I just need an introduction and a literature review totalling up to close to 2000 words.
In the intro talk about the what FSHD is, overview of symptoms, prevelance, natural history of progression of disorder, some stuff on genetics but dont go too indepth , but enough to write about, the paper is about the hearing not genetics, (maybe degree of genetics disorder impacts degree of hearing?), talk about hearing – this is where lit review comes in – focus of our study is what happens in auditory pathway – have a rational on why we think there might be auditory issues in people with FSHD, becuase it is a degenretive neuromotor disorder – other neuromotor disordr have hearing or auditory neuropathy as part of their persentation – talk about some disorder that have this such as friedreich’s ataxia etc. talk about it in relation to auditory neuropathy – talk about auditory neuropathy – what it is, how it is diagnosed, how it is tested – pre neural responses – otoacuaotics emissions and cochlear microphonics and neural measurements such as ABR. Auditory neuropathy is when there is normal cochlea function however ABR is defective. Talk about some of the test protocols, what the test broadly does and what it is finds – don’t go into too much detial. don’t mention how we do the test, just what type of info we will retrieve.
Need to argue why we are doing the study .
In lit review, talk about all literature you’d find, if you find 5, just talk about the 5.
Facioscapulohumeral
muscular dystrophy, or FSHD, is a
genetic disorder that leads to the weakening of skeletal muscles. Typically
beginning in early teenage years with the loss of muscles in the face (facio),
shoulders (scapula), upper arms (humerus), legs or core, FSHD can spread to any
muscle. Around 20 percent will need a wheelchair by age 50.
Hearing loss has been reported in some
patients (Lutz et al., 2013), but underlying mechanisms and auditory function
in affected individuals have not been thoroughly investigated.
Participants in this study will be
children and young people with FSHD aged 6 years+ and a selection of
adult participants with FSHD. Recruitment will be carried out through the Royal
Childrens’ Hospital, Department of Neurology.
Study Design: A case-control study where findings from each FSHD
participant will be compared with those of an age, gender and hearing-level
matched control.
Correlation analyses will be used to
compare findings across the various auditory measures
(see Rance et al. [2021] for details).
at the end of the intro, we will look at speech perception in noise and our study is about that.
Test Protocol
Data collection will be carried out by
students working in pairs. The protocol
should take around 60-90 mins to complete for each participant (including
breaks).
·
Sound detection
thresholds (audiogram)
·
Auditory evoked potentials
(ABR)
·
Monaural speech
perception (CNC-words in quiet and noise conditions)
·
Binaural speech
perception (LiSN-S – 4 test conditions)
·
Hearing disability
questionnaire (SSQ)