**Treatment**

328 Hearing Loss

Zallocchi M, Meehan DT, Delimont D, Askew C, Garige S, Gratton MA, Rothermund-

Franklin CA, Cosgrove D (2009) Localization and expression of clarin-1, the Clrn1 gene product, in auditory hair cells and photoreceptors. Hear Res 255:109-120. Zou J, Luo L, Shen Z, Chiodo VA, Ambati BK, Hauswirth WW, Yang J (2011) Whirlin

Replacement Restores the Formation of the USH2 Protein Complex in Whirlin

Young RW (1967) The renewal of photoreceptor cell outer segments. J Cell Biol 33:61-72. Young RW (1976) Visual cells and the concept of renewal. Invest Ophthalmol Vis Sci 15:700-

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725.

**15** 

Julia Sarant

*Australia* 

*The University of Melbourne* 

**Cochlear Implants in Children: A Review** 

In 1980, the first child in the world was implanted with the single-channel House cochlear implant device (Eisenberg & House, 1982). Children who initially received cochlear implants during this first paediatric clinical trial were quite old compared to current ages (the average age in the first House clinical trial was 8 years, whereas children are now being implanted as young as 6 months of age), and the majority communicated using sign language (Eisenberg & Johnson, 2008). It is now known that implanting older children who do not communicate orally gives little chance of speech perception or spoken language development. In 1985, the first children received a multichannel cochlear implant in Australia (Clark et al., 1987). This clinical trial selected children who had a higher potential for success, including shorter duration of deafness and a commitment to oral communication both at home and in their educational programs. At this time, it was unknown whether the speech processing schemes used with adults who had lost their hearing after developing language (ie. post-lingually deafened) would be appropriate for facilitating the speech perception and language development of young children with immature auditory systems. It is important also to note that the desired outcomes for adults and children differed; while the goal for adults was to improve auditory skills and communication using previously acquired cognitive, spoken language, and social skills, the goal for children was to develop these skills using the auditory information provided by the cochlear implant, having had no useful auditory experience (and therefore presumably no neural development of their auditory system) until they received their cochlear implant. The implantation of children was also highly controversial. For many years, cochlear implantation in children was opposed by the Deaf Community, on the grounds that deafness in children should be considered as a cultural and linguistic difference rather than as a disability that could be remediated by a cochlear implant. Over time, this view has changed such that in 2000, a position paper of the National Association of the Deaf in the U.S. stated that "cochlear implantation is a technology that represents a tool to be used in some forms of communication, and not a cure

It is now well documented that children with severe-profound hearing loss receive significant benefits from cochlear implants in terms of speech perception and language development (Blamey et al., 2006; Geers et al., 2008; Moog, 2002; Nicholas & Geers 2007). Cochlear implants are becoming the standard of care for children with severe-profound hearing loss, with increasing uptake of simultaneous bilateral implants over recent years. There is a large variation in implementation of cochlear implant technology around the

**1. Introduction** 

for deafness" (National Association of the Deaf, 2000).
