Importance of a multidisciplinary approach and monitoring in fetal warfarin syndrome.

Warfarin is a synthetic oral anticoagulant that crosses the placenta and can lead to a number of congenital abnormalities known as fetal warfarin syndrome. Our aim is to report on the follow-up from birth to age 8 years of a patient with fetal warfarin syndrome. He presented significant respiratory dysfunction, as well as dental and speech and language complications. The patient was the second child of a mother who took warfarin during pregnancy due to a metallic heart valve. The patient had respiratory dysfunction at birth. On physical examination, he had a hypoplastic nose, pectus excavatum, and clubbing of the fingers. Nasal fibrobronchoscopy showed upper airway obstruction due to narrowing of the nasal cavities. He underwent surgical correction with Max Pereira graft, zetaplasty, and osteotomies for the piriform aperture. At dental evaluation, he had caries and delayed eruption of the upper incisors. Speech and language assessment revealed high palate, mouth breathing, little nasal patency, and shortened upper lip. Auditory long latency and cognitive-related potential to auditory stimuli demonstrated functional changes in the cortical auditory pathways. We believe that the frequency of certain findings observed in our patient may be higher in fetal warfarin syndrome than is appreciated, since a significant number result in abortions, stillbirths, or children evaluated in the first year of life without a follow-up. Thus, a multidisciplinary approach and long-term monitoring of these patients may be necessary.

Cochlear implantation in a patient with sensori-neural deafness secondary to Charcot-Marie-Tooth disease.

OBJECTIVE AND IMPORTANCE: Charcot-Marie-Tooth (CMT) disease is the most common hereditary motor and sensory neuropathy and can result in profound sensori-neural hearing loss with deficiency in speech perception out of proportion to that which would be expected if the loss was cochlear in origin. This study investigates whether the reintroduction of auditory synchrony by means of cochlear implantation will improve speech perception in those with dys-synchrony related to impairment of temporal processing abilities secondary to CMT. CLINICAL PRESENTATION: A 67-year-old male presented with a gradual but significant decrease in his hearing as part of a slowly progressing demyelinating peripheral neuropathy. On open-set speech discrimination he scored 0%. INTERVENTION: A Med-el Flex(SOFT) cochlear implant (CI) was fully inserted into the left ear with no surgical complications. The CI speech processor was fitted 1 month post-implantation and standardized speech assessments conducted at 1 week, 3 months, 9 months, and 21 months following initial fitting, gave open-set speech discrimination scores of 0, 0, 53, and 54%, respectively. CONCLUSION: This report demonstrates that cochlear implantation is an option to rehabilitate severe-to-profound hearing loss in adults with auditory dys-synchrony secondary to CMT disease. Progress post-implantation is likely to be slower than for the average CI user.

Auditory neuropathy/Auditory dyssynchrony in children with Cochlear Implants.

UNLABELLED: The electrical stimulation generated by the Cochlear Implant (CI) may improve the neural synchrony and hence contribute to the development of auditory skills in patients with Auditory Neuropathy/Auditory Dyssynchrony (AN/AD). AIM: Prospective cohort cross-sectional study to evaluate the auditory performance and the characteristics of the electrically evoked compound action potential (ECAP) in 18 children with AN/AD and cochlear implants. MATERIAL AND METHODS: The auditory perception was evaluated by sound field thresholds and speech perception tests. To evaluate ECAP's characteristics, the threshold and amplitude of neural response were evaluated at 80 Hz and 35 Hz. RESULTS: No significant statistical difference was found concerning the development of auditory skills. The ECAP's characteristics differences at 80 and 35 Hz stimulation rate were also not statistically significant. CONCLUSIONS: The CI was seen as an efficient resource to develop auditory skills in 94% of the AN/AD patients studied. The auditory perception benefits and the possibility to measure ECAP showed that the electrical stimulation could compensate for the neural dyssynchrony caused by the AN/AD. However, a unique clinical procedure cannot be proposed at this point. Therefore, a careful and complete evaluation of each AN/AD patient before recommending a Cochlear Implant is advised. Clinical Trials: NCT01023932.

Auditory neuropathy/auditory dyssynchrony in children with cochlear implants / Neuropatia auditiva/dessincronia auditiva em crianças usuárias de implante coclear

The electrical stimulation generated by the Cochlear Implant (CI) may improve the neural synchrony and hence contribute to the development of auditory skills in patients with Auditory Neuropathy/Auditory Dyssynchrony (AN/AD). AIM: Prospective cohort cross-sectional study to evaluate the auditory performance and the characteristics of the electrically evoked compound action potential (ECAP) in 18 children with AN/AD and cochlear implants. MATERIAL AND METHODS: The auditory perception was evaluated by sound field thresholds and speech perception tests. To evaluate ECAP's characteristics, the threshold and amplitude of neural response were evaluated at 80Hz and 35Hz. RESULTS: No significant statistical difference was found concerning the development of auditory skills. The ECAP's characteristics differences at 80 and 35Hz stimulation rate were also not statistically significant. CONCLUSIONS: The CI was seen as an efficient resource to develop auditory skills in 94 percent of the AN/AD patients studied. The auditory perception benefits and the possibility to measure ECAP showed that the electrical stimulation could compensate for the neural dyssynchrony caused by the AN/AD. However, a unique clinical procedure cannot be proposed at this point. Therefore, a careful and complete evaluation of each AN/AD patient before recommending a Cochlear Implant is advised. Clinical Trials: NCT01023932.

A estimulação elétrica gerada pelo Implante Coclear (IC) pode ser capaz de melhorar a sincronia neural e contribuir para o desenvolvimento das habilidades auditivas de sujeitos portadores de Neuropatia Auditiva/Dessincronia Auditiva (NA/DA). OBJETIVO: Estudo de coorte prospectivo transversal para avaliar o desempenho auditivo e as características do Potencial de Ação Composto Eletricamente Evocado no Nervo Auditivo (ECAP) em 18 crianças portadoras de NA/DA e usuárias de IC. MATERIAL E MÉTODOS: Percepção auditiva e características do ECAP foram avaliadas respectivamente, por meio da determinação dos limiares tonais e testes de percepção de fala e pelas medidas de limiar e amplitude da resposta neural para as frequências de estimulação de 35 e 80Hz. Não foram encontradas diferenças estatisticamente significativas no desenvolvimento das habilidades auditivas entre os sujeitos avaliados. RESULTADOS: Não foram observadas modificações estatisticamente significativas nas características de limiar e amplitude do ECAP para as duas frequências de estimulação testadas. CONCLUSÕES: O IC caracterizou-se como um efetivo recurso para o desenvolvimento das habilidades auditivas em 94 por cento dos sujeitos, sendo a estimulação elétrica capaz de compensar a alteração da sincronia neural decorrente da NA/DA. A avaliação criteriosa de cada caso de maneira específica deve ser realizada no momento anterior à indicação cirúrgica. Clinical Trials: NCT01023932.

Estapedotomía con prótesis de titanio y teflón: análisis retrospectivo de los resultados / Stapedotomy prosthesis with titanium and teflon: retrospective analysis of results

Objetivo: describir los resultados obtenidos con la colocación de dos prótesis estapediales a pacientes sometidos a estapedotomía por otosclerosis. Tipo de estudio: descriptivo y retrospectivo de revisión de casos. Métodos: se utilizaron prótesis de titanio y de teflón-platino para el cierre de la diferencia entre las curvas ósea y aérea (gap) producido por la otosclerosis. Se revisaron 70 casos intervenidos en el Hospital de San José entre el 2002 y 2005, por tres cirujanos. Se utilizo técnica de pequeña fenestración realizada con perforadores manuales; 48 casos cumplieron con los criterios. Se excluyeron los casos de revisión, aquellos con anomalías anatómicas o datos inadecuados pre o postquirúrgicos. Se midió el gap óseo-aéreo con audiometría de tonos puros en cuatro frecuencias y el umbral límite de pérdida auditiva neurosensorial, tomando como base para evaluar los resultados el PTA (promedio tonal auditivo). Resultados: se agruparon 33 casos con prótesis de teflón y 15 de titanio. El cierre del gap óseo-aéreo menor o igual a 10 db fue del 80% para los casos de titanio y del 76% para los de teflón. La pérdida auditiva neurosensorial fue baja para ambos grupos sin diferencia significativa. Discusión: los resultados fueron similares para ambos grupos, pero se observó que la prótesis de titanio fue levemente superior. Los resultados descritos concuerdan con los reportados en la literatura, que muestran un rango en el cierre del gap óseo-aéreo menor de 10 db del 71% al 94%. El número pequeño de casos o la predilección por una de las prótesis puede influenciar el resultado descrito.

The effect of otitis media with effusion on perceptual masking.

OBJECTIVE: To determine the effect of otitis media with effusion (OME) on perceptual masking (a phenomenon in which spondee threshold for a 2-talker masker is poorer than for a speech-shaped noise masker). DESIGN: Longitudinal testing over a 1-year period following insertion of tympanostomy tubes, using clinical and normal-hearing control groups. SUBJECTS: Forty-seven children having a history of OME were tested. Possible testing intervals were just before the placement of tympanostomy tubes, and up to 3 separate occasions after the placement of the tubes. An age-matched control group of 19 children was tested. METHODS: A perceptual masking paradigm was used to measure the ability of the listener to recognize a spondee in either a speech-shaped noise or a 2-talker masker background. The masker was either continuous or gated on and off with the target spondee. RESULTS: In gated masking conditions, children with a history of normal hearing showed only slight perceptual masking, but the children with a history of OME showed relatively great perceptual masking before surgery and up to 6 months following surgery. In continuous masking conditions, both groups of children showed relatively great perceptual masking and did not differ significantly from each other in this respect either before or after surgery. However, before surgery, the OME group showed higher thresholds in both the 2-talker and speech-shaped noise maskers. CONCLUSIONS: In agreement with previous psychoacoustical findings, the relatively great perceptual masking in gated conditions shown by children with OME history may reflect a general deficit in complex auditory processing.