You are in: eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > INNER EAR Cochlear Implants, IndicationsArticle Last Updated: Jul 8, 2008AUTHOR AND EDITOR INFORMATIONAuthor: Brandon Isaacson, MD, Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center Brandon Isaacson is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Neurotology Society, North American Skull Base Society, and Texas Medical Association Coauthor(s): Kenneth H Lee, MD, PhD, Assistant Professor, Department of Otolaryngology Head and Neck Surgery, University of Texas Southwestern Medical Center; Consulting Staff, Division of Pediatric Otolaryngology, Children's Medical Center, Dallas; Joe Walter Kutz Jr, MD, Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center; Peter S Roland, MD, Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director of Clinical Center for Auditory, Vestibular and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Adjunct Professor of Communicative Disorders, School of Human Development; Eric W Sargent, MD, Clinical Associate Professor, Wayne State University, Otolaryngology - Head & Neck Surgery, Michigan Ear Institute Editors: Cliff A Megerian, MD, FACS, Medical Director of Adult and Pediatric Cochlear Implant Program, Vice-Chairman and Director of Otology and Neurotology, University Hospitals of Cleveland; Professor, Department of Otolaryngology-Head and Neck Surgery and Neurological Surgery, Case Western Reserve University School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Gerard J Gianoli, MD, Clinical Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Tulane University School of Medicine; Vice President, The Ear and Balance Institute; Chief Executive Officer, Ponchartrain Surgery Center; Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders; Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine Author and Editor Disclosure Synonyms and related keywords: cochlear implants, hearing impairment, auditory spiral ganglion, hearing loss, deafness, meningitis, candidates for cochlear implants, speech audiometry INTRODUCTIONBackground Hearing loss is one of the most common sensory impairments and affects 28 million Americans. Approximately 1-3 out of 1000 newborns has hearing impairment. The elderly are more commonly affected with 40-50% of people over age 75 having hearing loss. Depending on the degree of hearing loss, many affected individuals can be successfully fitted with hearing aids. For patients with hearing loss that is not mitigated with hearing aids, a cochlear implant may provide an opportunity for hearing. The cochlear implant is a surgically placed device that converts sound to an electrical signal. This electrical signal is transmitted via electrodes to the spiral ganglion cells in the cochlear modiolus. As of 2005, an estimated 85,000 patients worldwide have received cochlear implants. However, this number represents only a small number of individuals with hearing impairment who may potentially benefit from implantation (an estimated 250,000 people). Many candidates for cochlear implants often do not have access to this procedure due to failure of recognizing appropriate candidates or because of inadequate healthcare resources. Although individual responses to cochlear implants are highly variable and depend on a number of physical and psychosocial factors, the trend toward improved performance with increasingly sophisticated electrodes and programming strategies has dramatically expanded indications for cochlear implantation. Although cochlear implants originally were touted as an aid to speech reading for individuals with profound hearing impairment, a growing population of implanted patients are exceeding their preoperative hearing performance (which was aided with conventional hearing aids). Due to the overall success of cochlear implants and ongoing advances in performance, an article addressing indications for cochlear implantation attempts to describe a target that moves almost yearly. For excellent patient education resources, visit eMedicine's Ear, Nose, and Throat Center. Also, see eMedicine's patient education article Hearing Loss Throughout the 1970s, the Food and Drug Administration (FDA) recommended that devices be implanted only in adults with profound hearing loss. In 1980, the FDA allowed children at least 2 years of age to be implanted. The age limit has recently been lowered to 12 months for all 3 devices available in the PREOPERATIVE CONSIDERATIONSCochlear implantation is a collaborative effort involving patients, families, schools, audiologists, speech/hearing therapists, and surgeons. A patient with hearing impairment does not have a surgical problem that responds to the simple intervention of an implant surgeon. Because preoperative expectation affects the patient's postoperative satisfaction and use of the implant, all patients and families require attention and counseling from an implant team before they embark on the life-changing journey of cochlear implantation. Pure-tone audiometry The human ear is capable of hearing frequencies from 20-20,000 Hz. Pure-tone audiometry is used to assess a subject’s response to a frequency at a specific intensity measured in decibels. In most cases, frequencies from 250 Hz to 8000 Hz are assessed, as these are most important for speech perception. Speech audiometry Although a number of speech-recognition tests are currently used for different reasons, one of the most common speech-recognition tests is the hearing in noise test (HINT), which tests speech recognition in the context of sentences. In determining cochlear implant candidacy, HINT is performed without background noise, despite its name. HINT measures word-recognition abilities to assess the patient's candidacy for cochlear implantation, in conjunction with conventional pure-tone and speech audiometry. The HINT consists of 25 equivalent 10-sentence lists that may be presented in quiet or noise to assess the patient's understanding of sentences. Criteria For adults and children who can respond reliably, standard pure-tone and speech audiometry tests are used to screen likely candidates. For children aged 12-23 months, the pure-tone average (PTA) for both ears should equal or exceed 90 dB. For individuals older than 24 months, the PTA for both ears should equal or exceed 70 dB. If the patient can detect speech with best-fit hearing aids in place, a speech-recognition test in a sound field of 55-dB HL sound pressure level (SPL) is performed. A number of speech recognition tests are currently in use. Other tests Imaging Imaging with CT or MRI is performed prior to implantation to evaluate the inner ear, facial nerve, cochleovestibular nerve, brain, and brainstem. MRI may reveal hypoplasia or aplasia of the cochleovestibular nerve, whereas CT may show a narrow internal auditory canal or absence of the bony cochlear nerve canal at the modiolus. Inner ear malformations ranging from the rare cases of cochlear aplasia to the more common enlarged vestibular aqueduct are easily visualized on CT or MRI. Such results may alter the choice of side of implantation or raise other issues such as electrode selection. In pediatric or young adult patients with progressive hearing loss, exclude neurofibromatosis II by performing MRI before proceeding with implantation. MRI has more recently become the imaging study of choice at some institutions because the inner ear, cochleovestibular nerve, brain, and brainstem can all be visualized. MRI, unlike CT scanning, is also very useful in identifying early labyrinthitis ossificans, which typically begins with endoluminal fibrosis of the scala tympani at the basal turn. Preoperative counseling and education In addition to the purely audiologic criteria discussed above, pediatric candidates must be enrolled in an educational program that supports listening and speaking with aided hearing. For patients of all ages, no medical contraindications (eg, cochlear or auditory nerve aplasia, active middle-ear infection) may be present. Communication among patients, families, schools, audiologists, therapists, and surgeons is required. Immunization A study published in 2003 reported that pediatric and adult patients with cochlear implants are at increased risk of acquiring S. pneumoniae meningitis.1 In 2002, the CDC issued age-appropriate immunization guidelines for patients who have a cochlear implant or are going to be the recipient of a cochlear implant. These vaccines include the 7-valent pneumococcal conjugate vaccine (PCV7; Prevnar), and the 23-valent pneumococcal polysaccharide vaccine (PPV23; Pneumovax). The following recommended schedule was recommended:
Recommended Pneumococcal Vaccination Schedule for Persons With Cochlear Implants2
†For children vaccinated at less than 1 year, the minimum interval between doses is 4 weeks. §The additional dose should be administered at 8 or more weeks after the primary series has been completed. ¶Children older than 5 years should complete the PCV7 series first; 23 variant pneumococcal polysaccharide vaccine (PPv23) should be administered to children 24 months and older 8 weeks or more after the last dose of PCV7.3 **The minimum interval between doses is 8 weeks. ††PCV7 is generally not recommended for children 5 year and older. ETIOLOGIES OF SEVERE TO PROFOUND HEARING LOSSGenetic Genetic hearing loss is the most common etiology of childhood deafness (33-50%); and many of these cases can be attributed to single gene mutations. Seventy five to eighty percent of genetic deafness is secondary to autosomal recessive gene defects, 18-20% is secondary to autosomal dominant gene defects, and the remainders are X-linked gene defects. Another potential cause of genetic deafness is mitochondrial gene defects. Genetic hearing loss is generally divided into nonsyndromic and syndromic, with the former being twice as common as the latter.
Although 12 months is the current age limit the FDA has established for implantation, other factors may cause the implant team to treat infants younger than 12 months. In particular, a child with deafness due to meningitis may develop labyrinthitis ossificans, filling the cochlear duct or the entire cochlear labyrinth and usually the scala tympani starting near the round window, with bone. In cases of labyrinthitis ossificans, special techniques may be needed for successful cochlear implantation. However, even with special techniques, this condition often leads to a suboptimal outcome. Image 3 depicts a CT scan of a child with deafness due to meningitis whose left cochlea has ossified. In this patient, successful implantation of the patent right cochlea was accomplished. For patients at risk of labyrinthitis ossificans, implantation may be indicated soon after early ossification or fibrosis is diagnosed. Early implantation in the setting of labyrinthitis ossificans may allow a full electrode insertion and obviate the need for performing a cochlear drill-out procedure. Using serial imaging, implant teams may monitor patients with new deafness due to meningitis and perform implantation at the first sign of replacement of the scala tympani with fibrous tissue or bone. Several reports have described spontaneous hearing improvement after meningitis in patients with residual hearing. In patients with profound hearing loss after meningitis, the chance of hearing improvement is unlikely and cochlear implantation should proceed as soon as possible. Ossification can begin as early as 2 weeks after meningitis. In the setting of bilateral labyrinthitis ossificans, expeditious placement of bilateral implants should be considered as these patients are not likely to benefit from future technology. Ototoxicity Numerous medications can cause hearing loss. Perhaps the most well known ototoxic medications are the aminoglycoside antibiotics. Other commonly cited ototoxic medications include loop diuretics, erythromycin, salicylates, vancomycin, cisplatin, and quinine. Trauma Hearing loss from temporal bone trauma is most commonly conductive secondary to hemotympanum, tympanic membrane perforation, or ossicular discontinuity. The otic capsule is fairly resistant to trauma but occasionally fractures may involve the cochlear or labyrinth. Injuries to the otic capsule almost always results in profound sensorineural hearing loss. Bilateral otic capsule fractures are very uncommon but would be an indication for cochlear implantation. Intraluminal fibrosis or ossification may occur in the setting or otic capsule fractures, which can make electrode insertion more difficult. Preoperative imaging may provide additional information of fibrosis or ossification has occurred. Hyperbilirubinemia In the setting of neonatal jaundice, bilirubin may cross the blood-brain barrier. Bilirubin can deposit in the ventral cochlear nucleus and cause sensorineural hearing loss. Transient loss of wave IV and V on an ABR is seen in 33% of neonates with bilirubin levels of 15-25 mg/dL. Hyperbilirubinemia is also a risk factor of auditory neuropathy. Auditory neuropathy/dyssynchrony Patients with auditory neuropathy are characterized by intact outer hair cell function with abnormal or absent ABR, implicating the vestibulocochlear nerve as the site of pathology in this condition. Outer hair cell function can be assessed with otoacoustic emissions, the cochlear microphonic or pure-tone audiometry. A defect in inner hair cells, spiral ganglion cells or the synapse between the two is the cause of auditory neuropathy. Ménière disease Meniere disease is characterized by room spinning vertigo, fluctuating hearing, tinnitus, or aural fullness. The diagnosis is made with a thorough history and physical examination, as well as imaging studies to rule out retrocochlear pathology. Histology of postmortem temporal bones in subjects with Meniere disease reveals dilation of the endolymph compartment. Histologic endolymphatic hydrops may be the end result of some other pathologic process that is causing the symptoms of Meniere disease or may be the actual cause of the symptoms. Noise-induced hearing loss Presbycusis Hearing loss associated with aging initially begins with loss of high frequencies, with eventual progression to include lower frequency loss. Thirty to thirty five percent of 65 year olds have some hearing loss; this figure rises to 40-50% of individuals over 70 years. Men are more commonly affected with age-related hearing loss. Hearing aids often provide meaningful benefit for patients with presbycusis, but only one in five individuals who would benefit from a hearing aid actual wear one. Patients with severe to profound hearing loss who are unable to hear with traditional amplification may benefit from a cochlear implant. In cases where residual low frequency hearing is found, some individuals may benefit from a combined hearing aid /cochlear implant that provides both electrical and acoustic stimulation. Residual hearing Short implant electrodes that are under development may allow for implantation in patients with good low-frequency hearing and poor high-frequency hearing. Placing the electrode atraumatically in the basal turn of the cochlea through a small cochleostomy may preserve residual hearing, and a conventional hearing aid (for low-frequency hearing amplification) could be worn simultaneously. This approach allows for the auditory rehabilitation of patients who are not candidates for conventional implants because their low-frequency hearing exceeds current guidelines. These hybrid implants are a current focus of research. BILATERAL IMPLANTSPatients with unaided unilateral hearing loss often have difficulty in everyday listening situations. Significant disadvantages to unilateral hearing loss are well known and include the head shadow effect, difficulty with hearing in noise, and sound localization. Amplification for unilateral hearing loss is routinely recommended in children and adults. A multitude of studies have demonstrated significant performance improvement in patients with bilateral cochlear implants. OUTCOMESCritical outcome factors for pediatric patients receiving implants include (1) age at onset of deafness and duration of deafness prior to implantation, (2) progression of hearing loss, and (3) educational setting. In general, early implantation facilitates rapid development of oral communication ability. Progressive hearing loss, which allows for the development of speech-reading skills, favors performance after implantation. Placement in a school setting that stresses oral versus signed communication is important for optimal implantation outcome. However, many variables remain unknown because approximately one half of the variance in performance after implantation cannot be predicted. Children should be receptive to wearing a hearing aid before cochlear implantation because all current implants require an external processor. A period of hearing aid use to ascertain development of aided communication ability is an important criterion in determining candidacy of young children. After audiologic criteria have been met, parental expectations and attitudes must be assessed and addressed. Unrealistic expectations can frustrate the efforts of the child and the implant team. Families must be appropriately counseled about the need for long-term therapy, variable outcome of implantation, and the limitations of implantation. MULTIMEDIA
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Cochlear Implants, Indications excerpt Article Last Updated: Jul 8, 2008 | ||||||||||||||||||||||||||||||||||||||||||||||||||||