and bithermal caloric testing; visual acuity, ocular tonometry, slit-lamp examination, ophthalmoscopy, and electroretinography; and computed tomography of the temporal bone. RESULTS: Most affected individuals had moderate cochlear hearing loss beginning in the second decade and progressing at all frequencies. Variable degrees of asymptomatic vestibular dysfunction were present. Computed tomography showed normal inner and middle ear structures. No evidence suggested retinitis pigmentosa. CONCLUSIONS: The phenotype of DFNA11 is postlingual, nonsyndromic sensorineural hearing loss with gradual progression. Showing moderate hearing loss with asymptomatic variable vestibular dysfunction and no retinal degeneration, the DFNA11 phenotype is mildest among phenotypes caused by MYO7A mutations.     

Improving the otolaryngology consultation service in a teaching hospital

OBJECTIVE: To examine the type and quality of consultations requested from the otolaryngology service at a tertiary care hospital. STUDY DESIGN: Retrospective. METHOD: Review of written documentation of consultations over a 6-month period. RESULTS: One hundred eleven requests were received, and 107 written reports were made. Twenty services made requests. Thirty-two percent of requests had a legible requester or contact listed. Sixty-seven percent of requests stated why the patient was in hospital, and 85% stated the otolaryngological complaint. Thirty-two percent of requests made accurate reference to the otolaryngological history, and 6% recorded an ENT examination that was accurate. Seven percent of patients were intubated, and 16% had a tracheostomy prior to evaluation. Forty-eight percent of patients required flexible nasopharyngolaryngoscopy. Sixteen percent of patients required rhinoscopy, and 16% required tracheotomy. Twelve percent of patients needed audiograms, and small numbers of patients required biopsy, debridement of ears, ventilation tube insertion, nasal packing, or radiological studies. Reports were made by senior residents, and evidence that the case was discussed with or seen by an attending surgeon was present in 43% of reports. A diagnosis was stated in 85% of reports, and in 3% the diagnosis appeared to be inaccurate as compared with the history and physical examination recorded. A follow-up plan was stated in 70% of reports. CONCLUSIONS: There is a need to educate physicians about collegial communication regarding patients. This information can direct curriculum needed to prepare otolaryngology residents to provide a consultative service in a teaching hospital. This method of determining "true learning needs" can be used in other situations to improve resident training.     

The effect of mental alerting on gaze‐evoked nystagmus

Objectives: To determine whether mental alerting (MA) makes a significant difference to nystagmus recorded during gaze testing using electronystagmography (ENG). Design: A prospective study, blinded interpretation of test results, alternate order of testing sequence. Setting: Vestibular assessment clinic in a tertiary referral centre. Participants: A total of 80 patients were seen by the authors (R.L.H., K.F.L.M.) for vestibular assessment, between August 2002 and February 2004. Patients cognitively unable to perform MA tasks or with limited English were omitted. Patients all underwent gaze‐testing using ENG. Each patient was tested twice, once without MA and once with an MA task, in an alternate test order. Main outcome measures: Slow‐phase velocity values of recorded nystagmus, number of patients with clinically significant nystagmus (slow‐phase velocity ≥6 deg/s). Results: A total of eight patients had significant gaze‐evoked nystagmus when tested without MA and a total of six patients when tested with MA. Only four of these patients were common to both groups. When all nystagmus values (regardless of significance) were considered, no statistically significant differences between nystagmus measured with and without alerting were found (Wilcoxon signed rank test, P > 0.05). Conclusions: Mental alerting during gaze testing did not result in a significant difference in nystagmus and is just as likely to result in an exacerbation as a reduction in slow‐phase velocity. In addition, MA is likely to result in a noisy (and therefore difficult to interpret) recording and increases the impact of the test on the patient. In view of these findings, we propose that MA is contraindicated during the gaze‐testing component of the vestibular test battery.     

Audiovestibular findings in patients with mitochondrial A1555G mutation

OBJECTIVE: The aims of this study were to explore the prevalence of the A1555G mutation among a group of Japanese patients and to assess the pathophysiology of the hearing impairment associated with the mutation. STUDY DESIGN: Genetic study and retrospective chart review. METHODS: We screened for the mitochondrial DNA A1555G mutation in 138 unrelated Japanese deaf patients, including 63 sporadic cases and 75 familial cases with different patterns of inheritance. When available, patients carrying the mutation received audiovestibular examinations, including speech audiometry, distortion-product otoacoustic emission (DPOAE) testing, electrocochleography (ECochG), and electronystagmography. RESULTS: One of 63 sporadic cases (1.6%) and 6 of 75 familial cases (8.0%) carried the A1555G mutation. Patients with the mutation and a familial history included two with autosomal recessive inheritance and four with maternal inheritance. In addition, two of six patients (33.3%) presenting with aminoglycoside-induced sensorineural hearing loss (SNHL) were associated with the A1555G mutation. All but one of the patients carrying the mutation showed high-frequency SNHL. Distortion-product levels of DPOAE were reduced to the noise levels, suggesting the A1555G mutation caused cochlear deafness. Cochlear microphonics in ECochG showed elevation of the detection thresholds and corresponding audiometric thresholds. The ECochG data implied that patients with high-frequency SNHL had impairment of the cochlear hair cells that was most severe toward the basal turn. The electronystagmographic findings indicated no apparent vestibular dysfunction. CONCLUSIONS: Screening for the A1555G mutation, even in patients with idiopathic bilateral SNHL, likely would be useful for preventing further development and/or acceleration of the deafness.     

A longitudinal study of symptoms,anxiety and subjective well-being in patients with vertigo

In a prospective longitudinal study of the relationship between symptoms and anxiety in people with vertigo, 101 patients were evaluated on two occasions separated by a 7 month interval. At Time 1 the age, gender, vertigo type, duration of illness, medication, and audiovestibular test results of patients were recorded, and they completed questionnaires assessing handicap, emotional distress, predisposition to anxiety, and symptoms indicative of vertigo and of somatic anxiety. These questionnaires were re-administered at Time 2, and patients also indicated whether their vertigo was more or less severe than at Time 1. Although vertiginous symptoms at Time 2 were generally mild and intermittent, they were associated with significant handicap. Reported symptoms of somatic anxiety and arousal at Time 1 proved to be the only longitudinal predictors of perceived change in vertigo severity over the 7 month period.     

Vestibular disturbance in patients with large vestibular aqueduct syndrome (LVAS)

Large vestibular aqueduct syndrome (LVAS) is a common inner ear anomaly responsible for some unusual vestibular and audiological symptoms. The gross appearance of CT scan of the inner ear is generally normal. However, precise measurement of the inner ear components reveals abnormal dimensions, which may account for accompanying auditory or vestibular dysfunction. It has been reported that sudden increase in cerebrospinal fluid pressure can cause further deterioration of hearing due to transmission of pressure to the inner ear through the enlarged vestibular aqueduct. However, vestibular function is not often studied. In this report, audiovestibular function of 10 patients with large vestibular aqueducts was analysed and compared with the severity of the radiological deformity. The literature was reviewed and typical findings were discussed to emphasize varying aspects of audiovestibular function. It was found that some patients with LVAS have some spontaneous or provoked vestibular disturbance such as vertigo after watching revolving objects. The mean value of electronystagmographic abnormality in patients with hearing loss is greater than in patients with normal hearing. However, there is no statistical correlation between the level of hearing loss, electronystagmographic abnormality and severity of radiological deformity.     

Vertigo From Herpes Zoster Oticus: Superior or Inferior Vestibular Nerve Origin?

OBJECTIVE/HYPOTHESIS: This study aims to analyze which division of vestibular nerve in the internal auditory canal is responsible for inducing vertigo in patients with herpes zoster oticus (HZO). METHODS: Eight patients (three men and five women) suffered from auricular vesicles, otalgia, and facial palsy, and five of them also had vertigo. Each patient received a battery of tests, including neurological examination, blood examination, audiometry, caloric test, electronystagmography, and vestibular evoked myogenic potential (VEMP) test. RESULTS: All five HZO patients with vertigo had facial palsy on the lesioned side and spontaneous nystagmus beating toward the healthy side. Absent VEMPs were noted in five patients, absent caloric response was noted in four, and sensorineural HL was noted in three. Compared to another three HZO patients without vertigo, all revealed normal responses in both the caloric test and the VEMP test. On MRI scan, two out of four had abnormal gadolinium enhancement along the nerve segments within the internal auditory canal. Six months after treatment, a follow-up caloric test and VEMP test in these eight patients did not alter the results compared with before treatment. CONCLUSION: The nerve trunks within the internal auditory canal are widely affected in HZO patients with vertigo. Both superior division and inferior division of the vestibular nerve attribute to the vertiginous attack. Further, large numbers of HZO patients undergoing caloric testing and VEMP testing are required to support this tentative conclusion.     

Recovery of vestibular evoked myogenic potentials after a vertigo attack due to vestibular neuritis

Conclusions. Inferior vestibular nerve functions could recover in patients with vestibular neuritis (VN).

Objectives. Although the recovery of superior vestibular nerve functions has been reported, there is little information about the recovery of inferior vestibular nerve functions in patients with VN. This study was done to clarify if inferior vestibular nerve functions recover after an attack of VN.

Methods. Neuro-otological tests including vestibular evoked myogenic potential (VEMP) testing and caloric testing were sequentially performed in 13 patients with VN, who showed absence of VEMP on the affected side at the initial examination (7 men and 6 women, 28–82 years of age). VEMPs to click stimulation (95 dBnHL) were recorded with surface electrodes over each sternocleidomastoid muscle.

Results. Among the 13 patients, 5 patients (4 men and 1 woman) showed recovery of VEMP responses. Four of the five patients (three men and one woman) showed recovery of VEMP to the normal range. It takes 6 months to 2 years to recover within the normal range. On the other hand, caloric responses recovered to the normal range only in one patient.     

Vestibular Disturbance in Patients with Large Vestibular Aqueduct Syndrome (LVAS)

Large vestibular aqueduct syndrome (LVAS) is a common inner ear anomaly responsible for some unusual vestibular and audiological symptoms. The gross appearance of CT scan of the inner ear is generally normal. However, precise measurement of the inner ear components reveals abnormal dimensions, which may account for accompanying auditory or vestibular dysfunction. It has been reported that sudden increase in cerebrospinal fluid pressure can cause further deterioration of hearing due to transmission of pressure to the inner ear through the enlarged vestibular aqueduct. However, vestibular function is not often studied. In this report, audiovestibular function of 10 patients with large vestibular aqueducts was analysed and compared with the severity of the radiological deformity. The literature was reviewed and typical findings were discussed to emphasize varying aspects of audiovestibular function. It was found that some patients with LVAS have some spontaneous or provoked vestibular disturbance such as vertigo after watching revolving objects. The mean value of electronystagmographic abnormality in patients with hearing loss is greater than in patients with normal hearing. However, there is no statistical correlation between the level of hearing loss, electronystagmographic abnormality and severity of radiological deformity.     

High incidence of GJB2 mutations during screening of newborns for hearing loss in Austria

OBJECTIVES: The aim of the present study was to evaluate gap junction protein beta2 (GJB2) genetic testing within a national neonate screening program for hearing loss (HL) in a European population. DESIGN: Neonatal cases of nonsyndromic HL (N = 21) were identified by postpartal otoacoustic emissions (OAE) and brain stem electric response audiometry (BERA) analysis. GJB2 testing was performed by direct sequencing. RESULTS: Mutations in GJB2 were found in 15 of 21 children (71.4%) identified by neonatal audiological screening. The 35delG mutation in GJB2 was found homozygous in 10 cases (47.6%) and also as a clear cause of HL as the heterozygous alterations 35delG/del311-324 and 35delG/L90P. In a single case, L90P/R143Q was also identified as a cause of HL. In 3 HL cases that were not identifiable during initial OAE testing, homozygous 35delG and 35delG/R184P defined the genetic basis for HL in 2 cases, whereas one case had wild-type GJB2. CONCLUSIONS: Our findings of the high mutation rate in the Austrian population, especially in neonates identified during the newborn screening program, confirm the importance of screening for mutations in GJB2.     

Nasal and sinus manifestations of sarcoidosis

Nasal and sinus diseases are considered uncommon manifestations of sarcoidosis. We evaluated 159 consecutive patients with sarcoidosis for nasal symptoms. Sixty-three patients (39%) denied any nasal symptoms. Thirty-six patients (23%) had intermittent symptoms that lasted less than three weeks and required continuous intervention with nasal steroids or normal saline. Sixty patients (38%) were treated with nasal steroids and antibiotics for symptoms that lasted more than three weeks. Thirty-three patients (21%) had resolution of their symptoms after treatment with nasal steroids and antibiotics. Twenty-seven patients (17%) had symptoms that were unresponsive to three weeks of oral antibiotics and nasal steroids, and underwent CT scan. Based on the CT results, five patients underwent biopsy, which confirmed sarcoidosis. An additional patient who had a normal CT scan underwent a biopsy that was consistent with sarcoidosis. A retrospective review of 733 sarcoidosis patients was then performed, and an additional 12 patients were identified with biopsy-proven sarcoidosis. All of these patients required long-term therapy with prednisone (14 patients), methotrexate (13 patients), and/or azathioprine (8 patients). Our clinical study reveals a higher incidence of nasal and sinus disease in patients with sarcoidosis than has previously been described, and the recalcitrance of sarcoidosis when there is sinus involvement.     

Otolaryngologic Manifestations of Sarcoidosis: Presentation and Diagnosis

Neuro-otologic manifestations of sarcoidosis are rare. Dizziness in particular is a rare presenting complaint of the patient afflicted with this systemic granulomatous disorder. Head and neck and sinonasal presentations of this disease are more common. We reviewed our experience with six such patients who presented for management of their otolaryngologic (ORL) manifestations in order to delineate the involvement of the otolaryngologist in the treatment and diagnosis of these patients, with a focus upon the relevant tests and procedures in the otolaryngologist's de novo diagnosis of sarcoidosis. Studies ordered in the course of otolaryngologic evaluation and their utility in the diagnosis of sarcoidosis by the otolaryngologist are reviewed and classified into useful, supportive, and ancillary. The otolaryngologist played an important role in diagnosis, with four of six patients diagnosed to have sarcoidosis on the basis of their otolaryngologic presentations. Biopsy was performed by the otolaryngologist for diagnosis of sarcoidosis in all four of these cases. Steroids were central to treatment. Oral steroid therapy was the principal treatment: both patients with neuro-otologic sarcoid were sucessfully managed with oral steroids. Intralesional steroids were necessary to treat the skin lesion. One of six patients patients experienced complications related to steroid use.     

Dysequilibrium and audiovestibular function in panic disorder: symptom profiles and test findings

Patients with panic disorder commonly report symptoms of dizziness and imbalance. We studied the relationship between objective measures of audiovestibular function, phenomenologic, and self-report measures of dysequilibrium and related somatic symptoms in a sample of panic disorder patients with and without agoraphobia, unselected for the complaint of dysequilibrium. Of seventeen patients evaluated by electronystagmography, 71 percent exhibited abnormal vestibular test findings. These latter patients had higher total anxiety ratings than patients without vestibular abnormalities. We conclude that patients with panic disorder warrant evaluation of audiovestibular function.     

一家系遗传性进行性感音神经性聋耳声发射测试分析

目的 :探讨耳声发射 ( OAE)测试对遗传性进行性感音神经性聋的诊断意义。方法 :对一家系 6代中 5 4例 ( 10 8耳 )和 5 0例正常人进行了纯音测听、瞬态诱发耳声发射 ( TEOAE)和畸变产物耳声发射 ( DPOAE)测试 ,了解其耳蜗功能。结果 :本家系纯音听阈 >2 0 d B HL组中 ,84%出现 TEOAE反应幅度下降或消失 ,82 %出现DPOAE振幅下降或消失 ;纯音听阈正常组中 ,75 %出现 TEOAE反应幅度下降 ,6 4%出现 DPOAE某个频率的振幅下降或缺失 ;对照组 TEOAE和 DPOAE的检出率均为 10 0 %。结论 :OAE测试有助于遗传性进行性感音神经性聋的早期诊断     

Cochlear implantation in posttraumatic bilateral temporal bone fracture

Temporal bone (TB) fractures can cause loss of audiovestibular function. Four cases of profound hearing impairment following bilateral TB fracture are presented in this report. All patients received a cochlear implant. All 4 patients became regular users of their implants. None of the patients experienced facial nerve stimulation. Implant-aided audiometry demonstrated a hearing threshold of 28 dB HL. The performance in speech understanding was comparable to standard postlingual adult patients implanted. We believe that cochlear implantation in patients suffering from profound sensorineural hearing losses secondary to TB fractures can be an effective tool for rehabilitation.