77 3.2 Clinical evaluation and resonance frequency analysis in Down Syndrome 1. Introduction Down syndrome (DS) was first described in 1866 by Doctor John Langdon Down and the additional 47th (Trisomy 21) chromosome resulting in the associated physical characteristics was discovered in 1959 by Professor Jérome Lejeune. Affecting 1:1000 live births there are approximately 40 000 individuals with DS living in the UK [1]. The most common pathologies in children with DS are obstructive sleep apnoea, otitis media, hearing loss and cardiac disease [2]. Overall, Hearing impairment is reported to affect as many as 24.9–90% [3–6] of this group of children and can be permanent (15–24.9%) or transient (22–30%) [4,7]. Otitis media with effusion (OME) resulting in a moderate to severe conductive hearing loss affect 43–75.4% of children with DS [6,8,9], although the prevalence is dependent on age of the child. At the age of one year, 93% have a significant middle ear effusion [9]. By age of 7 years, 60% are affected and a declining trend is seen thereafter [10]. Longitudinal follow up of children with DS with hearing impairment demonstrated 88.8% receive ventilation tube insertion during their childhood (prior to the age of 18), often requiring multiple replacement procedures (mean 3.5) [11]. The presence of OME in children with DS results in significantly lower hearing levels with a mean pure tone average of 33.4 dB HL compared to those children with DS without OME 21.7 dB HL (p < 0.0001) [12]. Treatment of persistent OME results in remarkable improvement in hearing. These treatments include myringotomy, ventilation tubes insertion and hearing aids, either digital behind the ear or bone conducting hearing system. Ventilation tubes are required in 28.9%, a 13-fold higher requirement in DS patients as compared to an age matched control [5]. Interestingly patients are often rehabilitated with hearing aids regardless of surgical intervention [13]. As the majority of DS children require two or more sets of ventilation tubes, long term complications are common, especially in those who require 3 or more replacements. These include chronic tympanic membrane perforation 36.6%, atelectasis 29.3% and cholesteatoma 14.6% [14]. Spontaneous closure of perforations occurs in 33% of DS children which is increased to a 54.8% closure rate following primary tympanoplasty [15]. Considering the higher risks of ventilation tube in children with DS (infection, early extrusion, perforation and general anaesthetic) the National Institute of Clinical Excellence (NICE) recommends hearing aids as the initial treatment of choice for hearing impairment secondary to OME. Ventilation tube placement should only be offered as an alternative following multidisciplinary team discussion [16].
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