Effect of Recurrent Otitis Media on Language Profile in Children with Fragile X Syndrome

Abstract

ObjectiveLanguage is almost always affected in fragile X syndrome (FXS), and a delay in language acquisition is one of the first characteristics. The aim of this work was to study the effect of recurrent middle ear infections on the language profile in boys with FXS. Study design: Prospective case series.SettingAcademic Medical Center.Subjects and MethodsThe present study was conducted on 30 males, ranging in age from 4–10 years. They were diagnosed as having a full mutation of DNA. The males were divided into two groups: Group A included 15 children with a history of recurrent middle ear infections more than four times per year during the first 4 years of life, and Group B did not have a history of recurrent middle ear infections during the first 4 years of life. Language assessments were done for all participants using the Standardized Arabic Language test.ResultsResults showed significant delays in language development in children with FXS. Relative strengths in semantics compared to syntax and pragmatics were observed in all boys. The recurrent ear infections of the boys played an important role in the language development delay. The mean of receptive, expressive, and total language age was better and higher among boys without a history of recurrent middle ear infections compared to boys with recurrent middle ear infections.ConclusionRecurrent otitis media in boys with FXS exacerbates the language problems that exist in this syndrome.

Mechanisms underlying auditory processing deficits in Fragile X syndrome

“…Wisniewski et al 94 peripheral hearing function. 104,105 It is thus possible that the detected ABR alterations may have resulted indirectly from impaired middle ear function. Indeed, more recent studies from FXS individuals without middle ear infection did not find any differences in the absolute peak latencies and IPLs of ABRs.…”

“…Prolonged I‐III IPL could be explained by a delay in the transmission of the auditory signal from the periphery to the brainstem nuclei, or a delay in the activation of the superior olivary complex itself. In these studies, the participants were not screened for sensorineural hearing loss and, because individuals with FXS are prone to otitis media, presumably had a wide range of peripheral hearing function . It is thus possible that the detected ABR alterations may have resulted indirectly from impaired middle ear function.…”

Trastornos del habla en el síndrome X frágil. Una revisión bibliográfica

“…Respecto al tipo de estudio, se observa que son dos las tendencias principales, por un lado, los estudios comparativos entre diferentes trastornos o en ausencia de éstos y por otro, los estudios de campo, repartidos casi equitativamente a la mitad. En concreto, los estudios de campo encontrados son 10 ( Roberts et al 2002;Murphy y Abbeduto, 2007;Warren et al 2011;Robles, 2011;Sterling y Abbeduto, 2012;Robles et al 2013;Badran et al 2013;Klusek et al 2016;McDuffie et al 2016;Nelson et al 2018) frente a los 9 estudios comparativos (Comblain y Elbouz, 2002;Roberts et al 2007;Barnes et al 2009;Koover et al 2012;Herráiz et al 2014;Haebig et al 2016;Puente et al 2017;Huelmo et al 2016;Friedman et al 2018).…”

“…Además, respecto al género de la muestra, es destacable que únicamente en 1 artículo se estudia el femenino de manera exclusiva (Sterling y Abbeduto, 2012) frente a los 11 que estudian el género masculino (Comblain y Elbouz, 2002;Roberts et al 2002;Roberts et al 2007;Barnes et al 2009;Robles, 2011;Koover et al 2012;Robles et al 2013;Badran et al 2013;Herráiz et al 2014;Haebig et al 2016;Friedman et al 2018). Por tanto, el resto de las investigaciones estudian tanto el género femenino como el masculino, aunque no distribuido equitativamente.…”

“…En cuanto a la metodología de los estudios es remarcable que en 3 ésta es únicamente familiar (Warren et al 2011;Klussek et al 2016;Nelson et al 2018) mientras que en otros 6 se añade la metodología escolar (Robles, 2011;Sterling y Abbeduto 2012;Robles et al 2013;Badran et al 2013;Herráiz et al 2014;McDuffie et al 2016). El resto de las investigaciones se desarrollan únicamente en el ámbito escolar.…”