12/15/2014

Cochlear Implant Surgery

Cochlear Implant Surgery

Author: Cliff A Megerian, MD, FACS; Chief Editor: Arlen D Meyers, MD, MBA more...
Updated: May 21, 2013

Background
Cochlear implantation has become a routine procedure in the United States and worldwide for the management of
severe-to-profound sensorineural hearing loss. The decision to embark upon cochlear implantation is made either by
the patient (if adult) or by the parents or caregivers of a child. The 60- to 75minute procedure is well tolerated and
routinely performed on an outpatient basis in both adults and children.
The team concept in cochlear implant evaluation allows for an exchange of information between the surgeon and
other members of the implant and rehabilitation process, including audiologists, speech and language therapists,
social workers, and psychologists. Typically, the patient is referred to a cochlear implant center, and initial contact is
made. The patient may first be seen and identified as an implant candidate by an audiologist. Hence, a patient can
enter the evaluation process in a number of different ways. Nonetheless, various issues are taken into consideration,
including medical aspects of the patient's history, the audiologic evaluation, and radiographic studies.
An image depicting cochlear implant surgery can be seen below.

Postauricular incision for cochlear implant.

Although the team evaluation concept is explained at greater length in the Indications section, it is notable because it
allows for proper selection of patients, the continuous flow of pertinent dialogue, and the promotion of realistic
expectations on the part of the patient and the patient's family.
The evaluation process used by the authors at the implant center at the Case Medical Center/University Hospitals of
Cleveland and Rainbow Babies and Children's Hospital is summarized below. At the time of the medical evaluation,
the patient's general medical history and issues regarding hearing loss are reviewed. A complete neuro-otologic and
otolaryngologic examination is performed, and obvious conditions (eg, tympanic membrane perforation, chronic otitis
media, congenital anomalies) are noted. The patient's history is reviewed to establish the potential etiology of the
hearing loss. Audiologic tests are reviewed and repeated as necessary. Once the patient is deemed to be a potential
cochlear implant candidate, the various cochlear implant options are discussed, and audiologic evaluation
commences.
Typically, the audiologist measures the patient's hearing with and without hearing aids. Evaluation with pure-tone
audiometry and auditory brainstem response (ABR) testing (in the case of children) is often performed. Otoacoustic
emission (OAE) testing complements these studies; OAE results often indicate the need for a trial of newer and
sometimes stronger hearing aids.
A CT scan is obtained to evaluate the status of the cochlea and to establish the presence of a patent (nonossified)
cochlea or to identify a common cavity, Mondini dysplasia, enlarged vestibular aqueduct, or an ossified cochlea. In
some cases, an MRI is used instead of the CT when questions exist regarding the presence of the eight nerve or
severe ossification. In children and young adults, speech and language evaluation and educational placement
discussions are performed next. Finally, a psychosocial evaluation is completed. Once a patient has been evaluated,
a team meeting commences to recommend cochlear implantation advice. If the patient is cleared for cochlear
implantation, the patient proceeds with preoperative medical clearance, chooses a cochlear implant device, and
proceeds with surgery.

History of the Procedure

In 1957, Djourno and Eyries made the observation that activation of the auditory nerve with an electrified device
provides auditory stimulation in a patient. This observation is considered the seminal observation that paved the way
for modern cochlear implantation. In 1963, Doyle and Doyle's early experiments in scala tympani implantation
preceded the first House/3M single-channel implant in 1972.[1] Multichannel devices introduced in 1984 have
replaced single-channel devices by virtue of improved speech recognition capabilities. As of 2009, nearly 150,000
cochlear implants are estimated to have been performed worldwide, and approximately 7,000 procedures take place
annually in the United States. Three US Food and Drug Administration (FDA)approved multichannel devices are
routinely used in the United States currently, including the Nucleus 5 cochlear implant system (Cochlear
Corporation), the Clarion 90K (Advanced Bionics Corporation), and the Combi 40+ (MED-ELCorporation).

Problem
Severe-to-profound hearing loss, as evidenced by the lack of useful benefit from hearing aids, often determines
one's candidacy for cochlear implantation. In children, this is confirmed via auditory testing and failure to develop
basic auditory skills. In adults, candidates should receive limited or no benefit from appropriate hearing aids (ie, a
score of 50% or less on sentence recognition tests in the best-aided listening situation).

http://emedicine.medscape.com/article/857242-overview#showall

1/5

12/15/2014

Cochlear Implant Surgery

Epidemiology
Frequency
The incidence of congenital hearing loss varies by study. Niparko reviewed studies from the 1980s and 1990s and
noted that one of the most carefully performed epidemiologic studies was that of Van Naarden et al, which noted an
overall prevalence rate of serious hearing impairment of 1.1 cases per 1000 children aged 3-10 years.[2] By age 75
years, 360 of 1000 adults have a disabling hearing loss. According to the 1996 National Institute on Deafness and
Other Communications Disorders survey, more than 28 million Americans are deaf or hearing impaired.[3] This
statistic may reach 40 million by the year 2020.

Etiology
Common etiologies of deafness that lead to consideration of cochlear implantation in pediatric patients include
idiopathic, genetic, and acquired causes that result in congenital and delayed-onset hearing loss. Genetic hearing
loss can be dominant or recessive. Infectious etiologies, including bacterial and postviral meningitis, can lead to
severe hearing loss. Meningitis-related deafness has decreased with the routine use of the Haemophilus influenzae
vaccine in children. Adult patients presenting for implantation include those with progressive hearing loss that began
in childhood, viral-induced sudden hearing loss, ototoxicity, otosclerosis, Mnire disease, trauma, autoimmune
conditions, presbycusis, and bacterial infections.

Pathophysiology
Typically, patients presenting with severe-to-profound deafness have had a direct or indirect injury to the organ of
Corti, leading to degeneration or dysfunction of the hair cell system. Therefore, success of cochlear implantation
depends on stimulation of surviving spiral ganglion neurons. The number of surviving neuron populations needed for
successful implantation remains unclear. In 1991, Linthicum et al reported successful speech understanding in a
patient who demonstrated less than 10% of the normal complement of neurons via a temporal bone study.[4]
Therefore, despite the wide range of surviving neurons present in various pathologic causes of deafness (10-70% of
the normal 35,000-40,000 cells), most patients are likely potential implant candidates.

Presentation
In the past, children with hearing loss presented to the physician after their parents developed a concern about their
child's lack of response to noise and voices. This may have brought the child to the attention of an otolaryngologist
promptly (within a few weeks to months), or consultation may have been delayed up to a number of years. With the
addition of universal infant screening, babies are identified at birth as having a hearing loss. The loss is confirmed
and quantified with auditory brainstem testing, and, if profound, the patient is referred for cochlear implant evaluation.
Children are fitted with hearing aids, and a decision to implant is based on progress or lack of language development
and careful counseling of the family. If a child is clearly found to be an implant candidate, an earlier implantation
results in superior hearing and speech outcomes.
Thus, implantation at age 12 months is now considered ideal, and, in some instances, implantation at an earlier age
is performed. Adults with progressive loss that ultimately fails to be managed via amplification also may present for
implant consideration. Patients are increasingly informed of the various options for cochlear implantation via the
Internet and often have specific questions regarding different device options.
For excellent patient education resources, visit eMedicineHealth's Ear, Nose, and Throat Center. Also, see
eMedicineHealth's patient education article Hearing Loss.

Indications
The main indication for cochlear implantation is severe-to-profound hearing loss that is not adequately treated with
standard hearing aids. The clinical conditions that lead to such an indication include various scenarios, as follows:
Congenital hearing loss and prelingual deafness
Acquired hearing loss and postlingual deafness
Severe hearing loss that can be aided and that deteriorates to profound loss in childhood, adolescence, or
adulthood (perilingual) and coexists with various degrees of language development
Generally, the candidacy for implantation is considered separately for adults and children. As outlined in the 1995
National Institutes of Health (NIH) consensus statement on cochlear implantation, adult candidacy is noted as being
successful in postlingually deaf adults with severe-to-profound hearing loss with no speech perception benefit from
hearing aids.[5] In addition, the statement notes that "most marginally successful hearing aid users implanted with a
cochlear implant will have improved speech perception performance." Medicare guidelines as of January 2005 allow
for cochlear implantation in patients with 50% aided sentence discrimination scores and allow for 60% sentence
scores in clinical trials. Clearly, the trend over time is that relaxed guidelines are better, and better cochlear implant
performance and outcome have been demonstrated.
Prelingually deafened adults, although potentially suitable for cochlear implantation, must be counseled in regard to
realistic expectations, as language and open-set speech discrimination outcomes are less predictable. A strong
desire for oral communication is paramount for this group of patients
Children are considered candidates for cochlear implantation at age 12 months, and, because of meningitis-related
deafness with progressive cochlear ossification, occasional earlier implantation is necessary. Investigations are
ongoing into extending the age of early routine implantation to younger than 12 months. Audiologic criteria include
severe-to-profound sensorineural hearing loss bilaterally and poor speech perception under best-aided conditions,
with a failure to progress with hearing aids and an educational environment that stresses oral communication. The
use of objective testing in this age group includes auditory brainstem response (ABR) testing and otoacoustic
emission (OAE) testing in addition to trials of various auditory training programs, which are essential before cochlear
implantation. For further discussion, see the Medscape Reference article Indications for Cochlear Implants.

Relevant Anatomy
http://emedicine.medscape.com/article/857242-overview#showall

2/5

12/15/2014

Cochlear Implant Surgery

The surgeon performing cochlear implant surgery must be experienced in otologic surgery and, ideally, some
aspects of neurotologic surgery. Intimate knowledge of the relevant surgical anatomy of the mastoid cortex,
retromastoid region, and posterior/middle cranial fossa dura is important in properly performing the approach to the
facial recess and in properly creating an implant receiver well that provides low-profile placement of the internal
device.
In addition, the relationship of the facial nerve, incus, chorda tympani, and the facial recess needs to be properly
understood to safely perform the posterior tympanotomy to gain access to the middle ear. Once the facial recess has
been opened, knowledge of the round window anatomy as it relates to normal or abnormal middle ear topography is
vital. The ability to visualize the round window membrane by removing the bony round window niche is important for
creating a proper cochleostomy. Variations in anatomy, ossification of the scala tympani, and various strategies of
dealing with cerebrospinal fluid oozers and gushers should be anticipated.
For more information about the relevant anatomy, see Auditory System Anatomy, Skull Base Anatomy, and Facial
Nerve Anatomy.

Contraindications
Contraindications to cochlear implantation may include deafness due to lesions of the eighth cranial nerve or brain
stem. In addition, chronic infections of the middle ear and mastoid cavity or tympanic membrane perforation can be
contraindications. The absence of cochlear development as demonstrated on CT scans remains an absolute
contraindication. Certain medical conditions that preclude cochlear implant surgery (eg, specific hematologic,
pulmonary, and cardiac conditions) also may be contraindications. The lack of realistic expectations regarding the
benefits of cochlear implantation and/or a lack of strong desire to develop enhanced oral communication skills poses
a strong contraindication for implant surgery.

Contributor Information and Disclosures

Author
Cliff A Megerian, MD, FACS Medical Director of Adult and Pediatric Cochlear Implant Program, Director of
Otology and Neurotology, University Hospitals of Cleveland; Chairman of Otolaryngology-Head and Neck
Surgery, Professor of Otolaryngology-Head and Neck Surgery and Neurological Surgery, Case Western Reserve
University School of Medicine
Cliff A Megerian, MD, FACS is a member of the following medical societies: American Academy of
Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society,
American Otological Society, Association for Research in Otolaryngology, Massachusetts Medical Society,
Society for Neuroscience, Society of University Otolaryngologists-Head and Neck Surgeons, and Triological
Society
Disclosure: Cochlear Americas Consulting fee Board membership; Grace Corporation Consulting fee Board
membership
Coauthor(s)
Peter S Roland, MD Professor, Department of Neurological Surgery, Professor and Chairman, Department of
Otolaryngology-Head and Neck Surgery, Director, Clinical Center for Auditory, Vestibular, and Facial Nerve
Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Chief of Pediatric
Otology, Children's Medical Center of Dallas; President of Medical Staff, Parkland Memorial Hospital; Adjunct
Professor of Communicative Disorders, School of Behavioral and Brain Sciences, Chief of Medical Service, Callier
Center for Communicative Disorders, University of Texas School of Human Development
Peter S Roland, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of
Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society,
American Laryngological Rhinological and Otological Society, American Neurotology Society, American Otological
Society, North American Skull Base Society, and Society of University Otolaryngologists-Head and Neck
Surgeons
Disclosure: Alcon Labs Honoraria Consulting; Advanced Bionics Honoraria Board membership; Cochlear Corp
Honoraria Board membership; Med El Corp travel grants Consulting
Gail S Murray, PhD, MEd Clinical Director, Cochlear Implant Program, Director, Department of Audiology
Services, University Hospitals of Cleveland, Rainbow Babies, and Children's Hospital of Cleveland
Gail S Murray, PhD, MEd is a member of the following medical societies: American Academy of Audiology and
American Speech-Language-Hearing Association
Disclosure: Nothing to disclose.
Specialty Editor Board
Robert A Battista, MD, FACS Assistant Professor of Otolaryngology, Northwestern University, The Feinberg
School of Medicine; Physician, Ear Institute of Chicago, LLC
Robert A Battista, MD, FACS is a member of the following medical societies: American Academy of
Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, and
Illinois State Medical Society
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College
of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment
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

http://emedicine.medscape.com/article/857242-overview#showall

3/5

12/15/2014

Cochlear Implant Surgery

Gerard J Gianoli, MD is a member of the following medical societies: American Academy of Otolaryngology-Head
and Neck Surgery, American College of Surgeons, American Neurotology Society, American Otological Society,
Society of University Otolaryngologists-Head and Neck Surgeons, and Triological Society
Disclosure: Vesticon, Inc. None Board membership
Christopher L Slack, MD Private Practice in Otolaryngology and Facial Plastic Surgery, Associated Coastal
ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy
of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and
American Medical Association
Disclosure: Nothing to disclose.
Chief Editor
Arlen D Meyers, MD, MBA Professor of Otolaryngology, Dentistry, and Engineering, University of Colorado
School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic
and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head
and Neck Society
Disclosure: Axis Three Corporation Ownership interest Consulting; Medvoy Ownership interest Management
position; Cerescan Imaging Honoraria Consulting

References
1. Doyle J, Doyle D. Electrical stimulation of the nerve deafness. Bulletin of the Los Angeles Neurological
Society. 1963;28:148-150.
2. Van Naarden K, Decoufle P, Caldwell K. Prevalence and characteristics of children with serious hearing
impairment in metropolitan Atlanta, 1991-1993. Pediatrics. Mar 1999;103(3):570-5. [Medline].
3. National Institute on Deafness and Other Communcation Disorders. National Institute of Health. National
strategic research plan. In: US Department of Health and Human Services. Vol 5. 1996.
4. Linthicum FH Jr, Fayad J, Otto SR, et al. Cochlear implant histopathology. Am J Otol. Jul 1991;12(4):245311. [Medline].
5. National Institutes of Health. NIH Consensus Statement. Cochlear Implants in Adults and Children. 1995,
May15-17;13(2):1-30.
6. Gantz BJ, Tyler RS, Knutson JF, et al. Evaluation of five different cochlear implant designs: audiologic
assessment and predictors of performance. Laryngoscope. Oct 1988;98(10):1100-6. [Medline].
7. Roland JT Jr, Zeitler DM, Jethanamest D, et al. Evaluation of the short hybrid electrode in human temporal
bones. Otol Neurotol. Jun 2008;29(4):482-8. [Medline].
8. Balkany T, Telischi FF. Fixation of the electrode cable during cochlear implantation: the split bridge
technique. Laryngoscope. Feb 1995;105(2):217-8. [Medline].
9. Biernath KR, Reefhuis J, Whitney CG, et al. Bacterial meningitis among children with cochlear implants
beyond 24 months after implantation. Pediatrics. Feb 2006;117(2):284-9. [Medline].
10. Das S, Buchman CA. Bilateral cochlear implantation: current concepts. Curr Opin Otolaryngol Head Neck
Surg. Oct 2005;13(5):290-3. [Medline].
11. Djourno A, Eyries C. Prosthese auditive par excitation electrique a distance du nerf sensoriel al aid d un
bobinage inclus a demeure. [Auditory prosthesis by means of a distant electrical stimulation of the sensory
nerve with the use of an indwelling coil]. Presse Med. 1957;65:1417.
12. Gantz BJ, McCabe BF, Tyler RS. Use of multichannel cochlear implants in obstructed and obliterated
cochleas. Otolaryngol Head Neck Surg. Jan 1988;98(1):72-81. [Medline].
13. Green JD Jr, Marion MS, Hinojosa R. Labyrinthitis ossificans: histopathologic consideration for cochlear
implantation. Otolaryngol Head Neck Surg. Mar 1991;104(3):320-6. [Medline].
14. House WF, Urban J. Long term results of electrode implantation and electronic stimulation of the cochlea in
man. Ann Otol Rhinol Laryngol. Jul-Aug 1973;82(4):504-17. [Medline].
15. Nadol JB Jr. Hearing loss. N Engl J Med. Oct 7 1993;329(15):1092-102. [Medline].
16. Ng M, Niparko JK, Nager GT. Inner ear pathology in severe to profound sensorineural hearing loss. In:
Niparko JK, Mirk KI, Mellon NK, et al, eds. Cochlear Implant. Philadelphia: Lippincott Williams & Wilkins;
2000:57-87.
17. Niparko JK. The epidemiology of hearing loss. In: Niparko JK, Tucci DL, Robbins AM, Kirk KL, Mellon NK,
eds. Cochlear Implants: Principles and Practices. Philadelphia: Lippincott Williams & Wilkins; 2000:88-92.
18. Niparko JK, Wilson BS. History of cochlear implants. In: Niparko JK, Tucci DL, Robbins AM, Kirk KL, Mellon
NK, eds. Cochlear Implants: Principles and Practices. Philadelphia: Lippincott Williams & Wilkins; 2000:103107.
19. Rubinstein JT, Gantz BJ, Parkinson WS. Management of cochlear implant infections. Am J Otol. Jan
1999;20(1):46-9. [Medline].
20. Tambyraja RR, Gutman MA, Megerian CA. Cochlear implant complications: utility of federal database in
systematic analysis. Arch Otolaryngol Head Neck Surg. Mar 2005;131(3):245-50. [Medline].

http://emedicine.medscape.com/article/857242-overview#showall

4/5

12/15/2014

Cochlear Implant Surgery

21. Tucci Dl, Niparko JK. Medical and surgical aspects of cochlear implantation. In: Niparko JK, Tucci DL,
Robbins AM, Kirk KL, Mellon NK, eds. Cochlear Implants: Principles and Practices. Philadelphia: Lippincott
Williams & Wilkins; 2000:194-195.
Medscape Reference 2011 WebMD, LLC

http://emedicine.medscape.com/article/857242-overview#showall

5/5