The otological manifestations associated with autistic spectrum disorders

The otological manifestations associated with autistic spectrum disorders

International Journal of Pediatric Otorhinolaryngology 77 (2013) 629–634 Contents lists available at SciVerse ScienceDirect International Journal of...

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International Journal of Pediatric Otorhinolaryngology 77 (2013) 629–634

Contents lists available at SciVerse ScienceDirect

International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl

Review article

The otological manifestations associated with autistic spectrum disorders R.Y. Chin a,*, T. Moran b, J.E. Fenton c a

Department of Otolaryngology Head and Neck Surgery, Nepean Hospital Sydney, The University of Sydney, Australia Department of Otolaryngology Head and Neck Surgery, St. James’s Hospital, Dublin, Ireland c Department of Otolaryngology Head and Neck Surgery, GEMS University of Limerick, Limerick, Ireland b

A R T I C L E I N F O

A B S T R A C T

Article history: Received 17 September 2012 Received in revised form 4 February 2013 Accepted 6 February 2013 Available online 29 March 2013

Objectives: To elucidate the otological manifestations found in this increasingly commonly diagnosed condition. This paper will discuss the diagnosis, aetiology, pathogenesis, management and the outcomes of treatment. Study design: Systematic literature review. Materials and methods: The following databases were searched for articles pertaining to the otological manifestations of autistic spectrum disorders: MEDLINE, EMBASE, CURRENT CONTENTS, PSYCHLIT, CINAHL and HEALTHSTAR. Articles from 1965 to June 2012 were extracted. Relevant articles from the literature were selected and reviewed by two independent authors. Each paper was assessed as to its level of evidence and validity. The relevant results are presented and discussed in order to present a practical approach to the management of these patients. Results: Patients with ASD have an increased incidence of peripheral and central otological pathology. This pathology plays a key role in the behavioural, communication, and social aspects of the disease. ASD patients have a higher incidence of profound sensorineural hearing loss, middle ear infections, and abnormalities of the cochlear nerve and brainstem auditory pathways. There are cortical and brainstem neurodevelopmental abnormalities in the way auditory information is interpreted and processed in the ASD patient. Conclusions: The otolaryngologist plays a key role in the multidisciplinary management of individuals with ASD due to the high prevalence of otological pathology amongst these patients. Early diagnosis and expedient treatment focusing on normalisation of auditory input and development can maximise developmental outcomes. ß 2013 Elsevier Ireland Ltd. All rights reserved.

Keywords: Autistic spectrum Otological abnormalities Hearing loss ENT assessment of autistic spectrum disorders Auditory processing ASD and ENT

Contents 1. 2. 3. 4. 5. 6. 7. 8.

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Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Materials and methods . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Aetiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Introduction: practical clinical approach . . . . . . . . . . . . . . . . . . . . . . . . . . . Peripheral manifestations of auditory abnormalities in autistic spectrum . Central manifestations of auditory abnormalities in autistic spectrum . . . Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Consistency of auditory inputs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8.1. Addressing conductive hearing loss . . . . . . . . . . . . . . . . . . . . . . . . . . 8.2. Amplification. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8.3. Cochlea implantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Educational interventions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9.1. Applied behaviour analysis (ABA) . . . . . . . . . . . . . . . . . . . . . . . . . . . 9.2. Structured teaching (TEACCH) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Discussion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

* Corresponding author. E-mail address: [email protected] (R.Y. Chin). 0165-5876/$ – see front matter ß 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijporl.2013.02.006

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1. Introduction ASD is an umbrella term which includes impairments in three major domains. These are socialisation, communication, and behaviour [1]. ASD is a ‘‘Pervasive Developmental Disorder’’ (PDD) and includes autistic disorder, childhood disintegrative disorder (rare condition where a child learns skills, then loses them by age 10), Rett’s disorder (a rare developmental condition seen almost exclusively in females), Asperger syndrome (similar to autism, but with normal language development) and ‘‘Pervasive Developmental Disorder not otherwise specified’’ (PDD-NOS) or atypical autism. The otolaryngologist must appreciate that ASDs are associated with specific hearing and various auditory processing abnormalities along the entire length of the auditory pathway. An understanding of these abnormalities is essential to the early diagnosis and management of otological pathology in this group of patients. The inability to hear and effectively process auditory information is central to the deficits in socialisation, communication, and behaviour experienced by individuals with ASD. It is the role of the otolaryngologist to diagnose and expeditiously treat correctable otological pathology. This will maximise the effectiveness of any psychological and behavioural treatments. 2. Materials and methods The following databases were searched for articles pertaining to the otological manifestations of autistic spectrum disorders: MEDLINE, EMBASE, CURRENT CONTENTS, PSYCHLIT, CINAHL and HEALTHSTAR. Articles from 1965 to June 2012 were extracted. Relevant articles from the literature were selected and reviewed by two independent authors. Each paper was assessed as to its level of evidence and validity. The relevant results are presented and discussed in order to present a practical approach to the management of these patients. 3. Aetiology ASD has a prevalence of approximately 6.7 per 1000 live births; its incidence is most often quoted as 1:150 live births [2]. Since the 1990s its prevalence appears to be increasing and it is unclear whether this is due to an actual increase or to earlier and more accurate diagnosis of ASD [2]. It is becoming more apparent that the aetiology of ASD is multifactorial, with a variety of genetic and to lesser extent environmental factors playing a role. Phenotypic heterogeneity is a characteristic feature with multiple chromosomal abnormalities identified [3]. Zhao et al. has proposed a unified genetic theory for the inheritance patterns of autism. This theory proposes that there are two major patterns of inheritance in ASD – sporadic (simplex) and familial (multiplex) [4]. Both patterns involve mutations in multiple loci over the entire genome [5–7]. The theory may provide some explanation for ASD’s preponderance for males, its increased incidence in line with increased paternal age [8,9] and the high concordance rates in monozygotic twins of around 60–90% [7]. It is only since 1999 that a documented association between ASD and hearing loss has been appreciated. The incidence of sensorineural hearing loss is 10 times greater in ASD compared with the normal population; 3.5% vs. 0.1–0.2% [10,11]. Children with ASD have a pervasive developmental disorder which exists along a spectrum rather than as an isolated condition. Therefore it is reasonable to ask whether children of different subgroups of this condition are more likely to have differences in their hearing loss. Although no studies were identified which

specified differences in hearing loss for defined subgroups, differences in hearing loss have been explored along a spectrum of higher of lower levels of function within autism. Rosenhall et al. found that deficits in these children were similar across all levels of functioning, concluding it does not appear that the covariation with intellectual impairment can account for all of the variance of hearing deficit in autism [10]. Konstantareas and Homatidis however found that lower-functioning children had an earlier onset of ear infections than their higherfunctioning autistic peers and that ear infections coexisted with low-set ears, and with a higher autistic symptomatology score [12]. 4. Introduction: practical clinical approach In the otolaryngologists clinic there are two scenarios that we may be likely faced with. Firstly, our role in advising families when either we or the parents themselves suspect a behavioural disorder or global development delay which may be ASD. Secondly, we have a role in the management of a child with a previous diagnosis of ASD. In order to have any involvement in what is a very complex and emotive diagnosis, we must have a working knowledge of the behaviours and symptoms found in ASD. We must highlight the diagnosis is made as part of a multidisciplinary team. There are however certain behaviours which parents may describe which may lead us to suspect ASD. Firstly in the realm of communication, there are both verbal and non-verbal communication difficulties associated with ASD. Parents will likely first notice these as a developmental delay. At one year of age parents may notice their child cannot babble, point or respond to their own name and have no words by 18 months of age. There are also more subtle idiosyncrasies. An older child may display a very literal sense of language, interpreting words exactly as they mean. This may restrict their ability to understand jokes or sarcasm. Non-verbal communication difficulties would include inability to correctly interpret facial expressions or body language. Older children will have difficulty starting and maintaining the natural flow of conversation. From a social perspective parents may highlight that their child does not engage with the family and other children in a normal fashion, preferring to spend time alone. They have difficulty making and maintaining friendships with their peers. Parents may feel that their child resists affection and shows no understanding for other people’s thoughts and emotions. Older children with ASD often show little interest in play which involves make believe and imagination and may display a disregard for simple social rules and find it difficult to manage their emotions which may result in aggressive behaviour and outbursts of anger. Naturally, this may lead to difficulties at school. A key aspect to ASD is that of repetitive behaviours and a tendency to like sticking to the same routines. If these are disturbed, this can lead to significant distress to the child. Children with undiagnosed ASD may also present to otolaryngology outpatients for the assessment and or optimisation of hearing problems within the context of other conditions which may coexist with ASD. These include attention deficit hyperactivity disorder (ADHD), dyspraxias and epilepsy. It is likely that children with these problems will present to the otolaryngology outpatients clinic as often parents and others caring for the child may believe that difficulties are based on an audiological deficit. Children may also attend with quite the opposite complaint that of hypersensitivity to stimuli especially of sound, smell, touch and taste and this should pique the attention of the otolaryngologist to the potential of an underlying pathology.

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As highlighted earlier it is not the role of the otolaryngologist to make a diagnosis of ASD but to direct children whom we suspect to have these problems for appropriate assessment and management. This may be to the psychology department or a dedicated multidisciplinary unit depending on local availability of resources. The other likely scenario in which we may encounter these children is through the referral of children with a previous diagnosis of ASD. Motor dysfunction is associated with ASD and these children may be referred with balance problems. More commonly however, we are usually asked to see these patients for the assessment and potential treatment of otological problems as a contributory factor to their difficulties. ASD patients have higher incidences of recurrent middle ear infections, sensorineural hearing loss, hyperacusis, and corresponding deficits in auditory processing [10–17]. Firstly we must consider our approach to the child with ASD in the OPD. Practical issues which we must be conscious of in the setting of the clinic with these patients are based on established characteristics seen in these children. In responding to other people, we must anticipate that language deficits may be greater than expected for age. This may be characterised by delayed speech and limited vocabulary. Responses to the child’s name may be absent or delayed. These issues may be misinterpreted as a hearing deficit. Body language and facial expressions may not be interpreted as expected as may understanding of the intentions or the requests of others. Some children with ASD may unexpectedly reject affection from parents, carers or others. Interactions with others there may be a reduced or absent appreciation of the personal space of others, little social interest or a lack of initiation of play or enjoyment of situations which we would expect to see in other children of the same age. We may see socially unacceptable behaviour or a lack of turn taking, eye contact, pointing or other gestures. We may see unusual or restricted interests and repetitive behaviours and movements with highly specific interests and an excessive insistence on things being a certain way. When it comes to examination there may be an excessive reaction to any physical contact or discomfort. We may also see social and emotional development which is more immature than other areas of development. Thus, as much as is feasible, it is important to create an environment conducive to successfully assessing these children. This involves making the consultation as non-threatening and familiar as possible for the child. Key elements are:  Setting aside more time than normal – sometimes up to 45 min for a consultation.  Involving the parents, allied health and carers who the child is familiar with during the consultation.  Using an interview room rather than a normal consultation room as this provides a setting more in keeping with what the child is most familiar with.  The utilisation of the child’s favourite toys. We have found that following these relatively easy steps, that this enables the clinician to perform adequate otological examinations in a way which minimises stress to the child and allows accurate clinical diagnosis. Also as the child grows to know the clinician, he too becomes part of the child’s ‘‘team’’. As these children have been shown to have a higher incidence of middle ear effusions, where possible, all should undergo impedance audiometry to document objectively the status of the middle ear. Furthermore as it may be difficult to visualise the tympanic membrane in these children, a tympanogram may be possible where examination with an auroscope has failed.

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Tharpe et al. found that in audiometric evaluation of children with ASD, these children were more likely to indicate that they did not hear normally for at least one test stimulus when, in fact, other measures verified normal to near-normal hearing sensitivity [18]. When an abnormal pure tone audiogram is found in these children, auditory brainstem response (ABR) audiometry should be considered as an objective measure. Furthermore, in children who cannot be assessed properly due to behavioural issues already outlined, it is justified to perform an ABR in order to document hearing status objectively. Unfortunately the ABR is poor at picking up mild hearing loss so this may not be an ideal measure for some of these children. In children with mild to moderate hearing loss it is important that they are followed to make sure that the hearing loss is not progressive. Even following a normal ABR and in the absence of previous middle ear disease, an annual assessment including a pure tone audiogram is deemed reasonable by the senior author. This leads us to the auditory manifestations seen in ASD. These can be divided into peripheral and central. Peripheral abnormalities found more frequently in ASD are recurrent middle ear infections, an increased incidence of sensorineural hearing loss, and hyperacusis [12,14–18]. Central abnormalities are mainly related to the abnormal processing and interpretation of auditory stimuli in the cortex and limbic areas of the brain. As it is the peripheral deficits which have the most relevance to the paediatric otolaryngologist in terms of treatment, an in depth exploration of the cortical and brainstem abnormalities (central) in ASD is beyond the scope of this paper. 5. Peripheral manifestations of auditory abnormalities in autistic spectrum It is important for the otolaryngologist to have an understanding of the otological problems associated with ASD. Not only will this aid us in the interpretation of problems found in these children but within the context of behaviours discussed above. Compared to the general population, individuals with ASD appear to have a preponderance to upper and lower respiratory tract infections and an associated increased incidence of middle ear pathology such as otitis media [13]. This can exacerbate an already ten fold increased incidence of sensorineural hearing loss [19]. Thus, ASD patients with frequent ear infections are associated with a lower functional status than those with less frequent infections. Also, ASD is associated with external structural abnormalities, for example low set ears [13]. Multiple studies have demonstrated significant hearing loss within speech frequencies in these children. The majority of individuals had a 16–40 dB hearing loss, with approximately 8% with mild loss of 10 dB and 1% with severe loss of 50 dB. The consequences of this hearing loss in autistic individuals are profound with impairments of language, learning, attention and behavioural characteristics [13]. Speech-in-noise perception difficulties have been observed for individuals diagnosed with ASD, which in part are thought to reflect abnormal peripheral auditory processing [20]. Patients with ASD have pure tone audiograms which differ significantly from normal individuals. They appear to be characterised by having unusual peaks and troughs or perturbations [19,20]. These abnormal audiographic measurements have been attributed to dysfunctional middle ear muscle hyperactivity or hypersensitive groups of hair cells within the cochlea. It is suggested that the hyperacusis with subsequent aversive behaviours to sound reflect atypical auditory processing at a sensory or peripheral level. Children with ASD frequently exhibit this trait. Hyperacusis has been reported to be as high as 18% in the autistic

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population [10]. O’Riordan and Passetti report evidence of enhanced sensitivity for detecting differences in pitch in ASD [21]. Studies examining the auditory brainstem response from autistic individuals also seem to implicate functional deficits in the lower auditory brainstem [22–24]. Looking at objective measurements, the ‘‘Auditory Brain Stem Response’’ (ABR) which corresponds to peripheral structures in the brainstem and in the auditory nerve can highlight differences within this population. Several large studies involving the use of ABR suggest that a proportion of individuals with ASD may have a prolongation of Waves I–III, which may be a sign of retro-cochlear pathology or suggesting cochlear involvement [25–27]. The latency prolongation of Wave I seen in autistic individuals is often shared with patients with tinnitus [25] and may reflect a ‘‘slowing down’’ of synaptic processes in the organ of Corti. The Wave I abnormality may also be explained by pathology affecting the crossed olivocochlea bundle (COCB). This efferent system consists of crossed neurons in the medial olivary complex and vestibular nerve, which travel to the cochlea. It is thought that this system acts on the outer hair cells, which in turn modulate cochlear function. This system is thought to have a role in enabling the ear to develop a resistance and modulate noise trauma [25]. This may thus have a contributory effect to the reported hyperacusis of individuals with ASD. 6. Central manifestations of auditory abnormalities in autistic spectrum Neuropsychological studies show that the most commonly recognised auditory deficit in the ASD patient is the inability to filter auditory input [27]. In an extensive study of 101 children and young adults with mild autism, Rosenhall et al. reported that the majority were found to have abnormalities in the auditory brainstem [25]. This supports the findings of previous studies suggesting that brainstem dysfunction is involved in ASD. This also correlates with findings on ABR, which show abnormal patterns in children with ASD. One theory which may explain these differences is that of the classical and non classical pathway for auditory information transmission. The classical pathways are specific to each sense and are seen as more developed whereas the non-classical pathways are phylogenetically more primitive and the neurones within it less specialised and broadly tuned [28]. As part of this theory, the non-classical pathways contain multiple connections to the somatosensory pathways and other sensory systems and can thus respond to stimulation from these systems. A higher level of non-classical pathway activity in these patients could explain abnormal responses to auditory input. Furthermore, this theory has been used to explain other auditory processing abnormalities, for example in tinnitus, hyperacusis and phonophobia. These symptoms may be as a result of direct involvement of non-classical pathways. This would allow unprocessed auditory input to reach the amygdala through a subcortical route via the thalamus. This theory has also been used to explain the beneficial effects of therapeutic interventions used in autism [29]. A further contributory factor in autistic individuals may be morphological or functional abnormalities of the amygdala and its connections [30]. As the amygdala has long been associated with processing emotional and memory responses with specific reference to sensory stimuli from a variety of inputs, one can easily imagine how this may lead to distress. Another interesting aspect of auditory anomalies seen in ASD is that of auditory discrimination ability. Jones et al. studied the

auditory discrimination ability in ASD patients and the resultant effects on behaviour. They concluded that the variable ability in the population group to discriminate between frequency, pitch and duration of sound influences behaviour [31]. Bonnel et al. found that individuals with high-functioning autism were found to be superior to controls at frequency discrimination and frequency categorization [32]. This may lead to an abnormal focus of attention and a reported under-reactivity to other sounds, for example the sound of oncoming traffic when crossing the street. Failure to process auditory information automatically outside the individual’s current focus of attention has further significant impact on socialisation, cognition and aspects of language development [33]. 7. Management The best results are achieved by the co-operative efforts of the otolaryngologist, paediatrician, primary care physician, psychologists, and the patient’s family [34]. We will focus here on how the otolaryngologist can play a role in both the diagnosed ASD patient and in the patient who we are suspecting a behavioural disorder, which may be ASD. There is considerable evidence that early intensive appropriate treatment programmes will greatly improve the outlook for most young children with autism [35,36]. As we have seen, there are many reasons why these patients may present to the otolaryngology outpatients prior to a diagnosis. Thus, as discussed above it is vital that the otolaryngologist is familiar with both the otological manifestations seen in ASD and behaviours which could lead us to suspect ASD. This will facilitate expeditious referral to the appropriate services. As a key component of the multidisciplinary team the otolaryngologist must have an appreciation of the otological difficulties faced by children with this condition. This will aid in any interactions with the patient and in the interpretation of the results of audiological testing. As already discussed, there is a higher incidence of hearing loss in these patients. One of the key roles of the otolaryngologist is to identify hearing loss early and treat it aggressively. Another problem that we as otolaryngologists may be called on to address is that of hyperacusis. Often patients with ASD use earplugs or earmuffs to protect themselves from the discomfort of loud noise. This may however further poor tolerance to relatively soft sounds. A better treatment for some may be sound desensitisation. Patients undergoing this treatment listen to barely audible static noise for a set period of time each day. Over time, tolerance to sound is built up, and sensitivity to normal sounds is less painful. In order to enter into a meaningful dialogue with other members of the multidisciplinary team it is important that the otolaryngologist has an understanding of the behavioural modalities used in the treatment of patients diagnosed with ASD. Equally, any intervention by other members of the team will be greatly hindered if there is significant hearing impairment that should be addressed by the otolaryngologist. We will concentrate on two specific areas of treatment; firstly, interventions to maintain consistency of auditory inputs and secondly, specific educational interventions. 8. Consistency of auditory inputs In order to encourage neural plasticity and proper auditory development, appropriate management should attempt to make auditory input as consistent as possible. These strategies aid the effectiveness of educational or behavioural interventions being applied but is particularly important for therapy aimed at changing

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This evidence-based method applies psychological research to systematically change behaviour. ABA aims to teach children skills to adapt new situations and therefore reduce negative behaviours and increase positive behaviours [38]. Children who receive ABA have significant, long-lasting improvements in IQ, language, academic performance, and adaptive behaviours [39,40].

dysfunction can potentially exacerbate maladaptive behaviour, augment a negative relationship between the patient and their surrounding environment, and delay already impaired functional development. When this abnormality of peripheral processing is combined with brainstem and cortical auditory processing abnormalities, the effect becomes even more significant. In children suspected of ASD, it is important to delineate accurately the extent of hearing impairment and if possible treat this accordingly. Autistic individuals are difficult to test. They often have deficient co-operation, attention deficits, and cognitive dysfunction. Lack of cooperation may preclude psychoacoustical test procedures. Poor attention and cognitive problems make it difficult to perform reliable threshold estimates. Often it is necessary to perform a test several times until an assessment can be made as to the reliability of the tests performed. Lack of good reliable testing can result in over- or under-estimation of hearing impairment. Through an understanding of the central and peripheral otological abnormalities the otolaryngologist is of prime importance in liaising with audiological and paediatric colleagues in helping achieve accurate testing and diagnosis. It is known that there are central auditory processing abnormalities in individuals with even the mild form of ASD and these patients may not tolerate hearing impairments caused by otitis media (OM). Given that OM can cause speech and language delay in normal individuals without ASD, there may be a significantly more severe impact on those who have ASD and a conductive hearing loss. Individuals on the more severe end of the ASD spectrum may be more prone to recurrent infections of the upper respiratory tract and middle ear. These patients may have abnormalities in the middle ear muscle reflexes (contributing to hyperacusis), and an increased incidence of deafness and sensorineural hearing loss. Sensory experience is critical for the development of neuronal circuitry. Avoidance of essential sensory input or inappropriate focus on particular sensory experience is likely to compromise the typical neuro-developmental trajectory. This will have down-stream implications for perceptual, cognitive and social maturation. Thus, every opportunity should be given to maximising their peripheral auditory function by appropriate otolaryngological management. Expeditious treatment with antimicrobial agents and grommet insertion may provide a significant benefit and minimise time where audiological input is compromised and impairs behavioural treatment regimes.

9.2. Structured teaching (TEACCH)

11. Conclusion

This method was developed by Schopler and colleagues and whilst originally known as TEACCH is now most commonly called ‘‘structured teaching’’. Structured teaching utilises visual cues and schedules to provide an environment that is structured and predictable in its progression of events. The method utilises routines, with built-in flexibility to their structure. The aim is to maximise the child’s ability, whilst compensating for areas that are less developed in a given environment. Studies show that children who received this programme in their homes in addition to a day programme had better outcomes than children who only received a day programme [40].

ASD is a challenging and difficult management problem. Hence it necessitates multidisciplinary management. The otolaryngologist has a key role to play in helping to manage auditory pathology in individuals with this disease process. Patients need to be assessed clinically and tested on several occasions before an accurate assessment of the patient’s hearing ability is known. When an abnormality is found, such as middle ear effusions distorting auditory input, it should be managed expeditiously. The goal is to maintain optimal conditions for the individuals’ development and response to behavioural treatments. The brainstem and cortical auditory processing abnormalities, which are currently not amenable to surgical correction, may be easier to manage if the peripheral inputs feeding these areas are kept as consistent as possible. The aim is to enhance the patient’s quality of life and maximise their functional status through timely intervention by the otolaryngologist.

negative behavioural responses, for example, head banging associated with misinterpretation of auditory stimuli. Specific strategies to encourage consistent auditory input include: 8.1. Addressing conductive hearing loss The authors advocate the accurate diagnosis and effective and expeditious treatment of conductive hearing loss via appropriate treatment with antimicrobial therapy or grommet insertion. 8.2. Amplification ASD patients with hearing loss benefit from personal amplification devices to provide maximum access to all of the acoustic features of speech within an intensity range that is safe and comfortable [36]. 8.3. Cochlea implantation Cochlear implantation in ASD individuals is, at this stage, uncommon. To date, all studies published have small numbers and only one deals specifically with ASD patients. Donaldson et al. measured auditory gains made by children with ASD post cochlear implantation. These patients achieved gains in audiological and behavioural parameters post-implantation and generally demonstrated an improvement in their quality of life. However, these gains were less than those experienced by individuals without ASD and the post-op rehabilitation process was more challenging and involved [37]. 9. Educational interventions A detailed discussion of the educational interventions applied in the treatment of ASD is beyond the scope of this paper but the authors would recommend the following two therapies as they have the strongest evidence base of the available therapies [38]. 9.1. Applied behaviour analysis (ABA)

10. Discussion Increased awareness of the potential otological impairments in patients with ASD has important clinical implications. The behavioural and developmental effects of middle ear and cochlea

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