Audiovestibular Manifestations Research Articles

Otic Capsule Dehiscences Simulating Other Inner Ear Diseases: Characterization, Clinical Profile, and Follow-Up-Is Ménière's Disease the Sole Cause of Vertigo and Fluctuating Hearing Loss?

We present a series of six cases whose clinical presentations exhibited audiovestibular manifestations of a third mobile window mechanism, bearing a reasonable resemblance to Ménière's disease and otosclerosis. The occurrence of these cases in such a short period has prompted a review of the underlying causes of its development. Understanding the pathophysiology of third mobile window syndrome and considering these entities in the differential diagnosis of conditions presenting with vertigo and hearing loss with slight air-bone gaps is essential for comprehending this group of pathologies. A descriptive retrospective cohort study of six cases diagnosed at a tertiary center. All of them went through auditive and vestibular examinations before and after a therapeutic strategy was performed. Out of 84 cases of dehiscences described in our center during the period from 2014 to 2024, 78 belonged to superior semicircular canal dehiscence, while 6 were other otic capsule dehiscences. Among these six patients with a mean age of 47.17 years (range: 18-73), all had some form of otic capsule dehiscence with auditory and/or vestibular repercussions, measured through hearing and vestibular tests, with abnormalities in the results in five out of six patients. Two of them were diagnosed with Ménière's disease (MD). Another two had cochleo-vestibular hydrops without meeting the diagnostic criteria for MD. In two cases, the otic capsule dehiscence diagnosis resulted from an intraoperative complication due to a gusher phenomenon, while in one case, it was an accidental radiological finding. All responded well to the proposed treatment, whether medical or surgical, if needed. Otic capsule dehiscences are relatively new and unfamiliar entities that should be considered when faced with cases clinically suggestive of Ménière's disease, with discrepancies in complementary tests or a poor response to treatment. While high-sensitivity and specificity audiovestibular tests exist, completing the study with imaging, especially petrous bone CT scans, is necessary to locate and characterize the otic capsule defect responsible for the clinical presentation.

Prevalence of self-perceived audiovestibular symptoms in Egyptian COVID-19 patients

BackgroundAccording to National Institute for Health and Care Excellence (NICE), UK, common audiovestibular symptoms of COVID-19 include dizziness, tinnitus, and otalgia. The pathogenesis of otologic disorders ranges from direct damage to the inner ear structures to immune-mediated damage. Since the start of the pandemic, the prevalence of audiovestibular symptoms linked to COVID-19 has not been thoroughly investigated in Egypt. Our objective is to study and analyze the prevalence of the audiovestibular symptoms in the Egyptian population with history of COVID-19 infection.MethodsA cross-sectional study was conducted among Egyptian adults on the presence and nature of the audiovestibular manifestations in COVID-19 patients. An online questionnaire was used. The questionnaire was developed using Google Form. It was disseminated to the target population through social platforms from October 2021 till February 2022.ResultsData from 245 respondents were collected through online assessment of a convenient sample. The following de novo audiovestibular symptoms were experienced by the participants: Vertigo 20.8%, hearing loss 13.9%, tinnitus 12.7% and ear fullness 11.4%. No correlation was found between the investigations done (D-Dimer, CT chest) and the audiovestibular symptoms.ConclusionAudiovestibular symptoms are fairly common among COVID-19 patients, with higher prevalence, particularly of vertigo, in our study sample compared to the literature. It is recommended that patients with audiovestibular symptoms undergo early testing so that prompt interventions can be taken.

Audiovestibular Manifestations in Patients with Primary Raynaud's Phenomenon and Raynaud's Phenomenon Secondary to Systemic Sclerosis.

To address the prevalence of audiovestibular disorders in patients with primary Raynaud's Phenomenon (RP). A series of patients with primary RP and secondary RP in the context of systemic sclerosis (SSc) were compared with healthy controls. A prospective multicenter observational cross-sectional study was conducted in several Otolaryngology and Rheumatology Divisions of tertiary referral hospitals, recruiting 57 patients with RP and 57 age- and gender-matched controls. Twenty patients were classified as primary RP when unrelated to any other conditions and 37 patients who met the 2013 ACR/EULAR classification criteria for SSc were classified as having secondary RP associated with SSc. Audiometric and vestibular testing (vHIT), clinical sensory integration and balance testing (CTSIB), and Computerized Dynamic Posturography (CDP) were performed. As significant differences were found in the age of the two study groups, primary and secondary RP, no comparisons were made between both groups of RP but only with their control groups. No sensorineural hearing loss (SNHL) was recorded in any of our patients with primary RP and no differences were found in the voice audiometry tests with respect to controls. Four of 37 (10.8%) secondary RP patients presented SNHL. Those with SNHL were 7.03 times more likely to have a secondary RP than controls (p < 0.001). The audiometric curve revealed high-frequency hearing loss in 4 patients with RP secondary to SSc, and statistically significant differences were achieved when RP secondary was compared to controls in vHIT gain, caloric test, CTSIB, and CDP. Unlike patients with RP secondary to SSc, patients with primary RP do not show audiovestibular abnormalities. Regarding audiovestibular manifestations, primary RP can be considered a different condition than secondary RP.

Audiovestibular manifestations in patients with rheumatologic diseases: a review

Rheumatologic disorders are chronic and systemic disorders with an estimated global prevalence of 3,200 per 100,000 inhabitants. Several clinical manifestations are known and may lead to physical disability and altered quality of life. The most common audio vestibular manifestations commonly reported are hearing loss, tinnitus, vertigo, and fullness, however, there are some more still unregistered. The aim of this review is to describe the most common audio vestibular features in three of the most common rheumatologic conditions such as rheumatoid arthritis (RA), systemic erythematous lupus and systemic sclerosis (SSc). A literature search was performed with articles published between 2000 to 2021. In this literature review an increased prevalence of hearing loss for RA of 40% and 70% for systemic erythematous lupus was observed. In SSc, due to its early onset and progression, there is no estimated prevalence, however, a notorious hearing impairment in early stages of the disease was described. Vertigo was also described in these disorders, affecting more than 60% of patients. Further studies are in need to describe the affection on early stages of all disorders as well as its progression.

Audio-Vestibular Manifestations in COVID-19; A Systematic Review and Meta-analysis

Introduction Audio-Vestibular (AV) presentation as a consequence of COVID-19 is a less ventured zone. The aim of the study was systematically review the literature (both studies and case reports) published up to August 2021, in order to provide evidence on audio-vestibular symptoms in SARS-CoV-2, COVID-19 patients. Systematic review and meta-analysis was performed for assessment of prevalence of AV symptoms among COVID-19 patients. Materials and Methods A search of existing literature using key words and MeSH (Medical Subject Headings) terms was done. The methods were developed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. 36 qualitative studies were narratively synthesized. Meta-analysis was performed on 18 quantitative studies Results Hearing loss was most commonly reported AV manifestation among case series / case reports. In the quantitative studies, the pooled estimate of Hearing loss, Dizziness and Tinnitus was 4.558%, 4.226% and 5.513% respectively. Other reported AV symptoms included ear ache, aural fullness and facial paresis. Conclusion Prevalence of AV symptoms in COVID-19 differs from that of the published literature. This prevalence of hearing loss is less than that reported amongst the world population. Structured community-based studies with homogeneous working definitions have to be conducted to assess the true prevalence.

Comparative Study of Audiovestibular Symptoms between Early and Late Variants of COVID-19.

Audiovestibular symptoms during the acute phase of the corona virus disease 2019 (COVID-19), have been reported for earlier waves of the pandemic, while no studies investigated nor compared audiovestibular manifestations during subsequent waves of COVID-19. In the current study, we aimed to compare the occurrence of audiovestibular symptoms associated with COVID-19 between the alpha/beta, delta, and omicron variants. An online questionnaire was distributed to individuals with confirmed test results for COVID-19. We asked participants to report whether they experienced audiovestibular symptoms during the acute phase of the disease. The study included 939 participants; 120 un-infected controls and infected participants during alpha/beta (n = 301), delta (n = 102), and omicron (n = 416) predominance periods. Self-reported audiovestibular symptoms were found to be statistically significantly different between un-infected controls and COVID-19 infected individuals in all analyzed variants. Furthermore, our results showed no significant differences in audiovestibular symptoms among individuals infected during alpha/beta, delta, and omicron waves. Although individuals infected during the delta variant predominance period reported higher percentages of audiovestibular symptoms (ranging from 11.8% to 26.5% for auditory symptoms and from 12.7% to 34.3% for vestibular symptoms) than for the alpha/beta (ranging from 6.3% to 18.9% for auditory symptoms and 8.3% to 29.9% for vestibular symptoms) and omicron (ranging from 9.6% to 21.2% for auditory and 12.5 to 29.1% for vestibular symptoms) variants, this did not achieve statistical significance. With regards to auditory symptoms, the most commonly reported symptoms were aural fullness followed by hearing loss and tinnitus. With regards to vestibular symptoms, dizziness was the most commonly reported symptom followed by vertigo and unsteadiness. Logistic regression revealed that experiencing auditory symptoms were associated with other neurological symptoms, back and joint pain, and chest pain as COVID-19 symptoms. Vestibular symptoms were associated with anemia, gender, fatigue, headache, and breathing difficulties. In conclusion, our study shows that audiovestibular symptoms are common during the acute phase of early and late COVID-19 variants with no significant differences between them.

Audio-vestibular manifestations in Kawasaki disease (KD): a rare atypical case presentation

BackgroundKawasaki disease (KD) is an acute febrile, usually self-limiting illness of infancy and childhood, that can show audiological manifestation of sensorineural hearing loss (SNHL, 36% of patients), usually bilateral in nature.Case presentationWe report a 12-year-old female diagnosed with KD at 6 years, showing atypical manifestation of asymmetrical SNHL since 1.5 years after the episode of febrile illness. The patient was administered Immune Globulin intravenously and aspirin, which were tapered to a low dose. The patient later reported reduced hearing sensitivity, ear pain, blocking in both ears, and tinnitus (intermittent) in the left ear. The audio-vestibular manifestations in the case using test battery approach (Pure tone audiometry, speech audiometry, tympanometry, reflexometry, otoacoustic emissions, auditory brainstem response, and behavioral and objective vestibular tests) is discussed. The rehabilitative measures taken, and the need for sensitization of school teachers for early identification of hearing loss in children with KD is highlighted.ConclusionsThis case presentation concentrates on the rare asymmetrical SNHL and vestibular finding post-KD and emphasizes the need for health care professionals to refer patients with KD to an audiologist to trace the possible sequelae including hearing loss and vestibular disorders. The atypical manifestations of KD in hearing domain is suggestive of the need for early identification, adequate profiling of not just hearing but also balance-related manifestations, instigation of appropriate rehabilitative measures, effective counselling of parents and school teachers to facilitate better academic learning, and communication opportunities.

Four cases of audio-vestibular disorders related to immunisation with SARS-CoV-2 mRNA vaccines

Objective To gain medical insight into the clinical course and safety of otolaryngologic disorders following immunisation with severe acute respiratory coronavirus (SARS-CoV-2) mRNA-based vaccines. Design Case description. Study sample We report four cases of transient audio-vestibular symptoms, which occurred shortly after inoculation of two BNT162b2 (Pfizer-BioNTech®) and mRNA-1273 (Moderna®) vaccines. Results Hearing loss was unilateral in all cases and recovered at least partially: it was associated with persistent gait instability in two cases, after 1 and 7 months. Trigger mechanisms underpinning audio-vestibular impairment remain uncertain. Immune tolerance mechanisms with off-target innate activation of T-lymphocytes may be involved in vestibulocochlear nerve disorders, as for other cranial nerves involvement. Conclusions The occurrence of audio-vestibular manifestations following mRNA-based vaccines needs ENT monitoring to support their causality in such rare vaccine-related adverse events. Audio-vestibular disorders appeared of transitory nature, including hearing loss, and should not deter further efforts in large-scale vaccination campaigns against SARS-CoV-2.

Hearing disorders in lupus patients: correlation with duration and severity of the disease

BackgroundHearing loss can greatly affect the overall quality of life. Several studies have reported that patients with SLE may suffer from audio-vestibular manifestations. The aim of this study is to evaluate the influence of systemic lupus erythematosus (SLE) on hearing and association of hearing impairment with severity and duration of SLE.ResultsPure-tone audiometry (PTA) (air conduction and bone conduction) thresholds showed significant elevation in SLE group than controls (P ≤ 0.05). It was observed also that there is a significant association between SLE severity and duration with sensorineural hearing loss (SNHL) and its degree.ConclusionSLE can lead to hearing loss, and there is a positive association of SLE severity and duration with degree of hearing loss.

Short- and Long-Term Self-Reported Audiovestibular Symptoms of SARS-CoV-2 Infection in Hospitalized and Nonhospitalized Patients

Background: Audiovestibular symptoms during the acute stage have been reported in patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), while very few studies investigated the long-term audiovestibular manifestations of SARS-CoV-2. Objective: The objective of this study was to examine the occurrence of short- and long-term audiovestibular symptoms associated with SARS-CoV-2 infection. Method: In this cross-sectional study, a questionnaire was distributed to severe hospitalized cases and nonhospitalized patients with mild disease, all with confirmed SARS-CoV-2 test results. Participants were inquired to report audiovestibular symptoms during the acute phase and at 6-month follow-up after contracting SARS-CoV-2. Results: A total of 301 participants completed the questionnaire. Auditory symptoms were reported by 21.9% and 1.99% of patients during the acute phase and 6 months post SARS-CoV-2 infection, respectively. During the acute phase of SARS-CoV-2 infection, aural fullness represents the most common symptoms (18.94%) followed by tinnitus (9.97%) and hearing loss (6.31%). Vestibular symptoms were reported by 34% during the acute phase; most commonly was dizziness (29.9%) followed by vertigo (24.25%) and unsteadiness (8.31%). Long-term and persistent vestibular problems were reported by 3.99% patients. There were no statistically significant differences in self-reported audiovestibular symptoms between patients with severe SARS-CoV-2 disease compared to those with mild disease. Conclusion: The current study showed that audiovestibular symptoms are common among SARS-CoV-2 infected patients during the acute phase of the disease. However, these symptoms are mostly temporary and showed complete spontaneous recovery during the first 2 weeks postinfection.

The audiovestibular manifestations as early symptoms of multiple sclerosis: a scoping review of the literature.

Multiple sclerosis (MS) is an immune-mediated, demyelinating disease of the nervous system, which may impact the audiovestibular pathway at different stages of the disease. The auditory and vestibular manifestations of MS as a presenting or early symptom are an area in which more investigation is needed. The aim of this review is to determine the auditory and vestibular symptoms, which may occur at the presenting stage of multiple sclerosis. This clinical knowledge will allow a clinician to facilitate early diagnosis and intervention of MS through appropriate onward referral. Audiological and vestibular test results, as well as magnetic resonance imagery results, will also be examined to try to determine the impact of MS on the auditory and vestibular pathways. A scoping search of the electronic databases PubMed, Scopus, Web of Science, ScienceDirect, and EBSCO was conducted in March 2020 to obtain studies specifically of patients with audiovestibular symptoms at the early or presenting stages of multiple sclerosis. Data was extracted from studies which met the inclusion criteria and studies were subsequently critically appraised and assessed for risk of bias. Eighteen papers met the inclusion criteria for this study. Results of the study found that the most common audiovestibular manifestation as a presenting symptom of MS was unilateral, moderate-profound, fluctuating, and sudden sensorineural hearing loss across all frequencies (250Hz-8000Hz). Other symptoms include tinnitus, balance abnormalities, aural pain and aural fullness, which may accompany SSHL or occur independently. The peripheral involvement of the immune-mediated mechanisms of MS was suggested by peripheral findings in vestibular examination results and the involvement of wave I ABR in patients with irreversible hearing loss. Demyelinating lesions associated with MS were suggested by results obtained from evoked potentials measurements, including ABR, VEMPs, and MLR. An understanding of the sensitivity of evoked potentials in the detection of demyelinating lesions as well as the most common audiovestibular presentations of the disease allows the practitioner to provide an appropriate onward referral for MRI which may lead to early diagnosis and intervention of MS. We suggest that there is enough evidence to include evoked potentials complementary to MRI in the detection and monitoring of MS. As the review suggests evidence of involvement of the immune-mediated mechanisms of MS on peripheral structures like the inner ear, further clinical research is recommended to explore this mechanism. Key points 1. The most common audiovestibular manifestation as an early symptom of MS was unilateral moderate to profound SSHL across all frequencies (250Hz-8000Hz). 2. Findings of the review indicated the involvement of the immune-mediated mechanisms of MS in the peripheral structures of the inner ear. 3. These findings included peripheral results in the vestibular test of the patients involved in the study and secondly, the three studies which reported an absence of full recovery of hearing loss were also three papers which within their ABR results showed abnormality of wave I 4. This is the first paper to support the theory (Di Stadio et al. 2018) [32] that immune-mediated processes of MS can spread to peripheral inner ear structures 5. The review highlighted the sensitivity of evoked potentials in detecting MS lesions in the presenting stage, particularly ABR which demonstrated that in instances in which hearing loss recovered ABR results remained abnormal.

Syndrome de Cogan

“Typical” Cogan's syndrome is defined as a non-syphilitic interstitial keratitis associated with audio-vestibular resembling Ménière's disease with a 2-year maximum delay between these 2 organ impairment. Cogan syndrome is classified as “atypical” in the absence of interstitial keratitis and the presence of other inflammatory eye manifestations, an audio-vestibular impairment different from typical Menière-like disease, or a delay longer than 2 years between eye and audio-vestibular manifestations. Constitutional signs and large-vessel vasculitis is also possible, mostly affecting the thoracic aorta. The presence of acute-phase reactants is common, but no specific laboratory tests are available. The prognosis is dominated by the audio-vestibular impairment and in particular the risk of deafness, while other complications especially vascular complications being rare. Treatment with glucocorticoids is usually necessary and the combination to other immunosuppressive therapies or biological-targeted drugs needs to be determined.

Audiovestibular findings in autoimmune diseases

BackgroundAudiovestibular manifestations are reported in autoimmune diseases including hearing loss and vestibular symptoms.ObjectivesThis study is designed to evaluate the audiovestibular manifestations in patients with different autoimmune diseases especially asymptomatic cases.Subjects and MethodsThis work included two groups: study group (29 cases with different autoimmune diseases) and control group (20 healthy subjects). All participantswere subjected to basic audiologic evaluation, Sinusoidal harmonic acceleration (SHA) test of the rotatory chair at different frequencies (.01-.64Hz).Resultspatients with ADs showed elevated hearing thresholds (>25dBHL) at all tested frequencies. As regard SHA test, only 5 cases from the study grop showed normal results, while the rest of cases showed vestibular hypofunction (bilateral in 22 cases and unilateral in 3 cases).ConclusionAudiovestibular symptoms are common in different autoimmune diseases even asymptomatic cases. SHA test showed that vestibular affection is much more frequent than expected. So, regular screening of hearing and vestibular functions in patients with autoimmune should be done, for better and early management.

Evaluations of Audiovestibular Manifestations in Patients with Psoriasis

Evaluations of Audiovestibular Manifestations in Patients with Psoriasis

Cogan’s Syndrome and Other Ocular Vasculitides

The clinical presentation of Cogan's syndrome has been classified as typical and atypical. Like other forms of ocular vasculitis, Cogan's syndrome has been found to have autoimmune origins with antibodies against the cornea, inner ear, and endothelial antigens. Antineutrophil cytoplasmic antibody (ANCA) and rheumatoid factor (RF) have been associated with Cogan's syndrome as well as ocular-involving vasculitides not as strongly associated with the audiovestibular manifestations such as granulomatosis with polyangiitis and rheumatoid arthritis. The mainstay of therapy has been corticosteroids although other methods have been described in recalcitrant disease and to prevent development of systemic sequelae.

Audio-Vestibular Signs and Symptoms in Chiari Malformation Type I. Case Series and Literature Review

IntroductionChiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common presentation is occipital headache, the association of audio-vestibular symptoms is not rare. The aim of our study was to describe audio-vestibular signs and symptoms in Chiari malformation type I (CM-I). Materials and methodsWe performed a retrospective observational study of patients referred to our unit during the last 5 years. We also carried out a literature review of audio-vestibular signs and symptoms in this disease. ResultsThere were 9 patients (2 males and 7 females), with an average age of 42.8 years. Five patients presented a Ménière-like syndrome; 2 cases, a recurrent vertigo with peripheral features; 1 patient showed a sudden hearing loss; and 1 case suffered a sensorineural hearing loss with early childhood onset. The most common audio-vestibular symptom indicated in the literature in patients with CM-I is unsteadiness (49%), followed by dizziness (18%), nystagmus (15%) and hearing loss (15%). Nystagmus is frequently horizontal (74%) or down-beating (18%). Other audio-vestibular signs and symptoms are tinnitus (11%), aural fullness (10%) and hyperacusis (1%). Occipital headache that increases with Valsalva manoeuvres and hand paresthesias are very suggestive symptoms. ConclusionsThe appearance of audio-vestibular manifestations in CM-I makes it common to refer these patients to neurotologists. Unsteadiness, vertiginous syndromes and sensorineural hearing loss are frequent. Nystagmus, especially horizontal and down-beating, is not rare. It is important for neurotologists to familiarise themselves with CM-I symptoms to be able to consider it in differential diagnosis.

Manifestaciones audiovestibulares en la malformación de Chiari tipo i. Serie de casos y revisión bibliográfica

IntroductionChiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common presentation is occipital headache, the association of audio-vestibular symptoms is not rare. The aim of our study was to describe audio-vestibular signs and symptoms in Chiari malformation type i (CM-I). Materials and methodsWe performed a retrospective observational study of patients referred to our unit during the last 5 years. We also carried out a literature review of audio-vestibular signs and symptoms in this disease. ResultsThere were 9 patients (2 males and 7 females), with an average age of 42.8 years. Five patients presented a Ménière-like syndrome; 2 cases, a recurrent vertigo with peripheral features; one patient showed a sudden hearing loss; and one case suffered a sensorineural hearing loss with early childhood onset. The most common audio-vestibular symptom indicated in the literature in patients with CM-I is unsteadiness (49%), followed by dizziness (18%), nystagmus (15%) and hearing loss (15%). Nystagmus is frequently horizontal (74%) or down-beating (18%). Other audio-vestibular signs and symptoms are tinnitus (11%), aural fullness (10%) and hyperacusis (1%). Occipital headache that increases with Valsalva manoeuvres and hand paresthesias are very suggestive symptoms. ConclusionsThe appearance of audio-vestibular manifestations in CM-I makes it common to refer these patients to neurotologists. Unsteadiness, vertiginous syndromes and sensorineural hearing loss are frequent. Nystagmus, especially horizontal and down-beating, is not rare. It is important for neurotologists to familiarise themselves with CM-I symptoms to be able to consider it in differential diagnosis.

Investigations into audiovestibular manifestations in patients with psoriatic arthritis.

Although psoriatic arthritis (PsA) is a common chronic inflammatory rheumatic disease, little is known about audiovestibular impairment in this condition. We aimed to establish whether audiovestibular manifestations were present in patients with PsA. A set of 60 consecutive patients who fulfilled the Moll and Wright criteria for PsA and 60 matched controls were studied. During the period of recruitment, individuals were excluded who had a history of cardiovascular disease, cerebrovascular complications, peripheral artery disease, renal insufficiency, syphilis, Meniere disease and other vestibular syndromes, infections involving the inner ear, barotrauma, or were in treatment with ototoxic drugs. Most patients with PsA were men (63%). The mean age at the time of our study was 52.9 years and the mean age at the onset of symptoms was 33 years. Thirty-six (60%) of the 60 patients showed abnormal hearing loss in the audiogram compared to only 5 (8.3%) of the 60 controls (p < 0.001). Values of audiometric tests (pure-tone average and speech reception threshold) yielded significant differences between patients and controls (p < 0.001). The audiogram disclosed a bilateral and symmetrical sensorineural hearing loss (SNHL) in PsA with predominant pattern of high frequency SNHL in patients with PsA (46.7%) compared to controls (8.3%, p < 0.001). Patients with PsA exhibited abnormal vestibular tests more commonly than controls. A significantly increased frequency of abnormal computerized dynamic posturography with a predominant vestibular loss pattern was also observed in patients (23.3%) compared to controls (0%, p < 0.001). Our current study demonstrates strong evidence for inner ear damage in patients with PsA.

Development of a low grade lymphoma in the mastoid bone in a patient with atypical Cogan’s syndrome: A case report

Cogan’s syndrome is a rare disorder characterized by ocular and audiovestibular manifestations in its typical form and caries a wide variety of atypical manifestations. It is considered as an autoimmune disease. We present the first case in the literature of a 67year old woman with the development of low grade non-Hodgkin lymphoma (NHL) in the mastoid bone in a pre-existing history of atypical Cogan’s syndrome. The anatomical development of NHL was to a “target” organ of Cogan’s syndrome, which is the inner ear.

Auditory disorders in patients with systemic lupus erythematosus: Relation to clinical parameters

Aim of the workTo evaluate the hearing disorders in SLE patients with particular regard to their frequency and relationship to disease duration and activity. Patients and methodsTwenty female SLE patients were enrolled in the study. Assessment of disease activity was done using the SLE disease activity index (SLEDAI). Another 20 otologically healthy subjects of matched age and sex served as controls. Auditory assessment was performed and included otoscopic examination, pure tone audiometry (PTA), acoustic immittance testing and speech audiometry. ResultsThe PTA was abnormal in 13 (65%) patients; 4 had tinnitus and 1 vertigo. The PTA results showed a highly significant statistical difference from the control (p<0.001). Otoscopic examination, acoustic immittance testing and speech audiometry of all patients were normal. A significant difference was found in the age at disease onset between those with and without abnormal PTA (p=0.023). Moreover, there was a significantly lower hearing level (right ear) at 12,000Hz in juvenile-onset (N=6) (20.83±3.76db) compared to adult-onset cases (32.5±15.66db) (p=0.02). No significant difference was present in the audiovestibular manifestations (p=0.114), clinical, laboratory parameters or disease activity between those with or without hearing loss. However, hearing levels were significantly lower in those with lupus nephritis and those receiving hydroxychloroquine. ConclusionPure tone audiometry revealed SNHL in 65% of SLE patients. Absence of audiovestibular manifestations does not exclude inner ear affection. Age at disease onset is remarkably associated with hearing loss in SLE. Lupus nephritis and hydroxychloroquine use are associated with lower hearing levels and possible early hearing loss.