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Cochlear Implants and Auditory Brainstem Implants
Published in R James A England, Eamon Shamil, Rajeev Mathew, Manohar Bance, Pavol Surda, Jemy Jose, Omar Hilmi, Adam J Donne, Scott-Brown's Essential Otorhinolaryngology, 2022
Rajeev Mathew, Deborah Vickers, Patrick Axon, Manohar Bance
Assessment should be performed by a multidisciplinary team including but not limited to surgeons, audiologists and rehabilitationists. It is crucial that patients have realistic expectations of their hearing outcome and adequate pre-operative counselling is necessary. Behavioural audiological assessment involves age appropriate assessment of hearing thresholds and in adults also includes word recognition tests. This can be supplemented with objective measurements including otoacoustic emissions (assessment of auditory neuropathy spectrum disorder; see Chapter 16), auditory brainstem responses (ABRs; for hearing threshold estimation; see Chapter 4) and auditory cortical responses (for assessing benefit from hearing aids; see Chapter 4). This is particularly important in young children and in patients with suspected non-organic hearing loss. Transtympanic electrocochleography is occasionally used in patients in whom there is concern about the presence/function of the cochlear nerve.
Hearing Aids for the Pediatric Population
Published in Stavros Hatzopoulos, Andrea Ciorba, Mark Krumm, Advances in Audiology and Hearing Science, 2020
Katia de Almeida, Maria Cecíli Martinelli
Hearing loss of any type or cause may result in language deficits, poor academic achievement, reduced social skills and/or behavioral problems (Crandell, 1993). Therefore, hearing loss should be identified as soon as possible and remedied. The use of amplification is the first and most important resource that should be used to minimize the negative impacts of hearing loss on language development and academic performance (AAA, 2013, Bagatto et al., 2010; King, 2010). Therefore, a child needs hearing aids when there is a hearing loss of any type or degree of severity that may interfere with normal developmental processes, including minimal, mild, unilateral or Auditory Neuropathy Spectrum Disorder (AAA, 2013).
Paediatric Implantation Otology
Published in John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed, Paediatrics, The Ear, Skull Base, 2018
James Ramsden, Payal Mukherjee
First described in the mid-1990s,47 auditory neuropathy is a hearing disorder characterized by the presence of outer hair cell function (evidenced by the presence of intact evoked otoacoustic emissions and/or cochlear microphonics) but abnormal or absent auditory brainstem response. However, due to the multifaceted nature of the aetiology of this condition and the recognition that the abnormality is not localized to the cochlear nerve, but may involve other defects such as inner hair cell/synapse or a synchronization of the signals being transmitted, the terminology was expanded to auditory neuropathy spectrum disorder (ANSD).48
Chameleons, red herrings, and false localizing signs in neurocritical care
Published in British Journal of Neurosurgery, 2022
Boyi Li, Tolga Sursal, Christian Bowers, Chad Cole, Chirag Gandhi, Meic Schmidt, Stephan Mayer, Fawaz Al-Mufti
Damage to the vestibulo-cochlear nerve can cause auditory neuropathy, as the propagation of auditory information downstream of the mechanical and electrical conduction is disrupted.36 Notably, this damage leading to hearing loss can be caused by temporal bone fracture, trauma even in the absence of fracture, transient demyelination, and infarction due to stroke.37–39 As a FLS, hearing loss, in addition to the other otologic symptoms of pulsatile tinnitus and aural fullness, has been reported as a complication of IIH.3,40 The hearing loss is low-frequency and may also present with vertigo.40 About 1/3 of patients with IIH demonstrate prolonged interpeak latencies in auditory brainstem-evoked response, suggesting that the pathophysiology of this hearing loss is the ICP causing stretching and/or compression of the vestibulocochlear nerve and brainstem.40 Detection of this type of low-frequency sensorineural hearing loss may be especially helpful in diagnosing IIH, as it can be detected even when other symptoms are absent.41 Thus, audiological evaluations play an important role in the diagnosis of IIH, which should always be suspected in the presence of neurotological symptoms such as tinnitus, vertigo, aural fullness, and hearing disturbances.41
Auditory neuropathy in patients with features of tropical ataxic neuropathy in Tanzania
Published in Hearing, Balance and Communication, 2021
Ali F. Jaffer, Philip B. Adebayo
The following inclusion criteria were used to determine eligibility for this review:All patients were seen as first visits between 1 January 2019 and 31 December 2019.Complete audiometric and otoacoustic emission performed at the clinic with data recorded on the clinic’s practice management system.A probable diagnosis of auditory neuropathy is indicated on the patient’s report, based on the results of testing.Auditory neuropathy is suspected to be of late-onset and not attributed to any other known causes, e.g. hyperbilirubinemia requiring exchange transfusion.History of at least two of these symptoms indicated on their report: hearing difficulties, visual difficulties, peripheral neuropathy, gait ataxia, or imbalance.
Molecular testing for the study of non-syndromic hearing loss
Published in Hearing, Balance and Communication, 2020
Anna Morgan, Paolo Gasparini, Giorgia Girotto
To date, about 170 NSHL loci (67 autosomal dominant (DFNA) loci, 93 autosomal recessive (DFNB) loci, 6 X-linked loci, 2 modifier loci, 1 Y-linked locus, and 1 locus for auditory neuropathy), and 119 genes (39 DFNA genes, 64 DFNB genes, 11 DFNA/DFNB genes, and 5 X-linked genes) have been reported as causative (Hereditary Hearing Loss Homepage; http://hereditaryhearingloss.org/), and more than 400 syndromes associated with hearing loss have been described [5]. The introduction of next-generation sequencing technologies (NGS), such as targeted re-sequencing (TRS) and whole exome sequencing (WES) has dramatically increased the diagnostic rate of HL allowing the detection of both mutations in known deafness genes, as well as the discovery of new disease genes [6–9]. Moreover, thanks to the use of Comparative Genomic Hybridisation (CGH) and SNP arrays it has been possible to identify large Copy Number Variations (CNVs) that are now an emerging cause of hereditary HL [10].