Early experience and health related quality of life outcomes following auditory brainstem implantation in children

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Abstract

Objective

To assess auditory brainstem implant (ABI) outcomes in children with a prospective study.

Methods

Twelve patients with cochlear nerve deficiency received an auditory brainstem implant. Patients were evaluated with age appropriate speech perception and production assessments, and health related quality of life (HRQoL) surveys for parents of subjects and for subjects if age appropriate.

Results

Twelve patients received an ABI without major complications. Eleven out of twelve received some auditory benefit from their ABI. Parental HRQoL ratings were positive for all domains with the exception of communication. Self reported overall HRQoL metrics from two subjects were also positive.

Conclusions

ABI is a good option for patients who are not eligible for or fail CI. Our findings show that despite varying degrees of postoperative performance, HRQoL ratings were positive. The presence of additional disabilities and health problems resulted in less positive HRQoL outcomes. Our results emphasize the need to assess outcomes in these patients beyond speech perception and communication.

Introduction

Over the last several decades, cochlear implants (CI) have been used as a treatment for individuals with profound hearing loss often with tremendous benefit in speech and communication abilities [1]. Some with congenital profound hearing loss are not candidates for cochlear implantation because of absent cochleae or deficient cochlear nerves. The auditory brainstem implant (ABI) was initially designed for use in Neurofibromatosis 2 (NF2) patients with bilateral vestibular schwannomas and loss of their cochlear nerves [1]. Of patients undergoing ABI surgery in the US, 81% acquire auditory sensations [1]. At our center, we have implanted 40 deafened NF2 adults with an ABI. Adequate data from 31 patients, reveals 21 patients have sound detection, of which 9 have closed set discrimination and 2 have some open set speech perception.

More recently, the ABI is being explored as a treatment option for pediatric patients with cochlear nerve deficiency, bilateral cochlear ossification, and absent or severely malformed cochleae; conditions that preclude benefit from a CI. Centers with approval for investigational use of this device have published data concluding that ABI surgery is safe although outcomes have been variable [[2], [3], [4], [5], [6]]. In one study, 29 out of 35 children who received ABI had closed set word discrimination and 12 had open set discrimination above 50% [7]. A study by Colletti et al. reported that all 21 patients with cochlear nerve deficiency who initially failed CI and went on to ABI achieved environmental awareness and responded to speech sounds. Of the 21, 41% achieved open set speech perception [8].

In one United States institution, four pediatric patients implanted with an ABI achieved environmental sound awareness. One patient had spontaneous device failure. Another patient had device failure due to blunt trauma, a revision ABI and device failure a second time due to blunt trauma [3]. At another center in the United States, only one out five patients is frequently responding to environmental stimuli at the one-year post implantation stage [2].

While speech and hearing outcomes are a core part of ABI evaluation, they give a limited picture of a subject's outcomes. Hearing loss impacts psychosocial aspects of a person's life, such as communication, self-esteem and social relationships [9]. CI literature has explored these domains by creating CI specific tools to measure health related quality of life (HRQoL). HRQoL is defined as an individual's perceived mental and physical health and has become an increasingly important way of measuring outcomes and value of health interventions.

In attempt to better encapsulate outcomes in other domains in ABI pediatric patients, we collected data from the subjects' families regarding the HRQoL of their child following ABI. We used a validated CI parental survey to collect the data to report HRQoL data on this population.

Section snippets

Materials and methods

This study was reviewed and approved by the institutional review board of New York University School of Medicine. Informed consent was obtained from the parents of all subjects.

Surgical data and perception outcomes

Twelve patients underwent ABI surgery. Preoperative data is outlined for each subject in Table 1. All subjects had cochlear nerve deficiency. S6, S8 and S12 also had cochlear aplasia and did not have a CI trial. S1, S2 and S7 had complicated additional medical history or developmental difficulties. Nine patients trialed and failed a CI prior to ABI. The patients were determined to fail CI when they failed to make auditory or language progress on appropriate assessments after using the CI for at

Summary and discussion

Eleven out of the twelve subjects receive varying amounts of subjective and objective benefit from their ABI. The auditory outcomes for subjects ranged from no environmental sound awareness in one case to four cases of open set speech perception. No patients had any long-term complications. Our results are in agreement with other studies, which have found pediatric ABI surgery to be safe among experienced adult NF2 teams and provide varying degrees of auditory input to patients.

Overall, parents

Conclusions

An ABI may be considered in those children without other apparent disabilities who do not benefit from a cochlear implant or cannot receive one because of anatomic constraints. Appropriate preoperative counseling as to possible outcomes is imperative so that parents can make an informed choice. Our cohort demonstrates a range of auditory outcomes, with all but one receiving some benefit. While we found limited auditory and linguistic benefit from ABI, HRQoL measures seem to be somewhat more

Conflicts of interest

Dr. Roland is on the Cochlear Americas Surgical Advisory Board and Dr. Shapiro is on the Cochlear Americas Audiological Advisory Board.

Acknowledgements

This work was supported in part by the Children's Hearing Institute.

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    This study was reviewed and approved by the institutional review board of New York University School of Medicine.

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