Cochlear Implants: Optimizing Patient Benefit through Team Management and Family-Based Aural Rehabilitation

Introduction

The professional staff of Dallas Otolaryngology Associates and HEAR In Dallas joined forces to form the Dallas Otolaryngology Associates Cochlear Implant (DOA-CI) Team. Together they provide assessment and treatment to patients seeking cochlear implantation. The team maintains one philosophical goal-- the maximal use of sound for verbal communication and environmental monitoring for deaf patients ranging in age from infants to seniors. It is the team's philosophy that meaningful use of sound can enhance the quality of life for almost every deaf person.

The DOA-CI team includes the following: patient and their family members, a neurotologist, four audiologists, a nurse, billing/reimbursement people, two secretaries, a neuropsychologist, a rehabilitative audiologist/Certified Auditory-Verbal Therapist, two speech-language pathologists, an occupational therapist and the administrator of the Dallas Hearing Foundation. Other consultants and therapists are involved as dictated by individual patient needs. The team members pool their expertise to form long-term management programs for patients of all ages.

Upon conclusion of the initial audiometric evaluation, the audiologist recommends one of three options:

1. The patient participates in a program of aural rehabilitation with his/her current hearing aids. Patients referred for aural rehabilitation with hearing aids are monitored: their hearing aid progress is compared to the outcome anticipated with appropriate cochlear implantation and aural rehabilitation.



2. The patient tries new hearing aids, participates in short-term aural rehabilitation and is re-evaluated for implant candidacy as needed.



3. The patient proceeds with a full evaluation to determine cochlear implant candidacy and appropriate post-implant aural rehabilitation.

Implant centers vary greatly in the criteria used to determine patient candidacy for cochlear implantation. Consistent with their view of hearing as important for quality of life, the DOA-CI team views the implant as a tool that provides a range of benefit to patients. Long-term outcome or benefit from a cochlear implant is impacted by numerous variables. In determining patient candidacy for implantation and expected benefit, the team attempts to ascertain patient variables likely to impact their meaningful use of sound with an implant.

Significant factors include the patient's age, health, hearing history, hearing technology history, speech/language/reading/writing status, communication mode of patient/family/peers, psychological status, neurological/cognitive status, patient knowledge/goals for CI, educational options, motivation/compliance, support for developing auditory behavior, results of trial therapy, finances, time/availability and distance from the treatment program. Each of these is examined to formulate a complete picture of the patient and their life. This information is used to (a) determine candidacy for implantation, (b) counsel the patient and family members regarding expected benefit, (c) address issues that would need to change in order to proceed with implantation, (d) discuss how each issue would impact performance with the device and (e) explore options for optimizing benefit.

One key factor that leads to patient satisfaction is the free flow of communication among CI team members. Each member of the team recognizes and respects the expertise of the other team members and all regularly exchange information. The physician considers the opinion of each team member when deciding whether or not to proceed with surgery. The programming audiologists rely on feedback from the aural rehabilitation team regarding the patients' performance with each MAP on speech perception tasks. The insurance person requests progress reports to ensure continued coverage of needed services. The neuropsychologist assesses and treats patients for special learning needs and assists the team and patients with issues that may arise. Family members share situations and concerns about the patient's use of hearing and speech in daily life: these are used to individualize the aural rehabilitation program as well as modify the programming of the device. All members of the team work closely together for optimal care of each patient.

CI Patient Categories

With their broad criteria for candidacy, the CI Team assesses patients from the following categories:

• Infants identified with sensorineural (SN) hearing loss in the newborn nursery, toddlers and preschool-aged children identified with SN hearing loss: these patients are fitted with hearing aids, enrolled in Auditory-Verbal therapy and family education and response to hearing aids is monitored and compared to the expected benefit from implantation.



• Elementary school children and teens from mainstreamed, oral, or signing programs, with or without intelligible speech: some are referred for a trial period of aural rehabilitation with hearing aids in order to assess the child and significant family factors that impact candidacy.



• Young adults and middle-aged adults who have congenital or peri-linguistic deafness: patients may or may not have experience with hearing aids, and may or may not have intelligible speech. Patients may use speech, sign language or a combination of speech and sign for communication.



• Post-linguistically deafened children from mainstreamed, oral, or signing programs, with or without intelligible speech



• Post-linguistically deafened adults with short or long-standing deafness



• Senior citizens with progressive hearing loss who receive limited benefit from hearing aids

Etiologies & Associated Disorders (Peters, 2000)

With broadened cochlear implant candidacy criteria, there is an increased incidence of associated disorders in cochlear implant patients. Associated disorders can have serious impact on the patient's ability to process the signal provided by the cochlear implant; they also have significant implications on the individual's potential to learn to speak. Some examples of etiologies and associated communication disorders are presented below.

1. Congenital syndromes include CHARGE, CMV, rubella, cerebral palsy, Usher syndrome, fetal alcohol syndrome.


2. Acquired neonatal and childhood disorders include prematurity, hyperbilirubinemia, ECMO, meningitis, ototoxicity.


3. Developmental disorders of unknown etiology that co-occur with hearing loss includes sensory processing and sensory integrative disorders, feeding disorders, autism, auditory neuropathy, dyspraxia, cognitive delay/differences, attention deficits, language and learning differences, gross/fine motor delays, psycho-social differences and others.

Working with people who have associated disorders requires awareness, knowledge, experience; early involvement of team members; vigilance regarding medical and audiologic concerns; ongoing monitoring; realistic expectations from cochlear implantation; a high degree of parental/familial support and flexibility.

There are four concepts the team identifies as "key" when considering implantation of a patient with multiple disorders. They are:

1. Plasticity - The capacity to be pliable, reshaped, molded as new stimuli arrives and is internalized.


2. Adaptation/Generalization - The ability to modify response/behavior to changing conditions.


3. Compensation - The ability to offset deficit areas through maximal development of less impaired function.


4. Integration - The ability to bring together or incorporate separate parts into a unified, harmonious, and interrelated whole.

These concepts are used in family counseling and when designing the rehabilitation program.
Establishing Auditory Processes for Comprehension of Spoken Language and for Acquisition and Self-Monitoring of Speech Production

Post-linguistically deafened adults are typically seen for short-term aural rehabilitation. Since they already possess the cognitive processes of audition and the motor skills of speech, rehabilitation typically consists of counseling and adjustment issues and auditory training. Family members are included in therapy and are taught to administer home program assignments to support the patient's auditory development.

Many CI patients are pre-linguistically deaf. For this group of children and adults, a significant portion of therapy time is devoted to developing listening skills for the comprehension of spoken language as well as an auditory feedback system to guide the use of the three subsystems of speech (Pollack, 1985[a]). Learning to listen unfolds quite naturally for neurologically-intact children who are implanted within the first few years of life and managed in an Auditory-Verbal program. Therapy for this group incorporates parent education, whereby the parent learns to foster the child's natural patterns of audition and spoken language development (Daniel, 2000, Samson, 1994) throughout daily interactions.

For pre-linguistically deafened adults, the development of auditory processes is an arduous task following activation of a cochlear implant. Brain functions associated with hearing develop slowly in adults who have been deaf since birth. These individuals did not have the opportunity to develop the cognitive processes based on audition for receptive and expressive verbal language. Neither did they learn the respiratory, phonatory and articulatory functions for the production of intelligible speech (Hudgins and Numbers, 1942). Pre-linguistically deafened adults who begin hearing in adulthood must learn to perceive the prosodic and segmental aspects of their own speech and must learn to correct their own atypical speech habits as they learn to produce more appropriate speech sounds and patterns.

With a cochlear implant, children and adults must learn to process sound and develop meaningful use of acoustic input. Some of the cognitive processes that result in meaningful use of sound are as follows: attention to sound through time, the ability to judge two sounds as same or different, auditory imagery and memory for auditory images, memory span for auditory events, maintenance of the sound sequences, associating sounds with their referents, use of sound for linguistic purposes and retrieval of sound images for linguistic expression (Daniel, et. al., 1999, Pisoni and Geers, 1998, Baddeley and Logie, 1992, Pollack, 1985(a), Erber, 1982). Aural rehabilitation focuses on developing these skills in the patient and assisting family members in supporting the use of these functions in daily experiences.

In addition to using hearing for language comprehension, audition is the key sense through which individuals with normal hearing monitor their speech. From the first few months of life forward, infants proceed through cooing, vocal play, babbling, jargon and phonological maturation on their journey toward intelligible speech. Hearing is the primary sense that weaves the motor and tactile/kinesthetic aspects of the mother tongue into the child's developing sensory/motor system. Through audition, children experience the sounds produced by the three sub-systems of speech—respiration, phonation, and articulation. This auditory feedback system guides the infant in making necessary modifications in production for integrating the complex functions of the three subsystems of speech. Over time, automaticity of movement of the muscles of all three subsystems is achieved resulting in intelligible speech. Below are some aspects of speech that patients need to learn to control speech production through hearing (Ling, 2002).

Respiration: gaining control over the muscles involved in breathing for

Use of the breath in sustained phonation, build up oral pressure for plosives and fricatives, differentiation of voiced and voiceless sounds, modification of vocal loudness, and production of syllable strings for multisyllabic words, phrases and sentences.

Phonation: gaining control over the laryngeal muscles for

Production of vowels, voiced and voiceless consonants.

Vocal pitch that is appropriate to patient's age/sex, linguistic uses of pitch for speaking prosody, singing.

Voice quality.

Articulation: gaining control over the musculature of the lips, tongue, jaw and velopharynx for

Vowels: front/back, high/low:

• Requires proper movement of the lips, jaw, velopharynx, tongue tip, and middle and back portions of the tongue.

Consonants: stops, nasals, glides, plosives, fricatives, affricates:

• Requires complex control and coordination of all three subsystems of speech production.

Sequences of consonants and vowels:

• Requires smooth movements between vowels and consonants in repeated syllable production and alternated syllable production.


• Requires complex interactions of refined functions of all three subsystems.

Sensory Processing Disorders and Sensory Integration Dysfunction

Proper use and integration of information received by the brain from the various senses impacts a child's ability to learn (Kranowitz, 1998). The integration of sensory information from hearing, sight and touch is essential for meaningful use of the signal provided by hearing technologies.

Many children with hearing loss (Daniel, 1988) have atypical sensory processing and integrative functions that lead to a host of atypical behaviors. Among these are delays in gross and fine motor development, increase in self-stimulatory behaviors, poor eye contact, poor social interactions, feeding difficulties, sleeping problems, slow or disordered speech, language and learning disabilities and differences, and academic problems.

Sensory processing deficits require specialized forms of therapy to facilitate more typical use and integration of sensory information (Kranowitz, 1998). Some occupational therapists specialize in treating sensory integration dysfunction to help the child process and coordinate sensory input in order to learn more efficiently.

Characteristics of Deaf Children that may Indicate Sensory Processing Dysfunction

Many hearing impaired and deaf children exhibit sensory processing dysfunction as described by Kranowitz (1998). This may include over-sensitivity or under-sensitivity to touch, sight, movement, sound, smell and/or taste as well as poor integration of the sensory/motor systems. Children with sensory processing dysfunction may have delayed development in the areas of cognition, gross and fine motor skills, muscle tone and psycho/social interaction.

Sensory integration therapy helps children organize information entering the brain through the various senses. Sensory integration therapy can improve the child's ability to learn motor behaviors, speech/language and academic information. With appropriate therapy, children may improve their eating, playing and social behaviors as well. The purpose of sensory integration therapy is to help the child achieve and maintain an optimal state for learning, thereby developing at the fastest rate possible within the parameters of their physical and cognitive abilities. Outcomes vary from child to child and are influenced by the severity of the dysfunction of the particular child, the child's cognitive level, and environmental factors.

Kranowitz (1998) described treatment activities to improve body awareness, postural security, tactile discrimination, tactile defensiveness, balance, bilateral coordination, motor planning/movement sequences, fine motor skills, extension against gravity, flexion, ocular control and visual-spatial perception.

Educational Component of the Aural Rehabilitation Program

In working toward the goal of communicative competence and communicative independence, the aural rehabilitation staff on the DOA-CI team provides direct therapy to the patient and counsels parents regarding educational programs that support the child's use of hearing and spoken language. Parents are advised how to work with their school district to develop an Individualized Education Plan (IEP) that integrates the child's aural rehabilitation needs into the services provided by the school. The therapists find it useful to review the child's IEP and write goals for the parents to submit to the IEP committee. The goal of sharing information with the school staff is to create a maximal auditory learning environment throughout the school day. IEP goals typically address the child's need for classroom instruction via spoken language as well as increased opportunities for the child to practice speaking and interacting with hearing peers.

The educational and communicative limits imposed by an emphasis on sign language have been documented (COED, 1988). Most significant is that some ninety percent of children with hearing impairment are born to parents with normal hearing and the normal hearing parents rarely know or develop proficiency in sign language. Nonetheless, deaf education programs in the public schools usually teach signed rather than spoken language. This protocol results in a low proficiency in written or spoken English, an average reading level of third grade upon graduation, and a situation in which sixty percent of the graduates have a lifetime of unemployment or severe under-employment (COED, 1988).

Fortunately, new aural rehabilitation management procedures have raised the academic achievement levels of many deaf children -- even before the advent of cochlear implants (Goldberg and Flexer, 1993). With the fitting of hearing aids on young children several decades ago, a number of individuals have developed aural rehabilitative procedures that foster the use of audition for spoken language in severely and profoundly deaf children. Stunningly, the results reported in their 1993 study were obtained on children who (essentially) preceded the cochlear implant era.

These procedures are now referred to as "Auditory-Verbal" (AV) management (AVI, 1993) and are applied to children at some CI centers (Daniel and Sowers, in prep). The results are in sharp contrast to those obtained by the Commission on Education of the Deaf, who studied traditional deaf education programs (COED, 1988).

Goldberg and Flexer studied the long-term outcomes of 152 graduates of Auditory-Verbal programs throughout North America. Ninety-three percent of the subjects had severe-profound hearing impairment and were fitted with hearing aids at an average age of 23 months. 36.7% reported associated disorders in addition to their deafness. Respondents received an average of 11 years of AV therapy and parent education. None of the 152 had the benefit of a cochlear implant.

Outcomes for this group included the following: 70% graduated from regular high school between 16 and 18 years of age, over 75% of the subjects were fully mainstreamed in regular classrooms and 95% received education after high school with 88% attending colleges and universities for hearing students. All but one subject reported significant involvement of a mother in the rehabilitation process; 80% reported active participation of a father and 67% reported sibling involvement in their rehabilitation process. Seventy-eight percent used the telephone to send and receive messages and 49% used a telecommunications device for the deaf. Over half of the respondents reported early and continued involvement in community activities including scouting, churches/synagogues, clubs, sports, sororities, fraternities, etc. One individual reported self-perception as part of the Deaf community, 27% perceived themselves as part of the hearing mainstream and Deaf community and 73% perceived themselves as part of the hearing mainstream only. The AV program graduates entered careers of their choices including administrative assistant, computer programmer, finance manager, bank vice-president, truck driver, graphic artist courier, painter, social worker, engineers, teachers, attorneys, dentist, physician, etc.

Principles of Auditory-Verbal Management

Auditory-Verbal management is a form of aural rehabilitation that develops hearing as the foundation for verbal language learning (Estabrooks, 1994). It is strongly rooted in parent participation and the creation of an auditory learning environment at home and school. Individual patient needs dictate modifications of therapy goals and procedures. The therapist's individual areas of expertise, personality, mentor and interests guide the selection of therapy activities. Although specific techniques and procedures used in auditory-verbal development vary from one Auditory-Verbal therapist to another, the learning principles followed are consistent with those described in the cognitive sciences (Neisser, 1987).

Regardless of these differences, Auditory-Verbal therapists maintain the same guiding principles in their management of the child and family. The general concepts of audition as the driving sensory modality, verbal communication as the teaching tool and goal, and modification of the environment for auditory learning provide the consistency among professionals within AV. Below is an outline of the "ACE" concept of AV management (Daniel et. al., 1993)—audition as the basis for communication and environment.

AUDITION

• Support early diagnosis of the hearing impairment and hearing technology for optimal access to the acoustic speech spectrum


• Develop speech and environmental interactions through hearing


• Maintain use of hearing technology throughout the patient's waking hours

COMMUNICATION

• Develop a family-therapist partnership


• Promote spoken language as the primary means of communication


• Evaluate and monitor progress and treatment plan on an ongoing basis


• Guide the patient through normal stages of hearing, speech and language development


• Individualize therapy

ENVIRONMENT

• Maintain/foster a normal living environment within the hearing/speaking society


• Teach family members to create an auditory learning environment in daily living


• Facilitate independent functioning in the educational and employment mainstreaming


• Foster communication with typical, hearing/speaking peers and social groups


• Encourage auditory activities such as music and dance lessons, use of CD players, etc.

Conclusion

With the vastly heterogeneous population implanted at the DOA-CI center, management by a team of specialists is essential for optimizing patient benefit from a cochlear implant. In addition to the expertise offered by the professional staff, the patient and members of the patient's family play a critical role in optimizing benefit: they do so by maintaining an auditory learning environment at home, fulfilling the home program goals and updating the staff on patient performance in daily living. Although individual performance outcomes vary widely from patient to patient, satisfaction with the device is generally high due to the extensive assessment and family counseling program. As stated earlier, the professional cochlear implant team maintains one philosophical goal — the maximal use of sound for verbal communication and environmental monitoring. The team continues to pursue their goal of enhancing the quality of life for deaf individuals who want to hear.

References

Baddeley, A., and Logie, R., 1992, Auditory Imagery and Working Memory, In Reisberg, D., Ed., Auditory Imagery, Lawrence Erlbaum Assoiciates, Publ., Hillsdale New Jersey.

The Commission on Education of the Deaf, February 1988, Toward Equality: Education of the Deaf, A Report to the President and the Congress of the United States.

Daniel, L., in prep, DDOVS: A Rating Scale for Auditory-Verbal Competence. Monograph on Cochlear Implants. Alexander Graham Bell Association for the Deaf.

Daniel, L., 2003, Cochlear Implants Across the Lifespan: Assessment, Candidacy, Treatment and Outcomes, A Short Course Presented to the Texas Academy of Audiology, November 14, College Station, Texas

Daniel, L., 2000, MotherSong: Natural Language For Auditory-Verbal Development. The Listener. Journal of the Learning to Listen Foundation. Summer, Toronto, Canada.

Daniel, L., 1988-2003, Clinical notes of observations of deaf children who have cochlear implants.

Daniel, L., Ernst, M., Rothwell-Vivian, K., 1998, adapted from: Auditory-Verbal International, 1993, AVI Principles and Rules of Ethics.

Daniel, L., Daniloff, D., and Schuckers, G., 1999, ALPS: A Language Rehabilitation Program for Children with Cochlear Implants, The Journal of Louisiana Applied Health Professionals, Vol. II, Summer.

Daniel, L. and Sowers, J., in prep. Dallas Otolaryngology Associates and HEAR in Dallas: "The Heart of Hearing Team".

Estabrooks, W., 1994, Ed., In Auditory-Verbal Therapy for Parents and Professionals, Alexander Graham Bell Association for the Deaf, Wash. D.C.

Erber, N., 1982, Auditory Training, Alexander Graham Bell Association for the Deaf, Wash. D.C.

Goldberg, D. and Flexer, C., 1993, Outcome Survey of Auditory-Verbal Graduates: Study of Clinical Efficacy, Journal of the American Academy of Audiology, 4, pp.189-200.

Hudgins, C. V. & Numbers, F. C., 1942, An Investigation of Intelligibility of Speech of the Deaf. Genetic Psychology Monograph, 25, 289-392.

Kranowitz, C., 1998, The Out-of-Sync Child: Recognizing and Coping with Sensory Integration Dysfunction, Perigee Publishers.

Ling, D., 2002, Speech and the Hearing-Impaired Child: Theory and Practice, 2^nd Ed., Alexander Graham Bell Association for the Deaf, Wash. D.C.

Neisser, J.U., 1987, From Direct Perception to Conceptual Structure. In Neisser, U., ed. Concepts and Conceptual Development: Ecological and Intellectual Factors in Categorization. Cambridge, Cambridge University Press.

Peters, R., 2000, Hearing Loss and Associated Disorders, Seminar in Auditory-Verbal Development and Cochlear Implantation, Dallas, Texas.

Pisoni, D. B. and Geers, A., 1998, Working Memory in Deaf Children with Cochlear Implants: Correlation Between Digit Span and Measures of Spoken Language Processing. Paper presented at the 7^th Symposium on Cochlear Implants in Children, Iowa City, IA, June 4-6.

Pollack (a), D., 1985, Educational Audiology for the Limited Hearing Infant and Preschooler, 132-160, Chas. C. Thomas, Publ., Springfield, Illinois.

Pollack (b), D., 1985, Educational Audiology for the Limited Hearing Infant and Preschooler, 161-196, Chas. C. Thomas, Publ., Springfield, Illinois.

Samson, A. B., 1994, The Family-Professional Partnership: A Parent Perspective, In Auditory-Verbal Therapy for Parents and Professionals, Estabrooks, W., Ed., 195-215, Alexander Graham Bell Association for the Deaf, Wash. D.C.

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