Migraine and Cochlear Symptoms.

Migraine is one of the most common and highest burdens of disease. As a primary cerebral dysfunction illness, migraine might exhibit other system-related symptoms, including vestibular and cochlear symptoms. With the publication of the diagnostic criteria of vestibular migraine, the link between migraine and vestibular symptoms became clear. However, the relationship between migraine and cochlear symptoms is far from straightforward. Therefore, we focus on the correlation between migraine and deafness, sudden sensorineural hearing loss, acute tinnitus, and chronic tinnitus to better understand the relationship between migraine and cochlear symptoms.

Auditory and Olfactory Deficits in Essential Tremor - Review of the Current Evidence.

Background: Essential tremor (ET) is the most common adult movement disorder, characterized by several motor and increasingly well recognized non-motor symptoms. Sensory deficits, such as hearing impairment and olfactory dysfunction, are amongst them. This review analyzes the available evidence of these sensory deficits and their possible mechanistic basis in patients with ET. Method: A PubMed literature search on the topic was performed in the May 2019 database. Results: Nineteen articles on hearing impairment and olfactory dysfunction in ET patients were identified. The prevalence of hearing impairment is higher in ET patients than healthy controls or Parkinson disease. Cochlear pathologies are suggested as the underlying cause, but there is still a lack of information about retrocochlear pathologies and central auditory processing. Reports on olfactory dysfunction have conflicting results. The presence of mild olfactory dysfunction in ET was suggested. Conflicting results may be due to the lack of consideration of the disease's heterogeneity, but according to recent data, most studies do not find prominent evidence of olfactory loss in ET. Conclusion: Although there is increasing interest in studies on non-motor symptoms in ET, there are few studies on sensory deficits, which are of particularly high prevalence. More studies are needed on to investigate the basis of non-motor symptoms, including sensory deficits.

A long-term follow-up study on otoacoustic emissions testing in paediatric patients with severe malaria in Gabon.

BACKGROUND: In a previous study, severe and cerebral malaria have been connected with acute cochlear malfunction in children, demonstrated by a decrease of transitory evoked otoacoustic emissions (TEOAEs) reproducibility. This study aims to determine whether cochlear malfunction persists for 4 years after recovery from severe malaria in a subset of the previous study's collective. Follow-up TEOAEs were performed on site (CERMEL, Hôpital Albert Schweitzer, Lambaréné, Gabon) or at the participants' homes; 33 out of 90 participants included in the initial investigation by Schmutzhard et al. could be retrieved and were re-examined, 31/33 could be included. Of the 57 missing participants, 51 could not be contacted, 1 had moved away, 4 refused to cooperate, and 1 had died. METHODS: As in the initial investigation, participants of this prospective follow-up study were subjected to TEOAE examination on both ears separately. A wave correlation rate of > 60% on both ears was considered a "pass"; if one ear failed to pass, the examination was considered a "fail". The results were compared to the primary control group. Additionally, a questionnaire has been applied focusing on subsequent malaria infections between the primary inclusion and follow-up and subjective impairment of hearing and/or understanding. RESULTS: The cohort's mean age was 9 years, 14 children were female, 18 male. 31 had been originally admitted with severe, one with cerebral malaria. 83.8% of participants (n = 26) presented with a TEOAE correlation rate of > 60% on both ears (the cut-off for good cochlear function); in the control group, 92.2% (n = 83) had passed TEOAE examination on both ears. Recurrent severe malaria was associated with a worse TEOAE correlation rate. Age at infection and gender had no influence on the outcome. CONCLUSIONS: Cochlear malfunction seems to be persistent after 4 years in more than 16% of children hospitalized for malaria. In a healthy control group, this proportion was 7.8%. Yet, the severity of the initial TEOAE-decrease did not predict a worse outcome.

Association of Tinnitus and Other Cochlear Disorders With a History of Migraines.

Importance: A headache is a symptom of a migraine, but not all patients with migraine have headaches. It is still unclear whether a migraine might increase the risk of cochlear disorders, even though a migraine does not occur concurrently with cochlear disorders. Objective: To investigate the risk of cochlear disorders for patients with a history of migraines. Design, Setting, and Participants: This study used claims data from the Taiwan Longitudinal Health Insurance Database 2005 to identify 1056 patients with migraines diagnosed between January 1, 1996, and December 31, 2012. A total of 4224 controls were also identified from the same database based on propensity score matching. Statistical analysis was performed from January 23, 1996, to December 28, 2012. Main Outcomes and Measures: The incidence rate of cochlear disorders (tinnitus, sensorineural hearing impairment, and/or sudden deafness) was compared between the cohorts by use of the Kaplan-Meier method. The Cox proportional hazards regression model was also used to examine the association of cochlear disorders with migraines. Results: Of the 1056 patients with migraines, 672 were women and 384 were men, and the mean (SD) age was 36.7 (15.3) years. Compared with the nonmigraine cohort, the crude hazard ratio for cochlear disorders in the migraine cohort was 2.83 (95% CI, 2.01-3.99), and the adjusted hazard ratio was 2.71 (95% CI, 1.86-3.93). The incidence rates of cochlear disorders were 81.4 (95% CI, 81.1-81.8) per 1 million person-years for the migraine cohort and 29.4 (95% CI, 29.2-29.7) per 1 million person-years for the nonmigraine cohort. The cumulative incidence of cochlear disorders in the migraine cohort (12.2%) was significantly higher than that in the matched nonmigraine cohort (5.5%). Subgroup analysis showed that, compared with the nonmigraine cohort, the adjusted hazard ratios in the migraine cohort were 3.30 (95% CI, 2.17-5.00) for tinnitus, 1.03 (95% CI, 0.17-6.41) for sensorineural hearing impairment, and 1.22 (95% CI, 0.53-2.83) for sudden deafness. Conclusions and Relevance: In this population-based study, the risk of cochlear disorders, especially for tinnitus, was found to be significantly higher among patients with a history of migraines. This finding may support the presence and/or concept of "cochlear migraine."

Cochlear obliteration following a translabyrinthine approach and its implications in cochlear implantation.

The most frequent sequelae following a translabyrinthine approach for vestibular schwannoma resection is complete hearing loss on the affected side. Such patients could benefit from a cochlear implant, provided that two essential requisites are met before surgery: a preserved cochlear nerve and a patent cochlea to accommodate the electrode array. The goal of our study is to determine the prevalence and extent of cochlear ossification following a translabyrinthine approach. Postoperative MRI of 41 patients were retrospectively reviewed. Patients were classified according to the degree of cochlear obliteration into three groups (patent cochlea, partially obliterated cochlea and totally obliterated cochlea). The interval between surgery and the first MRI was studied as well as its relationship with the rate of cochlear ossification. At first postoperative MRI (mean interval of 20 months), 78% of patients showed some degree of cochlear ossification. Differences were found in the time interval between surgery and first MRI for each group, showing a smaller interval of time the patent cochlea group (p > 0.05). When MRI was performed before the first year after surgery, a larger rate of patent cochlea was found (p > 0.05). The present study suggests that cochlear ossification is a time-depending process, whose grounds are still to be defined.

Cochlear Dysfunction is not Common in Human Meningioma of the Internal Auditory Canal.

HYPOTHESIS: Cochlear dysfunction is not common in human meningioma of the internal auditory canal. BACKGROUND: Meningiomas arising from the cerebellopontine angle and internal auditory canal typically cause hearing loss. Cochlear dysfunction is known to contribute to sensorineural hearing loss induced by vestibular schwannoma, the most common tumor of the internal auditory canal. Detailed cochlear histopathology in meningioma has not been reported. METHODS: Retrospective analysis of cochlear histopathology in five unoperated and five operated meningiomas of the internal auditory canal identified after screening human temporal bone collections from three academic medical centers. RESULTS: While some dysfunction of all analyzed cochlear cell types was identified, a predominant or exclusive loss of hair cells was not observed in any meningioma. Only 14.3% of temporal bones showed significantly more hair cell damage on the side of the tumor when compared with the contralateral ear; cochlear neuronal damage was more prevalent in meningiomas. The incidence of hydrops, perilymphatic precipitate, or endolymphatic precipitate was low. CONCLUSIONS: Substantial cochlear damage in human meningioma of the internal auditory canal is not common. This may explain the anecdotal hearing improvement observed after surgical resection of meningioma. Our findings underline the importance of developing therapeutic strategies to prevent cochlear neuronal degeneration due to tumors of the internal auditory canal.

Olfactory and cochleovestibular dysfunction after head injury in the workplace: an updated series.

OBJECTIVES: The objective of this study was to determine the incidence of olfactory dysfunction in workers following head injury in the work place, to define its relationship to the site, severity of injury and direction of force. The demographics of head injured workers were also assessed to determine whether those with olfactory loss were more likely to have sustained a cochleovestibular injury. DESIGN: Retrospective case analysis. SETTING: Tertiary referral university hospital in Toronto, Ontario. PARTICIPANTS: A total of 3438 consecutive patients referred from the Workplace Safety and Insurance Board (WSIB) in the province of Ontario who sustained a work-related head injury were assessed between 1987 and 2014. MAIN OUTCOME MEASURES: Olfactory and cochleovestibular dysfunction assessed by history, clinical examination and subjective and objective tests. RESULTS: Olfactory dysfunction (OD) was identified in 413 of 3438 patients (12.0%) of which 321 were diagnosed with anosmia and 92 with hyposmia. In our series, injuries from a fall were the commonest cause for OD and a frontal or mid-face impact was more likely to result in OD than other regions (P = 0.0002). A loss of consciousness (LOC) of any duration correlated with OD. In those with olfactory dysfunction, an associated skull fracture occurred in 37.1% of patients and a CSF leak in 4.1%, which was significantly higher compared with those without OD(<0.0001). Patients with OD had a higher incidence of cochlear and vestibular loss (19.9% and 20.6%, respectively) compared with those without OD (14.3% and 17.1%, respectively). CONCLUSIONS: Post-traumatic olfactory dysfunction is more likely to occur in patients who experienced a moderate to severe head injury, LOC and more likely to result from a frontal or mid-face blow to the skull. Cochleovestibular dysfunction is likely to occur concurrently with olfactory dysfunction.

Increased prevalence of early cochlear damage in young patients with type 1 diabetes detected by distortion product otoacoustic emissions.

OBJECTIVE: To evaluate the hearing of adolescents with diabetes mellitus type 1(DM1) by otoacoustic emissions (OAEs), and by comparing different tests with pure-tone audiometry to identify potential early cochlear impairments. DESIGN: Pure-tone audiometry, transient evoked otoacoustic emissions (TEOAEs), and distortion product otoacoustic emissions (DPOAEs) were performed in a group of adolescents with and without DM1. Clinical characteristics, disease duration, and glycated haemoglobin levels were studied. STUDY SAMPLE: Participants were 40 adolescents with DM1 and 40 healthy subjects. RESULTS: Sensorineural hearing loss, affecting frequencies of 6000 and 8000 Hz, was found only in DM1 subjects when compared to the controls (7.7% vs. 0%, p < 0.05). A higher prevalence of cochlear damage was detected by DPOAE responses, 32% belonging from the diabetic group, vs. 3.7% in the control group. Absent TEOAE responses were observed in only three individuals, all from the diabetic group (5.1% of the tests performed in the diabetic group). Additionally, hearing thresholds were better in diabetic subjects with good control when compared to ones with regular or poor control (p = 0.00). Hearing thresholds were higher in poorly controlled diabetics when compared to subjects with good (p = 0.000) or regular control (p = 0.006). CONCLUSION: Early evidence of cochlear damage was detected in adolescents with DM1 leading to hearing loss at high frequencies. Abnormal DPOAEs responses were found more frequently than the alterations in TEOAEs and pure-tone audiometry, suggesting that DPOAEs evaluation is the most sensitive and it could be used for monitoring the progression of cochlear damage during the early stages of hearing impairment.

Cochlear involvement in Familial Mediterranean Fever: a new feature of an old disease.

OBJECTIVES: In this study we first aimed to assess the cochlear functions in children with Familial Mediterranean Fever. The second aim was to investigate the correlation between the hearing levels and some clinical features of Familial Mediterranean Fever including the duration of the disease, age at onset, genetic analysis and colchicine use. METHODS: Thirty-four children with Familial Mediterranean Fever and 27 age matched children were included in the study. Following otologic examination, all children underwent audiometric evaluation, including Pure Tone Average measurements and Distortion Product Otoaoustic Emission testing. Audiological results of the two groups were compared and correlation between the audiologic status and clinical parameters of the disease like the duration of disease, age at onset, mutations and colchicine treatment were studied. RESULTS: Pure tone audiometry hearing levels were within normal levels in both groups. Hearing thresholds of Familial Mediterranean Fever patients were found to be increased at frequencies 8000, 10,000, 12,500 and 16,000 (p<0.05). In otoacoustic emission evaluation, distortion products and signal-noise ratio of FMF children were lower in the tested frequencies, from 1400 Hz to 4000 Hz (p<0.05). Interaction of the disease duration and age of disease onset was found to predict hearing levels, distortion products and signal-noise ratios of children with Familial Mediterranean Fever (F value=2.034; p=0.033). CONCLUSIONS: To our knowledge this is the first study demonstrating cochlear involvement in children with Familial Mediterranean Fever which showed increased hearing thresholds at higher frequencies in audiometry together with decreased distortion products and signal-noise ratios demonstrated by distortion product otoacoustic emission testing. Similar studies must be carried out on adult patients to see if a clinical hearing impairment develops. The possible mechanisms that cause cochlear involvement and the effect of colchicine treatment on cochlear functions must be enlightened.

[Cochleovestibular dysfunction in patients with diabetes mellitus, hypertension and dyslipidemia]. / Disfuncion cócleo-vestibular en pacientes con diabetes mellitus, hipertensión arterial sistémica y dislipidemia.

INTRODUCTION AND OBJECTIVE: Polygenic or multifactorial inheritance of chronic disorders (MICD) contribute to irreversible cochleovestibular impairment. Our aim was to determine the type and degree of cochleovestibular dysfunction (CVD) in patients with MICD. METHODS: Cross-sectional. We studied 385 patients with type 2 diabetes mellitus, systemic arterial hypertension and dyslipidemia who were referred to Otorhinolaryngology Unit with hearing and vestibular symptoms. The auditory function was evaluated using conventional tonal audiometry and the vestibular function by electronystagmography. Duration of the disease and number of comorbidities, hearing thresholds at 125-8000 Hz pure tones, speech audiometry, oculomotor evaluation and thermal caloric tests were also analysed. RESULTS: A total of 66.7% (95% CI, 61.8-73.4) of patients had 1 comorbidity; 27.7% (95% CI, 23.3-32.5) had 2 and 5.4% (95% CI, 3.4-8.2) had systemic arterial hypertension, diabetes mellitus and dyslipidemia. The mean age was 62 years (SD 12.9) and 57.1% were women. The majority showed obesity, physical inactivity and smoking (77.4%; 95% CI, 72.8-81.4). Cochlear dysfunction was more common than CVD (98.9%; 95% CI, 97.3-99.7 versus 36.1%; 95%CI, 31.2-41.1; P=.001). However, the presence of CVD was significant in patients over 60 years (χ(2)(tend), P≤.001, odds ratio: 6.43) and with MICD ≥11 years old (χ(2)(tend), P≤.001, odds ratio: 4.57). CONCLUSIONS: Cochlear dysfunction occurs in patients with MICD and the impact is greater than that of vestibular dysfunction. However, the age factor, duration and number of MICDs contribute to CVD. It is necessary to act on the MICDs and lifestyles to improve CVD.

Study of auditory function in children with chronic lung diseases.

OBJECTIVE: Chronic hypoxia has an evident effect on cochlear function and hearing sensitivity. Otoacoustic emissions' testing is efficient in detecting subtle cochlear dysfunction. This cross sectional study was designed to assess the cochlear function in children with chronic lung diseases who were exposed to prolonged hypoxia and prolonged use of ototoxic drugs (as aminoglycosides) using basic audiological evaluation and transient evoked otoacoustic emissions testing. METHODS: The study was carried out on 30 Egyptian children with chronic lung disease recruited from the Pediatric Chest Clinic, Children's hospital, Ain Shams University. Twenty normal children were included as control. RESULTS: Six patients (20%) showed abnormal otoacoustic emissions. A significant effect of hypoxia on otoacoustic emissions findings was found (P<0.05). However, there was no significant effect of inhaled aminoglycosides on auditory functions whether pure tone audiometry, speech audiometry and transient evoked otoacoustic emissions testing. CONCLUSIONS: Children with chronic lung diseases are liable to cochlear dysfunction due to prolonged hypoxia. Inhaled aminoglycosides in chronic lung diseases is relatively safe on auditory functions.

Dead regions in the cochlea at 4 kHz in elderly adults: relation to absolute threshold, steepness of audiogram, and pure-tone average.

The aims of this study were (1) to investigate the prevalence of dead regions (DRs) at 4 kHz in elderly people with hearing loss and (2) to determine the extent to which the presence/absence of a DR can be predicted from the absolute threshold, the slope of the audiogram, or the pure-tone average (PTA) hearing loss at 0.5, 1, and 2 kHz. DRs were assessed for 98 ears with absolute thresholds between 60 and 85 dB HL at 4 kHz using the threshold equalizing noise test. Thirty-six ears had a DR at 4 kHz. There was no statistically significant difference in the slope of the audiogram or PTA between ears with and without DRs. However, the mean absolute threshold at 4 kHz was significantly higher for the group with DRs than for the group without DRs. The prevalence of DRs exceeded 50% for hearing losses greater than 70 dB.

[Otoacoustic emissions in clinical and surgical practice]. / Les otoémissions en pratique clinique et chirurgicale.

OBJECTIVES: Otoacoustic emissions (OAEs), discovered in 1978, have a well-established cochlear origin. They strongly depend on the outer hair cells and are widely used in experimental research as a means for testing cochlear function. However, outside screening, OAEs are only rarely used in clinical practice. The objective of this paper was to show their vast clinical utility. MATERIAL AND METHODS: First, a review of the biophysical and physiological knowledge on OAEs is provided, concerning transient OAEs as well as distortion-product OAEs, recalling the origin and the meanings of these acoustic signals. Several clinical situations are then presented, and the corresponding OAE alterations are explained, such as hearing screening in neonates, diagnosis of hearing impairment with particularities related to the age of the patient, situations critical to the cochlea such as ototoxic treatments, and surgical procedures to the cerebellopontine angle. RESULTS: OAEs appear to be a powerful tool in clinical practice, particularly in hearing screening and diagnosis of deafness. They can also be used to monitor hearing function during cerebellopontine angle tumor resection. CONCLUSION: OAEs are still rarely used as a diagnostic tool by clinicians despite their clinical value, which should make them a primary choice.

Valoración de las secuelas cocleovestibulares en pacientes con trauma craneal / Assessment of the cochleovestibular sequelae in patients with cranial trauma

Se hizo un estudio prospectivo de 30 pacientes atendidos en el Hospital Militar Central Dr Carlos J Finlay en el período comprendido de enero de 2001 a diciembre de 2003, que presentaron trauma craneal con secuela cocleovestibular asociada con la evolución de su enfermedad. El objetivo de esta investigación fue determinar la incidencia de las secuelas audiológicas presentes en estos casos, su comportamiento clínico y la efectividad de las técnicas empleadas para el diagnóstico de estas. De los pacientes estudiados, el 73,3 por ciento correspondió al sexo masculino y el 16,6 por ciento a la edad de 30 a 44 años. Según la naturaleza del trauma, las fracturas fueron las más frecuentes (56,7 por ciento); así como en la sintomatología postraumática, la hipoacusia (46,6 por ciento), la otorragia (30,0 por ciento) y los acúfenos (30,0 por ciento) estuvieron presentes en la mayoría de los enfermos. El 46,6 por ciento de los pacientes presentaron positiva la audiometría y las pruebas electronistagmográficas, en esta última predominaron la falta de inhibición a la fijación visual y la existencia de nistagmus vertical.

A prospective study was undertaken in 30 patients seen in Dr Carlos J Finlay Military Hospital from January 2001 to December 2003, that presented cranial trauma with cochleovestibular sequelae associated with the evolution of the disease. The aim of this research was to determine the incidence of the audiological sequelae in these cases, their clinical behaviour, and the effectiveness of the techniques used for the diagnosis. Of the studied patients, 73.3 percent were males, and 16.6 percent were between 30-and 44 years old. According to the trauma nature, fractures were the most frequent (56.7 percent); whereas in the posttraumatic symptomatology, hypoacusia (46.6 percent), otorrhagia (30.0 percent), and tinnittus (30.0 percent) were observed in most of the patients. A group of sick persons (46.6 percent) had a positive audiometry as well as electronystagmographic tests. The lack of inhibition to visual fixation, and the presence of vertical nystagmus prevailed in the latter.

Distribuição dos achados otoneurológicos em pacientes com disfunção vestíbulo-coclear

O equilíbrio corporal é fundamental no relacionamento espacial do organismo com o ambiente. Três sistemas são responsáveis pela manutenção do equilíbrio: a visão, o sistema proprioceptivo e o aparelho labiríntico ou vestibular. FORMA DE ESTUDO: retrospectivo clínico. Foi realizado um estudo retrospectivo de 3701 pacientes submetidos a exame clínico e exame cócleo-vestibular com registro vecto-eletronistagmográfico, em clínica particular de Otorrinolaringologia de Jundiaí, Estado de São Paulo, no período de 1979 a 2004. OBJETIVO: com a finalidade de se determinar a distribuição sindrômica dessa população e correlacioná-la com outros dados como sexo, faixa etária, sintomatologia, achados de exame clínico, audiológico, bem como quais são as especialidades médicas que solicitam essa avaliação com maior freqüência. RESULTADO: Na população estudada, foi encontrada uma maior prevalência do sexo feminino, numa proporção de 1,75:1. A faixa etária de indivíduos de 20 a 59 anos respondeu por 79 por cento dos pacientes estudados, incluindo portanto pessoas em idade laborativa, que apresentaram maior prevalência de síndromes periféricas, contudo não houve predominância de sexo feminino ou idade entre as diferentes síndromes. O estudo também demonstrou a presença de sintomas otoneurológicos comuns aos diferentes tipos de síndromes otoneurológicas, por exemplo, sem o predomínio clássico apontado na literatura das tonturas rotatórias predominarem nos casos de acometimento periférico e as não-rotatórias nos centrais. Alterações da acuidade auditiva, presença de zumbidos e sintomas neurovegetativos ocorreram com maior prevalência nos casos periféricos. Os desvios harmônicos às provas segmentares foram mais prevalentes nos pacientes portadores de síndromes periféricas e os desarmônicos, nas centrais, em consonância com a literatura pesquisada. CONCLUSÃO: As conclusões desta análise retrospectiva apontam para as especialidades de Otorrinolaringologia e Neurologia como as que mais freqüentemente solicitam este tipo de avaliação e o diagnóstico de síndrome periférica foi encontrado em 36 por cento dos pacientes, contudo » da população estudada não apresentou qualquer alteração na avaliação clínica e armada.

[Distribution of neurotological findings in patients with cochleovestibular dysfunction]. / Distribuição dos achados otoneurológicos em pacientes com disfunção vestíbulo-coclear.

UNLABELLED: The relationship between spatial body positioning and environment comes from perfect corporal balance. The three most important systems responsible for this relationship are: the optic system (sight), the proprioceptive system, and the labyrinthine system. STUDY DESIGN: Retrospective clinical. A retrospective study conducted on 3,701 patients of a private otolaryngologic clinic, in Jundiaí - São Paulo - Brazil, who underwent vestibular and cochlear labyrinthine function testing, from 1979 to 2004. AIM: To determinate the syndromic distribution occurred at that population and to correlate its relationship with sex, age, symptomatology, as well as otological, clinical and electronystagmographic findings and which were the most frequent medical specialties who asked for this investigation. RESULTS: There was found in the studied population a major prevalence on females (1.75:1). Seventy-nine percent of the patients were from 20 to 59 years old, therefore including people in labor age, with a major prevalence of peripheral syndromes, but there was no a preferential age or sex group among the different syndromes. This study also demonstrated that some otoneurological symptoms were common to all kind of otoneurological syndromes, in opposition to the data found in the world literature. Tinnitus, hearing loss, nausea and vomit as well as harmonic alterations on clinical examination were found with more frequency on peripheral syndrome and no harmonic on central syndromes, according to the reviewed literature. CONCLUSIONS: The study conclusions were able to show that the majority of the patients started their investigation with either otolaryngologists or neurologists and 36% of the patients had peripheral syndrome with almost 25% that had normal evaluation.

Sjögren's syndrome: an autoimmune disorder with otolaryngological involvement.

Sjorgen's syndrome (SS) is an autoimmune exocrinopathy characterized by lymphocyte infiltration of salivary and lacrimal glands that leads to progressive xerostomia and xerophtalmia. One-third of patients suffer of systemic manifestations including arthritis, fever, fatigue and mucosal dryness whereas those with major salivary involvement show an increased risk to develop low-grade non-Hpdgkin lymphomas. In addition, a minority of patients show symptoms related to progressive hearing loss whose pathogenesis remains undefined. Both deposition of autoantibodies to antigens of the inner-ear structures and infiltration by autoreactive T-cells have been implicated in its pathogenesis. In this context, high levels of autoantibodies to both cardiolipin and M3 muscarinic receprtors as well as to ciliar epitopes of the cochlear cells have been recently described. Here we review recent advances on the pathodgenesis of SS with a particular focus to otolaryngological manifestations.

Temporal bone histopathology in dominantly inherited audiovestibular syndrome.

OBJECTIVE: To describe the clinical and pathologic features of a new dominantly inherited audiovestibular syndrome. METHODS: History, examination, and audiometric testing in the proband, brother, and son; quantitative rotational testing in the proband and son; histopathology of the cochlea and vestibular labyrinth in the proband; sequencing candidate genes COCH and MYO7A in the brother and son. RESULTS: Affected family members developed slowly progressive hearing loss beginning in their late 30s and progressive imbalance in their early 70s. Three of four affected had brief (minutes) episodes of vertigo typically occurring a few times per year. Auditory and vestibular function testing documented a slowly progressive loss of auditory and vestibular function. Postmortem examination showed a loss of hair cells in the cochlea and vestibular receptor organs. There were no cellular infiltrates or acidophilic deposits. No mutations were found in the COCH or MYO7A genes. CONCLUSIONS: This dominantly inherited audiovestibular syndrome results in a selective loss of hair cells in the auditory and vestibular end organs. Finding the causative gene could have important implications for understanding the pathophysiology of presbycusis and dysequilibrium of aging.