[Immunomediation or hyperviscosity in rapidly progressive sensorineural hearing loss. A therapeutic approach]. / Inmunomediación o hiperviscosidad en la hipoacusia neurosensorial rápidamente progresiva. Una aproximación terapéutica.

INTRODUCTION: To evaluate the therapeutic response and the rheologic and immunological characteristics in subjects bearing of rapidly progressive sensorineural hearing loss (RPSHL). PATIENTS AND METHODS: A comparative prospective study on three groups of damaged ears treated with piracetam (n=29), corticosteroids (n=32) and plabcebo (n=25). Hearing loss and recovery level, whole blood filterability (WBF) and a Western-blot (WB) to anticochlear antibodies were evaluated. RESULTS: Recovery average was 20,8 +/- 55,9% in the first group, with progressive of deafness in the rest (p<0,01). WB did not show differences among groups. The higher WBF at the end of therapy was detected on group 1 (20,36 +/- 2,54 Vs 18,42 +/- 3,42 microl/seg on group 3; p<0,05). Moreover, it impaired only on those treated with piracetam, with statistically significative differences over the other two groups. CONCLUSIONS: We detected alterations on WBF in patients with RPSHL that justify the use of rheoactive measures as a pathophysiological therapy.

[Changes on blood filterability in patients with fluctuant hearing loss]. / Variaciones en la filtrabilidad sanguínea en pacientes con hipoacusia fluctuante.

We developed a two year follow-up in thirteen subjects bearing of fluctuant sensorineural hearing loss measuring their blood filterability (BF) and making a correlation to appearance of deafness episodes. We could observe a decrease on BF in all patients when they felt a hearing loss. When they recovered the hearing level, BF increased too. The use of reoactive techniques to avoid these decreases on BF in fluctuant deafness could potentially prevent or improve them.

Inmunomediación o hiperviscosidad en la hipoacusia neurosensorial rápidamente progresiva. Una aproximación terapéutica / Immunomediator or hiperviscosity in rapidly progressive sensorineural hearing loss. A therapeutic approach

Introducción: Valorar respuesta terapéutica y características reológicas e inmunológicas en sujetos con hipoacusia neurosensorial rápidamente progresiva (HNSRP). Pacientes y métodos: Estudio comparativo prospectivo sobre tres grupos de oídos dañados y tratados con piracetam (n=29), corticoides (n=32) y placebo (n=25). Se evaluó pérdida y recuperación auditiva, filtrabilidad en sangre total (FST) y un test de Western-blot (WB) para anticuerpos anticocleares. Resultados: El porcentaje de recuperación fue del 20,8±55,9% en el primer grupo, existiendo progresión de la hipoacusia en los otros dos (p<0,01). El WB no evidenció diferencias entre grupos. La mayor FST al final del tratamiento fue en el grupo 1 (20,36±2,54 Vs 18,42±3,42 μl/seg en grupo 3; p<0,05) y sólo mostró recuperación en tratados con piracetam, con diferencias estadísticamente significativas sobre los otros dos grupos. Conclusiones: En pacientes con HNSRP se detectan alteraciones en la FST que justifican el empleo de medidas reoactivas como tratamiento fisiopatológico

Introduction: To evaluate the therapeutic response and the rheologic and immunological characteristics in subjects bearing of rapidly progressive sensorineural hearing loss (RPSHL). Patients and methods: A comparative prospective study on three groups of damaged ears treated with piracetam (n=29), corticosteroids (n=32) and plabcebo (n=25). Hearing loss and recovery level, whole blood filterability (WBF) and a Western-blot (WB) to anticochlear antibodies were evaluated. Results: Recovery average was 20,8±55,9% in the first group, with progressive of deafness in the rest (p<0,01). WB did not show differences among groups. The higher WBF at the end of therapy was detected on group 1 (20,36±2,54 Vs 18,42±3,42 μl/seg on group 3; p<0,05). Moreover, it impaired only on those treated with piracetam, with statistically significative differences over the other two groups. Conclusions: We detected alterations on WBF in patients with RPSHL that justify the use of rheoactive measures as a pathophysiological therapy

Changes on blood filterability in patients with fluctuant hearing loss / Variaciones en la filtrabilidad sanguínea en pacientes con hipoacusia fluctuante

We developed a two year follow-up in thirteen subjects bearing of fluctuant sensorineural hearing loss measuring their blood filterability (BF) and making a correlation to appearance of deafness episodes. We could observe a decrease on BF in all patients when they felt a hearing loss. When they recovered the hearing level, BF increased too. The use of reoactive techniques to avoid these decreases on BF in fluctuant deafness could potentially prevent or improve them

En 13 sujetos afectos de hipoacusia neurosensorial fluctuante con Western-blot negativo se ha podido monitorizar durante dos años el valor de la filtrabilidad sanguínea (FS), correlacionándola con la aparición de episodios de hipoacusia. Pudo constatarse en todos los pacientes un descenso en la FS cuando observaban pérdida de audición. Con la recuperación auditiva el valor de la FS también se normalizaba. El empleo de procedimientos reoactivos que evitaran estos descensos en la FS en las hipoacusias fluctuantes podrían potencialmente prevenirlas o revertirlas

[Hearing level and intensive use of mobile phones]. / Nivel auditivo y uso intensivo de teléfonos móviles.

INTRODUCTION: Wide studies and substantial controversies build on utilization of actual mobile phones and appearance of systemic disorders or even tumours, but there is no knowledge about an eventual involvement on early hearing loss. PATIENTS AND METHODS: In a group of three hundred and twenty-three healthy and normoacoustic volunteers who were usual costumers of mobile phones an audiometric evaluation was made at the beginnig of its use and three years later, inquiring about the periods of time per day and year employed on direct contacts with phone. A healthy and normoacoustic control group of non users was studied too. RESULTS: Cases carried out 24.3 +/- 8.2 active contacts, reaching 50.4 +/- 27.8 days of mobile phone employment in three years. Audiometric curve was similar in cases and controls at the beginning of the study. After this follow-up, cases showed an increase on hearing threshold between 1 and 5 dB HL more than controls in speech tones (p<0.001). Moreover, there was a trend to correlate time of phone use to hearing impairment, but this finding did not result statistically significative. CONCLUSIONS: Frequent management of mobile phones in a middle period of time allows to detect a mild hearing loss, but the cause of this disorder keeps unclear.

Nivel auditivo y uso intensivo de teléfonos móviles / Hearing level and intensive use of mobile phones

Introducción: Existen amplios estudios e importantes controversias sobre el empleo de los actuales teléfonos móviles y eventuales afecciones sistémicas o aparición de tumores, pero no se conoce apenas sobre su posible implicación en la generación precoz de hipoacusia. Pacientes y métodos: En 323 voluntarios sanos y normoacúsicos usuarios habituales de telefonía móvil se efectuó evaluación audiométrica al inicio del empleo del aparato y a los tres años del mismo, así como una encuesta sobre el tiempo aproximado de uso diario y anual que efectuaban del sistema. Un grupo control sano y normoacúsico no usuario de telefonía móvil fue igualmente estudiado. Resultados: Los individuos estudiados efectuaron 24,3±8,2 contactos activos entre llamadas realizadas y recibidas, lo que supuso un total de 50,4±27,8 días de empleo del teléfono móvil en tres años. La gráfica audiométrica en casos y controles fue similar al inicio del estudio. En el seguimiento a tres años los casos mostraron una elevación del umbral de audición por vía aérea entre 1 y 5 dB HL más que los controles en las frecuencias conversacionales (p<0,001), aunque no se observó pérdida auditiva. Existió además una tendencia a correlacionar el tiempo de contacto directo con el teléfono y el deterioro en la audición, pero este hallazgo no resultó estadísticamente significativo. Conclusiones: El empleo de telefonía móvil entre usuarios frecuentes a medio plazo permite detectar ligeras pérdidas de audición no observadas en no usuarios, si bien el motivo último de este desorden auditivo no queda demostrado con los datos obtenidos

Introduction: Wide studies and substantial controversies build on utilization of actual mobile phones and appearance of systemic disorders or even tumours, but there is no knowledge about an eventual involvement on early hearing loss. Patients and methods: In a group of three hundred and twenty-three healthy and normoacoustic volunteers who were usual costumers of mobile phones an audiometric evaluation was made at the beginnig of its use and three years later, inquiring about the periods of time per day and year employed on direct contacts with phone. A healthy and normoacoustic control group of non users was studied too. Results: Cases carried out 24.3±8.2 active contacts, reaching 50.4±27.8 days of mobile phone employment in three years. Audiometric curve was similar in cases and controls at the beginning of the study. After this follow-up, cases showed an increase on hearing threshold between 1 and 5 dB HL more than controls in speech tones (p<0.001). Moreover, there was a trend to correlate time of phone use to hearing impairment, but this finding did not result statistically significative. Conclusions: Frequent management of mobile phones in a middle period of time allows to detect a mild hearing loss, but the cause of this disorder keeps unclear

[Otologic determining factors on infra-red tympanic thermometry in children]. / Condicionantes otológicos en termometría timpánica con infrarrojos en niños.

OBJECTIVE: To evaluate the influence of different otoscopic findings on body thermometry in children using an infra-red tympanic thermometer. PATIENTS AND METHODS: Body temperature was measured on healthy right and left ear canals with Thermoscan systems. Moreover, it was determined on children with altered otoscopy, companng to normal contralateral ear. To assess its diagnostic fiability, accuracy ratios were calculated in different temperatures. RESULTS: Temperature measured bilaterally did not offer differences, and intra/interassay variation ratios were always less than 0.34%. Acute otitis externa increased tympanic thermometry a mean of 0.36 degrees C (p < 0.001), and cerumen and previously radical surgery decreased a mean of 0.62 and 0.53 degrees C, respectively (p < 0.001). CONCLUSIONS: The conditions mentioned before modify body thermometry if measured by infra-red tympanic thermometer. So, this system must be avoided in these circumstances. Otitis media, fluid in middle ear, tympanic perforation and ventilation tubes did not modify thermometric results.

Condicionantes otológicos en termometría timpánica con infrarrojos en niños / Otologic determining factors on infra-red tympanic thermometry in children

Objetivo: Evaluar la influencia de diferentes hallazgos otoscópicos sobre la termometría corporal en niños con un sensor timpánico de infrarrojos. Pacientes y métodos: Con sistemas Thermoscan se comparó la temperatura corporal en oídos derecho e izquierdo sanos, así como en niños con otoscopias alteradas, comparando su valor con el oído contralateral normal. Su fiabilidad se estudió midiendo los índices de precisión y exactitud a diversas temperaturas. Resultados: La temperatura sobre oídos sanos no demostró diferencias derecha/izquierda, y los coeficientes de variación intra/interensayo fueron siempre inferiores a 0,34 por ciento. La termometría timpánica se elevó en la otitis externa aguda una media de 0,36ºC (p<0,001), y se redujo en la cera y el antecedente de cirugía radical 0,62 y 0,53ºC, respectivamente (p<0,001). Conclusiones: Estas condiciones modifican la temperatura medida por termometría timpánica con infrarrojos, no debiéndose emplear este sistema. La otitis media, la perforación timpánica y el drenaje transtimpánico no modificaron la termometría (AU)

OBJECTIVE: To evaluate the influence of different otoscopic findings on body thermometry in children using an infra-red tympanic thermometer. PATIENTS AND METHODS: Body temperature was measured on healthy right and left ear canals with Thermoscan systems. Moreover, it was determined on children with altered otoscopy, companng to normal contralateral ear. To assess its diagnostic fiability, accuracy ratios were calculated in different temperatures. RESULTS: Temperature measured bilaterally did not offer differences, and intra/interassay variation ratios were always less than 0.34%. Acute otitis externa increased tympanic thermometry a mean of 0.36 degrees C (p < 0.001), and cerumen and previously radical surgery decreased a mean of 0.62 and 0.53 degrees C, respectively (p < 0.001). CONCLUSIONS: The conditions mentioned before modify body thermometry if measured by infra-red tympanic thermometer. So, this system must be avoided in these circumstances. Otitis media, fluid in middle ear, tympanic perforation and ventilation tubes did not modify thermometric results (AU)

[Presentation of 2 cases of sudden deafness in patients with sickle-cell anemia and trait]. / Presentación de dos casos de sordera súbita en pacientes afectos de anemia y rasgo drepanocíticos.

We report two cases of sudden deafness which appeared during vaso-occlusive crisis of a sickle cell anaemia (homozygous form of disease) and a sickle cell trait (heterozygous form) in two males aged sixteen and twenty-two, respectively. A high erythrocyte aggregability and low deformability were proved in both young men. Although hearing disorders have been reported in this disease, a sudden onset is not common. The treatment used was useful in the removal of painful crisis and infections, but deafness did not improve in either of then.

[Atypical Cogan syndrome. Clinical and laboratory spectrum. Report of 2 cases]. / Síndrome atípico de Cogan. Espectro clínico y de laboratorio. Presentación de dos casos.

We show two atypical Cogan's syndrome cases in forty-seven and fifty-six years old individuals. Characteristical symptoms of the syndrome were detected in both (interstitial keratitis, sensorineural deafness and vestibular dysfunction), but they could be considered as unusual because of the association of systematic symptoms and an onset with hearing loss. In both cases there was an increase in erythrosedimentation rate and immunoglobulins levels, as well as high titles for rheumatoid factor and non-specific autoantibodies. By means of a Western-blot technique, several chochlear antibodies with distinct molecular weight were detected, but the 68 KD appeared in both. Metil-prednisolone treatment did not avoid ocular and auditive reactivation in ne of the patients.

Presentación de dos casos de sordera súbita en pacientes afectos de anemia y rasgo drepanocíticos / Report of two cases os sudden deafness in patients bearing of sickle cell anaemia and trait

Presentamos los dos casos de sordera súbita acontecidos durante el desarrollo de sendas crisis vasooclusivas en el contexto de una anemia de células falciformes (forma homocigota de la drepanocitosis) y de un rasgo drepanocítico (forma heterocigota) en dos varones de 16 y 22 años de edad, respectivamente. En ambos casos se constató el aumento en la agregabilidad de los eritrocitos y la disminución en su deformabilidad. Aunque está reconocida la afectación auditiva en esta enfermedad, la rapidez de instauración es rara. El tratamiento empleado alivió las crisis dolorosas y erradicó las infecciones, pero la hipoacusia resultó irreversible (AU)

We report two cases of sudden deafness which appeared during vaso-occlusive crisis of a sickle cell anaemia (homozygous form of disease) and a sickle cell trait (heterozygous form) in two males aged sixteen and twenty-two, respectively. A high erythrocyte aggregability and low deformability were proved in both young men. Although hearing disorders have been reported in this disease, a sudden onset is not common. The treatment used was useful in the removal of painful crisis and infections, but deafness did not improve in either of then (AU)

Síndrome atípico de Cogan. Espectro clínico y de laboratorio. Presentación de dos casos / Atypical Cogan's syndrome. Clinical features and laboratory findings. Report of two cases

Exponemos dos casos de presentación atípica de síndrome de Cogan en sendos individuos de 47 y 56 años de edad. En ambos se detecta la sintomatología característica del síndrome (queratitis intersticial, hipoacusia neurosensorial y vértigo), pero la asociación de trastornos generales y el inicio del cuadro por su deterioro audiovestibular los hacen infrecuentes. Los dos casos evidenciaron elevaciones en la VSG y niveles de inmunoglobulinas, y títulos positivos de factor de reumatoide y autoanticuerpos inespecíficos. Mediante un test de Western-blot pudieron determinarse anticuerpos contra antígenos clocleares de distinto peso molecular, siendo común el de 68 KD.El tratamiento de metil-prednisolona no evitó nuevas recidivas oftálmicas y auditivas en uno de los dos pacientes. (AU)

We show two atypical Cogan's syndrome cases in forty-seven and fifty-six years old individuals. Characteristical symptoms of the syndrome were detected in both (interstitial keratitis, sensorineural deafness and vestibular dysfunction), but they could be considered as unusual because of the association of systematic symptoms and an onset with hearing loss. In both cases there was an increase in erythrosedimentation rate and immunoglobulins levels, as well as high titles for rheumatoid factor and non-specific autoantibodies. By means of a Western-blot technique, several chochlear antibodies with distinct molecular weight were detected, but the 68 KD appeared in both. Metil-prednisolone treatment did not avoid ocular and auditive reactivation in one of the patients (AU)

Un caso pediátrico de trastorno autoinmune del oído interno (síndrome de Cogan) / Inner ear autoimmune disorder (Cogan's syndrome) in a pediatric patient

Se presenta un caso de síndrome de Cogan (queratitis intersticial no sifilítica, hipoacusia neurosensorial de rápida evolución y vértigo) en una niña de 12 años. La sospecha diagnóstica se fundamentó en la exploración clínica y audiológica y en los hallazgos inespecíficos de laboratorio (velocidad de sedimentación globular [VSG] elevada, y títulos positivos de inmunoglobulinas A y M [IgA e IgM] de factor reumatoide, así como anticuerpos anticitoplasma de neutrófilo [ANCA]). La detección de autoanticuerpos específicos frente a la fracción antigénica coclear de 68 kDa mediante Western-blot permitió confirmar la autoinmunidad organospecífica del trastorno laberíntico. El tratamiento con deflazacort en dosis de 1 mg/kg/día durante 3 semanas y la reducción progresiva revirtió la sintomatología y normalizó la exploración. Hasta la fecha el cuadro no ha presentado reagudizaciones (AU)

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[Inner ear autoimmune disorder. Cogan's syndrome]. / Un caso pediátrico de trastorno autoinmune del oído interno (síndrome de Cogan).

We present a case of Cogan's syndrome (interstitial keratitis, rapidly evolving sensorineural hearing loss and vestibular dysfunction) in a 12-year-old girl. Diagnosis was made by clinical and audiologic examination as well as by nonspecific laboratory findings (increased erythrosedimentation rate and positive IgA and IgM rheumatoid factor titers and antineutrophil cytoplasmic autoantibodies). Detection of specific autoantibodies against cochlear antigenic epitopes with a molecular weight of 68 kDa by Western-blot assay confirmed an organ-specific autoimmune mechanism. Therapy with deflazacort 1 mg/kg per day 3 three weeks decreasing progressively thereafter was successful and the patient remains symptom-free.