2017 Sec 1 Green Book

SINGLE-SIDED DEAFNESS COCHLEAR IMPLANTATION

of the hearing loss on functioning and learning, but this wait introduces a longer duration of deafness, a negative relationship in predicting CI outcomes. A recent review of the experience in Freiburg, Germany, with pediatric SSD indicates that children with acquired hearing loss and a shorter duration of hearing loss outperformed those with a longer duration of SSD (14). It is important that the family understands all of these considerations when making the decision with the cochlear implant team. Additionally, at this early stage of investigation, suc- cessfully obtaining financial reimbursement surrounding the surgery, the device and associated visits to the implant center represent an important obstacle to its wider adoption. Relative Contraindications After a certain period of time, as yet undefined, one might expect the length of deafness to be too long for the benefits of cochlear implants to be realized. Until data clarify such a cut-off, implantation with proper counsel- ing may be considered. SSD can have a significant impact on developmental spheres and various aspects of quality of life. An informed discussion to include all available therapies and their respective advantages and disadvantages with the family and CI team is essential to the decision- making process. Early experience with SSD CI recipients suggests that cochlear implantation, with appropriate preoperative assessment and counseling and postopera- tive management, may offer these patients the best opportunity to realize the benefits of binaural hearing. Although in our center, certain conditions seem like clear indications, further data will be necessary before this treatment modality is advocated more widely. CONCLUSIONS 1. Lieu JE, Tye-Murray N, Fu Q. Longitudinal study of children with unilateral hearing loss. Laryngoscope 2012;122:2088–95. 2. Bess FH, Tharpe AM. Performance and management of children with unilateral sensorineural hearing loss. Scand Audiol Suppl 1988;30:75–9. 3. Hawley ML, Litovsky RY, Culling JF. The benefit of binaural hearing in a cocktail party: Effect of location and type of interferer. J Acoust Soc Am 2004;115:833–43. 4. Brown KD, Balkany TJ. Benefits of bilateral cochlear implantation: a review. Curr Opin Otolaryngol Head Neck Surg 2007;15:315–8. 5. Arndt S, Aschendorff A, Laszig R, et al. Comparison of pseudo- binaural hearing to real binaural hearing rehabilitation after coch- lear implantation in patients with unilateral deafness and tinnitus. Otol Neurotol 2011;32:39–47. 6. Wazen JJ, Ghossaini SN, Spitzer JB, Kuller M. Localization by unilateral BAHA users. Otolaryngol Head Neck Surg 2005;132: 928–32. 7. Van de Heyning P, Vermeire K, Diebl M, et al. Incapacitating unilateral tinnitus in single-sided deafness treated by cochlear implantation. Ann Otol Rhinol Laryngol 2008;117:645–52. 8. Vermeire K, Van de Heyning P. Binaural hearing after cochlear implantation in subjects with unilateral sensorineural deafness and tinnitus. Audiol Neurootol 2009;14:163–71. REFERENCES

communication challenges that arise and not having previous experience or need to comprehend manual communication. Depending on the etiology, these patients may still be candidates for CI after bilateral hearing loss, but pre-emptive implantation at an early age can limit the duration of deafness in the worse hearing ear and hence improve likely outcomes if the threatened ear is not viable for implantation. Additionally, a cochlear implant can provide assurance that if and when the patient loses hearing in the threat- ened only hearing ear that they will not be completely ‘‘off line’’ with their cochlear implant. We have found this to be important in patients even in cases where the electric signal is not well integrated during the interval of persistent acoustic hearing as these patients quickly adapt to electric only hearing once further loss occurs. Although criteria continue to be defined, cochlear implant candidacy for SSD is most favored in younger patients with progressive conditions such as enlarged vestibular aqueduct (EVA), genetic conditions, auto- immune inner ear disease, ototoxicity, and certain meta- bolic diseases. Since the good ear is likely to decline eventually, re-establishing hearing in the poorer ear avoids the untoward sequelae of long duration of deaf- ness and total auditory deprivation. Counseling and Other Considerations Just as in any family with children undergoing evalu- ation for a cochlear implant, an important part of the preoperative counseling includes ensuring patients and their families understand the range of possible outcomes as well as the considerable time and effort required for optimal performance with the device. Additionally, particular consideration should include discussion about subjective performance and progress over time, in addition to objective testing. An assessment of functional impairments may be more important than objective audiologic testing, most of which may be relatively normal with one hearing ear. For those children who are school age, one should inquire of the family whether they have noted difficulty in particular listening con- ditions, in social interactions, or in reports from teachers. Another consideration is the very young child with SSD. With acknowledgement that some children with SSD grow up to be well-functioning adults and adapt well, these outcomes are difficult to predict. The devel- oping brain is at maximal neuroplasticity at a young age and so a prolonged period of auditory deprivation may compromise ultimate auditory performance with treat- ment. By analogy to adults, there are some adults who have lived with SSD without perceived difficulty, whereas others have found it challenging and no factors have yet been identified to know which patients fall into which group. Unfortunately, attempting to clarify these unknowns introduces a paradox. Waiting until a child gets older may allow a better determination of the impact Absolute Indication: Pediatric Progressive Hearing Loss

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