Question
I have been asked to follow a 2-year old with a diagnosed auditory neuropathy (diagnosed by two other outside pediatric Audiology centres). This child also has additional cognitive, developmental and motor delays. This child initially wore binaural hearing aids, which have subsequently removed by one of the pediatric audiology centres. I am requesting input on how to best assess her behaviourally (I do not have ABR, only OAE's and soundfield equipment) for long-term follow-up and ideas for supporting communication development with this child--with simple gesture, sign, or simple pictures, as there are other neurological deficits as well.
Answer
Behavioral methods of assessment, such as visual reinforcement audiometry and play audiometry, should be combined with functional hearing assessment scales in order to document auditory behavior development in this child. It is known that children with auditory neuropathy can demonstrate fluctuating hearing sensitivity (Sininger and Oba, 2002) and so "inconsistent" pure tone threshold and speech reception thresholds may be expected. It would be important, therefore, to systematically document the child's auditory behaviors. Several functional assessment scales are available including the Family Expectation Worksheet (FEW), Developmental Index of Audition and Listening (DIAL) and Pediatric Hearing Demand Ability and Need Profile (PHDANP), and the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS, McConkey-Robbins et al, 1998). These measures can be coupled with asking the parents to keep a journal of their child's auditory behaviors (English, Kooper & Bratt, 2004). The assessment of this child's hearing abilities will be the result of teamwork between the audiologist and the parents. As well, since auditory development and abilities are often demonstrated in speech development, a speech and language assessment may also provide valuable information about the child's hearing abilities. The MacArthur Communicative Development Inventories (Fensen et al, 1993) are used by speech-language pathologists to document speech and language abilities by systematic interview/questionnaire of the parents.
As for any child with a significant hearing impairment, language development must be the highest priority. How might we best provide language to a child with extremely limited or no access to an acoustic signal? If the parents have decided not to use audition as a means of language development (this is implied by the discontinuation of hearing aid use), then a visual-manual language, such as American Sign Language, should be used. Cued Speech (Leybaert & Alegria, 2003) has not yet been demonstrated to be a means of providing a "first" language, although it may enhance and support oral speech and language development. It is important to remember that the child with auditory neuropathy may be functioning like a child with a severe-profound sensorineural impairment, despite pure tone thresholds that indicate only a moderate sensitivity loss. So, the choice of language development method should be reasoned like that for a hard-of-hearing or deaf child. If access to an acoustic signal is provided, either through amplification or cochlear implantation, then a Total Communication or Aural-Oral method may used. In this case, however, it appears that the auditory modality will not be utilized, and ASL should be the recommended first language. Even if cochlear implantation is planned at a later date, the child will benefit from having a language system in place at the time of implantation.
References
English, K., Kooper, R., Bratt, G. (2004) Informing parents of their child's hearing loss. Audiology Today 16(2) 10-12.
Fenson L, Dale PS, Reznick JS, Thal D, Bates E, Hartung JP, Pathick S, Reilly JS. (1993). MacArthur Communicative Development Inventories. Singular Publishing Group: San Diego.
Leybaert, J & Alegria, J. (2003). The role of Cued Speech in the language development of deaf children. pp.261-274. In M. Marshark and P. Spencer (Eds). Handbook of Deaf Studies, Language and Education. New York, Oxford University Press.
McConkey-Robbins, Svirsky, M, Osberber MJ, & Pisoni, DB (1998) Beyond the audiogram: The role of functional assessments. In F. Bess (Ed.) Children with Hearing Impairment: Contemporary Trends. Nashville, Vanderbilt Bill Wilkerson Center Press.
Sininger, YS & Oba, S. (2002) Patients with auditory neuropathy: Who are they and what can they hear? In YS Sininger and A. Starr (Eds). Auditory Neuropathy: A New Perspective on Hearing Disorders. San Diego, Singular Press.
Dr. Barbara Cone-Wesson is associate professor of Speech and Hearing Science at the University of Arizona. She has used electrophysiological methods of auditory system assessment for clinical and research applications for over 25 years.