12/30/2017 Cochlear Implant Surgery: Background, History of the Procedure, Problem

Cochlear Implant Surgery

Updated: Oct 13, 2017
Author: Cliff A Megerian, MD, FACS; Chief Editor: Arlen D Meyers, MD, MBA more...

OVERVIEW

Background
Cochlear implantation has become a routine procedure in the United States and worldwide for the
management of severe-to-profound sensorineural hearing loss. It is a remarkable example of success
that was made possible through collaboration between engineers, surgeons, scientists and the
medical community. As of 2012, more than 300,000 implants have been performed worldwide. The
decision to embark upon cochlear implantation is made either by the patient (if adult) or by the parents
or caregivers of a child. This 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, radiographic studies, and overall expectations of
patient and involvement of family (in pediatric patients) in a rigorous rehabilitative process.

An image depicting cochlear implant surgery can be seen below.

Postauricular incision for cochlear implant.

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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,
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12/30/2017 Cochlear Implant Surgery: Background, History of the Procedure, Problem

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 Cleveland 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 bony anatomy of the cochlea and to establish the presence of a
patent (nonossified) cochlear duct. It is also used to identify various anomalies of cochlea-vestibular
anatomy (common cavity, incomplete partition defects, enlarged vestibular aqueduct, and cochlear
ossification. 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, 2] Multichannel devices introduced in 1984 have replaced single-channel devices by virtue of
improved speech recognition capabilities. As of 2012, nearly 300,000 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 HiRes 90K (Advanced Bionics Corporation), and the Synchrony
device (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 used for 3 months (ie, a score of 50% or less on sentence recognition
tests in the best-aided listening situation). Expanded indications include the use of hybrid devices
(with preserved hearing in low frequencies) which have more specific audiometric criteria, [3]
implantations in pediatric patients less than 12 months of age, and off-label use for unilaterial hearing
loss with or without bothersome tinnitus.

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12/30/2017 Cochlear Implant Surgery: Background, History of the Procedure, Problem

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. [4] 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. [5] This statistic may reach 44 million by
the year 2020 and 73.5 million in 20160. [6]

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, Ménière 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. [7] 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. However, studies do report better
post-implantation performance with higher residual spiral ganglion cells. [8] Also, delayed loss of
residual hearing in implantation done for hybrid implant procedures is linked with intracochlear fibrosis
and interventions aimed at reduction of cochlear trauma and inflammation, i.e., perioperative steroids
and hearing preservation approaches, are routinely used successfully. [9]

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 ABR and OAE
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.

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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. [10] 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. An evaluation for revised
indications for adult CMS population who do not meet current criteria for implantation is underway with
results expected in 2019. [11]

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
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
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12/30/2017 Cochlear Implant Surgery: Background, History of the Procedure, Problem

the mastoid cortex, retromastoid region, high riding jugular bulb, 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. The surgeon should be experienced in
placement of implants in malformed anatomy and be able to drill out or use split arrays implants in
cases of severe ossification. [12, 13]

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. Cochlear aplasia 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. The rigorous implant candidacy criteria and process helps to select prospective
patients who will yield the greatest benefit from it.

Patient Education
Various institue-specific online and device-specific literature is available and is discussed with the
patients and families at the time of initial evaluation and device selection. In addition, advocacy groups
such as the American Cochlear Implant Alliance provide further information and resources for further
utilization of implantation for hearing loss.

Clinical Presentation

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