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

Leena Asfour, David R. Friedmann•, William H. Shapiro, J. Thomas Roland Jr.,
Susan B. Waltzman
New York Universicy School of Medicine, 550 l .sr Ave, NY, NY 10016, USA

Keywords

Auditory brainstem implantation
Cochlear nerve deficiency
Health related quality of Life
Speech perception
Pediatric
Cocltlear implantation
Quality of life

Abstract

Objective: To assess auditory bralnstem 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 ARI without major complications. Eleven out of twelve received some auditory benefit from their ABI. Parcnlal HRQol. ratings were positive for all domains with the exception or communication. Self reported overall HRQoL mctrlcs from two subjects were also positive.

Conclu.sionJ: ABI is a good option for patients who are not eligible for orfail Cl. Our findings show that despite varying degrees of postoperative perfonnance, HI\QoL ratings were positive. The presence of addition al 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 communicalion.

1. Introduction

Over the last several decades, cochlear implants (CI) have been used as a treatment for individuals with profotmd 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 schwan·nomas and loss of their cochlear nerves [1]. Of patients undergoing AB! sW”gery in the US, 81% acquire auditory sensations [1]. At OW” center, we have implanted 40 deafened NF2 adults with an AB!. 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 malfom1edcochleae; conditions that preclude benefit from a Cl. Centers with approval for investigational use of this device have published data concluding that ABI surgery is safe although outcomes have been variable [2-6]. In one study, 29 out of 35 children who received ABl had closed set word d iscrimination and 12 had open set discrimination above 50% [7]. A study by Colletti eta!. reported tl1at all 21 patients with cochlear nerve deficiency who initially failed Cl 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 AB! and device failure a second time due to blunt trauma [3]. At nother center in tl1e United States, only one out five patients is frequently responding to envirorunental stimuli at the one-year post implantation stage [2]. While speech and hearing outcomes arc a core part of AB! cvalua·tion, 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]. Cl literature has explored these domains by creating Cl 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.