Healing criteria: How should an episode of benign paroxistic positional vertigo of posterior semicircular canal's resolution be defined? Prospective observational study.

OBJECTIVES: To compare the outcome of the Epley maneuver (EM) in benign paroxysmal positional vertigo of the posterior canal (CSP-BPPV) depending on the definition used for recovery. DESIGN: Multicenter observational prospective study. SETTING: Otoneurology Units of 5 tertiary reference hospitals. PARTICIPANTS: All patients presenting with unilateral CSP-BPPV assisted for 1-year period. EXCLUSION CRITERIA: Spontaneous nystagmus, positive McClure-Pagnini maneuver, positive bilateral Dix-Hallpike maneuver (DHM), positive DHM for vertigo but negative for nystagmus and atypical nystagmus. MAIN OUTCOME MEASURES: Response to EM was measured after 7 days in 3 different outcomes: disappearance of nystagmus during the DHM in the follow-up visit, disappearance of vertigo during the DHM and general status (GS) during daily life activities. RESULTS: 264 patients were recruited (68 male/166 female, mean age 62 years). After the EM, nystagmus disappeared in 67% of them, vertigo in 54% and 36% were asymptomatic in their daily life. These outcomes were strongly correlated, but they were not concordant in a clinically significant group of cases; only the 26% of patients met all of them. The healing process follows the next sequence: negativization of positional nystagmus, then disappearance of positional vertigo and, finally, the improvement of GS during daily life activities. CONCLUSION: Nowadays, healing criteria for the resolution of an PSC-BPPV episode have not been specifically defined yet. Provided that other otoneurological disorders have been ruled out, the next resolution criterion is proposed: absence of nystagmus and specifically during control DHM and disappearance of symptoms during daily life activities.

Analysis of risk factors influencing the outcome of the Epley maneuver.

Benign paroxysmal positional vertigo (BPPV) is the most frequent type of vertigo. The treatment of canalithiasis of the posterior semicircular canal consists in performing a particle-repositioning maneuver, such as the Epley maneuver (EM). However, the EM is not effective in all cases. The objective of this study is to identify risk factors, which predict the EM failure, among the clinical variables recorded in anamnesis and patient examination. This is an observational prospective multicentric study. All patients presenting with BPPV were recruited and applied the EM and appointed for a follow-up visit 7 days later. The following variables were recorded: sex, age, arterial hypertension, diabetes, hyperlipidemia, smoking habit, alcohol consumption, migraine, osteoporosis, diseases of the inner ear, previous ipsilateral BPPV, previous traumatic brain injury, previous sudden head deceleration, time of evolution, sulpiride or betahistine treatment, experienced symptoms, outcome of the Halmagyi maneuver, laterality, cephalic hyperextension of the neck, intensity of nystagmus, intensity of vertigo, duration of nystagmus, occurrence of orthotropic nystagmus, symptoms immediately after the EM, postural restrictions, and symptoms 7 days after the EM. Significant differences in the rate of loss of nystagmus were found for six variables: hyperlipidemia, previous ipsilateral BPPV, intensity of nystagmus, duration of nystagmus, post-maneuver sweating, and subjective status. The most useful significant variables in the clinical practice to predict the success of the EM are previous BPPV and intensity of nystagmus. In the other significant variables, no physiopathological hypothesis can be formulated or differences between groups are too small.

Probable benign paroxysmal positional vertigo, spontaneously resolved: Incidence in medical practice, patients' characteristics and the natural course.

BACKGROUND: Probable benign paroxysmal positional vertigo, spontaneously resolved (pBPPVsr), is a variant of benign paroxysmal positional vertigo (BPPV) in which there is no observable nystagmus and no vertigo with any positional maneuver. OBJECTIVES: To calculate the incidence pBPPVsr, compare the characteristics of the patients with pBPPVsr and BPPV not spontaneously resolved and describe the spontaneous resolution in the natural course of BPPV. METHODS: Multicenter prospective descriptive study. During a one-year period, all patients with suspected BPPV that presented to the Neurotology Units of five participating centers were recruited. The incidence of pBPPVsr was calculated as a percentage of the total number of patients with BPPV. The prevalence of several variables was compared between pBPPVsr and BPPV not spontaneously resolved. The timing of spontaneous resolution was estimated using Kaplan-Meier curves. RESULTS: 457 patients met the inclusion criteria. The incidence of pBPPVsr was 33.5%. It was significantly higher in males, in patients with normal bone mass and in patients who were not taking sulpiride. A rate of 18% of spontaneous resolution after the first month and 51% after the first year was found. This percentage did not change in a significant way after this moment. The curves for males, patients under 50 and patients with normal blood pressure decreased significantly faster. CONCLUSIONS: In our serie, BPPV spontaneously resolved in half of the patients with BPPV during the first year. This seemed to occur more commonly in males and could have been hindered by sulpiride intake, osteoporosis, advanced age and high blood pressure.

Stickler and branchio-oto-renal syndromes in a patient with mutations in EYA1 and COL2A1 genes.

Branchio-oto-renal (BOR) and Stickler (STL) syndromes are disorders that include hearing loss among their clinical features. STL syndrome type I (STL1) is a combination of ophthalmic, orofacial, articular, and auditory manifestations, caused by mutations in the COL2A1. BOR syndrome is an autosomal dominant trait encompassing branchial, otic and renal anomalies because of mutations in EYA1, SIX1 and SIX5. In this study, we have clinically and genetically diagnosed a proband that displayed STL1 and BOR syndromes. This patient and his younger brother exhibited hearing loss and cleft palate. Both siblings and their mother also showed myopia, congenital non-progressive vitreous anomaly and a flat face. Taken together, these clinical features are consistent with the diagnosis of a familial case of STL. Sequence analysis revealed in the three patients a novel COL2A1 mutation (c.1468_1475delinsT) that accounted for a STL1 phenotype. The proband also displayed pre-auricular pits, branchial fistulae and renal agenesis that define BOR syndrome. Interestingly, this patient carries an EYA1 mutation, p.R328X, which was not present in the two other patients or in his healthy father, supporting that the mutation arose de novo. In conclusion, this report highlights the importance of molecular testing and detailed clinical evaluation for the diagnosis of syndromes with overlapping phenotypic features.

[Open rhinolalia, a typical symptom of velo-cardio-facial syndrome]. / Rinolalia abierta, un síntoma característico del síndrome velocardiofacial.

The Velocardiofacial (VCF) syndrome is a relatively frequent cromosomopathy that usually associates various otorhinolaryngological features, as hipenasal speech, typical facies and auricular anomalies. We report a patient with VCF syndrome that before being diagnosed had undergone adenoidectomy with a postoperative worsening in speech. Otorhinolaryngological clinical features of the VCF syndrome are discussed and a diagnostic protocol is proposed to achieve an early diagnosis and to prevent iatrogenic interventions in these patients.

[Otorhinolaryngo logical manifestations in patients with Down syndrome]. / Manifestaciones otorrinolaringológicas en el síndrome de Down.

OBJECTIVE: The objective [corrected] of our study was to assess the most frequent otorhinolaryngological manifestations in patients with Down syndrome, and to propose diagnostic and management guidelines to improve their quality of life. METHODS: Patients with Down's syndrome referred to the ENT Department of two Spanish Hospitals during a 4-year period were retrospectively reviewed. Data of the following variables were collected: main symptoms, diagnosis, comorbidities, surgical procedures, and complications. RESULTS: Thirty patients with Down's syndrome were included in our study. The most frequent reasons for referral were hearing loss and newborns from the Hearing Impairment Screening Program. Otitis media with effusion, adenoid hypertrophy and obstructive sleep apnea were the most common diagnosis. Five patients underwent head and neck surgical procedures without complications. CONCLUSIONS: Hearing loss secondary to chronic otitis media with effusion and upper airway obstruction are frequent pathologies in patients with Down syndrome.

[Auditory neuropathy due to the Q829X mutation in the gene encoding otoferlin (OTOF) in an infant screened for newborn hearing impairment]. / Neuropatia auditiva secundaria a la mutación Q829X en el gen de la otoferlina (OTOF) en un lactante sometido a screening neonatal de hipoacusia.

We report an infant with auditory neuropathy secondary to the Q829X mutation in the gene encoding otoferlin (OTOF). Included in a universal newborn hearing screening program, the subject passed the otoacoustic emission (OAEs) test. Given that the infant had a familial history of deafness auditory brainstem response (ABR) testing was performed, revealing a profound hearing impairment. The genetic study confirmed that the subject was homozygous for the Q829X mutation in OTOF. The patient underwent a cochlear implant, obtaining satisfactory results. The moderately high prevalence of this mutation in the Spanish population could produce a significant false negative rate in newborn hearing screening programs using OAEs.

[Sensorineural hearing loss in cerebral palsy patients]. / Hipoacusia neurosensorial en pacientes con parálisis cerebral.

INTRODUCTION: Cerebral palsy (CP) is the most common chronic motor disorder in children and frequently associates sensorial pathology. The objective of our study was to establish the prevalence and characteristics of sensorineural hearing loss in children with CP. METHODS: We performed a retrospective study of patients born between the years 1975 and 2004, diagnosed of CP in the "Marqués de Valdecilla" University Hospital. Clinical data were collected including the presence of sensorineural hearing loss, age at diagnosis, treatment and associated pathology. RESULTS: Sixty four patients had confirmed CP. Audiological testing had been performed in thirty patients (47%) of them 18 (60%) had sensorineural hearing loss (12 bilateral and 6 unilateral). In thirteen cases hearing loss was associated with mental retardation. The age at diagnosis ranged from 3 months to 7 years (mean 23.2 months). Eight patients were treated with hearing aids and one with a cochlear implant. CONCLUSIONS: Sensorineural hearing loss is frequent in CP patients. Management of this problem is difficult in this setting because of the motor disorder and the associated pathology. Early audiological assessment is very important to improve the language outcome in these children.

[Universal newborn hearing screening in Cantabria (Spain): results of the first two years]. / Cribado universal de la hipoacusia congénita en Cantabria: resultados de los dos primeros años.

AIMS: We present the results of the first 2 years of universal newborn hearing screening in Cantabria. MATERIAL AND METHODS: We performed a descriptive study of screening with two levels of transient evoked otoacoustic emissions in 8,836 newborns, diagnostic confirmation with auditory brainstem response, and treatment. RESULTS: The coverage of the first two levels of otoacoustic emissions was 98.4 % and 99.5 %. The incidence of risk factors was 3.08 %. A total of 6.7 % of those studied in the first stage were referred to the second, and 0.7 % of those studied in the second stage were referred to testing of auditory brainstem responses. Of the patents referred to the second stage, 97.6 % attended, and of those referred to the third stage 87.1 % attended. The positive predictive value after the second session of otoemissions was 7.9 %, and the false positive rate was 3.3 %. Sensorineural and bilateral hearing loss was diagnosed in 11 children, and permanent unilateral hypoacousia was diagnosed in one child, representing an incidence of 1.38/1,000 newborns. Sixty percent were diagnosed before the age of 3 months and 100 % before the age of 7 months. Fifty percent began treatment before the age of 6 months and 90 % before the age of 1 year. Of three cochlear implants indicated, two were implanted at 11 and 13 months. The cost was 1.3 3 per child screened and 867 3 for each case diagnosed. CONCLUSIONS: All the objectives of the first and second stages of screening were achieved. The continuity index anticipated for the third stage (87.1 vs 95 %) and access to treatment at 6 months (50 % vs 100 %) were less satisfactory, although these results compare favorably with those of previously published studies.

[Vestibular drop attacks or Tumarkin's otolithic crisis in patients with Meniere's disease]. / Crisis otolíticas de Tumarkin o drop attacks en pacientes con enfermedad de Meniere.

INTRODUCTION: Drop attacks (DA) are a sudden fall that comes without warning and without loss of consciousness, with no associated neurological symptoms and normal neurological examination. A certain number of patients with Meniere's disease, develop Tumarkin's otolithic crisis or DA. The purpose of this study is to document the frequency and clinical features of DA in patients with Meniere's disease. METHODS: A cohort of 40 patients with "definitive" Meniere's Disease were followed up between six months and 12 years. The presence and characteristics of Tumarkin's otolithic crisis were recorded. RESULTS: Thirteen (32.5%) patients developed DA during the outcome of their Meniere's disease. The interval between the onset of typical symptoms of Meniere's disease and the DA ranged from less than one year to 18 years. The number of DA varied from 1 to 14. The attacks typically occurred in a flurry during a period of 1 year or less. Six patients had only one DA. No patient requiered treatment for the DA. CONCLUSIONS: Tumarkin's otolithic crisis in Meniere's disease are not uncommon. They can occur at any time during the course of the disease, generally in a flurry of less than one year. Most patients have a spontaneus remission of the DA.

[Prevalence of the 35delG mutation in the GJB2 gene, del (GJB6-D13S1830) in the GJB6 gene, Q829X in the OTOF gene and A1555G in the mitochondrial 12S rRNA gene in subjects with non-syndromic sensorineural hearing impairment of congenital/childhood onset]. / Prevalencia de las mutaciones 35delG en el gen GJB2, del (GJB6-D13S1830) en el gen GJB6, Q829X en el gen OTOF y A1555G en el gen del ARNr 12S mitocondrial en sujetos con hipoacusia neurosensorial no sindrómica de inicio congénito o en la infancia.

INTRODUCTION: The most frequent mutations responsible for non-syndromic hearing impairment in the Spanish population are the 35delG mutation in the connexin 26 gene (GJB2), the del(GJB6-D13S1830) deletion in the connexin 30 gene (GJB6), the Q829X mutation in the otoferlin gene (OTOF), and the A1555G mutation in the 12S rRNA gene of the mitochondrial genome. PATIENTS AND METHODS: Screening for these mutations was performed on 38 patients from Cantabria with non-syndromic sensorineural hearing impairment of congenital/childhood onset. RESULTS: The A1555G mutation was detected in homoplasmy in 9 patients (23.7%). Three individuals were heterozygous for the 35delG mutation (7.9%). The heterozygous del(GJB6-D13S1830) deletion was present in one case (2.6%). One subject was homozygous for the Q829X mutation (2.6%). CONCLUSIONS: These four mutations are present in 36.8% of all cases of non-syndromic hearing impairment in our population.

Prognostic factors in supraglottic laryngeal carcinoma.

We carried out a retrospective study of patients with supraglottic carcinomas who were treated surgically at the Marques de Valdecilla Hospital (Santander, Spain) between 1978 and 1987 and who were followed up for at least 5 years. The Kaplan-Meier survival curves were calculated for 24 clinical, histologic, and morphometric parameters. Multivariate analysis was then performed by means of the Cox regression model. In the univariate analysis, survival was related to presence of capsule rupture of the involved lymph nodes (p = 0.00001), number of metastatic lymph nodes (p = 0.0002), postoperative TNM stage (p = 0.004), grade of cell differentiation (p = 0.001), presence of intratumoral necrosis (p = 0.01), and type of invasion (p = 0.04). The nuclear area did not have an influence on survival. Only the presence or absence of capsule rupture of the metastatic lymph nodes and the grade of cell differentiation were included in the final Cox model and proved to be parameters with independent prognostic significance.

Mucormycosis of the sphenoid sinus in an otherwise healthy patient. Case report and literature review.

Paranasal and rhinocerebral mucormycosis refers to uncommon opportunistic fungal infections, reported to occur especially in association with diabetic acidosis (the most common), immunosuppressive therapy, malignancy, or other chronic debilitating disorders. However, patients who have no underlying disease have occasionally been affected. According to the literature reviewed, only 13 well-documented cases without any predisposing factor have been previously reported. We describe a unique case of sphenoidal mucormycosis in an otherwise healthy individual, and the first patient to present with headache as the only symptom. We emphasize the importance of a high index of suspicion for early diagnosis and prompt management.

[Usefulness of the flexible fibroscope with working channel in the treatment of pedunculated polyps of the vocal cords]. / Utilidad del fibroscopio flexible con canal de trabajo en el tratamiento de pólipos pediculados de cuerda vocal.

INTRODUCTION: Suspension laryngoscopy by general anesthesia is the universally method of performing microlaryngeal surgery for the treatment of vocal cord polyps. Recently a new technique using a flexible endoscope with working channel has been introduced and popularized in Spain for treating these lesions. MATERIAL AND METHODS: A prospective study was undertaken to asses the efficacy of this technique, something modified, for removal either pedunculated vocal cord polyps or with minute implantation basis. RESULTS: Twenty-six consecutive patients with pedunculated cord polyps were surgically removed, at the ENT-Department of Sierrallana Hospital (Torrelavega, Cantabria) from January 1995 till September 1996. In 4 patients with strong gang reflexes the procedure could not be finished. Twenty-two patients improved their voice and were satisfied with the result. In 2 other cases, of the group, the voice regain was not complete, but owing to the feelings of the individuals no more surgical procedures were scheduled. No postoperative aftermath were observed. One year later all cases were checked and all proclaimed their well-being. CONCLUSIONS: We suggest that removal of pedunculated polyps of the vocal cord employing the fibroendoscope with working channel and local anesthetic should be the choix procedure for the lot of patients suffering this pathology, and the microsurgery with general anesthesia must be reserved for failure cases or for those patients using voice professionally.

[Head and neck abscesses]. / Abscesos de cabeza y cuello.

OBJECTIVE: To review the clinical, microbiological and therapeutic characteristics of all head and neck abscess seen in a Community Hospital. MATERIALS AND METHODS: Medical records of all patients admitted to Sierrallana Hospital (Torrelavega, Cantabria) from January 1995 till June 1998, because of head and neck abscess were reviewed. RESULTS: Fyfty-seven head and neck abscess of 54 patients were considered. Age of patients ranged from 14 to 78-year-old. Periamygdaline region was the most frequent location, where as tonsillar and dental infections were the origins of the abscess in the large majority of cases. Most of the abscess cultures yield mixed flora. Intravenous penicillin was used in 77.3 percent of patients and surgical drainage was required in almost 80 percent of cases. Treatment response was good with no serious complications in any case. CONCLUSIONS: Head and neck abscess are a relative common cause of hospital admissions. Among them periamygdaline abscess are frequently found. The treatment is surgical drainage with local or general anesthesia (depending of location) and simultaneous i.v. course of antibiotic (Penicillin G sodium salt) efficacious in the great number of patients. Serious complications are very rare.

[Hypoglossal nerve palsy and infectious mononucleosis]. / Parálisis del nervio hipogloso en la mononucleosis infecciosa.

Neurologic manifestations are infrequent in infectious mononucleosis and specially hypoglossal nerve involvement is very rare. To the best of our knowledge only 6 cases have been previously reported. We describe herein the case of a 20-years-old man who presented an unilateral hypoglossal nerve palsy secondary to infectious mononucleosis. He was treated i.v. steroids without recovery, but the palsy resolved spontaneously 2 months after the onset.

[Early diagnosis of nasopharyngeal carcinoma]. / Diagnóstico precoz del carcinoma de nasofaringe.

For making an early diagnosis of nasopharyngeal carcinoma it is fundamental to fulfill three requirements. First a high suspicion index based in the knowledge of symptoms and signs of the malady. Secondly it is necessary the routinely use in the consulting room of a nasopharyngoscope. An in third place is to make, in doubtful cases, an enlarged biopsy under endoscopic control and local anesthesia.

[Ambulatory septoplasty]. / Septoplastia ambulatoria.

The AA. admit that the septoplasty under general anaesthesia is a surgical procedure that can be realized ambulatory, without added risks to patient. With viewpoint they have done a retrospective survey of the whole group of septoplasties performed under general anaesthesia at Torrelavega Hospital (Santander) in two years, between April 1995 and August 1997. The total number of cases amounted 56 (49 men and 7 women), lasting the procedure, more or less, one hour; postanaesthesia recovery also about 86 minutes and the hospital permanence about six hours and a half. Readmitted were 14 cases, because vomiting (7), for laryngeal spasm (2), bleeding (1), retention of urine (1) and other because vertigo (1), fever (1) and headache (1).

[Major ambulatory surgery in otorhinolaryngology]. / Cirugía mayor ambulatoria en otorrinolaringología.

In the last years we have witnessed a great increase of major ambulatory surgery procedures in our specialty. Some of them are very common as adenoidectomies, tonsillectomies and also the insertion of transtympanic tubes. Nevertheless there is no agreement about which of the procedures should be realized through AMS. The aim of this work is to do a review on the most important features involved in AMS entlightening what kind of operations can be included i such programmes.

[Benign cervical mesenchymoma]. / Mesenquimoma benigno cervical.

Mesenchymoma are growths made up for two or more mesenchyme derivatives that normally not joint in the same tumor, with the exception of the connective tissue which appears in all tumors of mesenchymal lineage. Benign mesenchymoma is a rare tumor more frequent in extremities and peri-renal area or, very rarely in head and neck territories, being the oropharynx the most encountered sitting in the latter place. The paper reports a clinical case of benign cervical mesenchymoma of a long course, more than 20 years, and big size, asymptomatic and diagnosed through fine needle biopsy. After total removal the histological examination showed a composite growth of extended areas of fibrous tissue associated to vessels and other zones of ripe fat tissue.