The (Educational) Needs of Children with Comorbid Autism and Deafness


A description is offered in these notes of the frequency of the association of autism and deafness.

Reference is made to the complexity of evaluating hearing deficits among children who are autistic, and to two assessment measures based upon evoked neural activation. 

There follows a sampling of observations or concerns expressed by professionals and parents concerning the means of determining the relative effects of autism and of deafness (and their confoundability), and of planning intervention.

The conclusion highlights the significance of a series of consultations and reviews involving multi-contributions by which to monitor progress and modify strategies on the basis of observed responses to the treatment for the hearing deficit and to the initial autism-oriented educational provision.


M.J.Connor                                                                                              January 2008


The (Educational) Needs of Children with Comorbid Autism and Deafness



Joint Diagnoses of Autism and Deafness


In their introduction to a study of autism and hearing loss, Rosenhall et al (1999) note that evidence for the aetiology of autism has included significant support for an organic basis in that various clusters of symptoms characteristic of autism implicate both cortical and sub-cortical regions.


One hypothesis has it that the anomalous responses among children with autism to sensory stimuli (either an under- or an over-reaction), and a predilection for stereotyped behaviour and motor activity, are a function of abnormalities in the brainstem.

Meanwhile, the identification of impairment of communication as a core deficit in autism has led to much debate concerning the aetiological role of deficits in the hearing system ... and reference is made to cases where there is a common underlying source of both deafness and autism (such as the impact of rubella). 


In respect of existing statistics concerning the frequency of an autism-deafness comorbidity, the authors referred to the study of Jure et al (1991) whose large sample of children identified with a hearing impairment included 5·3% who were also identified with autism. 

Those children showing both autism and a hearing impairment were found more commonly to show neurological or congenital anomalies than children identified with only one or other of the conditions. 


There has been the suggestion (see, for example, Smith et al 1988) that deprivation of sensory input, notably the experience of a hearing deficit in early childhood, may be a contributory factor in the development of autism. 

However, in the study by Jure et al there was shown to be no correlation between the severity of the hearing impairment and severity of autistic symptomatology. 

In other words, this finding may be taken as a contradiction of the hypothesis linking autism with sensory deprivation ... (and, presumably, the implication is for avoiding any assumption that, among children with a validly firm diagnosis of autism, there will be significant reductions in the presenting symptoms of autism as a result of improvements brought about in their hearing capacity). 


One could reinforce this point by citing the evidence from the review of Iarocci and McDonald (2005) that parents of infants identified with autism frequently report sensory abnormalities observable at a very early developmental stage, such as an over-reaction or pre-occupation in respect of certain stimuli but a marked non-responsiveness to others (including the calling of their names).   Atypical behaviours may persist throughout the development of children with autism, in the absence of any sensory deficits such as a hearing loss. 


Nevertheless, it should be acknowledged that one should not underestimate, either, the additive and negative impacts of a combination of autism and hearing loss, with implications for diagnostic precision in determining the spectrum of needs.


One should also recognise that, among children who have experienced a profound hearing loss but who do not meet the criteria for a diagnosis of autism or ASD, there is evidence that their social relationships may well improve following a successful cochlear implantation. The “mechanism” is assumed to involve an improvement in communication skills and correspondingly better interactions with peers. 


However, in their review of evidence, Bat-Chava et al (2005) describe how the outcomes may vary widely across even these children who have had no other condition diagnosed alongside the hearing loss, and that long-term residual social weaknesses may be observed. 

Their own study highlighted the variability of functioning among children with implants, reflecting differences in the age of the children at the time of the implant, duration of implant use, the mode of communication, and cognitive factors.

(Among the studies cited in this review was that of Delgado et al [1982] who described commonly lower levels of social maturity among children with a history of deafness compared to normally-hearing children, but the evidence suggested that this weakness was more attributable to such variables as family or school environments, the quality of inter-communications, or parental handling, rather than directly to the hearing loss.)    


It has also been demonstrated that autism and mental retardation difficulty are frequently associated.  Relevant to the current issue is a review of findings by these present authors suggesting an incidence of around 7% of hearing impairment among samples of people with mental retardation, further reinforcing the view of a greater than chance probability of some linkage between autism and deafness. 


Accordingly, Rosenhall et al completed a large scale study to determine the prevalence of hearing deficits or hearing loss in individuals identified with autism.

Children and adolescents identified with ASD and subject to ongoing neuro-psychiatric and medical monitoring were selected for inclusion in the study.

(The study was acknowledged to be clinic-based, but reference was made to existing evidence [Gillberg 1992] that, in the region selected, population-based and clinic-based samples of children and young people with autism have not shown significant differences.)


The study sample included 153 boys and 46 girls with a mean age at the time of audiological assessment of 7 years for the boys and 8·4 years for the girls (albeit with a wide range across cases); and the diagnosis of autistic disorder had been confirmed at or after 3 years of age in all cases. 

Assessment procedures included audiometry and auditory brainstem response examination; tympanometry; testing for hyperacusis; and a defining of the degree of hearing loss.


(The authors describe how one major problem in a study of the prevalence of hearing loss among individuals with autism is that of ensuring validity of results given difficulties in respect of limited cooperation, attention deficits, and cognitive anomalies.  However, repeated assessment sessions and  psycho-acoustical or auditory brainstem response techniques were implemented to increase the meaningfulness of the data, although the sample was subdivided into those who had been reliably tested and those considered difficult to evaluate (among whom a severe hearing loss was readily identifiable, but mild to moderate losses were not so accurately assessed).


The results included a prevalence figure of “pronounced” or “profound” hearing loss or deafness of 3·5% which is higher that that typically observed in samples drawn from the population at large. 

Among an unselected population of children, the incidence of a profound hearing loss is more likely to be around 0·1 or 0·2%, thus indicating that hearing disorders are much more prevalent in populations identified with neurological and developmental dysfunctions. 


The authors went on to describe how hearing deficits in autism occurred at similar rates at all levels of intellectual functioning. Thus, even if mental retardation is also associated with a hearing loss, it appears that the covariation with intellectual impairment cannot account for all of the variance of hearing deficits in autism.


Their conclusion emphasised the point that those children with a combination of autism and hearing loss will have communication problems of a much greater severity than would be the case with either condition alone. 

Their recommendation is for careful assessment of the hearing status of all children identified with autism and ASD, and to implement the aural habilitation as necessary while establishing the most suitable communication system to meet the needs. 


(The implication that one might tentatively draw is that, in the case of a child with a clear diagnosis of both a significant/severe level of autism and of hearing loss, it is necessary to await the outcomes of the intervention concerned with the hearing needs ... such as the activation of an cochlear implant ... in order to determine the most appropriate system and support by which to maximise communication. 

However, whatever the gains observed in this domain, and whatever the changes in the available level of communication and in associated day to day functioning, the symptoms and needs associated with the autism will remain.  The curricular goals and the associated steps and strategies will still need to be appropriate for a child with autism, albeit delivered in such a way as to take account of the hearing status and of the required access to the aids and techniques and support recommended.)


Assessment of Auditory Abilities


As a brief addendum to these notes, one can cite descriptive studies highlighting the nature of the acoustic and brainstem-response methodologies which have been described as important in the identification of hearing deficits among children with autism.  


The notes by Tas et al (2007), for example, begin with an acknowledgement that the assessment of hearing is important for the full diagnosis and comprehensive treatment of children showing autistic behaviours.  However, their frequent unresponsiveness makes for difficulties in gaining meaningful data from standard assessment procedures ... hence the recourse to alternative measures.


The Auditory Brainstem Response (ABR) involves the detection and recording of activity in the auditory pathway from the acoustic nerve to the higher mid-brain structures (ie there is no dependence upon a “conscious” response on the part of the child to some auditory stimulus). 

Converging evidence has attested to the reliability of this approach.


Transient Evoked Otoacoustic Emission (TEOAE) reflects the cochlear’s active response to sound stimulation.  This method is also described as accurate, while non-invasive and easy to administer. 

The evoked emissions are reduced where there is a sensorineural impairment, and usually absent in the case of a sensorineural hearing loss exceeding 30dB hearing level.  


Tas et al note that an audiology clinic may well be the first setting in which a child, ultimately diagnosed with autism or ASD, is presented; and they confirm that hearing loss appears to be more common in children with autism than among typically-developing children.

What may be thought to be of particular significance is their conclusion about the importance of detecting any hearing loss to prevent any possible failure to respond to the education of autism.

While the diagnosis of autism is eased and more confidently made when hearing is normal, the presence of a hearing impairment does not reduce the significance of the autism.


Diagnostic Considerations    


The study by Roper et al (2003) begins by referring to existing findings in respect of the higher than chance rates of comorbidity involving autism and deafness, and of the likelihood that early communication difficulties may lead to the consideration of either diagnostic possibility, with the possible implications for missing one or other of the conditions when they co-exist or for a delay in completing a diagnosis (and for the provision of the most effective intervention). 


Their own study investigated whether late diagnoses of autism, developmental delay and/or deafness, were evident among three groups ... children both deaf and autistic, children with deafness and learning disability, and autistic children with normal hearing. 

It was predicted that diagnosis would be more difficult (and, thus, more likely to be delayed) among children with comorbid autism and deafness. 


Their results included the pattern among parents of a higher mean age of the children suspected of having developmental disabilities in the case of the hearing autistic children compared to the deaf-autistic or deaf-learning disabled group, indicative of greater or more observable difficulties among children experiencing these “joint” conditions ... particularly so among the deaf-autistic group.

However, the median age at which parents first began to suspect hearing problems was the same for the deaf-autistic and deaf-learning disabled group ... (with the possible implication that the matter of deafness may have diverted attention from alternative attributions for some of the observed difficulties).


No significant differences were found between the deaf autistic and hearing autistic groups in respect of the (current) level of autistic behaviours, consistent with existing findings of no difference between those with and without deafness in the severity of autistic symptoms ... (ie it would seem dangerous to assume that successful intervention in regard to compensating for the deafness would lead to a moderation in overall symptomatology given that the autistic symptoms remain significant in their own right).     

Further, there was no sign or symptom specific to the deaf-autistic group that would have allowed for an earlier identification. 


The conclusion from Roper et al, with the proviso of caution in relying upon retrospective parental information amongst other methodological issues, was that late diagnosis has major implications for the assessment and remediation of these children and in determining whether they are to be educated as primarily deaf, or deaf and autistic.

They hold that the presence of autism will necessarily require specificity in the nature of educational provision. 

As argued by Jordan and Powell (1995), children with autism require access as early as practicable to educational strategies that are adapted to their particular style; but a failure to recognise the autism in comorbid deafness and autism may lead to placement in a provision designed primarily for children who are deaf or partially hearing such that their idiosyncratic and autism-related needs are not met.


As far as differential diagnosis is concerned, observations on the part of a small sample of professionals would suggest that children whose “primary” need is deafness or hearing loss do not usually demonstrate significant stereotyped behaviours and activities. However, this kind of symptom cluster is typically a characteristic of children with autism and ASD whatever their hearing status.


Meanwhile, evidence from case studies at the Kennedy Centre for Research (Einstein College of Medicine, New York) has reinforced the view that, among children both deaf and autistic, the severity of the autistic behaviour may be related to the severity of cognitive deficits but not to the severity of the hearing loss.  It was also noted that the (early) recognition of one of the conditions inhibited the identification of the second in around 25% of the cases described, with implications for a failure to match the full profile of needs with appropriate intervention and methodology.


A set of personal experiences described by a parent of a child diagnosed first as deaf and later as autistic (Beals 2004) includes the observation that neither the “deaf intervention” nor the “autistic intervention” provided the appropriately sensitive match to the joint needs.  The implication is that it is necessary to clarify the range of needs and to plan intervention accordingly in terms of goals and the precise nature of the manner in which the strategies are organised .... complicated, Beals believes, by controversies in both deafness and autism concerning, respectively, signing versus oral approaches, and developmental versus behaviourist approaches.


Her experience indicates that deafness may be considered as a more straightforward disability in terms of the basic needs... ie inaccessibility to sounds and speech ... and of the range of well-tried methods for fostering signed and spoken language.


The problem concerns how best to manage a combination of needs, and Beals describes how the family’s reaction in her son’s case was to attribute all the difficulties observed to the deafness ... the motor activities and the stereotyped behaviours were attributed to his need to compensate for the impaired sensory input by producing his own visual stimulation; and the aloofness and retreat into his own world were seen as a consequence of the lack of communication in the early days before the deafness was recognised.

However, the maintenance of such symptoms ... the lack of attunement to other people ... well after the (successful) activation of a cochlear implant provided the impetus for ongoing assessment and the subsequent diagnosis of autism.


Beals refers to the dilemma in respect of determining the most appropriate form of language to target, and the initial decision concerned the desirability of a heterogeneous approach involving the use of signing in the first instance with speech and lip-reading to be considered over time and in accordance with observed performance in the use of the hearing aids  ... perhaps with cued speech to be considered as a further option.


In other words, it is a matter of determining via observation the best match between the idiosyncratic needs and available strategies. However, the critical (probably more critical) issues included how to address the severe social and interactional deficits, further supporting the view that the autistic traits are central and to be targeted albeit with methodology that is adapted to meet the particular type and level of the accompanying hearing loss. 


Meanwhile, albeit acknowledging the risks of seeking to generalise from a small number of (anecdotal) cases, one notes separate references on a special needs’ (website) forum to instances where a child with comorbid autism and deafness has started at a school for the deaf or hearing impaired only to fall increasingly behind the mean level of progress and to be transferred to a setting geared for children with autism and ASD.  These reports also concur in respect of the perceived value of the visual emphasis in the teaching methodology, notably the  Picture Exchange Communication System, which is typical, anyway, of specialist schools or classes for children with autism and ASD.


Other references describe the gains achieved as a result of Applied Behavioural Analysis (Verbal Behaviour Approach).


Again, therefore, there is the implication for cases of comorbid autism and deafness that it is the autism that defines the overall targets and approaches, while the degree and nature of the hearing loss guides the nature of modifications necessary to the specific teaching and learning strategies. 


It is relevant here to cite Samuels (2007) who describes the need to create individualised programmes for children with idiosyncratic permutations of difficulties and disabilities ... the interventionist provision is to be fitted to the child as opposed to seeking to fit the child to an existing provision. 

The example is given of pressure being exerted upon staff of some specialist schools for the deaf to accept pupils with significant additional needs who cannot benefit from their environments in which sign language is the primary mode of communication. Reference is made to comments from staff who describe the range of needs already attending the schools, but to an inability to be all things to all people in centres established to provide for deafness as the primary need.


One might reiterate that, where there is a valid diagnosis of (severe) autism, the needs will persist, albeit, in some or many cases, with moderated or modified patterns of symptoms and behaviours over time; and they will require ongoing specialist intervention whatever provisions are made to compensate for the deafness and whatever changes are observed in the hearing status. 




While hearing deficits or deafness are more prevalent in an autistic population than in the population as a whole, they are still relatively uncommon; and comorbid autism and hearing loss, each at a severe level, will represent a markedly complex permutation of needs.


Where such a complexity is observed, the requirement would suggest particular relevance in a clinical rather than a categorical approach thus to tap, via consultations, the experience of all those concerned with the child (parents and professionals) to set out the precise profile of needs and the targets for intervention.


In the case of a pre-school child already identified with severe autism, and recently provided with a cochlear implant, one has to balance the desirability of early intervention against the requirement to monitor performance following the activation of the implant thus to determine the precise needs (including how the presentation of the autistic symptomatology may be affected). 


What matters is not simply early intervention but appropriately targeted intervention in a setting which is most suited (or can most readily be modified) to provide for the particular profile of needs. This is important not only for the child and the maximising of progress, but also for the family as a whole given that the resilience of the parents may well be reinforced if they can assist in, and be reassured by, the implementation of a plan of action that reflects the existing needs and which can be modified as or if the needs are observed to change.


Meanwhile, acknowledging the limited data base upon which to draw, it would appear that initial decisions (subject to continual review) about school provision in such a case would involve an emphasis upon the autism in determining the most appropriate school and the educational programme, but the degree and nature of the ongoing hearing and communication levels would be significant for decisions about how the programme is to be delivered.


                                *          *          *          *          *          *          *


M.J.Connor                                                                                              January 2008 








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This article is reproduced by kind permission of the author.

© Mike Connor 2008.

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