Sordera neurosensorial congénita con malformación de oído interno ligada al X en una familia mexicana / Congenital sensorineural deafness X-linked, with inner ear malformation in a Mexican family

Introducción. Las sorderas o hipoacusias prelinguales son de etiología genética entre el 60 y el 68% de los casos; de estos, del 20 al 40% son malformaciones del oído interno. De los casos de hipoacusia no sindrómica ligada al X se han descrito siete tipos. De las malformaciones de oído interno, la partición coclear incompleta tipo III es la menos frecuente.Objetivo. Presentar el reporte clínico-genético de una familia mexicana, con indi-viduos varones afectados por sordera neurosensorial congénita con malformación de oído interno. Material y Métodos. Se realizó estudio de una familia en la que nueve miembros presentaban sordera. Se estudiaron cuatro de ellos y una madre sin manifestaciones, a través del estudio clínico general por médico genetista, el estudio audiológico (otos-copía y audiometría) por médico audiólogo y el estudio de tomografía computada (TC) por médico radiólogo.Resultados. Los pacientes estudiados presentaron sordera neurosensorial congéni-ta, de severa a profunda bilateral. A través de la TC, se evidenció malformación de oído interno. Tres pacientes presentaron partición coclear incompleta tipo III y un paciente partición incompleta tipo I. Debido al estudio clínico y al árbol genealógico, se definió diagnóstico de hipoacusia neurosensorial no sindrómica ligada al X. La TC de la madre sin manifestaciones no presentó evidencia de malformaciones en oído interno (MOI).Conclusión. El estudio de imagen es fundamental para definir presencia o no de MOI en todos los pacientes con hipoacusia y así poder guiar la terapéutica y el aseso-ramiento genético, así como realizar los estudios moleculares más adecuados

Introduction. The pre-lingual deafness or hearing loss are of genetic cause in be-tween 60% and 68% of cases, among these, between 20% and 40% are malforma-tion of the inner ear. From the non-syndromic hearing loss cases that are linked to the X chromosome, seven types have been described. Among these inner ear malforma-tions, incomplete cochlear partition type III is the less frequent.Objective. Present the clinical genetical report of a Mexican family, with male in-dividuals affected by congenital neurosensory deafness with inner ear malformation.Materials and methodology. A study on a family in which nine members were affected by deafness was done. Four of them, plus a mother without manifestation, were studied through a general clinical study by a geneticist, an audiological study (otoscopy and audiometry) by an audiologist, and a computed tomography (CT) scan by a radiologist.Results. The studied patients presented congenital neurosensory deafness, from se-vere to deep bilateral. Via the CT, the inner ear malformation was made clear. Three of the patients presented incomplete cochlear partition type III and one patient in-complete cochlear partition type I. Due to the clinical study and the family tree, it was diagnosed non-syndromic neurosensory deafness linked to X. The CT of the mother without manifestation did not show evidence of inner ear malformations.Conclusion. The study by image is fundamental to define whether there is or not a presence of inner ear malformations in any patient with heading loss to be able to guide the therapeutics and the genetic counseling, as well as to make more accurate molecular studies

Les surdites brusques idiopathiques facteurs pronostiques / Sudden idiopathic deafness prognostic factors

Les surdités brusques idiopathiques (SBI) constituent l'un des sujets les plus débattus en otologie. Plusieurs problèmes d'ordre physiopathologiques, thérapeutiques et pronostiques restent non résolus. But : Identifier les facteurs pronostiques, cliniques et audiométriques de récupération auditive après traitement d'une SBI. Malades : Etude rétrospective portée sur 27 malades (29 cas de SBI) traités dans le service d'ORL et chirurgie cervicofaciale du CHU Habib Bourguiba Sfax durant la période comprise entre les années 1990 et 2005. Méthodes : C'est une étude statistique recherchant une corrélation significative entre certains facteurs cliniques et audiométriques et la récupération auditive. Les facteurs étudiés étaient : l'âge, l'aspect de la courbe audiométrique, la perte auditive initiale et le délai de prise en charge thérapeutique. Résultats : L'âge inférieur à 50 ans, la courbe ascendante et la perte auditive inférieure à 70 dB étaient les facteurs de meilleur pronostic. Le délai de prise en charge thérapeutique semble avoir peu de rôle dans la récupération auditive. Discussion : La majorité des auteurs ont signalé que l'âge jeune, les surdités légères ou moyennes, la courbe ascendante et la précocité de la prise en charge sont associés à un meilleur pronostic. Nos résultats rejoignent ceux de Tran Ba Huy qui ne trouve pas de corrélation entre le délai de prise en charge thérapeutique et le pronostic de récupération auditive.

Morfología de la curva audiométrica en la enfermedad de Ménière al momento del diagnóstico / Audiometric curve configurations in Ménière disease at the first visit / Morfologia do desenho audiometrico na doença de Ménière no momento do diagnostico

INTRODUCCIÓN: La enfermedad de Ménière es una vestibulopatía frecuente que se manifiesta con crisis de vértigo, acúfenos, plenitud aural y pérdida auditiva neurosensorial. La hipoacusia es un síntoma fundamental para realizar el diagnóstico y se puede presentar de diversas formas. OBJETIVO: Describir las morfologías de las curvas audiométricas de los pacientes con enfermedad de Méniére al momento del diagnóstico. MATERIAL Y MÉTODOS: Estudio retrospectivo. Se evaluaron 99 pacientes. Se les realizaron estudios audiométricos y se registró el umbral tonal medio, el estadio correspondiente, la presencia de hipoacusia conductiva y la morfología de la curva audiométrica...

INTRODUCTION: Ménière disease is a frequent vestibular desorder that causes vertigo attacks, tinnitus, aural fullness and sensorineural hearing loss. Hearing loss is an essential symptom to get certain diagnosis and it can appear in several shapes. OBJECTIVE: to describe the configurations of the audiometric curves of patients with Ménière desease at the first visit. MATERIAL AND METHODS: A retrospective study included 99 patients. Audiometric studies were performed and mean tonal thresholds, corresponding stage, conductive hearing loss and audiometric curve configurations were recorded…

INTRODUÇÃO: A doença de Ménière é uma vestibulopatia freqüente que se manifesta com crise de vertigem, zumbido, plenitude aural e perda auditiva neurossensorial. A perda de audição é um sintoma fundamental para fazer o diagnóstico mais pode se apresentar com muitas formas. OBJETIVO: Descrever as morfologias das curvas audiométricas em pacientes com doença de Méniére no momento do diagnóstico. MATERIAL E MÉTODOS: Estudo retrospectivo. 99 pacientes foram avaliados. Estudos audiológicos foram feitos e o limiar tonal meia, o estágio, a presença de perda auditiva condutiva e o desenho da curva audiométrica foram analisados...

The Relationships of Tone Burst -ABR Thresholds and the Pure-Tone Audiograms in Normal Hearing Adults / 听力学及言语疾病杂志

Objective To investigate the relationships of tone burst -ABR Thresholds and the pure -tone audiograms in normal hearing adults .Methods The response thresholds of Tone burst -ABR and the pure -tone audiogram were recorded from 34 adult(68 ears) at 0 .5 ,1 .0 ,2 .0 ,and 4 .0 kHz .The results were statistically ana-lyzed .The correlation of the response thresholds of tone burst -ABR and the pure-tone audiogram were studied . Results At 0 .5 ,1 .0 ,2 .0 ,4 .0 kHz ,the averages of the response thresholds of tone burst -ABR were 27 .43 ± 3 .29 ,25 .98 ± 2 .76 ,16 .78 ± 2 .37 ,and 12 .42 ± 2 .64 dB nHL ,respectively .The averages of the pure -tone audio-gram were 12 .23 ± 3 .99 ,11 .82 ± 2 .56 ,9 .58 ± 3 .23 ,and 9 .92 ± 2 .59 dB nHL ,respectively .The differences between the response thresholds of tone burst -ABR and the pure-tone audiogram were 12 .13 ± 4 .51 ,11 .43 ± 3 .66 ,7 .61 ± 2 .43 ,and 7 .17 ± 1 .32 dB ,respectively .At 0 .5 ,1 .0 ,2 .0 ,and 4 .0 kHz ,Pearson correlation coefficients were 0 .69 ,0 .79 ,0 .84 ,0 .89 ,respectively .Conclusion The tone burst -ABR and the pure -tone audiogram thresholds were a highly correlated with each other ,especially at 2 .0 kHz and 4 .0 kHz .Tone burst -ABR has the frequency specificity ,which can response the hearing level better .

The Best-Matched Pure Tone Average and Speech Recognition Threshold for Different Audiometric Configurations / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The agreement between pure-tone average (PTA) and speech recognition threshold (SRT) has become more important with the increasing demands for medical certification. The purpose of this study was to explore the relationships between the SRT and several variations of PTA, and to determine which PTA formula would provide the best agreement with SRT for different audiometric configurations. SUBJECTS AND METHOD: Audiological data on 783 ears were retrospectively collected. The air-conduction PTAs were calculated using five different formulas: three-frequency average (3FA), weighted three-frequency average (W3FA), four-frequency average (4FA), weighted four-frequency average (W4FA), and six-frequency average (6FA). The audiometric configuration was classified into five categories. The PTA-SRT relationships were analyzed using correlation and simple linear regression for each audiometric configuration. RESULTS: Highest correlation was observed between the SRT and W3FA for all audiometric configurations with the correlation coefficient of 0.964 as a whole. The SRT and 3FA were best-matched in the linear regression models for overall/flat/high frequency gently sloping/low frequency ascending; the SRT and W3FA were best-matched for high frequency steeply sloping (HFSS); the SRT and 4FA were best-matched for miscellaneous audiograms. CONCLUSION: The most stable PTA variations that make the best-matched pairs with SRT for any audiogram are the conventional 3FA and W3FA doubling 1 kHz threshold. The addition of frequencies higher than 2 kHz to a PTA formula seems to have impeded the PTA-SRT agreement, especially for HFSS audiograms. W3FA should be the method of choice in predicting SRT from PTA for HFSS audiograms.

Efifcacy of early combination treatment with intratympanic glucocorticoid injection for sudden hearing loss / 中国耳鼻咽喉头颈外科

[ABSTRACT]OBJECTIVETo investigate the therapeutic effect of early combination treatment with intratympanic glucocorticoid injection for sudden sensorineural hearing loss(SSNHL).METHODSTotal of 178 patients with diagnosis of SSNHL, including 44 with hearing loss at middle-low frequencies,50 at high frequencies,44 at all frequencies and 40 at total deafness, were randomly divided into combination treatment group (CT group) and systemic oral prednisone group (OP group).CT group (82 patients) received intratympanic administration of methylprednisolone and oral prednisone in tapering doses. Pure-tone threshold audiometry (PTA) was performed at week 8 after beginning of therapy. RESULTSIn middle-low frequencies, the final PTA and PTA improvement were 26.14±24.82 dB and 42.23±1.61 dB in CT group respectively, and were 37.91±13.98 dB and 30.00±13.30 dB in OP group respectively (both P<0.05). In high frequencies, all frequencies and total deafness SSNHL group, the differences of the final PTA and PTA improvement were not statistically significant in both groups. The PTA improvement in CT group for total deafness (26.52±14.03) dB was more than that of OP group (22.06±11.17)dB (t=2.361,P<0.05). In middle-low frequencies, the recovery rate was 72.73%(16 cases) in CT group, and 40.91% (9 cases) in OP group, the difference was significant (χ2=4.539,P<0.05), but not in apparent effective rate, effective rate and total effective rate. In high frequencies, all frequencies and total deafness, there were no significant differences in therapeutic effect. The recovery rate of total CT group (40.24%) was significantly increased compared with that of OP group (26.04%)(χ2=4.061,P<0.05), the differences of apparent effective rate, effective rate and total effective rate were not statistically significant in both groups.CONCLUSIONFor low-middle frequencies SSNHL, the early combination treatment with intratympanic glucocorticoid injection leads to higher hearing recovery and therapeutic effect compared with traditional oral prednisone. Systemic application should be applied for high frequencies, all frequencies and total deafness of SSNHL.

Correlation of numeric rating scale with pure tone audiogram for assessing hearing loss.

Objective: Hearing loss is the public health problem affecting all the age groups. For the assessment of hearing loss pure tone audiogram (PTA) is the gold standard but cannot be easily available in rural areas. So, the main aim of our study is to see the correlation of numeric rating scale with the pure tone audiogram for assessing the hearing loss. Materials and methods: This was the prospective, non randomized and longitudinal study performed in two hundred patients with unilateral hearing loss. For the study purpose, the numeric rating scale (NRS) was divided into 5 parts as that of degree of hearing loss and the PTAwas also divided into 5 parts to see the correlation with the NRS findings. The analysis was done using SPSS 16.0 Software. Results: The total frequency of unilateral hearing loss was 1.79%. 125(62.5%) patients fell within 16 – 25years.The mean age was 23.2+/-9.7.Females were affected more than males. Most of the patients who were in high school had unilateral hearing loss. Students and housewives accounted for 87.5%. 89% patients had conductive hearing loss, 10% had sensorineural hearing loss whereas only 1% patients had mixed hearing loss. The most of patients reported NRS2 and NRS3 which was 85.5%. Regarding the PTA analysis, 91% patients fell within mild and moderate hearing loss. There was high degree of correspondence in mild hearing loss between NRS and PTA. The Pearson’s correlation showed the statistically significant correlation between NRS and PTA with p=0.00 Conclusion: The NRS scale can be used as an optional measure for PTAin assessing the hearing loss mainly in rural areas for screening where there is lack of PTA.

Signal-to-noise Ratio Loss in the People with Normal Audiograms / 听力学及言语疾病杂志

Objective To explore the signal-to-noise ratio loss (SNR loss) of the people with normal pure-tone audiograms .Methods A group of 10 patients were recruited into the study ,who complained they could not hear speech clearly in the real world environment but had normal pure-tone audiograms ,immittance and word rec-ognition score (WRS) results .The Mandarin hearing in noise test (M-HINT ) was administered to them and the results were compared to the normal ranges published in the journal .Results All of them had SNR loss when noise (speech spectrum) came from front of them ,but 9 patients had SNR loss when noise came from 90 degree left or 90 degree right of them .Conclusion The current study showed the patients had speech hearing loss in the noisy envi-ronment ,regardless of whether they had normal audiograms ,immittance and WRS results .So we should pay atten-tion to such patients and adopt some special tests (eg M-HINT) .It is better for us to communicate and counsel with them .

Migraine and Sudden Hearing Loss

On rare occasions, sudden hearing loss may be associated with a migraine. To our knowledge, there have only been two prior reports of sudden hearing loss attributed to migraines. A 40 years old man had suffered from recurrent headaches with hemiparesis, vertigo, and hearing loss for the past three years. At this time, he presented with severe bilateral sensorineural hearing loss of sudden onset, anarthria, right hemiplegia, tinnitus, and vertigo. An audiogram showed a down-sloping sensorineural hearing loss of 77dB by pure tone average on the right side and 72dB on the left. Sixteen days later, audiographic findings improved to 30dB of pure tone average on both sides. Vasospasm of the labyrinthine arteries instigated by migraine might explain these inner ear symptoms. A history of migraine should be sought in patients with sudden hearing loss and when found, anti-migraine medications such as calcium channel block-ers should be considered.

Clinical Observation on Hearing Change in Hydromyringa / 听力学及言语疾病杂志

Clinical Observation on pure-tone audiogram shape,preauripuncture and postau-ripuncture change of pure-tone threshold average (PTA) ,A-B gap was conducted in 97 patients (117 ears) with hydromyringa.Bone conduction audibility threshold change in some cases was discussed.