Constantino Morera

Electric acoustic stimulation of the auditory system: results of a multi-centre investigation.

Conclusion. A high rate of hearing preservation during cochlear implantation for electric acoustic stimulation (EAS) is possible, even when surgery is conducted by a number of different surgeons. Objectives. This study aimed to determine the degree of hearing preservation using surgery for EAS in a European multi-centre clinical investigation. It also aimed to demonstrate the effect of EAS in individuals with residual low frequency hearing, both on speech perception and on subjective quality of life measures. Patients and methods. Eighteen patients with profound high frequency hearing loss were recruited in five participating European centres. Subjects were assessed based on an audiologic test battery, as well as on a subjective hearing aid benefit questionnaire. Each subject underwent attempted hearing preservation cochlear implantation using the MED-EL C40+ device with a Medium electrode. Residual ipsilateral hearing and speech discrimination abilities were assessed at defined intervals up to 12 months after the combined electric-acoustic mode was introduced. Results. Results showed that some degree of hearing preservation was possible in 15718 patients. All subjects showed statistically significant benefit on all three speech perception tests over time. These significant benefits were also reflected in the subjective benefit outcomes.

A multicenter study on the prevalence and spectrum of mutations in the otoferlin gene (OTOF) in subjects with nonsyndromic hearing impairment and auditory neuropathy

Autosomal recessive nonsyndromic hearing impairment (NSHI) is a heterogeneous condition, for which 53 genetic loci have been reported, and 29 genes have been identified to date. One of these, OTOF, encodes otoferlin, a membrane‐anchored calcium‐binding protein that plays a role in the exocytosis of synaptic vesicles at the auditory inner hair cell ribbon synapse. We have investigated the prevalence and spectrum of deafness‐causing mutations in the OTOF gene. Cohorts of 708 Spanish, 83 Colombian, and 30 Argentinean unrelated subjects with autosomal recessive NSHI were screened for the common p.Gln829X mutation. In compound heterozygotes, the second mutant allele was identified by DNA sequencing. In total, 23 Spanish, two Colombian and two Argentinean subjects were shown to carry two mutant alleles of OTOF. Of these, one Colombian and 13 Spanish subjects presented with auditory neuropathy. In addition, a cohort of 20 unrelated subjects with a diagnosis of auditory neuropathy, from several countries, was screened for mutations in OTOF by DNA sequencing. A total of 11 of these subjects were shown to carry two mutant alleles of OTOF. In total, 18 pathogenic and four neutral novel alleles of the OTOF gene were identified. Haplotype analysis for markers close to OTOF suggests a common founder for the novel c.2905_2923delinsCTCCGAGCGCA mutation, frequently found in Argentina. Our results confirm that mutation of the OTOF gene correlates with a phenotype of prelingual, profound NSHI, and indicate that OTOF mutations are a major cause of inherited auditory neuropathy. Hum Mutat 29(6), 823–831, 2008.

Advantages of binaural hearing provided through bimodal stimulation via a cochlear implant and a conventional hearing aid: A 6-month comparative study

Conclusions Our study data demonstrate the additional benefit derived from continued use of a contralateral hearing aid (HA) post-cochlear implantation for speech recognition ability in quiet and in noise. Postoperative bimodal stimulation is recommended for all subjects who show some speech recognition ability in the contralateral ear as it may offer binaural listening advantages in various listening situations encountered in everyday life. Objectives To assess the benefits derived from bimodal stimulation for experienced HA users implanted with a cochlear implant (CI) (score ≥ 20% in disyllabic test). The correlation between pre- and postoperative performance on speech perception measures was examined to determine additional criteria for recommending bimodal stimulation postoperatively. Material and methods A within-subject repeated-measures design was used, with each subject acting as their own control. Assessments were carried out preoperatively in aided monaural and best-aided conditions and at 6 months postoperatively in CI-alone, contralateral HA-alone and bimodal listening conditions. Speech recognition using Spanish words and sentences materials was assessed at conversational level and for soft speech in quiet. Speech comprehension in noise was assessed using word materials at a signal:noise ratio of +10, for coincident speech in noise and for spatially separated speech in noise. Twelve adult native Spanish subjects with a severe-to-profound hearing impairment who were experienced with optimally fitted conventional amplification and who displayed suboptimal speech understanding preoperatively were enrolled in the study. Preoperatively, conventional amplification was worn by five subjects binaurally and by seven monaurally. Results Postoperatively, superior speech recognition ability in quiet and in noise for disyllabic words was achieved using bimodal stimulation in comparison to performance for either monaural aided condition. Mean improvement in speech recognition in the bimodal condition was significant over performance in the CI-alone condition for disyllabic words in quiet at 70 (p=0.006) and 55 dB SPL (p=0.028), for disyllabic words in noise at +10 dB with speech and noise spatially separated with the noise source closest to the contralateral HA (S0NHA) (p=0.0005) and when the noise source was closest to the CI ear (S0NCI) (p=0.002). When testing word recognition in noise with speech and noise sources coincident in space, word scores were superior in the bimodal condition relative to the CI-alone condition but this improvement was not significant (p=0.07). The advantages of bimodal stimulation included significant effects of binaural summation in quiet and significant binaural squelch effects in both the S0NHA and S0NCI test conditions. All subjects showed superior performance in the binaural situation postoperatively relative to the best-aided condition preoperatively for one or more test situations.

International consensus on bilateral cochlear implants and bimodal stimulation

Binaural hearing allows listeners with normal hearing to understand speech better in silence and noisy conditions and is an essential requirement for spatial hearing and sound localization. Other benefits of binaural hearing are more natural hearing, reduced listening effort and an improved quality of life. The cortical projection of the ear is fundamentally contralateral and only a small proportion of fibers in the auditory neural pathway have homolateral cortical projection. This results in insufficient homolateral neural and cortical development in cases of monaural stimulation.

An evaluation of the preservation of residual hearing with the Nucleus® Contour Advance™ electrode

Conclusion. Our study results confirm that it is possible to preserve preoperative hearing levels in the majority of subjects when using the Nucleus 24 Contour Advance provided that there is adherence to the major principles of ‘soft surgery’. Our study group demonstrated that 71–86% of subjects showed preservation of preoperative hearing thresholds at 6 months to varying degree. Objectives. The aim of the study was to assess the degree of residual hearing preserved postoperatively in a group of standard cochlear implant (CI) candidates following implantation via soft surgery with a Nucleus® 24 Contour Advance™ CI. Surgical technique variations from the soft surgery guidelines provided were assessed and their potential impact upon the conservation of residual hearing was examined. Subjects and methods. A prospective multicentre study involving a within-subject repeated measures design with each subject acting as their own control was performed. Pure-tone audiometric thresholds were assessed and compared in both implanted and contralateral ears for each subject preoperatively as baseline measures and at 6 months postoperatively. Surgeons were asked to complete a questionnaire to capture various aspects of the surgical technique used for each subject. Variations in the surgical technique performed were examined for potential correlation with conservation of residual hearing. Twenty-eight adult subjects, with a severe to profound hearing impairment, were enrolled in the study across eight implant clinics in four countries. Results. In all, 36% of subjects demonstrated preservation of thresholds to within 10 dB of preoperative thresholds across the frequency range (0.25, 0.5, 1.0, 2.0 and 4.0 KHz) and for the low frequency range (0.25–1.0 KHz). Approximately two-thirds of subjects demonstrated preservation of preoperative thresholds to within 20 dB. Preservation of low frequency thresholds post-implant was shown to correlate moderately with cochleostomy site, being more likely for subjects with a site anterior-inferior to the round window but also possible with inferior locations; weakly with cochleostomy size, being more likely when smaller than 1.2 mm; and also with the use of Healon® as a sealant and lubricant. Preservation of hearing thresholds across up to 4000 Hz was shown to correlate weakly with the use of suction following opening of the endostium and with bone dust contamination, both having a negative effect upon preservation, while no correlation was observed with the preservation of thresholds for low frequencies alone.

Outcomes in adults implanted with the FLEXsoft electrode

Conclusion. Achieving deep insertions, as well as good speech perception results, the FLEXsoft electrode array allows for some preservation in subjects with measurable low frequency hearing, even after a period of time. This opens the door for future research in electrode design, hearing preservation research and drug delivery systems. Objectives. The FLEXsoft electrode is designed to be atraumatic to the structures of the cochlea during deep insertion of a cochlear implant electrode. This paper reports on the surgical and functional outcomes in implantations with the FLEXsoft electrode array. Patients and methods. Twenty-three adult subjects received a FLEXsoft electrode array and were assessed on speech perception tests (monosyllables, sentences in quiet and in noise), a subjective questionnaire (Nijmegen Cochlear Implant Questionnaire) and a pure-tone audiogram. Results at 1, 3, 6 and 12 months post first fitting were compared to scores from the preoperative interval. Results. Surgery was uneventful in all cases, the surgical handling was satisfactory and correct position of the electrode was achieved in all cases. Hearing could be preserved (as determined by the audiogram) in half of the subjects who had measurable audiograms preoperatively at the 1 month test interval, and in a quarter of subjects after 12 months of device use, despite deep insertion of the electrode. Speech perception scores showed significant improvement over time, as did quality of life scores, and were comparable to results with the standard electrode array as used in the COMBI 40+ and PULSARCI100.

Time Course of Episodes of Definitive Vertigo in Ménière’s Disease

OBJECTIVE To evaluate the frequency and duration of episodes of definitive vertigo in Ménières disease. DESIGN Prospective longitudinal study. SETTING Multiple tertiary referral centers. PATIENTS Five hundred ten individuals from 8 hospitals that met the American Academy of Otolaryngology-Head and Neck Surgery diagnostic criteria for definitive Ménières disease. INTERVENTION Conservative treatment. MAIN OUTCOME MEASURE Frequency and duration of episodes of definitive vertigo during follow-up. RESULTS Ménières disease affects both sexes and both ears equally, with onset generally in the fourth decade of life. The number of episodes of vertigo is greater in the first few years of the disease. Although episodes of vertigo that last longer than 6 hours are less frequent than shorter episodes, they occur with similar frequency throughout the natural course of the disease. The percentage of patients without episodes of vertigo increases as the disease progresses, and 70% of patients who did not have an episode of vertigo for 1 year will continue to be free of episodes during the following year. Thus, there is a relationship between the frequency of episodes in consecutive years, although this association decreases rapidly as the number of years increases. CONCLUSION The frequency of definitive episodes of vertigo in Ménières disease decreased during follow-up, and many individuals reached a steady-state phase free of vertigo.

Evaluación del implante coclear como técnica de tratamiento de la hipoacusia profunda en pacientes pre y post locutivos

Resumen Introduccion Los objetivos son los siguientes: 1. Evaluacion de los resultados en el ambito del lenguaje. 2. Impacto de los implantes cocleares (IC) en la calidad de vida. 3. Evaluacion de las complicaciones medicas y fallos tecnicos del IC. 4. Evaluacion de los costes directos e indirectos generados en las diferentes fases de un programa de IC. 5. Determinar factores que repercuten en la evolucion clinica y en el gasto economico Material y Metodos Se estudia una poblacion de 877 pacientes con hipoacusia pre o postlocutiva, ninos y adultos, tratados en 5 centros espanoles con programas de IC. Se han utilizado pruebas audiometricas y cuestionarios globales de calidad de vida. Se han contabilizado las complicaciones medicas y tecnologicas de los IC. Se han calculado los costes economicos directos e indirectos de una implantacion coclear Resultados Los pacientes postlocutivos alcanzaron umbrales de 40 dB SPL, estables a lo largo de 12 anos de evolucion. En Vocales pasaron de un 30% en el preoperatorio al 80-90%, en Bisilabas de un 10% a un 50-60% y en Frases CID de un 18% a un 60-70%. En la poblacion de prelocutivos la edad de implantacion influyo decisivamente en los resultados. Los mejores fueron los obtenidos en ninos implantados mas tempranamente. Asi, aquellos implantados entre 0 y 3 anos de edad en Vocales pasaron de un 0% en el preoperatorio a un 95%, en Bisilabas de un 0% a un 90% y en Frases CID de un 0% a un 90-95%. El desarrollo del lenguaje de la poblacion prelocutiva tambien estuvo marcado por la edad de implantacion. En torno al 80% de los pacientes adultos postlocutivos manifestaron haber mejorado su estado de animo y su sociabilidad. Su estado general de salud no se vio deteriorado, ni precisaron mas ayudas de familiares y amigos. Se registro un 3,42% de complicaciones medico-quirurgicas mayores, un 7,06% de complicaciones medico-quirurgicas menores y un 3,07% de fallos tecnicos en los componentes internos del IC. El costo de todo el proceso de implantacion en un adulto postlocutivo oscilo entre 36.912 y 37.048 euros y en ninos prelocutivos entre 37.689 y 44.273 euros Conclusiones Los IC benefician la capacidad de comunicacion de los pacientes implantados. Los resultados en la poblacion prelocutiva justifican la puesta en marcha de programas de deteccion precoz de la hipoacusia infantil. Los pacientes postlocutivos adultos se muestran satisfechos con los resultados obtenidos, aunque perciben limitaciones en ambientes acusticos no favorables. Se expone un analisis de costes directos e indirectos util para la realizacion de estudios coste-beneficio sobre IC en nuestro medio. El bajo indice de complicaciones indica unos margenes de seguridad adecuados. Entre los factores que mas influyen en la evolucion estan: la duracion de la hipoacusia, la edad de implantacion, la morfologia coclear y la funcionalidad de la via auditiva, la motivacion del paciente y de su familia y la coexistencia de otras minusvalias asociadas a la hipoacusia

Maternally inherited non-syndromic hearing impairment in a Spanish family with the 7510T>C mutation in the mitochondrial tRNA Ser(UCN) gene

Inherited hearing impairment is a highly heterogeneous group of disorders.1,2 In a majority of cases (about 70%), the hearing loss is non-syndromic, that is, it is not associated with any other clinical feature. It can be transmitted following autosomal (recessive or dominant), X linked, or maternal inheritance patterns. In the nuclear genome, more than 70 loci have been reported to be involved in non-syndromic hearing impairment, and 27 genes have been isolated from their critical intervals.2 In addition, a number of different mutations in several genes of the mitochondrial genome are responsible for hearing impairment.3,4 Some of these mutations result in a variety of additional clinical features in diverse organs. Mitochondrial syndromic hearing loss includes Kearns-Sayre syndrome (MIM 530000), MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes; MIM 540000), MERRF (myoclonus epilepsy and ragged red fibres; MIM 545000), and MIDD (maternally inherited diabetes mellitus and deafness; MIM 520000).3–5 In other cases, intrafamilial and interfamilial phenotypic variation is observed, a mutation causing syndromic or non-syndromic hearing impairment in different patients. This is the case for the 7445A>G mutation, causing hearing loss and palmoplantar keratoderma,6–10 and for 7472insC, responsible for hearing loss and neurological disorders (myoclonus epilepsy, ataxia, and cognitive impairment).11–14 Finally, other mutations have been associated so far only with hearing loss. These include mutations 7510T>C15 and 7511T>C16 in the tRNASer(UCN) gene, and 1095T>C17 and 1555A>G18 in the gene for the 12S rRNA. This last mutation is responsible for a dual phenotype, since it also confers increased susceptibility to the ototoxic action of aminoglycoside antibiotics.18 Most of the mutations causing maternally inherited non-syndromic …

Contralateral hearing aid use in cochlear implanted patients: Multicenter study of bimodal benefit

Abstract Conclusion: The use of a hearing aid (HA) in combination with a cochlear implant (CI) significantly improved performance for speech perception in quiet, in noise, and for localization compared with monaural conditions. No significant differences in functional performance were observed following optimization of HA fitting. Objectives: To evaluate the binaural benefits derived from using a contralateral HA in conjunction with a CI in subjects with significant functional hearing in the nonimplanted ear and the effects of HA fitting optimization. Methods: Fifteen adult CI users, intra-subject controls, were enrolled in a prospective repeated-measure multicenter study. Evaluation of performance for speech understanding, localization, and subjective impressions was conducted before and following HA fitting optimization for CI alone, HA alone, and CI + HA. Results: For speech testing in quiet, bimodal scores were significantly better than for HA alone and CI alone conditions (p < 0.01). For speech and noise (S0N0) at 0° azimuth the scores were significantly better in the bimodal condition than for CI alone (p = 0.01), indicating binaural summation. When noise was presented to the HA side (S0NHA) bimodal scores were significantly better than for CI alone (p < 0.01 and p < 0.05, respectively), suggesting a significant binaural squelch effect. Sound localization ability was significantly improved in the bimodal condition compared with the CI alone condition (p = 0.002).