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A 76 year old woman was referred to our unit after presenting with intermittent dysphonia of 1 year duration. There was neither a relevant medical history nor did she have any toxic habits such as consumption of alcohol or tobacco. There were no B symptoms. Clinical nasopharynx, Waldeyer ring, and cervical examinations all showed normal results; the nasofibroscopy performed revealed a single laryngeal polyp in the left vocal cord (Fig. 1) with a preservation of laryngeal movement. Findings from a blood test and a general clinical examination were normal. Laryngeal micro surgery with the use of suspension laryngoscopy according to the Kleinasser technique was indicated and the before-mentioned polyp was extirpated. The subgloggic region, the supraglottis, and the hypopharynx were disease free. A bronchoscopy was also performed which also resulted normal. The biopsy from the anatomopathological study showed a lymphoid infiltration of the tissue with characteristics which are typical of MALT type extraganglionar B cell lymphoma (Fig. 2) and the immunohistochemical staining confirmed B lymphocytes (CD20 + CD79 + BCL + BCL6, CD5, CD3, CD23, D1 cyclin). The proliferation index (MIB-1) was low. PCR expansion of IGH reordering, in accordance with BIOMED-2

Objectives/Hypothesis To evaluate the vestibulo-ocular reflex and its relationship with subjective balance in a long-term follow-up after vestibular schwannoma surgery. Study Design Retrospective cohorts study in a tertiary referral hospital. Forty-nine consecutive patients on which vestibular schwannoma surgery was performed at least 1 year before. Methods Patients are classified in accordance with the morphology of the vestibulo-ocular reflex (VOR) into two groups: Group A, in which covert and overt saccades always occur in an organized fashion; and group B, in which covert and overt saccades randomly occur during head impulse and once finished. We evaluate the presence of covert and overt saccades and the morphology of the VOR measured by the video head impulse test (vHIT) and its relationship with subjective balance measured by Dizziness Handicap Inventory (DHI). Results The group B patients returned a higher score in total DHI and all three subscales without any predominant subscale (P = 0,0002; t test). Group B patients were older than those in group A (P = 0,024; t test). No differences were found in sex distribution, tumor size, or side or time interval after surgery. Regarding the unaffected side, overt saccades were found to be more frequent in group B patients (P = 0.05; X2). Conclusions Long-term follow-up after vestibular schwannoma surgery has shown that 22% of the patients display a particular abnormality in the VOR because refixation saccades occur in a random fashion after elicitation of the reflex in the HIT test. These patients report the higher level of vestibular disability and handicap. Level of Evidence 2b. Laryngoscope, 124:1431–1435, 2014

The aims of this study were to estimate the prevalence of familial cases in patients with Meniere's disease (MD) and to identify clinical differences between sporadic and familial MD. We recruited 1375 patients with definite MD according to the American Academy of Otolaryngology-Head and Neck Surgery criteria, obtaining the familial history of hearing loss or episodic vertigo by direct interview or a postal survey in 1245 cases in a multicenter study. Familial clustering was estimated by the recurrence risk ratio in siblings (λs ) and offspring (λo ) using intermediate and high prevalence values for MD in European population. A total of 431 patients (34%) reported a familial history of hearing loss or recurrent vertigo and 133 patients had a relative with possible MD. After clinical reevaluation, 93 relatives in 76 families were diagnosed of definite MD (8.4%), including three pairs of monozygotic twins. λs and λo were 16-48 and 4-12, respectively. We observed genetic heterogeneity, but most families had an autosomal dominant inheritance with anticipation. No clinical differences were found between sporadic and familial MD, except for an early onset in familial cases. We may conclude that MD has a strong familial aggregation and that sporadic and familial MDs are clinically identical.

Acute vestibular deficit as the first sign of leukemia is extremely rare. The literature shows some cases of sudden hearing loss accompanied by instability and associated with hyperviscosity syndrome. We present the case of a patient who presents a harmonic vestibular deficit of the right ear. The complementary studies revealed an abnormally high level of leukocytes. A peripheral blood cytogenetic analysis is performed due to a high suspicion of leukemia, and the results show BCR/ABL fusion gene with a cut point in the M-BCR region, which confirms the diagnosis of chronic myeloid leukemia. In this case we detail the importance of taking hematological disorders into consideration in the differential diagnosis of patients with otoneurological symptoms, and we also review the etiopathogenic mechanisms, symptoms, diagnosis, and therapeutic options for chronic myeloid leukemia with sudden hearing loss and vertigo.

Autosomal dominant (AD) familial Meniere's disease (FMD) is a rare disorder involving the inner ear defined by sensorineural hearing loss, tinnitus and episodic vertigo. Here, we have identified two novel and rare heterozygous variants in the SEMA3D and DPT genes segregating with the complete phenotype that have variable expressivity in two pedigrees with AD-FMD. A detailed characterization of the phenotype within each family illustrates the clinical heterogeneity in the onset and progression of the disease. We also showed the expression of both genes in the human cochlea and performed in silico analyses of these variants. Three-dimensional protein modelling showed changes in the structure of the protein indicating potential physical interactions. These results confirm a genetic heterogeneity in FMD with incomplete penetrance and variable expressivity.

Table of records containing missing data that was queried to the corresponding author by email. (DOCX 21 kb)

PRISMA checklist of items to include when reporting a systematic review. (DOCX 19 kb)

Outcome instruments used only once either for primary or secondary outcomes. (DOCX 19 kb)

Table reporting the full reference list for all 228 included records. (DOCX 39 kb)

Tabulation of the evaluation of outcome reporting bias. ‘✓’ denotes consistent reporting across publications; ‘✗’ denotes inconsistent reporting; ‘o’ denotes partial reporting whereby the instrument remains consistent but the time frame does not; ‘P-only’ denotes that the outcome was specified in the protocol, but not reported as a study finding; ‘F-only’ denotes that the outcome was not specified in the protocol, but was reported as a study finding. For P-only, we cannot distinguish cases where an outcome was measured and analysed but not reported, measured but not analysed or reported, or not measured. (DOCX 25 kb)

Meniere disease (MD) is a heterogeneous clinical condition characterized by sensorineural hearing loss, episodic vestibular symptoms, and tinnitus associated with several comorbidities, such as migraine or autoimmune disorders (AD). The frequency of bilateral involvement may range from 5 to 50%, and it depends on the duration of the disease. We have performed a two-step cluster analysis in 398 patients with bilateral MD (BMD) to identify the best predictors to define clinical subgroups with a potential different etiology to improve the phenotyping of BMD and to develop new treatments. We have defined five clinical variants in BMD. Group 1 is the most frequently found, includes 46% of patients, and is defined by metachronic hearing loss without migraine and without AD. Group 2 is found in 17% of patients, and it is defined by synchronic hearing loss without migraine or AD. Group 3, with 13% of patients, is characterized by familial MD, while group 4, that includes 12% of patients, is associated by the presence of migraine in all cases. Group 5 is found in 11% of patients and is defined by AD. This approach can be helpful in selecting patients for genetic and clinical research. However, further studies will be required to improve the phenotyping in these clinical variants for a better understanding of the diverse etiological factors contributing to BMD.

BACKGROUND: There is no evidence-based guidance to facilitate design decisions for confirmatory trials or systematic reviews investigating treatment efficacy for adults with tinnitus. This systematic review therefore seeks to ascertain the current status of trial designs by identifying and evaluating the reporting of outcome domains and instruments in the treatment of adults with tinnitus. METHODS: Records were identified by searching PubMed, EMBASE CINAHL, EBSCO, and CENTRAL clinical trial registries (ClinicalTrials.gov, ISRCTN, ICTRP) and the Cochrane Database of Systematic Reviews. Eligible records were those published from 1 July 2006 to 12 March 2015. Included studies were those reporting adults aged 18 years or older who reported tinnitus as a primary complaint, and who were enrolled into a randomised controlled trial, a before and after study, a non-randomised controlled trial, a case-controlled study or a cohort study, and written in English. Studies with fewer than 20 participants were excluded. RESULTS: Two hundred and twenty-eight studies were included. Thirty-five different primary outcome domains were identified spanning seven categories (tinnitus percept, impact of tinnitus, co-occurring complaints, quality of life, body structures and function, treatment-related outcomes and unclear or not specified). Over half the studies (55 %) did not clearly define the complaint of interest. Tinnitus loudness was the domain most often reported (14 %), followed by tinnitus distress (7 %). Seventy-eight different primary outcome instruments were identified. Instruments assessing multiple attributes of the impact of tinnitus were most common (34 %). Overall, 24 different patient-reported tools were used, predominantly the Tinnitus Handicap Inventory (15 %). Loudness was measured in diverse ways including a numerical rating scale (8 %), loudness matching (4 %), minimum masking level (1 %) and loudness discomfort level (1 %). Ten percent of studies did not clearly report the instrument used. CONCLUSIONS: Our findings indicate poor appreciation of the basic principles of good trial design, particularly the importance of specifying what aspect of therapeutic benefit is the main outcome. No single outcome was reported in all studies and there was a broad diversity of outcome instruments. PROSPERO REGISTRATION: The systematic review protocol is registered on PROSPERO (International Prospective Register of Systematic Reviews): CRD42015017525. Registered on 12 March 2015 revised on 15 March 2016. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s13063-016-1399-9) contains supplementary material, which is available to authorized users.

Electrode array misplacement is an infrequent complication in cochlear implant surgery. A case report of electrode array insertion into the posterior semicircular canal, and its effects on the vestibular function is described. Video head impulse test (vHIT) has become an exceptional diagnostic tool to study vestibular function. We present our experience using the vHIT to detect a misplaced electrode array after cochlear implant surgery.

The appearance of a primary germinoma in the central nervous system but not on or near the midline or within the brain is exceptional. It may occur at any age; however, it is rare in patients over 50 years old. Only a handful of cases of germinomas located in the cerebellopontine angle were presented, but to our knowledge, there has been no description of an isolated germinoma in the internal auditory canal. We report a case of germinoma in the internal auditory canal in a 51-year-old man simulating the clinical and radiological characteristics of a vestibular schwannoma.

Ménière’s disease (MD) represents a heterogeneous group of relatively rare disorders with three core symptoms: episodic vertigo, tinnitus, and sensorineural hearing loss involving 125 to 2,000 Hz frequencies. The majority of cases are considered sporadic, although familial aggregation has been recognized in European and Korean populations, and the search for familial MD genes has been elusive until the last few years. Detailed phenotyping and cluster analyses have found several clinical predictors for different subgroups of patients, which may indicate different mechanisms, including genetic and immune factors. The genes associated with familial MD areCOCH,FAM136A,DTNA,PRKCB,SEMA3D, andDPT. At least two mechanisms have been involved in MD: (a) a pro-inflammatory immune response mediated by interleukin-1 beta (IL-1β), tumor necrosis factor alpha (TNFα), and IL-6, and (b) a nuclear factor-kappa B (NF-κB)-mediated inflammation in the carriers of the single-nucleotide variant rs4947296. It is conceivable that microbial antigens trigger inflammation with release of pro-inflammatory cytokines at different sites within the cochlea, such as the endolymphatic sac, the stria vascularis, or the spiral ligament, leading to fluid imbalance with an accumulation of endolymph. Computational integration of clinical and “omics” data eventually should transform the management of MD from “one pill fits all” to precise patient stratification and a personalized approach. This article lays out a proposal for an algorithm for the genetic diagnosis of MD. This approach will facilitate the identification of new molecular targets for individualized treatment, including immunosuppressant and gene therapy, in the near future.

After forced prolonged position type one (FPP-one) and the appropriate repositioning maneuvers, or FPP-two, 95.45% of patients with cupulolithiasis of the horizontal semicircular canal benign paroxysmal positional vertigo (HSC-BPPV).were symptom-free.To treat patients with cupulolithiasis of the HSC-BPPV.This was a prospective study including 22 subjects with HSC-BPPV (cupulolithiasis) based on apogeotropic direction-changing positional nystagmus (apo-DCPN). Patients adopted FPP-one, which means lying down on the side of the weaker nystagmus during nightly rest for 2 weeks. If apo-DCPN persisted, subjects adopted type two FPP (FPP-two), which means lying on the strongest nystagmus side during nightly rest for 2 weeks.No vertigo or nystagmus was observed in 15 subjects after FPP-one. One subject experienced geotropic DCPN (geo-DCPN), two subjects had posterior BPPV, and another had superior BPPV. Three subjects had persistent apo-DCPN and they were subjected to FPP-two. After that, no vertigo or nystagmus was detected in two subjects. Apo-DCPN persisted in the other remaining subject after FPP-two.

Introduccion y objetivos Los autores describen y tratan de extender el conocimiento de una frecuente afeccion a la que denominan inseguridad fobica ortostatica que, en su unidad de otorrinolaringologia-neurootologia, resulta ser la segunda causa (22,3 %) de consultas por vertigos reales o aparentes, asi como de establecer analogias y diferencias de dicho trastorno con otras afecciones similares descritas en psiquiatria (agorafobia, vertigo somatomorfo y vertigo espacial) y en neurologia (vertigo postural fobico). Proponen para su diagnostico un sencillo metodo, y exponen su tratamiento y resultados. Pacientes y metodo Se analiza a 151 pacientes con sintomas indefinidos de “mareo”, “vertigo” o “inseguridad”, seleccionados entre 1999 y 2005, a los que, ademas de una anamnesis y una exploracion neurootologica adecuadas, realizaron un interrogatorio estandarizado especifico, un test de Beck (de referencia psiquiatrica) y un tratamiento farmacologico ansiolitico-antidepresivo. Resultados Encontraron, entre otros, 3 sintomas y 1 condicion exploratoria que se cumplen en todos los enfermos y constituyen los 4 pilares del diagnostico positivo. Este se convierte en seguro cuando el tratamiento consigue una remision total (en el 69,53 % de todos los pacientes) o subtotal (en el 24,49 %), segun su escala de valoracion de la inseguridad para todas las situaciones. Conclusiones El estudio estadistico permite extraer una concordancia sintomatica dentro del grupo analizado, una equivalencia sindromica entre los enfermos y unos resultados satisfactorios del tratamiento antidepresivo (en el 94 %), lo que permite verificar las hipotesis etiopatogenica y diagnostica sospechadas ante el trastorno y obtener, consecuentemente, un metodo logico de diagnostico y tratamiento. Los autores proponen asimilar a este protocolo diagnostico, incluso terapeutico —cuando no se disponga de equipos psicoterapeuticos especializados—, la mayor parte de los sindromes psicogenos de inseguridad descritos.

Background and objectives The authors attempt to expand knowledge about a subjective balance disorder they have called Phobic Orthostatic Insecurity, a condition representing the second cause of medical visits (22.3%) to their ENT & Neuro-otology clinic, and attempt to identify relationships with similar conditions described in psychiatry (agoraphobia, somatoform vertigo, and space-phobia) and in neurology (phobic postural vertigo). They also propose a simple diagnostic method and present their therapies and results. Patients and method A total of 151 patients with an indefinite symptomatology of “dizziness,” “vertigo,” or “insecurity” were evaluated (from 1999 to 2005) by means of a full medical history and an appropriate neurological examination, pharmacological treatments with anxiolyticsantidepressives, a measurement of the degree of depression with the Beck test (a kind of psychiatric benchmark), and with a specific standardized test. Results Three symptoms and 1 exploratory condition, among others, were found in all 151 patients studied; these constitute the 4 bases for a positive diagnosis. This is confirmed if the treatment achieves total remission (this occurred in 69.53% of all patients) or a sub-total remission (24.49%), according to valuation scale for insecurity in all situations. Conclusions The statistical analysis showed a symptomatic concordance within the group analyzed, a syndromic equivalence between patients and satisfactory results with the antidepressive treatments (94%), thus confirming the diagnostic and aetiopathogenic hypotheses for the disorder and, later, providing a logical method for diagnosis. The authors propose to assimilate this diagnostic protocol (and therapeutic when no specialist psychotherapy teams are available) to most of the psychogenic insecurity syndromes described.

The application described in this study appears to be accurate and valid, thus allowing calculation of a hearing handicap and assessment of the pure-tone air conduction threshold with iPhone/iPad devices.To develop and evaluate a newly developed professional, computer-based hearing handicap calculator and a manual hearing sensitivity assessment test for the iPhone and iPad (AudCal).Multi-center prospective non-randomized validation study. One hundred and ten consecutive adult participants underwent two hearing evaluations, a standard audiometry and a pure-tone air conduction test using AudCal with an iOS device. The hearing handicap calculation accuracy was evaluated comparing AudCal vs a web-based calculator.Hearing loss was found in 83 and 84 out of 220 standard audiometries and AudCal hearing tests (Cohen's Kappa = 0.89). The mean difference between AudCal and standard audiogram thresholds was -0.21 ± 6.38 dB HL. Excellent reliability and concordance between standard audiometry and the application's hearing loss assessment test were obtained (Cronbach's alpha = 0.96; intra-class correlation coefficient = 0.93). AudCal vs a web-based calculator were perfectly correlated (Pearson's r = 1).

[EN] The developed software (HITCal) may be a useful tool in the analysis and measurement of the saccadic video head impulse test (vHIT) responses and with the experience obtained during its use the authors suggest that HITCal is an excellent method for enhanced exploration of vHIT outputs. Objective: To develop a (software) method to analyze and explore the vHIT responses, mainly saccades. Methods: HITCal was written using a computational development program; the function to access a vHIT file was programmed; extended head impulse exploration and measurement tools were created and an automated saccade analysis was developed using an experimental algorithm. For pre-release HITCal laboratory tests, a database of head impulse tests (HITs) was created with the data collected retrospectively in three reference centers. This HITs database was evaluated by humans and was also computed with HITCal. Results: The authors have successfully built HITCal and it has been released as open source software; the developed software was fully operative and all the proposed characteristics were incorporated in the released version. The automated saccades algorithm implemented in HITCal has good concordance with the assessment by human observers (Cohen’s kappa coefficient = 0.7).

Functional evaluation of the vestibulo-ocular reflex (VOR) is one of the most important issues in the examination of the dizzy patient. The video Head Impulse Test (vHIT) has become an essential tool at the bedside and in the laboratory examination of the VOR. Most of vHITs devices only outputs the results of the head and eye movements based on velocity data and graphs, we consider that the position and acceleration data and graphs could have some clinical interest. With this interest we developed a standardized computer-based method to calculate position and acceleration graphs with the velocity data obtained from the vHIT. With the help of a specific computational development program a Graphical User Interface (GUI) was designed. Position and acceleration graphs were done using the classical mechanics methods. The GUI was tested measuring the head and eye displacements in normal and altered vHIT responses. The source files of the developed GUI are available on web: http://mlibra. com. The developed tool can be a useful tool to investigate some vHIT responses.

Resumen Objetivo: El linfoma no Hodgkin (LNH) es una tumoracion frecuente que afecta con asiduidad al area ORL unque no existen muchos trabajos con series grandes sobre el tema. Pacientes y metodologia: Hemos estudiado 300 pacientes con LNH y hemos analizado su afectacion cervicofacial, del anillo de Waldeyer, sus sintomas de aparicion y el estadio de la enfermedad. Resultados: Mas del 50% tenian afectacion cervico-facial. De ellos, la manifestacion clinica mas frecuente era la presencia de adenopatias. Dentro de la afectacion del anillo de Waldeyer, lo mas frecuente es la afectacion de la amigdala palatina. La mayoria de los paciente se encontraba en un estadio IV de su enfermedad. Conclusiones: Existe una relacion clara entre el LNH y el area ORL por lo que es necesario tenerlo en cuenta en la aparicion de adenopatias cervico-faciales.

Our last cases of cochlear implants in obliterated or ossified cochleas are presented, and we had described the surgical technique for double-array or compressed system. In our opinion the best surgical method is the com- bined approach (endoaural with aticotomy view and poste- rior timpanotomy) for increased promontory view. This technique provides the advantage that it always preserve the posterior wall of the external ear canal, and the array is positioned easily.

Objective To evaluate vestibular function before and after cochlear implantation (CI) Study Design A prospective descriptive study. Material and Methods: Thirty consecutive patients with profound sensorineural hearing loss undergoing CI. Objective assessment of vestibular function was performed with the caloric test and video head impulse test (vHIT) in patients before and after CI. Dizziness Handicap Inventory (DHI) was used for subjective assessment before and after CI. Results Thirty patients received CI with 21 by round window approach and nine by anteroinferior cochleostomy. Vestibular results were categorized into four groups: no changes (20 patients), changes in the caloric test and vHIT (3 patients, all with DHI changes; P = 0.0001), changes in vHIT gain but not in caloric test (3 patients, all with DHI changes; P = 0.005), no changes in the caloric test and vHIT gain but only saccades appear (4 patients, all with DHI changes; P = 0.011). Conclusions Although CI is a safe surgery with few major complications, it is a procedure that can produce dizziness. The vHIT reveals that 30% of patients demonstrate postoperative change in vestibular function. Therefore, when examining a CI patient with postoperative symptoms of dizziness, results of the vHIT test and gain as well as the presence of saccades, along with an increase DHI score, are parameters to consider in their evaluation. Level of Evidence 4. Laryngoscope, 125:2371–2375, 2015

Nina de 6 anos diagnosticada al nacimiento de sindrome de CHARGE con ausencia completa y bilateral de conductos semicirculares (fig. 1). La paciente presenta una buena deambulacion con ampliacion de la base de sustentacion. Se le realizo un Video Head Impulse Test (vHIT) donde se objetivo un deficit vestibular bilateral severo con un reflejo vestibulo-oculomotor completamente anomalo (fig. 2). En el sindrome de CHARGE las malformaciones mas frecuentes son las de oido1 y, dentro de ellas, la ausencia de canales semicirculares laterales, aunque tambien puede aparecer una displasia de todos ellos2. Estas malformaciones son faciles de detectar mediante tomografia computarizada, y clinicamente se traducen en que los ninos presentan una alteracion del equilibrio, que se manifiesta como torpeza y amplia base de sustentacion con el consiguiente retraso en el desarrollo psicomotor3. El vHIT es un metodo diagnostico rapido y no invasivo para evaluar la efectividad del reflejo vestibulo-oculomotor en pacientes con sintomatologia vestibular y que permite su seguimiento. Es muy importante instaurar programas de rehabilitacion precoces, que deben tener en cuenta el patron especifico del desarrollo motor inducido por el deficit vestibular y estimular precozmente la vision y propiocepcion que ayudan a compensar los defectos vestibulares. Si los pacientes tienen

The third branquial arch anomalies represent less than 1% of this deformites. The clinical manifestation can be varied althouth the formation of cysts is the commonest one and may appear at any time in life. We report the clinical case of a 7 year old boy with an neck mass. This mass was diagnosed as a branquial cyst. The patologist found a "timic reminescences". We carry out a review of the literature for this pathology.

Frey syndrome has been observed especially in patients who have undergone a parotidectomy operation, but also in zoster herpes, in parotiditis, condilea fractures, obstetric traumatisms with forceps and in surgery of the meningioma of the cerebellopontine angle. It also appears without previous surgery, like in our case. In these circumstances it is believed that a clinical neuritis, primary or secondary to a neighbouring inflammation may cause the start of this disorder. Several treatments have been suggested which highlights the difficulty of them. The most effective one is the intradermic injection of botulinum toxin type A. Its use in Frey's syndrome was initiated by Drobik and Laskawi in 1995. Since then the references to its use are numerous. Nevertheless, it is a treatment which has been introduced very few times in our country.

The degree of caloric weakness before surgery influences faster or slower recovery of patients undergoing vestibular schwannoma surgery. The Dizziness Handicap Inventory (DHI) is a good index to show the recovery of patients as it relates directly to an improvement or not of the subjective visual vertical (SVV).To evaluate the process of recovery of patients as measured by the SVV and the DHI after surgical removal of vestibular schwannoma.We studied 24 consecutive patients of the University Hospital of Salamanca who underwent vestibular schwannoma surgery. We assessed age, tumour size, degree of canalicular weakness and preoperative SVV, and their relationship with DHI and SVV at discharge and also at 1, 3 and 6 months postoperatively.Patients with lesser degrees of caloric weakness took longer to normalize SVV than those with a higher caloric weakness before surgery (p0.05). There was a significant correlation between DHI and improvements in SVV with time. The differences disappeared in 6 months where all patients, with greater or lesser degree of caloric weakness, had the same results.

Acute vestibular deficit as the first sign of leukemia is extremely rare. The literature shows some cases of sudden hearing loss accompanied by instability and associated with hyperviscosity syndrome. We present the case of a patient who presents a harmonic vestibular deficit of the right ear. The complementary studies revealed an abnormally high level of leukocytes. A peripheral blood cytogenetic analysis is performed due to a high suspicion of leukemia, and the results show BCR/ABL fusion gene with a cut point in the M-BCR region, which confirms the diagnosis of chronic myeloid leukemia. In this case we detail the importance of taking hematological disorders into consideration in the differential diagnosis of patients with otoneurological symptoms, and we also review the etiopathogenic mechanisms, symptoms, diagnosis, and therapeutic options for chronic myeloid leukemia with sudden hearing loss and vertigo.

Introduction Benign Paroxysmal Vertigo of Childhood (BPVC) is a common cause of vertigo in children and it is characterized by recurrent attacks of vertigo without warning resolving spontaneously after minutes to hours. It has been considered the equivalent of migraine in childhood. Methods Twenty-seven patients diagnosed with BPVC were recruited between 1991 and 1997 with a follow up of at least 15 years. The incidence of migraine, inner ear disorders and family medical history is analyzed. Results The average age for the onset of the attacks of BPVC was 3 years and 11 months, and for spontaneous resolution, it was around 5 years and 7 months. The average age for follow up was 18 years. Nine patients developed migraine during adulthood. Nineteen of them had a family history of migraine. Conclusion We have observed that the prevalence of migraine in patients that had been diagnosed with BPVC is higher than in the general population, which leads us to propose BPVC as a precursor of migraine during childhood.

Evaluation of the audiological outcome and subjective satisfaction of BAHA Cordelle II in the treatment of patients with severe to profound bilateral mixed hearing loss.Retrospective study of 12 patients suffering a severe to profound bilateral sensorineural hearing loss, using pure tone audiometry (PTA), speech audiometry and subjective evaluation before and after the implantation of a BAHA Cordelle II (Cochlear(®)).The average gain in conversational frequencies (0.5 to 4kHz) with BAHA in free field was 43, 51, 47 and 44dB, respectively. We observed a GAP over closure in 10 of the 12 patients. Speech audiometry improved from 85% at 83dB of maximum discrimination to 96% at 62dB. The subjective evaluation questionnaires showed great satisfaction with a slight decrease in noisy or windy environments. The great majority of our patients used the BAHA device throughout the entire day.The BAHA Cordelle II (Cochlear(®)) is a good option in the treatment of severe to profound bilateral mixed hearing loss. Its best advantages are a low risk of labyrinthization, high result predictability, easy and step-by-step surgery, no need for general anaesthesia, and the GAP over closure in all frequencies. Active middle ear devices represent another alternative, but specific indications have not been defined yet because of low universal experience. When the intelligibility of the patient is poor, cochlear implantation should be considered.