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BACKGROUND: Immune dysregulation in individuals with Down syndrome (DS) leads to an increased risk for hospitalization and death due to coronavirus disease 2019 (COVID-19) and may impair the generation of protective immunity after vaccine administration. METHODS: The cellular and humoral responses of 55 individuals with DS who received a complete SARS-CoV-2 vaccination regime at 1 to 3 (visit [V 1]) and 6 (V2) months were characterized. RESULTS: SARS-CoV-2-reactive CD4+ and CD8+ T lymphocytes with a predominant Th1 phenotype were observed at V1 and increased at V2. Likewise, an increase in SARS-CoV-2-specific circulating Tfh (cTfh) cells and CD8+ CXCR5+ PD-1hi lymphocytes was already observed at V1 after vaccine administration. Specific immunoglobulin G (IgG) antibodies against SARS-CoV-2 S protein were detected in 96% and 98% of subjects at V1 and V2, respectively, although IgG titers decreased significantly between both time points. CONCLUSIONS: Our findings show that DS individuals develop an effective immune response to usual regimes of SARS-CoV-2 vaccination.
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Antígenos de Grupos Sanguíneos , COVID-19 , Síndrome de Down , Síndrome de Quebra de Nijmegen , Humanos , Anticorpos Antivirais , COVID-19/prevenção & controle , Vacinas contra COVID-19 , Imunidade , Imunoglobulina G , SARS-CoV-2 , Vacinação , AdultoRESUMO
Methane production by microbial fermentation of municipal waste is a challenge for better yield processes. This work describes the characterization of a hydrogenotrophic methanogen microbial community used in a bioaugmentation procedure to improve the methane yield in a thermophilic anaerobic process, digesting the organic fraction of municipal solid waste. The performance of the bioaugmentation was assessed in terms of methane production and changes in the microbial community structure. The results showed that bioaugmentation slightly improved the cumulative methane yield (+ 4%) in comparison to the control, and its use led to an acceleration of the methanogenesis stage. We observed associated significant changes in the relative abundance of taxa and their interactions, using high throughput DNA sequencing of V3-16S rRNA gene libraries, where the abundance of the archaeal hydrogenotrophic genus Methanoculleus (class Methanomicrobia, phylum Euryarchaeota) and the bacterial order MBA08 (class Clostridia, phylum Firmicutes) were dominant. The relevant predicted metabolic pathways agreed with substrate degradation and the anaerobic methanogenic process. The purpose of the study was to evaluate the effect of the addition of hydrogenotrophic methanogens in the generation of methane, while treating organic waste through anaerobic digestion.
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Euryarchaeota , Microbiota , Anaerobiose , Metano/metabolismo , RNA Ribossômico 16S/genética , Reatores Biológicos/microbiologia , Euryarchaeota/genética , Euryarchaeota/metabolismo , Microbiota/genética , Firmicutes/metabolismoRESUMO
INTRODUCTION: We evaluate the impact of COVID-epidemic in colorectal cancer (CRC) diagnosis during Spain's state of emergency. METHODS: We compared newly diagnosed patients with patients diagnosed in the same period of 2019. RESULTS: A new diagnosis of CRC decreased 48% with a higher rate of patients diagnosed in the emergency setting (12.1% vs. 3.6%; p = .048) and a lower rate diagnosed in the screening program (5.2% vs. 33.3%; p = .000). CONCLUSIONS: Fewer patients have been diagnosed with CRC, with a higher rate of patients diagnosed in an emergency setting.
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COVID-19/epidemiologia , Neoplasias Colorretais/diagnóstico , Serviço Hospitalar de Emergência , SARS-CoV-2 , Idoso , Feminino , Humanos , Masculino , Espanha/epidemiologiaRESUMO
Optical fiber with YPO4:Pr3+ nanocrystals (NCs) is presented for the first time using the glass powder-NCs doping method. The method's advantage is separate preparation of NCs and glass to preserve luminescent and optical properties of NCs once they are incorporated into optical fiber. The YPO4:Pr3+ nanocrystals were synthesized by the co-precipitation and hydrothermal methods, optimized for size (< 100 nm), shape, Pr3+ ions concentration (0.2 mol%), and emission lifetime. The core glass was selected from the non-silica P2O5-containing system with refractive index (n = 1.788) close to the NCs (no = 1.657, ne = 1.838). Optical fiber was drawn by modified powder-in-tube method after pre-sintering of glass powder-YPO4:Pr3+ (wt 3%) mixture to form optical fiber preform. Luminescent properties of YPO4:Pr3+ and optical fiber showed their excellent agreement, including sharp Pr3+ emission at 600 nm (1D2-3H4) and 1D2 level lifetime (τ = 156 ± 5 µs) under 488 nm excitation. The distribution of the YPO4:Pr3+ NCs in optical fiber were analyzed by TEM-EDS in the core region (FIB-SEM-prepared). The successful usage of glass powder-NCs doping method was discussed in the aspect of promising properties of the first YPO4:Pr3+ doped optical fiber as a new way to develop active materials for lasing applications, among others.
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INTRODUCTION: Vestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy. MATERIAL AND METHODS: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC. RESULTS: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found. CONCLUSIONS: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.
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Neuroma Acústico , Neuroma Acústico/terapia , Neuroma Acústico/diagnóstico , Neuroma Acústico/diagnóstico por imagem , Humanos , Conduta ExpectanteRESUMO
An investigation of fluoroindate glass and fiber co-doped with Yb3+/Er3+ ions as a potential temperature sensor was assessed using the fluorescence intensity ratio (FIR) technique. Analysis of thermally coupled levels (TCLs-2H11/2 and 4S3/2), non-thermally coupled levels (non-TCLs-4F7/2 and 4F9/2), and their combination were examined. Additionally, the luminescent stability of the samples under constant NIR excitation using different density power at three different temperatures was carried out. The obtained values of absolute sensitivity (0.003 K-1-glass, 0.0019 K-1-glass fiber 2H11/2 â 4S3/2 transition) and relative sensitivity (2.05% K-1-glass, 1.64% K-1-glass fiber 4F7/2 â 4F9/2 transition), as well as high repeatability of the signal, indicate that this material could be used in temperature sensing applications.
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The effect of BaF2, MgF2, and AlF3 on the structural and luminescent properties of gallo-germanate glass (BGG) doped with erbium ions was investigated. A detailed analysis of infrared and Raman spectra shows that the local environment of erbium ions in the glass was influenced mainly by [GeO]4 and [GeO]6 units. Moreover, the highest number of non-bridging oxygens was found in the network of the BGG glass modified by MgF2. The 27Al MAS NMR spectrum of BGG glass with AlF3 suggests the presence of aluminum in tetra-, penta-, and octahedral coordination geometry. Therefore, the probability of the 4I13/2â4I15/2 transition of Er3+ ions increases in the BGG + MgF2 glass system. On the other hand, the luminescence spectra showed that the fluoride modifiers lead to an enhancement in the emission of each analyzed transition when different excitation sources are employed (808 nm and 980 nm). The analysis of energy transfer mechanisms shows that the fluoride compounds promote the emission intensity in different channels. These results represent a strong base for designing glasses with unique luminescent properties.
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(1) Background. Patients with bilateral vestibular disease (BVD) experience oscillopsia with a detriment to visual acuity (VA). This VA is driven mainly by the VOR that has two components: rotational and translational. VA can be tested by using dynamic visual acuity (DVA) on a treadmill because both systems are activated. The aim of this study is to compare VA before and after chronic electrical stimulation of the otolith organ. (2) Materials and Method. Five patients suffering from bilateral vestibular dysfunction (BVD), previously implanted with a new vestibular implant prototype, were included in this study with the aim to check VA with and without vestibular implant use (W and W/O) in static, 2 km/h and 4 km/h walking situations. DVAtreadmill was measured on a treadmill with a dynamic illegible E (DIE) test in static and dynamic conditions (while walking on the treadmill at 2 and 4 km/h). The DVA score was registered in a logarithm of the minimum angle of resolution (LogMAR) for each speed. In addition, every patient completed the oscillopsia severity questionnaire (OSQ) and video head impulse test (vHIT) before and after activation of the vestibular implant. (3) Results. The analysis shows a significant difference in OSQ scores and DVA with an improvement in dynamic conditions. Organized corrective saccades during the use of a vestibular implant with no changes in gain were also detected in the video head impulse tests (vHIT). (4) Conclusion. The vestibular implant with otolithic stimulation offers changes in the response of DVA, which makes this paper one of the first to address the possible restoration of it. It is not possible to rule out other contributing factors (presence of covert saccades, somatosensory system, ). More work seems necessary to understand the neurophysiological basis of these findings, but this implant is added as a therapeutic alternative for the improvement of oscillopsia.
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OBJECTIVES: The aim of this study was to assess whether a virtual rehabilitation program using Nintendo Wii added to conventional physical therapy improved functionality, balance, and daily activities in chronic stroke survivors, when compared with conventional physical therapy. DESIGN: We undertook a randomized controlled clinical trial. The participants of this study were randomized to 2 groups: (1) conventional physical therapy (CPTG), which included exercises related to functionality, balance, and activities of daily living; and (2) virtual reality with Nintendo Wii (VRWiiG), which included balance training with the Wii Balance Board and upper limb exercises with the Wii Sports package, added to conventional physical therapy. SETTING AND PARTICIPANTS: This study was conducted in a university rehabilitation clinic and 29 stroke survivors were admitted. METHODS: Both interventions lasted 4 weeks, 2 sessions per week. Assessments were performed at baseline and at the end of the study, including functionality [Timed up and go (TUG)], balance [Tinetti Performance-Oriented Mobility Assessment (POMA)], Berg Balance Scale (BBS), and activities of daily living [Fugl-Meyer Upper Limb Motor Assessment, Barthel Index, Frenchay Activity Index (FAI)]. RESULTS: Regarding TUG, POMA, and BBS, the analysis of variance showed significant differences for time and group∗time interaction. Post hoc analysis showed between-group differences (P = .044, d = -0.78; P = .012, d = 1.00; P = .042, d = 0.79, respectively) and within-group differences only in the VRWiiG (P < .001, d = 0.75; P < .001, d = -0.76; P < .001, d = -0.57, respectively). Regarding activities of daily living, post hoc analysis showed within-group differences only in VRWiiG. CONCLUSIONS AND IMPLICATIONS: Our study showed promising results in functionality, balance, and activities of daily living when adding virtual reality with Nintendo Wii to conventional physical therapy in chronic stroke survivors. All procedures were approved by the Human Research Ethics Committee of the University of Valencia (H1518177391901). ClinicalTrials.gov database (NLM identifier NCT04144556).
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Reabilitação do Acidente Vascular Cerebral , Acidente Vascular Cerebral , Jogos de Vídeo , Atividades Cotidianas , Terapia por Exercício , Humanos , Modalidades de Fisioterapia , Equilíbrio PosturalRESUMO
INTRODUCTION: Patients with episodic vestibular syndrome (EVS) whose symptoms resemble those of vestibular migraine (VM) but who do not meet the criteria for it are common. OBJECTIVE: To describe those patients suffering from EVS in whom defined etiologies have been ruled out in order to determine if their symptoms can be linked to VM. MATERIAL AND METHODS: Prospective multicenter study. The medical records of patients with VM and patients with EVS suggestive of VM but not meeting the criteria for it were examined. The characteristics of headache, the number and the length of attacks, the association of vestibular symptoms and headache, the intensity of symptoms and the response to treatment were recorded. RESULTS: 58 patients met the criteria for VM or probable VM; 30 did not. All of the symptoms improved significantly in the treated patients with VM or probable VM; in the rest of the treated patients, only the vestibular symptoms improved. CONCLUSION: A subgroup of patients that cannot be attributed to any known vestibulopathy according to present day VM criteria profited from migraine treatment, suggesting that their vestibular symptoms belong to the migraine spectrum; whereas some do not, yet our analysis could not identify distinctive features that allowed subgroup attribution.
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Transtornos de Enxaqueca , Doenças Vestibulares , Vestíbulo do Labirinto , Humanos , Transtornos de Enxaqueca/diagnóstico , Estudos Prospectivos , Vertigem/diagnóstico , Doenças Vestibulares/diagnósticoRESUMO
INTRODUCTION: Subjective benign paroxysmal positional vertigo is a form of benign paroxysmal positional vertigo in which during the diagnostic positional maneuvers patients only present vertigo symptoms with no nystagmus. OBJECTIVE: To study the characteristics of subjects with subjective benign paroxysmal positional vertigo. METHODS: Prospective multicenter case-control study. All patients presenting with vertigo in the Dix-Hallpike test that presented to the participating hospitals were included. The patients were separated into two groups depending on whether nystagmus was present or not. An Epley Maneuver of the affected side was performed. In the follow-up visit, patients were checked to see if nystagmus and vertigo were present. Both groups of patients were compared to assess the success rate of the Epley maneuver and also to compare the presence of 19 variables. RESULTS: 259 patients were recruited, of which 64 belonged to the subjective group. Nystagmus was eliminated in 67.2% of the patients with benign paroxysmal positional vertigo. 89.1% of the patients with subjective benign paroxysmal positional vertigo remained unaffected by nystagmus, thus showing a significant difference (p=0.001). Osteoporosis and migraine were the variables which reached the closest to the significance level. In those patients who were taking vestibular suppressors, the percentage of subjective benign paroxysmal positional vertigo was not significantly higher. CONCLUSIONS: Subjective benign paroxysmal positional vertigo should be treated using the Epley maneuver. More studies are needed to establish a relationship between osteoporosis, migraine and subjective benign paroxysmal positional vertigo. The use of vestibular suppressants does not affect the detection of nystagmus.
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Vertigem Posicional Paroxística Benigna/fisiopatologia , Transtornos de Enxaqueca/fisiopatologia , Osteoporose/fisiopatologia , Adolescente , Adulto , Vertigem Posicional Paroxística Benigna/complicações , Vertigem Posicional Paroxística Benigna/tratamento farmacológico , beta-Histina/uso terapêutico , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Transtornos de Enxaqueca/complicações , Nistagmo Fisiológico/fisiologia , Osteoporose/complicações , Modalidades de Fisioterapia , Postura/fisiologia , Estudos Prospectivos , Sulpirida/uso terapêutico , Adulto JovemRESUMO
INTRODUCTION: Community-acquired pneumonia (CAP) is a common serious infection. This study aimed to evaluate the prognostic utility of neutrophil count percentage (NCP) and neutrophil-lymphocyte ratio (NLR) in patients with CAP. METHODS: Retrospective study of hospitalized patients with CAP. Patients had a blood test at admission and 3-5 days after hospitalization (early-stage test). The main outcome variables were 30-day and 90-day mortality. RESULTS: Two hundred and 9patients were included. Patients who survived had significant reductions in both NCP and NLR between admission and the day 3-5 blood tests (from 85.8% to 65.4% for NCP and from 10.1 to 3.2 for NLR). Twenty-five patients died in the first 90 days. Patients who died had lower, non-significant reductions in NCP (from 84.8% to 74%) and NLR (from 9.9 to 6.9) and significantly higher early-stage NCP and NLR than those who survived. NCP values higher than 85% and NLR values higher than 10 in the early-stage blood test were associated with a higher risk of mortality, even after multivariate adjustment (HR for NCP: 12; HR for NLR: 6.5). CONCLUSION: NCP and NLR are simple, low-cost parameters with prognostic utility, especially when measured 3-5 days after CAP diagnosis. High NLR and/or NCP levels are associated with a greater risk of mortality at 90 days.
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Infecções Comunitárias Adquiridas/sangue , Infecções Comunitárias Adquiridas/diagnóstico , Linfócitos , Neutrófilos , Idoso , Idoso de 80 Anos ou mais , Feminino , Hospitalização , Humanos , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos RetrospectivosAssuntos
COVID-19 , Síndrome de Down , Adulto , Humanos , Vacinas contra COVID-19 , SARS-CoV-2 , COVID-19/prevenção & controle , Imunidade , Anticorpos Antivirais , VacinaçãoRESUMO
Resumen El síndrome de Mayer-Rokitansky-Küster-Hauser (SMRKH) es una anomalía del tracto genital femenino caracterizada por ausencia congénita del útero y porción superior de la vagina. Ocurre en uno de cada 4,500 nacimientos y se diagnostica normalmente durante la adolescencia al presentarse amenorrea primaria. Su función ovárica está preservada, pero la información actual respecto al potencial reproductivo de estas pacientes es limitada. Se presenta el caso de una mujer con diagnóstico de SMRKH sometida a estimulación ovárica para transferencia de embriones a útero subrogado y se discute su potencial reproductivo: técnicas de reproducción asistida, intervenciones e impacto psicológico.
Abstract Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a congenital anomaly of the female genital tract characterized by congenital absence of the uterus and upper part of the vagina. It occurs in 4,500 female births and diagnosis is usually made during adolescence when primary amenorrhea presents. They have functioning ovaries but data regarding their reproductive potential is limited. We hereby report the case of a woman diagnosed with MRKH syndrome in whom assisted reproductive techniques were used to try to achieve pregnancy by gestational surrogacy and their reproductive potential is discussed: assisted reproductive techniques, procedures, and psychological impact.
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INTRODUCTION AND OBJECTIVES: The set of symptoms relating to disorders of the balance system are common in the general population. However, there are few studies quantifying the frequency of onset of the various vestibular disorders that present in specialist otoneurology units in the Spanish population. The aim of this study is to establish the epidemiology of vestibular disorders, their form of presentation, and the expected care burden in a specialist otoneurology clinic. MATERIAL AND METHODS: A retrospective, descriptive, observational study of patients referred to the otoneurology unit of a third level hospital between 1/1/2015 and 31/12/2015. RESULTS: One hundred and seventy-four patients were assessed (121 women and 53 men) with a mean age of 53±17 years. Forty-three individuals per 100,000 inhabitants were assessed over the study period. The patients were referred in the majority from external ENT (36.8%) and primary care (28.7%) clinics. The most frequent reason for consultation was dizziness (40.2%) followed by vertigo (31%). The most frequent diagnoses were benign paroxysmal positional vertigo (28.2%) and vestibular migraine (28.2%) -defined (59%) or probable (41%)-, followed by Ménière's disease (13.8%), vascular disorders (5.7%), chronic subjective dizziness (4.6%) and vestibular neuritis (4%). CONCLUSIONS: The availability of precise information on the prevalence and impact of vestibular disorders is important to enable the health services to plan an appropriate response to the expected care demand in the community. Benign paroxysmal positional vertigo and vestibular migraine are the most commonly diagnosed disorders in otoneurology clinics. Systematic terminology is essential for the comparison of results.
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Unidades Hospitalares/estatística & dados numéricos , Neurologia , Otolaringologia , Doenças Vestibulares/epidemiologia , Adulto , Idoso , Comorbidade , Grupos Diagnósticos Relacionados , Tontura/epidemiologia , Feminino , Humanos , Masculino , Doença de Meniere/epidemiologia , Pessoa de Meia-Idade , Transtornos de Enxaqueca/epidemiologia , Estudos Retrospectivos , Espanha/epidemiologia , Centros de Atenção Terciária/estatística & dados numéricosRESUMO
INTRODUCTION: and objectives: In-depth assessment of the vestibulo-ocular reflex with the new video systems available provides information on various previously-unidentified characteristics in patients with peripheral vestibular disorder. The aim of this work is to quantify how often pathological gain (uni- or bilateral) in the posterior semicircular canal (PSC) gain appears in video head-impulse testing (vHIT) with preservation of the gain in the rest of the semicircular canals, and to analyse the relation of this frequency with patient age and/or diagnosis. METHODS: This was a prospective observational study on adults referred to our unit between June 2012 and February 2014. Age, sex, pathological antecedents, prior history of vestibular pathology, diagnosis and time of evolution of the clinical picture and its characteristics were recorded. Each patient underwent a complete otoneurological examination, including vHIT (Otometrics®). Depending on suspected diagnosis, patients also received cervical vestibular evoked myogenic potential (VEMP) testing, tonal audiometry and speech test, as well as assessment by Neurology and/or nuclear magnetic resonance or computed axial tomography scan imaging study. RESULTS: In all, 363 patients were assessed; 57 (16%) patients (33 males and 24 females; mean age, 57 ± 15 years) showed pathological PSC gains, 46 (81%) unilateral and 11 (19%) bilateral. Ménière's Disease was the most frequent diagnosis, followed by benign paroxysmal positional vertigo (27%). Patients with unilateral involvement were younger than those with bilateral (55 vs. 68 years; p=0.03). In the patients with bilateral involvement, PSC gain and age were related (-0.7; 0.04) and the gain value was symmetrical in both ears (0.7; p=0.05). The gain in the anterior and horizontal canals tended to decrease with age, but without statistical significance in our sample. CONCLUSIONS: Of the patients referred for an otoneurological consultation, 16% showed pathological PSC gain (unilateral in 81% and bilateral in 19% of the cases). This gain involvement was attributable to age when the gain was bilateral and symmetrical.
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Canais Semicirculares , Idoso , Feminino , Teste do Impulso da Cabeça , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Reflexo Vestíbulo-Ocular , Doenças Vestibulares/diagnósticoRESUMO
OBJECTIVE: Prediction of speech recognition (SR) and quality of life (QoL) outcomes after cochlear implantation is one of the most important challenges for otologists. By sifting through very large amounts of data, data mining reveals trends, patterns, and relationships that might otherwise have remained undetected. There are identifiable pre-implantational factors that condition the cochlear implantation outcome. Our objective is to design a data mining system to predict and classify cochlear implant (CI) predictable benefits in terms of SR and QoL in each patient. MATERIALS AND METHODS: This is an observational study of CI users for at least one year. Audiological benefits and its relation to QoL are analyzed using the Glasgow Benefit Inventory (GBI) and the Specific Questionnaire (SQ). Sociodemographic and medical variables are processed in SPSS Statistics 19.0, MatLab® and Weka®. Classifiers are designed using the nearest neighbour and decision tree algorithms. Estimators are created by linear logistic regression. RESULTS: A total of 29 patients (mean age, 55.3 years; 52% female and 48% male) including 48% unilateral CI users and 51% bimodal CI users were included in the study. GBI improved by 36 points and SQ by 1.7 (p<0.05). Using Nearest Neighbour (IB1) algorithm for classifiers, interesting attributes were identified for SR and SQ result classification (success rate: 80.7%). Decision tree algorithm (J48) showed influencing variables for GBI (success rate: 81%). Estimators by linear logistic regression analysis disclosed a precision of 85%, 68%, and 71% for SR, GBI, and SQ, respectively. CONCLUSION: Our study proposes a systematized system to classify and estimate SR and QoL improvement based on our initial evaluation to complement decision making and patients' information.
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Implante Coclear/psicologia , Mineração de Dados , Surdez/psicologia , Surdez/reabilitação , Qualidade de Vida/psicologia , Teste do Limiar de Recepção da Fala , Algoritmos , Árvores de Decisões , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Satisfação do Paciente , Prognóstico , Inquéritos e QuestionáriosRESUMO
INTRODUCTION: Chiari 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 METHODS: We 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. RESULTS: There 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. CONCLUSIONS: The 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.